Section 8 Flashcards

1
Q

A patient complains of having a unilateral, persistent, stabbing pain in the neck the radiates over the back of the skull. Upon physical examination, she has hypoesthesia and tenderness in the affected area. Which of the following best describes the cervical spinal segment originates of the nerve involved in the patient’s condition?

A
  1. C2 and C3

  • The greater occipital nerve, which innervates the skin of the back of the scalp up to the vertex of the skull and the ear, branches from the C2 spinal nerve.
  • The lesser occipital nerve arises from the ventral rami of the C2 and C3 spinal nerves.
  • The lesser occipital nerve is a sensory nerve and courses behind the sternocleidomastoid muscle.
  • Damage to the lesser occipital nerve may result in occipital neuralgia.
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2
Q

A 28-year-old male is brought to the hospital after a motor vehicle collision. He was riding a motorcycle without a helmet and was hit in the frontoparietal region. On examination, he has a Battle sign and haemotympanum. He is also noted to have right-sided facial nerve palsy and sensorineural hearing loss. CT scan reveals a temporal bone fracture. He is appropriately treated and discharged the next day. Two days later, he returned to the clinic and was noted to have horizontal nystagmus with the fast phase to the left side. What is the most likely underlying pathology for this finding?

A
  1. Right-sided perilymphatic fistula secondary to a transverse fracture

  • Perilymphatic fistula is a known complication of transverse temporal fractures.
  • The nystagmus has its fast phase away from the side of the lesion.
  • Facial nerve palsy occurs in approximately 20% of longitudinal fractures compared to 50% of transverse temporal bone fractures.
  • Cerebellar lesions are also known to cause nystagmus; however, this is a less likely cause in this patient because of the mechanism of injury and the direction (horizontal) of the nystagmus.
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3
Q

A 37-year-old man presents with right arm pain and weakness in the right shoulder and hand for one week. On examination, the patient is unable to abduct the right shoulder and is unable to make a pincer grasp (‘OK’ sign) with his right index finger and thumb. The clinician orders a nerve conduction study and electromyography (NCS/EMG) to evaluate for brachial plexopathy. Which of the following findings are likely to be seen if the study is performed on the same day?

  1. Fibrillation potentials in the flexor pollicis longus muscle.
  2. Greater than 50% reduction of the compound muscle action potential of the abductor pollicis brevis at the forearm stimulation site, as compared to the wrist stimulation site of the median nerve.
  3. Normal study except for possible reduced motor unit recruitment in the flexor pollicis longus and flexor digitorum indicis muscles.
  4. Positive sharp waves, fibrillation potentials, increased motor unit action potential amplitude and duration in the flexor pollicis longus and flexor digitorum indicis muscles.
A

3. Normal study except for possible reduced motor unit recruitment in the flexor pollicis longus and flexor digitorum indicis muscles.

  • In this case, the anterior interosseus nerve is affected as the patient is unable to make a pincer grasp with the thumb and index finger (‘OK’ sign).
  • Brachial plexopathy usually leads to axonal changes (although some demyelinating features may be present). However, it has only been one week since the onset of symptoms. Thus, axonal changes will not manifest on the EMG as it takes about 3 weeks for axonal changes to manifest. Demyelinating changes, on the other hand, may manifest earlier and are usually seen as reduced recruitment of motor units.
  • It is not easy to differentiate early axonal injury from demyelinating changes unless one waits for about 3 weeks to perform an EMG. At that time, due to Wallerian degeneration, early denervation changes (axonal injury) can be seen on EMG. These manifest as positive sharp waves and fibrillation potentials. In chronic axonal neuropathies, chronic denervation and reinnervation changes can be seen which manifest as reduced motor unit recruitment, increased motor unit action potential, and amplitude.
  • Thus, option 1 would have been correct if the EMG was performed about 3 weeks after the onset of symptoms. Option 4 would be correct if the EMG was performed a few months after the onset of symptoms. Option 2 indicates a demyelinating lesion in the median nerve between the forearm and wrist segment. This would not be expected in a brachial plexus injury.
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4
Q

A 16-year-old female suffers a significant head injury after falling off a trampoline. Her past medical history is significant for type 1 diabetes mellitus and asthma. She initially has post-concussive symptoms in the hospital. Three days into her hospital stay, she starts feeling very lethargic and begins to look dehydrated. On examination, her blood pressure is 110/60 mmHg, her pulse is 89/min regular, and she has signs of dehydration. Her labs suggest serum sodium of 127 mEq/L and urinary sodium of 54 mEq/L. What should be the treatment plan for her underlying disorder?

  1. Restriction of salt and fluids
  2. Intravascular saline with increased oral intake of salt
  3. Salt tablets and added salt in diet alone
  4. Demeclocycline
A

2. Intravascular saline with increased oral intake of salt

  • This is a case of cerebral salt wasting that presents with hyponatremia (serum sodium less than 135 meq/L) and usually increased urine sodium (urine sodium more than 40 meq/L).
  • In cerebral salt wasting urine osmolality is typically elevated (more than 100 mosmol/kg).
  • Syndrome of inappropriate secretion of antidiuretic hormone (SIADH) will have a similar laboratory picture as cerebral salt wasting with hyponatremia and increased urine sodium. However, with SIADH, the patient is euvolemic to hypervolemic from the retained free water as compared to the hypovolemic picture of cerebral salt wasting.
  • Other causes for hyponatremia should be excluded including polydipsia, renal disease, use of diuretics, heart failure, hypothyroidism, heart failure, malignancies, hormone deficiency, and pseudohyponatremia.
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5
Q

A 65-year-old-female with a history of hypertension presents to the clinic complaining about back pain getting worse for six months. Pain is constant, 10/10, burning, tightening sensation, radiating from tailbone area to buttock then to left leg, worsened by sitting, bending, walking, alleviated by stretching, ice/heat pad. Pain is also accompanied by numbness/weakness on the left foot. On detailed history, she went through 3 major spinal surgeries for severe scoliosis. The first surgery that was done five years ago lasted 10 hours, involved instrumented fusion from T12 to the pelvis. Subsequent surgery was done four years ago due to hardware failure, and the last one was done one year ago to remove loose iliac bolts. Her physical exam shows a positive straight leg test on the left side and decreased sensation on the dorsal of the left foot. The laboratory tests show normal basic metabolic panel (BMP) and complete blood count (CBC). She was told no more surgeries are needed to solve the issue and feel disappointed. She comes for a second opinion regarding her treatment plan. Which of the following will be preferred advice to give?
1. Surgery
2. Steroid epidural injection
3. Pain management and psychotherapy
4. Immunoglobulin

A

3. Pain management and psychotherapy

  • Arachnoiditis is a persistent inflammation of the arachnoid mater as well as subarachnoid space due to many possible causes such as prior spinal surgery, infection.
  • It is an incurable condition that is difficult to treat. Treatment mainly focuses on alleviating pain, improving quality of life, managing symptoms, helping patients cope with the difficulties it presents.
  • Pain management ranges from traditional treatment modalities like medication, physical therapy, cognitive, behavior psychotherapy, to modern treatment techniques such as thecaloscopy and neurostimulation.
  • Long-term prognosis after surgery remains poor with immediate improvement followed by relapse and progressively worsening occurs later.
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6
Q

A 36-year-old female comes to your primary care office for daily headaches. The headaches are pulsatile, unilateral, and associated with photophobia and nausea, but without aura. The problems occur daily and significantly interfere with her quality of life. She has been experiencing these symptoms over the last 18 months. On exam, she is neurologically intact with a reasonable range of motion of her neck. She has tried multiple medications without any relief, including preventative and abortive therapy. She has gone to cognitive behavioral therapy for her headaches and recently underwent a botulinum injection, but it provided minimal relief. The clinician is considering a procedure to relieve her pain. What is the most common location of the pain, targeted for this procedure?

  1. Left supraorbital and supratrochlear nerves
  2. Bilateral greater and lesser occipital nerves
  3. Right greater and lesser occipital nerves
  4. Bilateral supraorbital and supratrochlear nerves
A

2. Bilateral greater and lesser occipital nerves

  • This patient has chronic daily migraine headaches, given the refractory nature of her pain and failure to conservative management. The patient would be a candidate for a pericranial radiofrequency ablative nerve block. The most common location to target is the bilaterally greater and lesser occipital nerves.
  • According to a 2016 study, 35% percent of pericranial ablative nerve blocks for chronic headaches target the greater and lesser occipital nerves.
  • For probe placement, the greater occipital nerve is slightly inferior to the superior nuchal line and medial to the occipital artery. Lesser occipital nerve is lateral to the occipital artery and medial to the mastoid process.
  • The lesser occipital nerve is located lateral to the occipital artery and medial to the mastoid process. Separately the bilateral supraorbital and supratrochlear nerves are targeted in about 14 percent of cases of pericranial nerve blocks.
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7
Q

A 30-year-old male patient presents to the hospital following a wakeboarding injury with an acute type II odontoid fracture in good alignment. The CT spine revealed a posteriorly oblique fracture type with a fracture gap of 2.5 mm and an angulation of 12 degrees. Magnetic resonance imaging scan (MRI) images revealed an intact transverse ligament. His body mass index (BMI) is 20 kg/m2. Which of the following is the most rational management strategy for patients presenting with such clinical characteristics?

  1. Halo fixation
  2. Rigid cervical collar
  3. C1-C2 posterior instrumentation
  4. Odontoid screw
A

4. Odontoid screw

  • The patient has sustained an unstable type II posterior oblique odontoid fracture.
  • The patient has an intact transverse ligament allowing for odontoid screw placement.
  • The anterior odontoid screw fixation preserves the C1-C2 movements and is therefore preferred compared to the posterior fixation.
  • The patient is otherwise thin without anatomical barriers to the technical placement of the odontoid screw.
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8
Q

A 58-year-old man is brought to the emergency department after having a 4-foot fall from a hammock. On physical examination, there is mild low back pain and no neurologic deficit. On computed tomographic imaging, there is an L1 fracture in the posterior vertebral body with a 25% height reduction with a widened interpedicular distance. Magnetic resonance imaging shows that the posterior ligamentous complex is intact. Which of the following is the most appropriate management strategy for this patient?

  1. Decompression and fusion
  2. Vertebroplasty
  3. Adequate pain control and early ambulation
  4. Fusion without decompression at 2 levels above and below the injured level
A

3. Adequate pain control and early ambulation

  • In patients with an intact posterior ligamentous complex and no neurological symptoms, nonoperative treatment is recommended.
  • Patients with adequate pain control can begin early mobilization without bracing.
  • The use of bracing with a spinal orthosis can provide symptomatic pain relief but does not affect the outcome.
  • Vertebroplasty is indicated for patients with intractable back pain after 4-6 weeks of conservative treatment.
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9
Q

A 24-year-old female who had delivered a healthy male baby through normal vaginal delivery one week back presents to the emergency department with severe headache and vomiting. She is not having any comorbidities and is not on any drugs other than folate supplementation. On clinical examination, her Glasgow coma scale score is 15, and she does not have any neurological deficits. Computed tomogram of the brain does not reveal any abnormalities. Neurology consultation is done, and a magnetic resonance imaging of the brain with magnetic resonance venogram is ordered. The report comes as thrombosis involving the superior sagittal sinus and right transverse sinus. The brain parenchyma is reported to be normal. Blood investigations do not show any evidence of thrombophilia. How should the patient be managed?

  1. Start on warfarin with target INR 2-3 and continue for 1 year
  2. Start on body-weighted subcutaneous low-molecular-weight heparin followed by warfarin for 3 months
  3. Local intravenous thrombolysis
  4. Mechanical thrombectomy
A

2. Start on body-weighted subcutaneous low-molecular-weight heparin followed by warfarin for 3 months

  • The treatment of a patient with cortical venous sinus thrombosis (CVST) in the acute phase consists of either body weight- adjusted subcutaneous low-molecular-weight heparin (LMWH) or dose-adjusted intravenous heparin, provided there is no contraindication for anticoagulation.
  • If CVST is due to a transient risk factor, oral anticoagulation may be given for 3 months. In idiopathic CVST and in patients with mild thrombophilia (heterozygous factor V Leiden or prothrombin G20210A mutation, and high plasma levels of factor VIII) oral anticoagulation is continued for 6–12 months. In this patient, the risk factor is transient (post-puerperal). Hence anticoagulation may be given for three months only.
  • Life long anticoagulation should be given in patients with (1) recurrent CVST, (2) CVST with severe thrombophilia including antithrombin, protein C or protein S deficiency, homozygous factor V Leiden or prothrombin G20210A mutation, and antiphospholipid antibodies.
  • Systemic or local thrombolysis and mechanical thrombectomy are not indicated in patients with CVST. These are tried if there is medical deterioration due to CVST, provided other causes are ruled out.
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10
Q

A 44-year-old male patient is being seen in the emergency department (ED) with complaints of a left eyelid drooping for two days. In the ED, his vitals were found to be stable. He underwent basic workup with CBC, metabolic profile, and chest radiograph, all of which came to be normal. The emergency team was concerned about his symptoms, so a CT head was ordered, which was normal as well. Based on these findings, neuro was consulted, and the patient was found to have left periorbital pain and restriction of the eye movements. MRI of the brain reveals contrast enhancement with thickening in the cavernous sinus region. While going over the results and the diagnosis, the patient asks about the prognosis of his condition. What should be the response?

  1. It is an end-stage disease and there is no treatment
  2. There is no recurrence of this condition
  3. Relapses tend to occur in about 40% to 50% of patients
  4. Symptoms will not get better and he will have a permanent deficit
A

3. Relapses tend to occur in about 40% to 50% of patients

  • The patient has symptoms of Tolosa-Hunt Syndrome (THS) that is described as severe and unilateral periorbital headache associated with painful and restricted eye movements. Relapses tend to occur in about 40% to 50% of patients and can be ipsilateral, contralateral, or bilateral.
  • Tolosa Hunt Syndrome is characterized by the dramatic symptomatic improvement seen with Glucocorticoids. Symptom improvement, especially pain relief, is usually seen 24 to 72 hours after starting steroids, with the majority of patients reporting improvement within one week. Cranial nerve palsies improve gradually and can take anywhere from two to eight weeks for recovery. It is unusual to have residual deficits after steroid treatment.
  • Relapses are more common in younger patients than in older patients. Every relapse should ideally be investigated with a full workup as Tolosa Hunt syndrome is a diagnosis of exclusion.
  • Tolosa Hunt Syndrome is characterized by the dramatic symptomatic improvement seen with glucocorticoids. Symptom improvement, especially pain relief, is usually seen 24 to 72 hours after starting steroids, with the majority of patients reporting improvement within one week. Cranial nerve palsies improve gradually and can take anywhere from two to eight weeks for recovery. It is unusual to have residual deficits after steroid treatment.
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11
Q

A 35-year-old who is pregnant with no previous screening is evaluated with antenatal ultrasound. The ultrasound reveals a frontal bone indentation and cerebellum wrapping around the brain stem. If an MRI is completed at a later date, which of the following would be the most likely finding?
1. Agenesis of the corpus callosum
2. Lissencephaly
3. Large posterior fossa
4. Small posterior fossa

A

4. Small posterior fossa

  • This fetus has a Chiari II malformation. Neuroimaging with magnetic resonance imaging (MRI) is the most important modality to evaluate these patients.
  • MRI posterior fossa classic findings include a small posterior fossa, brainstem appearing downwardly displaced, low-lying and elongated fourth ventricles, beaked tectal plate, and inferior displaced cerebellar tonsil and vermis through the foramen magnum.
  • MRI spine fossa classic findings include spina bifida aperta, myelomeningocele, and tethered core.
  • Antenatal ultrasound classic findings include the lemon sign (frontal bone indentation depicting a lemon) and banana cerebellum sign (cerebellum wrapping around the brain stem).
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12
Q

A 5-year-old boy presented with defective vision and with a deviated RE. The parents noticed the defective vision one year ago. On examination, vision in RE was 6/60 and LE-6/6. RE had esotropia. RE anterior segment examination was within the normal limit. RE fundus examination revealed a funnel-shaped optic nerve head. It had a central whitish fibrous tissue and was surrounded by a ring-shaped area of chorioretinal pigmentary disturbance. The retinal vessels originated as multiple straight narrow branches at the edge of the disk. Systemic association was suspected, and an MRI brain was ordered. What findings are expected on the MRI brain?
1. Herniation of brain tissue and overlying meninges through a defect in the cranium
2. Deep white matter lesions near the ventricles
3. An intracranial space-occupying lesion with obstructive lateral ventricle hydrocephalus
4. A dural-based mass which is isointense on T1 and T2 and enhances vividly

A

1. Herniation of brain tissue and overlying meninges through a defect in the cranium

  • RE fundus evaluation reveals features of morning glory syndrome (MGS). It is a primary mesenchymal abnormality with a partial development of the lamina cribrosa and incomplete closure of the posterior sclera wall. It is characterized by a funnel-shaped optic nerve head. It has a central whitish fibrous tissue and is surrounded by a ring-shaped area of chorioretinal pigmentary disturbance. The retinal vessels originate as multiple straight narrow branches at the edge of the disk. MGS can be associated with basal encephalocele.
  • Basal encephalocele is the herniation of neural tissue through a defect in the basal skull. MGS is usually associated with the trans-sphenoidal type. Other reported basal encephaloceles include spheno-ethmoidal, sphenopharyngeal, and trans- ethmoidal. These defects can be misdiagnosed as nasal polyps; it is thus important not to biopsy such lesions.
  • MRI is the modality of choice for a case of encephalocele. It helps to delineate the location of the encephalocele, characterize the hernia sac (based on the size, contents, and status of the herniated neuroparenchyma), and identify other associated intracranial malformation. All the above factors help in preoperative planning and prognosis.
  • The child has MGS. MGS can be associated with basal encephalocele. On MRI, it is identified by herniation of brain tissue and overlying meninges through a defect in the cranium. MRI deep white matter lesions near the ventricles are seen in multiple sclerosis. An intracranial space-occupying lesion with obstructive lateral ventricle hydrocephalus can be seen in tuberculoma or neoplasm. A dural-based mass that is isointense on T1 and T2 and enhances vividly suggests meningioma. Thus herniation of brain tissue and overlying meninges through a defect in the cranium is correct.
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13
Q

A 45-year-old man is admitted to the hospital and managed supportively following a severe head injury. The neurological examination revealed brainstem areflexia. The apnea test did not elicit any spontaneous respiratory drive in the patient despite the arterial blood PCO2 level of 60 mm Hg. However, on painful stimulus, the patient occasionally shows flexion and crossing of his arms over his chest. What is the next step in the management of this patient?

  1. Declare brain death
  2. Continue supportive management
  3. The patient is in a persistent vegetative state
  4. Do ancillary tests for diagnosing brain death
A

1. Declare brain death

  • The patient with brain stem death can sometimes show limb movements secondary to spinal reflexes. This is still is compatible with the diagnosis of brain death.
  • The characteristic movement shown by the patient is typical of Lazarus sign sometimes seen in brain death patients.
  • Coma, brain stem areflexia, and a positive apnea test is confirmatory of a brain death status.
  • The ancillary tests are only justified in cases wherein the apnea test is inconclusive, hazardous, or in patients in whom brainstem reflexes should not be elicited such as vestibulo-ocular reflex in patients with cervical cord injury.
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14
Q

A 16-year-old patient was being managed with tracheostomy following a severe head injury. The patient was later decannulated following his neurological improvements. However, the patient kept having stridor with tachypnea uniquely when placed in the supine position. There was some improvement when the patient was nursed in the lateral position. What might be the probable cause for such findings in the patients?

  1. Subglottic stenosis
  2. Tracheoesophageal fistula
  3. Vocal cord paralysis
  4. Tracheomalacia
A

4. Tracheomalacia

  • The symptoms of respiratory stridor in patients, especially in a supine position, are characteristic following removal of the tracheostomy tube is characteristic of tracheomalacia.
  • Tracheomalacia results from an ischaemic injury to the tracheal cartilages with subsequent destruction and necrosis. This leads to a gravity-mediated collapse of the weakened trachea, especially in a supine position.
  • Making a high incision in the trachea while creating a stoma leads to damage to tracheal cartilages in the first and second tracheal rings, thereby predisposing to tracheomalacia.
  • Stridor due to subglottic stenosis will not improve upon the lateral or prone positioning of the patient. Tracheoesophageal fistula presents with choking and coughing right after each feeding of the patient.
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15
Q

52-year-old female presents with cognitive issues. She claims that she has difficulty finding words and forgets the names of familiar people. She would frequently lose small objects like her wallet and keys and would have to search for them as she cannot recall where she placed them. She is still able to do daily activities without issues and still handles her finances. Her mother had a stroke at 56-years-old, and her older brother has dementia. She has a history of migraine with aura for the past 20 years, mild stroke 5 years ago with no deficits, no hypertension or diabetes. She does not smoke or take alcohol. Mini-mental status exam is 24, no motor and sensory deficits, (+) Babinski bilaterally. A cranial MRI was requested. Which of the following would you expect to see?
1. T2/Fluid-attenuated inversion recovery (FLAIR) hyperintensities on the corpus callosum and optic nerve lesions
2. T2/FLAIR hyperintensities in the occipital lobe
3. T2/FLAIR hyperintensities in the anterior temporal pole
4. Atrophy of the frontal and temporal lobe

A

3. T2/FLAIR hyperintensities in the anterior temporal pole

  • Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) should be suspected in patients with a strong family history of early strokes and dementia. The most common symptoms are migraine headaches, cognitive deficits, ischemic episodes, and psychiatric disorders. The average age of onset is around 30 years. A migraine with aura is the most common initial symptom, present in about 55% of people diagnosed with CADASIL, and slightly more common in women.
  • CADASIL has a somewhat characteristic appearance on MRI, and a radiologist may suggest the diagnosis. Abnormal T2/FLAIR white matter hyperintensities, similar in appearance to those seen with the chronic microvascular disease, are present in CADASIL in the anterior temporal, external capsule, and paramedian superior frontal subcortical white matter. Microbleeds are seen with a greater prevalence in CADASIL patients. These are best seen on a T2*- gradient recalled echo (GRE) MRI sequence as multiple punctate foci of susceptibility/hypointensity.
  • The lesions in the anterior temporal pole distinguish CADASIL from ischemic leukoaraiosis.
  • Optic nerve and corpus callosum lesions are more commonly found in multiple sclerosis.
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16
Q

A 16-year-old male patient was brought to the emergency department after a motor vehicular accident. On examination, vital signs reveal blood pressure 140/85 mmHg, heart rate 112 bpm, the regular, temperature was afebrile. He was drowsy but easily arousable, oriented, and able to follow commands. He has a 3 cm laceration on the left parietal region, and he complains of severe headache. The cranial nerve examination was normal. The motor exam shows a drift on the right upper and lower extremity with a 40% sensory deficit on the right. Babinski was positive on the right. A head CT was requested, which shows a crescent-shaped hyperdensity on the left cerebral convexity with mass effect and midline shift of structures. Surgical intervention should be done urgently in this patient in which of the following scenario?
1. GCS 14
2. Hematoma thickness of 9mm
3. Midline shift of 7mm
4. ICP of 18mmHg

A

3. Midline shift of 7mm

  • Surgery for decompression of subdural hematoma is done if there is a midline shift of 5 mm or greater.
  • Surgery also is indicated if there is an acute subdural hematoma that exceeds 10 mm in thickness.
  • Sometimes, if the midline shift is 5 mm and there are no neurological deficits like dilated pupils or elevated intracranial pressure, observation can be adequate.
  • Surgery is recommended when a subdural hematoma is associated with compressed basilar cisterns.
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17
Q

A 3-month-old baby boy had a ventriculoperitoneal shunt placed when he was 21 days old. He was pre-term and had a grade III intraventricular hemorrhage, which was initially treated with serial anterior fontanelle taps but eventually required the shunt. On a routine visit, the mother asked the pediatrician what signs should she evaluate to confirm adequate shunt function. Which of the following represents evidence of proper function?
1. Good urine output
2. Loud crying sounds
3. Bulging fontanelle
4. Normal head circumference

A

4. Normal head circumference

  • Shunt malfunction will accumulate cerebrospinal fluid, which may lead to an increased in head circumference.
  • Shunt dysfunction in toddlers and infants presents with enlargement of the head, full tense fontanel when the baby is upright, prominent scalp veins, swelling along shunt tract, vomiting, irritability, sleepiness, downward deviation of eyes, less interest in feeding, loss of previous milestones.
  • Children and adults with shunt malfunction present with nausea, vomiting, headache, vision problems, irritability, tiredness, swelling along shunt tract, personality change, loss of balance, difficulty waking up or staying awake, a decline in academic or job performance.
  • Urine output does not have a correlation with shunt functionality as the cerebrospinal fluid is absorbed in the peritoneal cavity
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18
Q

A 61-year-old man is brought to the emergency department for abrupt loss of consciousness after a fall at his home that happened 2 hours ago. His past medical history is notable for atrial fibrillation and New York Heart Association class 3 congestive heart failure. He has otherwise been well without additional medical problems. His medications include warfarin and metoprolol. Currently, his temperature is 98.6, his blood pressure is 125/75 mm Hg, his pulse rate is 77/min and irregular, and his respiration rate is 17/min. The patient is obtunded without localizing neurologic findings. The cardiac examination reveals an irregularly irregular rhythm. The remainder of the examination is unremarkable. A head CT scan shows a large subdural hematoma. Lab values indicate a hemoglobin level of 12.8 g/dL, platelet count of 283,000/μL, and INR of 3.0. Intravenous vitamin K is administered, and plans are made for emergent neurosurgery. Which of the following is the next most appropriate step in managing this patient?
1. Fresh frozen plasma
2. Factor IX complex
3. Cryoprecipitate
4. Idarucizumab

A

2. Factor IX complex

  • Factor IX complex, known as prothrombin complex concentrate (PCC), should be used to reverse the effects of warfarin anticoagulation in patients experiencing severe bleeding and those requiring urgent surgery.
  • Although vitamin K alone can be effective in reversing the effect of warfarin, its hemostatic effect can take several hours.
  • In urgent situations, simultaneous replacement of the vitamin K– dependent coagulation factors are necessary. Fresh frozen plasma treatment involves a large volume infusion and may cause problems in a patient with congestive heart failure.
  • Cryoprecipitate would be indicated to treat severe hypofibrinogenemia, usually arising as a consequence of disseminated intravascular coagulation (DIC) or severe liver disease. Idarucizumab is a monoclonal antibody that binds the non–vitamin K antagonist oral anticoagulant dabigatran and causes a rapid reduction in available dabigatran in the body for up to 24 hours. Idarucizumab will not reverse warfarin anticoagulation.
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19
Q

A 65-year-old patient with a past medical history of a previous extensive laminectomy with medial facetectomy at L4 and L5 levels presents to your office with persistent, localized pain at the operative site. The patient complains of shooting back pain in his lower lumbar region that radiates into his posterior left leg. The patient states his pain is worse with prolonged walking or standing up straight and improves with bending forward. On physical exam, he is tender to palpation over the area of pain, and he has a decreased sensation in the distribution of the L4 dermatome on the left. Muscle strength is intact bilaterally for his lower extremities, but his deep tendon reflex is 1/4 for L4 on the left, and 2/4 bilaterally for S1. Straight leg raise is positive on the left. The dynamic x-ray films degenerative arthritis of the lumbar spine. What is the origin of the characteristic pain in the patient?

  1. Faulty hardware
  2. Traversing nerve root
  3. Nerve of Luschka
  4. Infective pathology
A

2. Traversing nerve root

  • When discussing radicular pain, it is crucial to review human neuroanatomy, where nerve roots exit the spinal cord.
  • Patients with symptomatic spinal stenosis will experience symptoms of neurogenic claudication due to compression of the transversing nerve root.
  • Patients with neurogenic claudication often have bilateral and asymmetrical radicular pain, weakness, and or sensory loss of the lower extremities. These symptoms are made worse by walking or standing for prolonged periods. An L5 nerve root compression occurs from central disc protrusion of L2-L3 or L3- L4, a lateral disc protrusion at L4-L5, or from a far-lateral protrusion at the foramen at L5-S1.
  • The involvement of the existing and the traversing nerve roots leads to radicular pain characterized, such as sciatica. The areas of the body which we can contribute to a single nerve root are called autonomous zones. These independent areas of lumbosacral radiculopathy include the anterior thigh (L2 and L3), the medial calf (L4), and the dorsum of the foot (L5), and the sole (S1).
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20
Q

A 30-year-old primiparous woman with a BMI of 32 kg/m2 requests epidural analgesia for labor pain. After obtaining consent, the epidural space is located at a depth of 7 cm with loss of saline resistance. A catheter is inserted successfully into the epidural space through a Tuohy needle despite initial slight resistance. On removal of the needle, it is noted that the catheter broke at the 6 cm mark, and the missing segment is not seen either on the drapes or the floor. Which of the following is the next most appropriate step in the further management of the broken epidural catheter?

  1. CT scan of the lumbar region
  2. Plain x-ray of the lumbar region
  3. Surgical exploration of the back for the missing segment of the catheter
  4. Perform an epidural at a different space
A

1. CT scan of the lumbar region

  • The epidural catheter is radioopaque; it may be visible on plain x-ray, but a CT scan is the most helpful in delineating the broken catheter tip location.
  • The catheter fragment is unlikely to cause any further problems to the patient, but fibrosis around the nerve root may produce radicular irritation signs.
  • An immediate management plan for labor analgesia should be instituted. This may include a discussion with the patient and obstetrician and depends on the progress of labor.
  • Epidural anesthesia is a safe procedure and is routinely performed by anesthesiologists. Breakage of an epidural catheter is rare but a worrisome complication. However, if this happens, the presence of retained epidural catheter fragment should be properly documented and informed to the surgical team and the patient.
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21
Q

A 65-year-old man with a history of peripheral artery disease and hypertension is brought to the clinic for evaluation. His wife reports sudden personality changes, nausea, third cranial nerve palsy, and vision loss. Which artery is most likely affected?
1. Anterior cerebral artery
2. Middle cerebral artery
3. Vertebral artery
4. Basilar artery

A

4. Basilar artery

  • Rostral brainstem infarction (top of the basilar syndrome) is known to cause sudden behavioral changes, oculomotor deficits, and visual loss.
  • Motor function is usually preserved.
  • This is usually a result of atherosclerotic disease.
  • Posterior circulation strokes often present with nausea.
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22
Q

A 38-year-old woman presents to the clinic complaining of spontaneous galactorrhea. The patient has a history notable for severe claustrophobia. The review of systems is unremarkable. Visual fields and cranial nerves are intact. Her serum prolactin level is 177 ng/mL (normal range: 2 – 29 ng/mL) and urine HCG is negative. What is the next best step in the evaluation of this patient?
1. CT sella protocol with intravenous contrast
2. CT sella protocol without intravenous contrast
3. CT angiogram of the head
4. Radionuclide cisternography

A

1. CT sella protocol with intravenous contrast

  • A dedicated MRI protocol with and without intravenous contrast is the first-line choice for evaluating for a functional pituitary microadenoma. However, if patients have absolute contraindications to MRI (claustrophobia, a noncompatible pacemaker or metallic foreign body), a CT with intravenous contrast may be obtained.
  • Noncontrast CT imaging of the pituitary adds little diagnostic value. This repeat imaging increases the patient’s radiation dose without clinical benefit. This is even more relevant to newer CT scanners, which can generate virtual noncontrast images without any added radiation dose to the patient.
  • A CTA head with intravenous contrast is an ideal study to evaluate for vascular regions within the head. However, this is not a recommended imaging study for the initial evaluation of possible pituitary microadenoma.
  • Radionuclide cisternography is not indicated for the evaluation of pituitary adenomas.
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23
Q

A 58-year-old woman with a past medical history significant for insulin-dependent type 2 diabetes, hypertension, hyperlipidemia, and morbid obesity presents to the office for further evaluation of right leg burning, tingling, and difficulty ambulating over the last two months. She denies any sharp or dull achy pain. Pain is worse with walking, states she has been having difficulty standing on her toes. Her symptoms have not been getting worse or better. She states she had similar symptoms a few years ago, but they went away on their own. She denies any bowel or bladder incontinence or saddle anesthesia. On physical exam, she is noted to have diminished sensation along the S1 dermatome of her right leg. Lower extremity muscle strength is 5/5 on the left and 3/5 on the right in leg extension. Her deep tendon reflexes are 2/4 for L4 but diminished 1/4 for S1 on the right and 2/4 on the left. The straight leg raise is negative. She has an antalgic gait upon ambulation and difficulty walking on her toes. Which of the following is the next best step in the management of this patient?
1. Electromyography
2. MRI of the lumbar spine
3. Physical therapy
4. X-ray of the foot 3 views

A

2. MRI of the lumbar spine

  • The median duration of diabetes is four years before being diagnosed with diabetic amyotrophy. Diabetic amyotrophy is a specific type of lumbosacral plexopathy. MRI with gadolinium contrast is the best test for the evaluation of the lumbosacral plexus.
  • The differential diagnosis of lumbosacral plexopathy is broad, including radiculopathy. An MRI can help differentiate plexopathy from radiculopathy.
  • Conventional magnetic resonance imaging (MRI) is useful to localize the area of injury or disease and identify the likely cause of lumbosacral plexopathy.
  • In diabetic or idiopathic amyotrophy, symptoms of plexopathy are likely due to either ischemic injury secondary to a microscopic vasculitis or possible demyelination secondary to axonal dystrophy. These findings could be seen on MRI.
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24
Q

A 62-year-old man presents with a three-day history of a new-onset frontal headache that progressed to a generalized headache. He describes the pain as a nine out of ten, throbbing, and does not improve with over the counter medications. He denies any changes in vision, vomiting, seizures, limb weakness, behavioral changes, or weight loss. He has a past medical history of hypertension, type 1 diabetes mellitus, and a 37 pack-year smoking history. However, he is not compliant with his medications. He denies any family history of a cerebral hemorrhage. His vital signs show oxygen saturation 98% on room air, respiratory rate 26 per minute, heart rate 79 bpm, blood pressure 162/96 mmHg, and temperature 98 F. The physical examination is unremarkable. A CT scan of the brain reveals an isolated hyperdense right temporal occipital lobe lesion measuring 1.7 cm in its longest axis. An MRI shows a large right temporal occipital lesion, with several smaller lesions bilaterally with surrounding edema. What is the most appropriate initial step in the management of this patient’s condition?
1. Administer intravenous dexamethasone
2. Surgical resection
3. Whole-brain radiotherapy
4. Stereotactic radiosurgery

A

1. Administer intravenous dexamethasone

  • The first step in the management of newly diagnosed brain metastases is the treatment of intracranial edema.
  • Oral or intravenous steroids, such as dexamethasone, are commonly used. A loading dose of 10 mg intravenous (IV) dexamethasone followed by 4 mg IV every six hours is one dosing regimen.
  • After the initial clinical response, which can occur rapidly, the dose may be tapered to avoid many of the long-term high-dose steroid administration’s adverse effects.
  • Following the initiation of steroids, definitive management may be initiated. Treatment options include surgical resection (for limited brain metastases in patients with good performance status and surgically accessible lesions), whole-brain radiotherapy, and stereotactic radiosurgery.
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25
Q

A 25-year-old rugby player, who sustained a head trauma two days ago, presents to the hospital with complaints of a vertical double vision, which increases with the downward gaze. On examination, the left eye is tilted upward as compared to the right eye. Which direction should the patient asked to look to test the function of the paralyzed muscle?
1. Upward only
2. Downward only
3. Down and in
4. Down and out

A

3. Down and in

  • Looking ‘down and in’ isolates the function of the superior oblique muscle and is the proper way to test the function of this muscle.
  • Injury to the trochlear nerve (CN IV) results in weakness of the downward movement of the eye.
  • Trochlear nerve palsy also results in the tilting of the affected eye upward due to the unopposed action of the rest of the eye muscles.
  • ‘Upward only’ movement of the eye is due to the action of the superior rectus muscle. ‘Down and out’ movement of the eye tests the action of the inferior rectus muscle. ‘Downward only’ movement of the eye is due to the action of the inferior rectus muscle.
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26
Q

A 48-year-old woman with a history of asthma and known ovarian tumor presents to the clinic with new back pain and bilateral lower extremity weakness. She is status-post hysterectomy with salpingo-oophorectomy and additional chemo- radiation 2 years prior to the current presentation. On examination, she has mild hip flexor weakness which is fairly symmetric bilaterally. She has preserved sensation and deep tendon reflexes. During her initial workup, hematology and metabolic labs are normal and her neuroimaging does not show any abnormality with gadolinium enhancement of the nerve roots. EMG nerve conduction studies are planned to help diagnose the underlying etiology. Which of the following sets of findings is most consistent with radiation-induced lumbosacral plexopathy in this patient?

  1. Nerve conduction studies: normal sensory and reduced amplitude of compound motor action potentials; needle electromyography: fibrillation and polyphasic potentials.7
  2. Nerve conduction studies: normal sensory and reduced amplitude of compound motor action potentials; needle electromyography; fibrillation potentials with positive sharp waves and no polyphasia
  3. Nerve conduction studies: absent sensory and reduced amplitude of compound motor action potentials: needle electromyography shows myokymic potentials
  4. Nerve conduction studies: normal sensory and motor nerve action potentials; needle electromyography: fibrillation potentials with polyphasic potentials
A

3. Nerve conduction studies: absent sensory and reduced amplitude of compound motor action potentials: needle electromyography shows myokymic potentials

  • Myokymic potentials in conjunction with other findings of plexus involvement noted on nerve conduction studies (such as low amplitude or absent sensory and motor nerve action potentials) are pathognomonic of radiation-induced plexopathy.
  • Bilateral involvement is likely more indicative of radiation- induced plexopathy as opposed to neoplastic lumbosacral plexopathy which presents unilaterally.
  • The nerve conduction study and EMG have to be taken in conjunction with the clinical presentation and not independently.
  • Radiation-induced plexopathy typically presents about a year or later from the time of initial radiation to the pelvis.
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27
Q

A 50-year-old male with a past medical history of HIV and HAART therapy presented with worsening headaches for the last 4 weeks. He underwent a CT head, which showed a left- sided mass in the frontal lobe, and an MRI confirms the mass. CSF shows atypical cells and positive for the Ebstein Barr virus. Flow cytometry shows CD 20 +ve cells. Opthalmology evaluation shows a possible retinal involvement, the biopsy of which showed a diffuse large B-cell lymphoma. What should be done next?
1. A repeat lumbar puncture for further testing
2. Radiation to the mass in the frontal lobe
3. High-dose methotrexate based therapy
4. Surgery with stereotactic radiosurgery

A

3. High-dose methotrexate based therapy

  • Primary CNS lymphoma should be aggressively treated with high-dose methotrexate based therapy.
  • Ebstein Barr virus may have a causal link for the development of primary CNS lymphoma.
  • About 25% of patients with primary CNS lymphoma, has the involvement of the eye.
  • Radiation or surgery is not first-line therapy for the treatment of CNS lymphoma.
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28
Q

A 60-year-old man is brought to the outpatient department with a complaint of severe headache for the past two hours associated with vomiting. On examination, he does not have any neurological deficits. A computed tomogram scan of the head shows hyperdensity in the left Sylvian fissure. Which of the following drugs is approved by the FDA to reduce the incidence and severity of ischemic deficits in this patient?
1. Nicardipine
2. Nisoldipine
3. Clevidipine
4. Nimodipine

A

4. Nimodipine

  • Nimodipine is FDA approved to improve the neurological outcome in patients treated for subarachnoid hemorrhage.
  • Nicardipine is FDA approved to treat chronic stable angina as an immediate-release product and management of hypertension as an immediate and sustained-release product.
  • Nisoldipine is FDA approved to treat hypertension.
  • Clevidipine is FDA approved to treat hypertension.
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29
Q

A 40-year-old man presented to the emergency department with acute, sudden onset back pain radiating down both legs. He reports some altered sensations in his genitals and has not passed urine. He has a history of chronic back pain and takes paracetamol, tramadol, and pregabalin. He tells you that he has had an MRI scan of his spine in the past, but surgical intervention was not recommended. Examination of his lower limbs reveals 4 out of 5 MRC power bilaterally and altered sensation along the L4, L5, and S1 nerve root bilaterally. There is some melted sensation in the saddle area, but the sensation remains intact. A PR exam reveals normal tone and reflex is present. A bladder scan reveals a residual volume of 100 mL. His current presentation is then superseded by an interval MRI scan of his lumbosacral spine. The scan is compared to the previous and shows that the large 5 cm multiloculated cyst arising from the L5 nerve root sheet has changed in appearance and shows changes that represent an acute hemorrhage within the cyst long. There is incomplete compression of the cauda equina. The scan is compared to the previous and shows that the large 5 cm multiloculated cyst arising from the L5 nerve root sheet has changed in appearance and represents an acute hemorrhage within the cyst wall. There is incomplete compression of the cauda equina. How would you proceed with management?
1. Reassurance and discharge
2. Admission for monitoring
3. Urgent decompression of L5
4. L5 Nerve root block for relief

A
  1. Reassurance and discharge

  • Tarlov cysts are usually incidental, asymptomatic findings on an MRI scan. However, some may be causing the patient symptoms, and some may have complications such as bleeding in this case.
  • There is limited evidence in the literature on the optimal management of partial cauda equina compression secondary to hemorrhage within a Tarlov cyst. However, it is noted that without significant signs and symptoms of cauda equina clinically, Conservative management may be an option.
  • Bleeding is a recognized complication and may be the pathophysiology behind developing a Tarlov cyst, i.e., secondary to trauma. However, they may mimic cauda equina syndrome, admission and monitoring of symptoms would provide the least invasive option. Yates et al. have described successful management this way, with symptom-free disease at five years.
  • Reassurance and discharge are incorrect due to signs and symptoms and a radiological indication of partial compression of cauda equina. Decompression surgery at this stage may be justifiable depending on severity and discussion with the patient. But the clinical signs for cauda equina compression are not as severe and may initially be amenable to conservative management. An L5 nerve root block at this stage is not advisable given an acute hemorrhage.
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30
Q

The orthopedic and neurosurgical teams are called to the operating room for a 34-year-old male patient who suffered an ejection from a motor vehicle rollover accident. While neuromonitoring is available and being used, the team notices a decrease in amplitude, and latency is noted with the N14 potential. What is the most likely source of these changes requiring surgical attention?
1. A brachial plexus injury sustained during prolonged labor on a macrosomic neonate
2. An unstable cervical spine in a patient extracted from an MVC
3. An elderly patient who has fallen and sustained an expanding subdural hematoma
4. Crush injury to the lower extremity in a factory worker

A

2. An unstable cervical spine in a patient extracted from an MVC

  • Although this patient’s cervical spine status is unknown, were they to have an injury to the area it would be most likely to affect the N14 potential, which is generated at the cervicomedullary junction.
  • This should be a concern while evacuating the patient from the motor vehicle. The N14 potential is generated at the cervicomedullary area.
  • An expanding subdural hematoma would most likely affect the N20 potential, which is generated at the cortex.
  • A lower extremity would most likely affect peripheral nerve potentials from the lower extremity, such as N8 generated from the tibial and sciatic nerves.
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31
Q

A 30-year-old primigravida undergoes a lower segment cesarian section under spinal anesthesia due to the non- progression of labor. Postoperatively, she has severe headaches, especially on standing up. She subsequently undergoes an epidural blood patch for the management of her problem. What is the most common problem with this treatment?
1. The requirement of several blood patches to obtain relief
2. They often require surgical exploration
3. Infection at the injection site
4. Back pain

A
  1. Back pain

  • It is very uncommon to require more than two epidural blood patches (EBPs) to resolve a post-dural puncture headache (PDPH).
  • Surgical exploration for persistent PDPH is rare.
  • Infection at the site of EBP occurs in less than 1% of patients.
  • Back pain from autologous blood injection may limit the volume of blood to be injected. Most patients will have some degree of back discomfort, usually lasting up to 2 weeks
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32
Q

A 10-year-old boy is under treatment for acute lymphoblastic leukemia and is planned for intrathecal methotrexate to prevent CNS relapse. Where is the most appropriate point of access to the intrathecal space in this patient?
1. Lumbar puncture
2. Right frontal
3. Right parietal
4. Ventriculoperitoneal shunt

A

2. Right frontal

  • Ommaya reservoir, a dome-shaped port with an intraventricular catheter attached to it, is commonly used for delivery for antineoplastic drugs into the intrathecal space.
  • Ommaya reservoir is routinely placed subcutaneously over the right Kocher’s point, which is approximately 2-3 cms away from the midline and 1 cm anterior to the coronal suture. Unless indicated for a tumor cyst or in the presence of anatomical distortion, the intraventricular catheter is placed through a burr hole over right Kocher’s point.
  • The bulge over the scalp is palpated, and under aseptic precautions, a butterfly cannula is inserted at an oblique angle to penetrate the reservoir.
  • Ommaya reservoir helps prevent repeated lumbar punctures. Unless indicated for a tumor cyst or by the presence of anatomical difficulty, the intraventricular catheter is placed through a burr hole over right Kocher’s point.
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33
Q

A 40-year-old male was brought by an ambulance to the emergency department after a fall and accompanying head trauma. He denies any loss of consciousness. The initial primary survey showed his blood pressure was 105/74 mmHg, heart rate was 110/min, respiratory rate was 18 breaths/min, and oxygen saturation was 96% at room air. On physical examination, the patient was noted to have periorbital ecchymosis with tarsal plate sparing and hemotympanum. There was also a left-sided complete facial weakness. What will be the most likely finding on the CT-scan of the skull?
1. Comminuted fracture of the ethmoid bone
2. Discontinuity in the left petrous temporal bone
3. Transverse fracture of the right temporal bone
4. Osseous defect in the cribriform plate

A

2. Discontinuity in the left petrous temporal bone

  • The base of the skull includes at least five bones; the cribriform plate of the ethmoid bone, the orbital plate of the frontal bone, the petrous and squamous portions of the temporal bone, the sphenoid, and the occipital bones.
  • The most common bone involved in basilar skull fractures is the petrous portion of temporal bone. A CT-scan of the skull is most likely to show a fracture of the petrous temporal bone,
  • Fractures that involve the petrous ridge of the temporal bone will cause blood to pool behind the tympanic membrane causing it to appear purple, which is called hemotympanum. Periorbital ecchymosis (raccoon eyes): Pooling of blood surrounding the eyes is most commonly associated with fractures of the anterior cranial fossa.
  • Temporal fractures, which are most common, are associated with carotid injury, injury to cranial nerves VII or VIII, and mastoid cerebrospinal fluid leak.
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34
Q

A 40-year-old man presents with severe lower back pain for the past week, which started after trying to lift a weight at the gym. The pain is radiating down the left lower limb, and he is feeling weakness in his limb. He comes with a magnetic resonance imaging scan of the lumbar spine, which reveals a far lateral disc herniation at L4-5 on the left side. What can the neurological deficit be expected in this patient in his left lower limb?
1. Difficulty in ankle dorsiflexion
2. Difficulty in ankle plantarflexion
3. Difficulty in great toe dorsiflexion
4. No weakness

A

1. Difficulty in ankle dorsiflexion

  • Paracentral disc herniation in the lumbar region characteristically strikes the root exiting below the disc space. An L4-5 disc herniation most often compresses the traversing L5 nerve root.
  • Very lateral herniated disks may compress the upper root. A far lateral L4-5 herniation can compress the L4 root (producing ankle dorsiflexion weakness).
  • Free fragments can migrate and compress roots at other levels.
  • MRI imaging is superior to myelography, CT, or CT myelography in the diagnosis of disc bulge, protrusion, herniation, extrusion, or migration.
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35
Q

A 35-year-old female presents to the outpatient department with the complaint of occasional mild to moderate frontal headaches over the past few months. She also gives a history of blurring of vision. She does not have galactorrhea or amenorrhea. She comes with a magnetic resonance imaging scan of the head, which shows a sellar lesion extending to the suprasellar location and displacing the chiasm. Which of the following is the most sensitive ophthalmologic test for detecting optic pathway compromise secondary to this tumor?
1. Peripheral visual field testing
2. Central visual field testing
3. Examination of the fundus
4. Color vision testing

A

1. Peripheral visual field testing

  • The patient is having a pituitary macroadenoma.
  • The compression of the chiasm causes visual field abnormalities in a pituitary adenoma.
  • The most sensitive test for detecting optic pathway compromise due to a pituitary tumor is peripheral visual field testing.
  • Decreases in color perception are much less sensitive in the diagnosis of optic nerve compression.
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36
Q

A one-month baby boy is rushed to an emergency department following an accidental fall injury at home. CT head revealed an indentation of 1 cm without any fracture line at the left parietal bone. There were no intracranial lesions. What is the recommended plan of management for the child?
1. Surgical elevation
2. Vacuum-assisted elevation
3. Conservative management
4. Craniotomy and mesh repair

A

3. Conservative management

  • The presence of an indentation of the skull without any fracture line indicates a ping pong fracture.
  • The skulls in newborn and young infants are elastic compared to that of the skull in the adult. This leads to a ping pong pattern of fracture.
  • The elastic nature of the skull also allows for the slow remodeling of the fracture owing to the growth of the brain, especially in small-sized lesions with no other intracranial traumatic pathologies.
  • Surgical management of ping pong fracture is only indicated in cases of 1) large defect, 2) when associated with other traumatic intracranial lesions, and 3) aesthetic purposes.
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37
Q

A 50-year-old female with a 20-year-history of intermittent intorsion of the left eye and vertical episodic double vision is referred for extraocular muscle surgery to improve the symptoms. Which operation is the best procedure for this patient?

  1. Left superior oblique tenotomy and ipsilateral recession of the inferior oblique muscle
  2. Left superior oblique tenectomy with contralateral inferior rectus recession
  3. Left inferior rectus recession and left inferior oblique recession
  4. Left trochlectomy with right inferior rectus recession
A
  1. Left superior oblique tenotomy and ipsilateral recession of the inferior oblique muscle

  • The cause of the symptoms in this patient is the repeated overaction of the left superior oblique muscle causing left superior oblique myokymia.
  • Surgery to weaken the superior oblique muscle alone will increase double vision because of the overaction of the inferior oblique muscle.
  • Weakening of the left superior oblique muscle combined with the weakening of the ipsilateral inferior oblique muscle with recession will give the best result in this patient.
  • The contralateral inferior rectus (the right inferior rectus muscle in this case) sometimes needs to be recessed in addition to the ipsilateral surgery if there is persistent double vision.
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38
Q

A 38-year-old woman in her first pregnancy delivers a 4800-gm baby boy. When she attempts to walk, she scuffs the toes of her right foot. She elevates her right foot higher than normal to avoid scuffing her toes (steppage gait). Neurological examination reveals a weakness in the muscles of the anterior crural compartment. Sensory losses are present in the lateral leg and dorsum of the foot. Damage to which of the following could account for the patient’s condition?

  1. Deep fibular nerve
  2. Lumbosacral trunk
  3. Superficial fibular nerve
  4. Tibial nerve
A

2. Lumbosacral trunk

  • The patient has suffered a foot drop in her right foot.
  • The patient’s first pregnancy and very large baby boy are risk factors for neurological damage during parturition.
  • The patient’s sensory losses are consistent either with a lesion of the lumbosacral trunk (L5) or the superficial fibular nerve.
  • The deep fibular nerve could account for the foot drop, but the only sensory innervation by this nerve is the area between the big and second toes. The tibial nerve has no role in the development of foot drop.
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39
Q

A 62-year-old man presents to the clinic after sustaining an injury in a motor vehicle accident. He is having difficulty heel-walking and has diminished sensation along the anterolateral aspect of his right foot. Which of the following electrodiagnostic test finding suggests is most consistent with a diagnosis of isolated radiculopathy in this patient?
1. Normal SNAPs, normal CMAPs, positive sharp waves and/or fibrillations on needle electromyography
2. Abnormal SNAPs, normal CMAPs, positive sharp waves and/or fibrillations on needle electromyography
3. Abnormal CMAPs, normal SNAPs, normal needle electromyography
4. Normal SNAPs, normal CMAPs, normal needle electromyography

A

1. Normal SNAPs, normal CMAPs, positive sharp waves and/or fibrillations on needle electromyography

  • The nerve conduction study is typically normal in lumbosacral radiculopathy.
  • This is due to the preservation of the dorsal root ganglion, as the lesion of lumbosacral radiculopathy is typically proximal to the dorsal root ganglion.
  • Positive sharp waves and/or fibrillations are typically seen during the needle EMG portion of the test.
  • SNAPs and CMAPs on nerve conduction study are normal in lumbosacral radiculopathy.
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40
Q

A 10-year-old boy is brought to the clinic for follow-up. He sustained a hyperextension head injury during a motor vehicle accident five months ago. Vital signs include blood pressure 115/60 mmHg, heart rate 70/min, and temperature 98.6 F (37 C C). During a physical examination, the patient has a full range of motion to his cervical spine and full motor strength to his bilateral upper and lower extremities. The patient has intact sensation to light and painful stimuli to his bilateral upper and lower extremities. He is noted to have decreased vibration and fine touch sensations below the cervical spine level of C7. Which of the following additional features is most likely to be present in this patient?

  1. Loss of temperature sensation below the level of injury
  2. Normal gait
  3. Hyperreflexia
  4. Difficulty ambulating in the dark
A

4. Difficulty ambulating in the dark

  • Mechanism of injury and clinical findings are all important when making an accurate spinal cord injury diagnosis. In this question, the patient sustained a hyperextension injury, and on physical exam, he had fine touch sensation and vibration sensation loss below cervical spine level C7.
  • This patient had intact motor and light touch. This leads one to make the diagnosis of posterior spinal cord injury. Posterior cord injury is defined by the preservation of motor function, pain, and temperature, light touch, but proprioception and vibration sensations and fine touch are lost below the level of the injury.
  • The posterior portion of the spinal cord can be damaged either by direct trauma or because of a secondary injury involving the posterior spinal arteries. This syndrome is linked to injuries associated with hyperextension of the neck. Patients with this injury may ambulate, but since they lose proprioception and vibratory sensations, direct visualization of their feet is required during walking. Therefore, they may not be able to ambulate in the dark.
  • Loss of proprioception below the injured level clinically results in gait abnormalities. It is important first to make an accurate diagnosis and then know all clinical findings that are present from this diagnosis. Posterior spinal cord syndrome affects the dorsal column of the spinal cord, which is responsible for fine touch, proprioception, and vibration. It would not affect temperature sensation, which is transmitted by the lateral spinothalamic tract and is a common finding of anterior cord syndrome. Hyperreflexia is not a clinical finding of posterior spinal cord syndrome.
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41
Q

A 65-year-old female patient is brought to the hospital with severe headaches. The patient indicates that she was cooking when the pain started, and there is no trauma in the history. However, she is able to recall several family members who died of strokes, and they were all beyond the age of 60. On exam, she has no weakness but appears mildly confused. The imaging is suggestive of subcortical hemorrhages, and the appropriate treatment is started. Two days later, she is found unconscious in the hospital and is found to have additional brain hemorrhages. The patient passes away despite treatment. If pathology is obtained, what would be the expected findings?

  1. Gliosis
  2. Neurofibrillary tangles
  3. Amyloid-beta peptides
  4. Malignant cells
A
  1. Amyloid-beta peptides

  • Probable cerebral amyloid angiopathy (CAA) (with pathology) is defined when clinical data and pathologic tissue demonstrates lobar, cortical-subcortical, or cortical hemorrhage, pathological evidence of CAA, and absence of another diagnostic lesion.
  • Brain biopsies are rarely utilized in the diagnosis of CAA, except when CAA-related inflammation is suspected.
  • If a sample is obtained, it should be examined with beta-amyloid immunostain or Congo red stain.
  • By using these techniques, the deposition of amyloid beta- peptide can be identified to help support the diagnosis of CAA.
42
Q

A 45-year-old patient has been diagnosed with diffuse traumatic subarachnoid hemorrhages and diffuse axonal injury following a motor vehicle collision. The patient is intubated and has been sedated for minimizing the risk of raised intracranial pressure following malignant brain edema. The treating clinician advises the attending nurse to initiate the nasogastric feeding after a repeat CT head 48 hours later showed resolution in the traumatic brain injuries and the concurrent brain edema. The nurse, however, found almost 75 percent of the previous feed as the gastric residual while aspirating from the nasogastric tube, four hours of the prior feeding time, before attempting the next feed. The bowel sounds were also diminished in the patient. What is the recommended next step to facilitate enteral feeding in the patient?

  1. Delay the enteral feeding
  2. Start the total parenteral nutrition
  3. Feeding jejunostomy
  4. Prokinetic agent
A
  1. Prokinetic agent

  • The patient has the typical features of the enteral feeding intolerance owing to the presence of delayed gastric emptying. This is seen in almost 46 percent of patients undergoing enteral feeding.
  • Larger gastric residual content and the diminished intestinal motility predisposes the risk of aspiration pneumonia in such patients.
  • The use of prokinetic agents and small bowel feeding regimes are the most rational approaches in managing such enteral feeding intolerance.
  • The small bowel feeding, such as the feeding jejunostomy, is only tried once the enteral feeding intolerance persists despite the use of the prokinetic agents. The use of total parenteral nutrition is only advisable in patients who cannot tolerate or are contraindicated for enteral nutrition therapy.
43
Q

A 35-year-old man presents with sudden onset neck pain radiating to his left hand following a minor fall injury. The neurological examination reveals weakness during shoulder shrugging, elbow flexion, elbow extension, and handgrip. MRI shows disc desiccation in multiple segments of the cervical spine with features of associated canal stenosis. What is the most appropriate next step in management for this patient’s radiculopathy?

  1. CT spine
  2. Epidural corticosteroid injection
  3. Laminectomy
  4. Multiple level anterior cervical discectomy
A

1. CT spine

  • This patient has disc desiccation in multiple segments of the cervical spine with features of associated canal stenosis following a minor fall injury.
  • It is imperative to rule out an ossified posterior longitudinal ligament (OPLL) in this patient, which is best seen in CT images.
  • CT helps in knowing the pattern (localized vs. continuous) and length of segments involved in OPLL and thereby helps form a management plan in the patient (anterior vs. posterior approach).
  • Corticosteroid for pain management is justified only after ruling out OPLL and severe foraminal stenosis by CT imaging.
44
Q

A 12-year-old boy is brought to the hospital after closed head trauma suffered after he was hit by a car while on a crosswalk. On emergent CT, a diffuse anterior interhemispheric subarachnoid hemorrhage is noted. Which of the following is most likely to be found on further imaging in this patient?

  1. An anterior communicating artery aneurysm
  2. An aneurysm located at the cerebral convexity at the injury site
  3. An aneurysm located at the internal carotid artery cerebral ipsilateral to the injury site
  4. An aneurysm located at the internal carotid artery cerebral contralateral to the injury site
A

1. An anterior communicating artery aneurysm

  • Aneurysms involving the anterior cerebral artery distribution and its distal branches can often result from injury along the falx.
  • Aneurysms involving the posterior cerebral artery can occur near the free edge of the tentorium cerebelli following closed head trauma in a child.
  • Aneurysms involving the middle cerebral artery can occur along its course on the sphenoid ridge following closed head trauma in a child.
  • False traumatic aneurysms result from a fibrous organization around a prior hematoma encasing the parent vessel. False lesions often develop after direct penetrating injury.
45
Q

A 45-year-old female presents with numbness and a painful, shooting sensation in her left leg. During the neurological exam, a test of the Gordon reflex on her left side was positive. What is the most likely diagnosis?

  1. Diabetic peripheral neuropathy
  2. Myasthenia gravis
  3. L5 radiculopathy
  4. Multiple sclerosis
A

4. Multiple sclerosis

  • The Gordon reflex correlates to a lesion in the pyramidal tract or corticospinal tract and is an upper motor neuron sign.
  • Multiple sclerosis is a disease that affects the corticospinal or pyramidal tract.
  • A positive Gordon reflex is an extensor plantar response on the tested side.
  • The Gordon reflex is a variant of the Babinski sign.
46
Q

A 45-year-old woman is undergoing a fluoroscopy-guided stellate ganglion block with local anesthetic for the treatment of an upper extremity complex regional pain syndrome. After injecting 2 mL of contrast, a prominent cephalad spread along longus colli muscle is noted. 20 minutes following completion of the procedure, the patient exhibits ipsilateral Horner syndrome, yet reports only minimal pain relief in the upper extremity. No significant temperature change in extremity is observed. Which of the following best explains this outcome?

  1. Intramuscular injection of the anesthetic
  2. Inadequate spread of anesthetic solution to upper thoracic ganglia
  3. Inadequate time allowed after the procedure for upper extremity assessment
  4. Unusual anatomical location of stellate ganglion
A

2. Inadequate spread of anesthetic solution to upper thoracic ganglia

  • The caudal spread of the anesthetic to the T2 - T3 level during the stellate ganglion block is required to interrupt the sympathetic activity to the upper extremity.
  • The spread of the solution depends on the correct positioning of the needle tip and the volume of anesthetic administered.
  • Pain relief, warmth, decrease in perspiration, and change in color of the area supplied by the sympathetic nerves blocked are subjective indicators of a successful procedure. Objective tests are recommended and include measuring skin temperature and blood flow, skin conductance, and provocative sweat tests. Comparing the before and after procedure difference in temperature at the fingertips of both hands is the most common and simple method used to assess the effect of the block.
  • The stellate ganglion is located anterior to the neck of the first rib and the C7 transverse process, under the prevertebral fascia. Injection at the C6 or C7 levels relies on the spread of anesthetic to reach the stellate ganglion. This can be sufficient to block the sympathetic outflow to the ipsilateral head and neck. Yet, the majority of the upper extremity sympathetic innervation comes from the upper thoracic ganglia, which occasionally may not be covered by local anesthetic spreading from levels above.
47
Q

A 28-year-old is undergoing corrective spinal surgery for excessive scoliosis. During the surgical procedure, several monitoring electrodes are placed at the popliteal fossa, lower back, and scalp. In addition, a stimulator electrode is inserted over the medial malleolus. Stimulation of this area generated a waveform recorded at the popliteal, lower back, and scalp electrodes. The procedure is carried out using propofol and opiate intravenous anesthesia. While inserting a surgical screw, the surgeon is alerted to a decrease in waveform amplitude recorded at the scalp level. There is also an increase in latency in the same electrode. Which of the following structures is likely disrupted in this scenario?

  1. Posterior spinal artery
  2. Anterior spinal root nerve
  3. Anterolateral white column
  4. Motor nerve endplate
A

1. Posterior spinal artery

  • This patient is undergoing spinal surgery with an inherent risk to the underlying cord. During the procedure, monitoring is carried out in this patient by using somatosensory evoked potentials. In this case, a reduction in waveform amplitude suggests an interruption of the neural arc.
  • The somatosensory evoked potential measures the integrity of the dorsal column pathway. Thus, a reduction in amplitude will suggest an interruption of this pathway from the periphery to the cortex. The posterior columns are supplied by the posterior spinal artery, and manipulation can cause an avascular compromise in this patient.
  • The surgeon should be warned if the amplitude falls below 50 % and there is an increase in latency. Local retraction, dissection, and clipping can all cause increased local pressure or local ischemia, which results in impaired conduction. A change in technique and positioning may result in improvement in the recorded potentials.
  • These potentials provide real-time neurosurgical feedback to the surgeon. However, the somatosensory evoked potentials do not measure the spinothalamic or corticospinal tracts. Thus interruption/damage to these pathways is still possible with standard somatosensory evoked potentials.
48
Q

A 65-year-old man suffered a high-speed motor vehicle collision and was originally taken to an outside hospital. There he was intubated and sedated and treated for cerebral edema with multiple medications and modalities. Approximately 24 hours later, he is transferred to a higher level hospital with increasing B- type natriuretic peptide and an ejection fraction of 20%. What is the most likely offending agent causing his current cardiac abnormalities?

  1. Mannitol
  2. Midazolam
  3. Fentanyl
  4. Propofol
A
  1. Mannitol

  • Mannitol is commonly used to treat cerebral edema and is a diuretic.
  • Mannitol is contraindicated in progressive heart failure as the fluid shifts mannitol causes can precipitate worsening heart failure.
  • Mannitol can cause significant electrolyte shifts. First it can cause hyponatremia as water in drawn into the intravascular space then hypernatremia as free water is excreted with mannitol by the kidneys.
  • Propofol infusion syndrome typically occurs after more than 24 hours of administraiton of propofol and is typified by rhyabdomyolysis, metabolic acidosis, renal failure and cardiac faliure with hyperkalemia, hypertrigliceridemia and liver enlargement.
49
Q

A 55-year-old female is admitted to the neurosurgery intensive care unit for a large ischemic stroke. An external ventriculostomy drain has been placed. The monitor reveals the onset of waves lasting 10 minutes with an amplitude of 60 mmHg above the baseline. After the wave dissipates, you note that the baseline intracranial pressure is higher than when the wave began. What is the best next step in the management of this patient?

  1. Administer mannitol
  2. Place the patient in the supine position
  3. Start isotonic intravenous fluids at 150 ml/hour
  4. Obtain an emergent computed tomography scan
A
  1. Administer mannitol

  • A-waves, also known as plateau waves, are waveforms of great concern in patients with a ventriculostomy.
  • These waves have a duration of 5 to 20 minutes and an amplitude of over 50 mmHg.
  • When the waves subside, the baseline usually rests at a higher level.
  • The presence of an A-wave signals herniation. Therefore, one must do something urgently to relieve the intracranial pressure, such as administering mannitol.
50
Q

A 36-year-old man presents to the pain clinic with complaints of low back pain. His symptoms were of acute onset and began after he attempted to lift a heavy piece of furniture two weeks back. He reports that the pain “shoots down” his left leg. Which of the following physical exam findings would be most consistent with L4 radiculopathy?

  1. Bilateral wasting of extensor digitorum brevis
  2. Diminished left Achilles reflex
  3. Left foot drop
  4. Positive Babinski sign on the left
A

3. Left foot drop

  • Lumbar radiculopathy commonly presents as low back pain that radiates down a lower extremity. Other symptoms may include numbness in a dermatomal pattern and weakness along the affected myotome.
  • Diminished reflexes are considered to be a lower motor neuron sign and can be seen in radiculopathy.
  • On neurologic exam, the sensation should be assessed. Impaired sensation in a dermatomal pattern is suggestive of radiculopathy.
  • Upper motor neuron signs such as a positive Babinski sign or clonus can indicate spinal cord involvement and shoulder be investigated further. Motor weakness can be seen and will follow a specific myotome in lumbar radiculopathy: L2- hip flexion, L3- knee extension, L4- dorsiflexion, L5- great toe dorsiflexion, and S1- plantar flexion.
51
Q

A 50-year-old man was admitted to the hospital after he presented to the emergency department with a severe headache. A CT of the head was noted to have a large 4 cm frontal mass with surrounding edema. He was started on steroids to help with symptoms. He was evaluated by neurosurgery and underwent surgical resection of the frontal mass. The pathology of the lesion turned out to be a melanoma. Further evaluation by scans did not show any other metastatic lesion. Which of the following is recommended to decrease the local recurrence at the surgical site?

  1. Whole-brain radiation therapy (WBRT)
  2. Pembrolizumab
  3. Stereotactic radiosurgery (SRS)
  4. Dabrafenib and trametinib
A

3. Stereotactic radiosurgery (SRS)

  • Stereotactic radiosurgery is recommended to the local surgical bed after the surgical resection of the brain metastases.
  • SRS to the surgical bed decreases the local recurrence of the melanoma at the site of the surgery.
  • Whole-brain radiation therapy (WBRT) is not recommended post-surgical resection of the brain metastases.
  • Systemic therapy options like the pembrolizumab and BRAF and MEK inhibitors are useful for systemic control of metastatic melanoma.
52
Q

A 7-year-old girl with protrusion over the lower back present since birth has a ventriculoperitoneal shunt that was last revised when she was one and a half years old. She has a seizure disorder well controlled with levetiracetam. She uses crutches for ambulation. Over the past three months, she complained that her legs and back hurt, and she refuses to walk for more than five minutes. On examination, she has atrophic lower extremities with decreased reflexes. She also has a weakness and bilateral pes cavus deformity. The remaining exam findings are within normal limits. Non-contrast computed tomography of the head reveals that her shunt tubing is in an appropriate position, and ventricles size was the same from the previous imaging 18 months ago. What is the most likely cause of her new symptoms?

  1. Chronic urinary tract infection
  2. Hydrocephalus
  3. Shunt failure
  4. Tethered cord
A

4. Tethered cord

  • Myelomeningocele is commonly associated with the tethered cord.
  • The tethered cord usually presents late during periods of growth.
  • The tethered cord’s symptoms may include worsening of gait, leg or back pain, bladder or bowel dysfunction, progressive scoliosis, or worsening atrophy of limbs.
  • The best test for diagnosing the tethered cord is magnetic resonance imaging of the spine.
53
Q

A 40-year-old man presents to the hospital with a 2-day history of fever and back pain. The MRI of the lumbar spine shows abnormal T2 signal and enhancement in the disc space. There is also an abnormal marrow signal in the adjacent vertebral bodies. Which of the following complications is most often associated with the patient’s diagnosis?

  1. Epidural abscess
  2. Osteoporosis
  3. Arachnoiditis
  4. Meningitis
A

1. Epidural abscess

  • The patient demonstrates classic MRI findings of discitis osteomyelitis, which is characterized by abnormal T2 signal within the disc space and enhancement on the post-contrast images. There is an abnormal marrow signal with enhancement and irregularity in the adjacent vertebral bodies.
  • MRI is very sensitive for diagnosing discitis osteomyelitis. The next step in the progression of the disease is the development of an epidural or paraspinal abscess. The posterior extension of the infection as an epidural abscess can compress on the thecal sac, spinal cord, and cauda equina nerve roots.
  • The infection in the spinal canal can extend to meninges and cauda equina nerve roots and cause meningitis and arachnoiditis, which present as enhancement of meninges and abnormal clumping and enhancement of nerve roots in thecal sac.
  • The common etiologies include systemic source, spinal instrumentation, trauma, intravenous drug use, and immunosuppression.
54
Q

A 68-year-old woman presents with headaches. For the past 6 months, she complained of progressive headaches located in the right parietal region, initially graded 3/10 that worsened to 7/10. Her medical history is non-contributory. On examination, vital signs are normal. Her pupils are isochoric and briskly reactive to light. There is bilateral papilledema. The rest of the cranial nerve examination is normal. There are no motor or sensory deficits. A cranial MRI with contrast reveals an extra-axial, homogenously enhancing mass lesion with a dural tail on the right parietal convexity measuring 3.6 cm. Which of the following is the correct origin of the cells responsible for this tumor?

  1. Arachnoidal cap
  2. Central nervous system macrophages
  3. Ependymal cells
  4. Astrocytes
A

1. Arachnoidal cap

  • Meningiomas are mostly benign central nervous system (CNS) tumors. About 90% of these tumors are slowly growing benign tumors. Malignant meningiomas constitute less than 10%.
  • Meningiomas originate from meningothelial cells of the arachnoid cap.
  • MRI with contrast helps to distinguish an extra-axial from an intra-axial lesion. Meningiomas resemble a homogenous enhancement with a feature called a dural tail.
  • One of the pathohistological characteristics seen in meningiomas is the growth of meningothelial cells that mineralize to form psammoma body.
55
Q

A 35-year-old female presents for MRI evaluation of the lumbar spine due to chronic back pain. A 2 cm lesion is noted within the T11 vertebral body, distant from the site of pain at the L5 vertebral body. CT comparison demonstrates prominent trabeculae on axial images with a “polka-dot” appearance. This lesion is consistent with an MRI comparison from eight years ago. In addition to the above mentioned findings, today’s MRI lumbar spine will most likely show which of the following?

  1. Lack of enhancement
  2. T1/T2 isointensity
  3. T2 dark signal
  4. T1 hyperintensity
A

4. T1 hyperintensity

  • Vertebral body hemangiomas are T1 high signal, frequently due to their high-fat content. The water content of vertebral hemangiomas causes these lesions to have a high T2 signal.
  • Hemangiomas are generally asymptomatic. On occasion, vertebral body hemangiomas can extend into the neuroforamina and subsequently cause neurologic symptoms. Compression fractures of the affected vertebral body, although rare, can also cause pain.
  • Due to their high vascularity, vertebral body hemangiomas also demonstrate enhancement.
  • Due to their thickened trabecular appearance, vertebral body hemangiomas demonstrate a “polka-doted” appearance.
56
Q

A 66-year-old man has presented with headaches for the last four months. He describes the pain as four out of ten, located in the frontal region bilaterally. One month ago, the headaches increased in intensity, became more prominent upon waking up, and are accompanied by double vision. He has no past medical history. On examination, his pupils are isocoric briskly reactive to light, with no visual field cuts. There is bilateral papilledema on fundoscopy. The extraocular movement shows decreased abduction of the left eye when looking to the left and decreased abduction on the right eye when looking to the right. The motor and sensory examinations are normal. He ambulates on his own with a wide-based gait and has difficulty doing tandem gait. An MRI of the brain shows a well-defined homogenously enhancing lesion inside a ventricle with hydrocephalus. In which of the following is this lesion most likely located?

  1. The frontal horn of the lateral ventricle
  2. The fourth ventricle
  3. The trigone of the lateral ventricle
  4. The third ventricle
A

3. The trigone of the lateral ventricle

  • Intraventricular meningiomas arise from choroid plexus stromal cells.
  • The choroid plexus is located in the lateral ventricular atria (trigone), the third, and the fourth ventricles. Its most significant volume is in the lateral ventricle atria.
  • This is the most common location for intraventricular meningiomas.
  • There is no choroid plexus in the frontal horns.
57
Q

A 17-year-old patient sustained a fracture over the frontal sinus following a motor vehicle collision. The patient was managed conservatively and was subsequently discharged from the hospital. Two weeks later, he presented with a severe headache associated with multiple episodes of vomiting. Computed tomography head revealed the presence of a hypodense lesion with a Hounsfield unit of -1000 in the frontal lobe, without evidence of subfalcine herniation. What is the next management strategy for the patient?

  1. Burr hole evacuation
  2. 100% oxygen by a rebreather mask
  3. Craniotomy
  4. Antibiotics
A

2. 100% oxygen by a rebreather mask

  • The presence of a hypodense lesion with a Hounsfield unit of -1000 is suggestive of a pneumocephalus.
  • The presence of fracture over the frontal sinus sometimes creates communication of the frontal sinus with the epidural region of the brain. This leads to pneumocephalus.
  • 100% oxygen therapy may lead to a resolution in the size of the pneumocephalus. Positive pressure should be avoided. The nitrogen gradient dependent flow of the air from the epidural region to the blood vessels helps minimize signs and symptoms due to tension pneumocephalus.
  • Burr hole evacuation is justified only in cases of tension pneumocephalus with herniation syndrome. A craniotomy is seldom required for the management of pneumocephalus.
58
Q

A 65-year-old woman presents with a two-year history of lower back pain. The patient describes the pain as a seven out of ten. She has a past medical history of spondylolisthesis due to arthritis, for which she had a spinal fusion two years ago. The pain got better at first, but it never resolved completely. If a spinal cord stimulator (SCS) was considered as a treatment option for this patient, what is the most common complication?

  1. Loss of pain coverage
  2. Infection
  3. Spinal epidural hematoma
  4. Headaches
A

1. Loss of pain coverage

  • The complication rate following spinal cord stimulator implantation is relatively high.
  • The most common complications are due to the hardware malfunction, such as lead migration.
  • Spinal cord stimulators are designed to treat chronic pain. The implantable device offers a nonpharmacological approach to various pain conditions.
  • Failed back surgery is the most common indication for spinal cord stimulator implant, which is an effective analgesic and is used for persistent radicular pain following surgery.
58
Q

A 17-year-old patient presented to the clinic complaining of lower back pain during and after her gymnastics training. She has olympic aspirations but is not able to progress with training due to pain. She has complained of a gait alteration due to this. Increase in what radiological parameter on a lumbosacral radiograph is most likely to be responsible for her symptoms?

  1. Pelvic obliquity
  2. Sacral slope
  3. Slip angle
  4. Pelvic incidence
A

4. Pelvic incidence

  • Pelvic incidence is a constant angle between the line drawn from the center of rotation of the femoral head toward a superior endplate of S1 and another line drawn downwards perpendicular to the superior endplate of the same vertebra. Pelvic incidence value does not change with regards to the position of the pelvis (sitting/standing). It can not be changed with surgery either.
  • Increased pelvic incidence angle is a significant factor in the etiology of spondylolisthesis. The normal value of approximately 50 degrees is usually increased to 70 degrees to 80 degrees in patients with spondylolisthesis. Increased pelvic incidence requires lumbar spine hyperextension in order to balance the trunk over the pelves. This puts the posterior spinal elements under increased stress and can cause the failure of the system (pars interarticularis stress fracture), leading to a forward slip of the superior vertebra over the inferior one.
  • If the femoral heads are not interposed on the lateral pelvic radiograph, the line joining the two centers of femoral heads should be used as initiating points for drawing the pelvic incidence angle.
  • Slip angle is another important parameter in the pathophysiology of spondylolisthesis. If significantly increased (to greater than 45 degrees), it is a factor predicting the progression of the slip.
59
Q

A 55-year-old man presents with numbness on the right side. The symptoms started 2 hours ago. His past medical history includes hypertension, type 2 diabetes mellitus, and cardiac arrhythmia. Vital signs show oxygen saturation 98% on room air, respiratory rate 16 per minute, blood pressure 160/95 mmHg, heart rate 122 beats per minute irregularly irregular, and temperature 98 F. On examination, the cranial nerves were intact. Power was intact with 5/5 in all extremities. There is a 20% sensory deficit on the right face, arm, and leg. Imaging studies reveal an infarct on the left parietal region. Which of the following further neurological symptoms would you expect this patient to exhibit?

  1. Involuntary dance-like movements on the right
  2. Inability to write
  3. Inability to name objects by touch alone
  4. Inability to name familiar objects by sight
A

3. Inability to name objects by touch alone

  • The inability to recognize an object by touch without visual input is known as astereognosis.
  • Astereognosis is associated with lesions in the parietal lobe.
  • An isolated infarct in the parietal area can cause such a deficit.
  • Testing may not be appropriate when there is a loss of primary modality of touch sensation as with peripheral neuropathy.
60
Q

A 52-year-old man is brought in by emergency medical services (EMS) after being involved in a motor vehicle collision. Examination demonstrates slow flexion of the elbow, wrist, and fingers with adduction and internal rotation of the shoulders. The legs are extended and internally rotated at the hip, with the extension of the knee and plantar flexion of the feet. Which of the following is the most likely position of the toes in this pathological posturing observed in this patient?

  1. Adducted and hyperextended
  2. Abducted and hyperextended
  3. Abducted and flexed
  4. Adducted and flexed
A

2. Abducted and hyperextended

  • Decorticate posturing is described as abnormal flexion of the arms with the extension of the legs.
  • It involves slow flexion of the elbow, wrist, and fingers with adduction and internal rotation at the shoulder.
  • The lower limbs show extension and internal rotation at the hip, knee extension, and plantar flexion of the feet. Toes are typically abducted and hyperextended.
  • Decorticate posturing is a clinical diagnosis and should be considered a pathological sign of a neurological injury.
60
Q

A 60-year-old male presents with severe back pain and ataxia for the past ten days. The pain is in the lumbar region and radiates around his lower abdomen. He has also been experiencing difficulty walking and has fallen two times in the past three days. He also says that he feels weakness, numbness, and tingling in both of his lower limbs. He denies fever, nausea, vomiting, and bowel/bladder dysfunction. The patient has never had similar back pain before. His past medical history is significant for renal cell carcinoma, which was discovered after a few episodes of hematuria and flank pain. He had the tumor surgically removed eight months ago, and there were no signs of recurrence during his follow-up last month. On examination, blood pressure is 131/83 mmHg, pulse is 81 beats/min, and respiratory rate is 17 breaths/min. Neurological examination shows motor strength of 2/5 and loss of pain and temperature sensations in both lower limbs. Babinski reflex is upgoing bilaterally. Which of the following is the best next step in the management of this patient?

  1. High-dose corticosteroids and MRI of the spine
  2. High-dose corticosteroids and neurosurgery consult
  3. MRI of the spine and neurosurgery consult
  4. High-dose corticosteroids and a lumbar puncture
A

1. High-dose corticosteroids and MRI of the spine

  • This patient with a history of renal cell carcinoma and acute onset back pain accompanied by weakness and sensory changes in the lower limbs is consistent with epidural spinal cord compression most likely due to tumor metastasis.
  • Cancers that metastasize to the spine include prostate, renal cell carcinoma, non-Hodgkins lymphoma, and breast cancer.
  • Epidural spinal cord compression is a medical emergency. High- dose corticosteroids (e.g., dexamethasone) should be administered immediately to decrease swelling and edema around the tumor, which will relieve the spinal cord compression.
  • MRI of the spine should also be done to confirm the diagnosis, assess spinal stability, and to plan the appropriate treatment. Patients with a stable spine or radiation-sensitive tumors can be treated with radiation therapy only. Patients with an unstable spine or radiation-resistant tumors should have an urgent neurosurgical consult.
61
Q

A 41-year-old man with chronic low back pain presents to the office for follow-up. Previous extensive workups, including X-ray and MRI, are unremarkable. However, he hurt his back four months ago while working on his car. His pain is a 4/10 and does not improve with acetaminophen, ibuprofen, or muscle relaxers. He denies any lower extremity weakness, sensation changes, or bowel or bladder changes. On exam, he has right paraspinal muscle hypertonicity and a restricted range of motion of the lumbar spine. His strength, sensation, and deep tendon reflexes are all within normal limits. He recently finished physical therapy, but the pain has persisted. He does not want to take any additional medications. Which of the following is the most common side effect of the next best nonpharmacologic step in the management of this patient’s back pain?

  1. Infection
  2. Bleeding
  3. Pain
  4. Numbness
A
  1. Infection
  2. Bleeding
  3. Pain
  4. Numbness

  • Acupuncture is indicated for cases of chronic low back pain, especially mechanical pain. The negative MRI and muscle hypertonicity helps establish mechanical nature. The patient has failed initial conservative management in pharmacological and physical therapy. Second-line treatments include acupuncture. The most common side effect is needle pain.
  • Needle pain occurs in 3.3% of cases of acupuncture.
  • Needle entry pain typically resolves with the removal of the acupuncture needle.
  • The second most common side effect of acupuncture is hematoma formation (bleeding). It occurs in 3% of patients. Infection is the most common serious side effect but occurs in less than one percent of cases.
62
Q

A 28-year-old pregnant female presents with a headache that started when she got out of bed this morning. The headache is worse when she is upright and ambulating. She complains of mild nausea and slightly diminished hearing. Her systemic examination is normal. MRI head shows dural venous engorgement, minimal subdural fluid collections, and downward sagging of the brain. What is the most appropriate initial management plan for the patient?

  1. Epidural blood patch
  2. Bedrest, caffeine, and oral hydration
  3. Fibrin sealant placed percutaneously
  4. Surgical repair
A

2. Bedrest, caffeine, and oral hydration

  • The patient has spontaneous intracranial hypotension. The headache associated with SIH is often orthostatic, occasionally triggered by Valsalva.
  • Spontaneous intracranial hypotension is characterized by low cerebrospinal fluid (CSF) opening pressure, orthostatic headache, and brain “sag” with diffuse pachymeningeal enhancement in radiological images.
  • Most patients improve with a resolution of symptoms following bed rest, sufficient oral fluid intake, caffeine intake, and time.
  • If there is no improvement, an epidural blood patch would be appropriate. Only patients with intractable symptoms require fibrin sealant or surgery.
63
Q

A 39-year-old man presents to the clinic with pain in the neck that shoots to the right side of his head. MRI of the C spine shows multilevel facet arthropathy. He has had mild relief with occipital nerve blocks in the past and reported an 80% improvement in pain after C2-3 facet joint injection. Peripheral nerve stimulation is considered for the long term relief of his symptoms. What is the most appropriate target of stimulation for this patient?

  1. Right occipital nerve
  2. Right third cervical nerve
  3. Right paraspinal muscles
  4. Right C7 nerve root
A

2. Right third cervical nerve

  • This case shows the classical presentation of cervicogenic headache.
  • The third cervical nerve is a branch of C2-3 dorsal ramus that supplies the C2-3 facet joint.
  • While the patient had mild relief with an occipital nerve block, pain relief is much better with C2-3 facet joint injections.
  • Even though occipital nerve stimulators might still help with headaches, it would not be useful to manage the patient’s neck pain. Therefore, the most appropriate answer is the right third occipital nerve stimulation.
64
Q

A 66-year-old female presented to the emergency department with sudden onset of weakness in the left side of the body. She also gave a history of hypertension and hyperlipidemia. On examination, she had aphasia, right-sided gaze preference, dysarthria, and left-sided hemiplegia. Which of the following structure is most likely affected?

  1. Right middle cerebral artery
  2. Left middle cerebral artery
  3. Left anterior cerebral artery
  4. Circle of Willis
A

1. Right middle cerebral artery

  • In this patient, the most likely diagnosis is middle cerebral artery syndrome causing contralateral hemiparesis and sensory loss, which are more in upper extremities than the lower extremities, contralateral hemianopia, Wernicke aphasia, and gaze abnormalities.
  • The major blood supply of the brain is by two sets of arteries, the vertebral arteries, and the carotid arteries. Inside the skull, the carotids divide into two major arteries, the anterior and the posterior cerebral arteries, and many smaller arteries like the ophthalmic artery and the anterior choroidal arteries. These arteries supply blood to the anterior two-thirds of the brain. The ophthalmic artery supplies the structures of the orbit while the posterior communicating artery acts as an anastomotic ‘connecting vessel’ in the Circle of Willis. Anterior choroidal artery supply structures in the brain important for motor control and vision, and the anterior cerebral artery supplies part of the cerebrum.
  • The vertebral arteries join to form a basilar artery which is located near the base of the skull. They give off many small branches in the brain stem and forms the posterior cerebellar and the posterior meningeal arteries. Meningeal branch supplies the falx cerebelli, a sheet of dura mater, while the anterior and posterior spinal arteries supply the spinal cord, spanning its entire length. The posterior inferior cerebellar artery supplies the cerebellum.
  • The anterior cerebral artery supplies the anteromedial portion of the cerebrum. The middle cerebral artery is situated laterally, supplying the majority of the lateral part of the brain. The posterior cerebral artery supplies both the medial and lateral parts of the posterior cerebrum.
65
Q

A 57-year-old man presents for a followup of left- sided C7 radiculopathy of 3 months duration. He has tried adequate conservative measures over the past 3 months, without much relief. He had previously undergone C4-5, C5-6 anterior cervical discectomy, and fusion 6 years back through a right-sided approach. He is significantly bothered by his symptoms and wishes to go ahead with the surgery. Which of the following is the most appropriate approach in such a scenario?

  1. Never approach the C6-7 disc in this patient from the left side, as the previous surgery was performed through a right-sided approach
  2. There is no contraindication to left-sided approach in this patient
  3. Obtain a consultation with an otolaryngologist to evaluate the status of his vocal cords before deciding upon the approach
  4. Avoid any anterior cervical surgeries in this patient
A

3. Obtain a consultation with an otolaryngologist to evaluate the status of his vocal cords before deciding upon the approach

  • Anterior cervical discectomy and fusion can be performed through right or left sided approaches.
  • There are pros and cons for both these approaches, and in general, the approach is decided based on the surgeon’s preference and the individual clinical scenario. The right-sided approach is more convenient for right-handed surgeons. While this approach has traditionally been associated with recurrent laryngeal nerve palsy, recent evidence does not show any statistically significant difference in recurrent laryngeal nerve palsy rates between these two approaches. The left-sided approach for distal cervical discs is associated with high rates of thoracic duct injuries.
  • In individuals undergoing revision anterior cervical discectomies, the cervical spine can be approached from the ipsilateral side (as the previous incision) or the contralateral side. However, whenever a decision is made to approach the spine from the contralateral side, it is preferable to have the vocal cord examined by an otolaryngologist so as to rule out any partial vocal cord palsy from unilateral recurrent laryngeal nerve injury during the first operation. Bilateral recurrent laryngeal nerve injury with bilateral vocal cord paralysis carries very high morbidity.
  • The incisions for anterior cervical approaches may be transverse or vertical. This choice depends upon the previous incision, although whenever feasible transverse incisions are preferred in view of a more pleasing, cosmetic scar.
66
Q

A 38-year-old man presents with difficulty walking for the past year. The difficulty has gradually progressed to the point where he requires a walker for support. Examination demonstrates exaggerated deep tendon reflexes bilaterally in the upper and lower extremities. MRI of the cervical spine shows a C4-5 disc prolapse with cord compression. What is the Nurick grade for this patient’s condition?

  1. II
  2. III
  3. IV
  4. V
A

3. IV

  • Nurik is a six-grade system (0-5) grading system is based on “difficulty in walking.”
  • Nurick grading is one of the functional grading systems used to classify the severity of myelopathy based on the ambulatory status of the patient.
  • This patient presents with difficulty walking, exaggerated reflexes, and cord compression on MRI suggestive of cervical myelopathy.
  • Grade IV is consistent with the patient requiring support from a walker plus cord compression.
67
Q

A 17-year-old male is brought to an emergency department after a motorbike accident. On arrival, his blood pressure is 98/62 mmHg, pulse rate is 124 bpm, respiratory rate is 11 breaths/min, and oxygen saturation is 84% at room air. His Glasgow coma scale (GCS) score is 7/15. On physical examination, there is purplish discoloration of his left tympanic membrane with bloody otorrhea. There is bruising of both eyes with tarsal plate sparing and behind the left ear. Left ear drainage is also noted, and fluid is mixed with blood. After initial management in the emergency department, a CT scan of the head is taken, which reveals pneumocephalus. What emergency intervention is most likely to worsen this radiological finding?

  1. Nasogastric tube intubation
  2. Endotracheal tube intubation
  3. Nasal intermittent positive pressure ventilation
  4. Air embolization via a central line
A

3. Nasal intermittent positive pressure ventilation

  • Hemotympanum usually appears within hours of injury and may be the earliest clinical finding. Periorbital ecchymosis (raccoon eyes) is typically not present during the initial evaluation and is delayed by 1 to 3 days. If bilateral, this finding is highly predictive of a basilar skull fracture. Retroauricular or mastoid ecchymosis (Battle sign) is highly predictive of basilar skull fracture.
  • Nasal intermittent positive pressure ventilation (NIPPV) should be avoided as it may induce or worsen pneumocephalus.
  • Basilar skull fractures are usually due to significant trauma. A thorough trauma evaluation with interventions to stabilize the airway, ventilation, and circulatory issues is the priority. Associated cervical spine injury is common, so attention to cervical spine immobilization, particularly during airway management, is necessary.
  • Nasogastric tubes and nasotracheal intubation should be avoided because of the risk of inadvertent intracranial tube placement, but this is rare.
68
Q

A 38-year-old man is brought in after he fell from the roof of his house. The paramedics brought him on a spinal board with a cervical collar. The cervical x-ray reveals a fracture of the C6 vertebrae. The cervical MRI shows focal cord enlargement at the level of C6-C7 with hyperintensity in the T2 sequence. Which of the following findings does one expect to see in this patient?

  1. Altered sensation
  2. Upper limb weakness prominent distally more than the proximally
  3. Lower limb weakness prominent proximally more than distally
  4. Respiratory muscle compromise
A

2. Upper limb weakness prominent distally more than the proximally

  • In patients with a lesion on the C6 cervical cord, the upper extremities will only have biceps functioning and weakness of the wrist and hands.
  • In patients with complete C6 quadriplegia, there is complete paralysis of the legs, and patients most likely will be unable to control bladder and bowel function.
  • There are no expected alterations to the sensation as the brain and brainstem are not affected. There are no intercostals available to assist with respiration, but the diaphragm will still function as its innervation is derived from C2 to C4.
  • Patients may also have spinal shock, where the neurologic findings are accompanied by hyporeflexia. This progresses to hyperreflexia at a later date.
69
Q

A 36-year-old woman presents with an eight- month history of low back pain exacerbated by flexion and sitting. She denies radicular pain in either lower extremity. She is a competitive weightlifter and trains most days of the week. Conservative medical management and physical therapy have provided minimal relief. MRI shows loss of disc height and significant disc desiccation in the L4-5 and L5-S1 intervertebral discs. Which of the following is most likely involved in this patient’s presentation?

  1. Sinuvertebral nerve
  2. Basivertebral nerve
  3. Dorsal rami
  4. Sciatic nerve
A

1. Sinuvertebral nerve

  • The interverbal discs are supplied by the sinuvertebral nerve, which innervates the posterior annulus and posterior longitudinal ligament. It is a mixed nerve containing a somatic root from the ventral ramus and an autonomic root from the grey ramus. Once the nerve takes a recurrent course through the intervertebral foramen and enters the spinal canal, it divides into superficial and deep networks. The superficial network branches to multiple vertebral levels and contains mostly sympathetic fibers, while the deep network contains primarily somatic fibers and innervates the corresponding segment.
  • Provocative discography is a fluoroscopically-guided diagnostic procedure involving deliberate administration of contrast into the nucleus pulposus of the suspected painful disc. The pressurized injection of contrast simulates mechanical loading and induces pain. Additionally, the pattern of contrast spread is indicative of the degree of disc disruption. This procedure is only performed when there is a high pretest probability of discogenic pain based on patient presentation and imaging. This is in part due to potentially significant post-procedural complications, including diskitis, dural puncture, disc rupture from contrast overpressurization, and worsening of underlying disc degeneration.
  • Disc pain arises from sensitization secondary to the ingrowth of sinuvertebral nerve fibers within annular fissures. Degenerative discs have shown penetration deep into the inner third and even nucleus of the disc. Exposure of the nucleus to the outer annulus and neuronal tissue results in the attraction of inflammatory mediators. The result is hyperinnervation and hyperalgesia related to the leakage of proinflammatory cytokines.
  • The basivertebral nerve is an intraosseous nerve that enters the posterior vertebral body via the basivertebral foramen and arborizes near the center of the vertebral body, sending branches to innervate the superior and inferior vertebral endplates. These nerve endings have been shown to proliferate in damaged and degenerated endplates.
70
Q

A 24-year-old man is brought to the emergency department following an assault. His vital signs are within normal limits. He reports severe pain on his face, epistaxis and edema. After clinical and radiographic examination, any eye complications are ruled out, and a non-displaced fracture is suspected. There are no functional or aesthetic defects identified. What is the most appropriate management strategy for this patient?

  1. Conservative treatment
  2. Emergency tracheostomy
  3. Open reduction and internal fixation
  4. Splinting teeth
A

1. Conservative treatment

  • Conservative treatment is indicated for non-displaced fractures.
  • Surgical intervention is indicated if there are eye concerns or functional or aesthetic concerns.
  • The patient should be advised to have a soft diet to allow for healing and attend for frequent review.
  • Advise the patient no nose blowing and to sneeze with mouth open to allow for bony healing.
71
Q

A 40-year-old man presents with a hand tremor that gets worse when trying to perform a task. He started noticing it three years ago; however, at that time, it did not affect his daily activities. The patient has no past medical history and does not smoke, drink alcohol, or take recreational drugs. On the other hand, he has a family history of tremors and myocardial infarction on his father’s side. On examination, there were no tremors at rest, but when asked to reach for objects, broad large-amplitude tremors were noticed. This type of tremor is most likely due to pathology at which structure?

  1. Cerebral cortex
  2. Cerebellum
  3. Substantia nigra
  4. Internal capsule
A

2. Cerebellum

  • The patient has a kinetic or intention tremor that is usually due to cerebellar damage.
  • Intention tremors can develop because of lesions in the brain, including stroke or a tumor.
  • Patients with a history of alcohol use disorder may also have intention tremors. Other illnesses that can manifest with tremors include multiple sclerosis.
  • Parkinson’s disease is due to a disease of the substantia nigra and is characterized by a resting tremor that improves with intentional movements. Lesions of the internal capsule result in weakness.
72
Q

A 54-year-old male presents to the emergency department with a droopy eyelid on the right side. This began several hours ago and is associated with diplopia in multiple directions. Physical exam reveals right-sided ptosis, dilated pupil, and down and out positioning of the affected eye. What is the most likely etiology of the condition affecting this nerve?

  1. Hypertension
  2. Diabetes
  3. Intracranial aneurysm
  4. Multiple sclerosis
A

3. Intracranial aneurysm

  • This patient, with a pupil-involving lesion of the oculomotor nerve, most likely suffers from an intracranial aneurysm. The parasympathetic fibers run at the periphery of the oculomotor nerve and are often affected by compression due to aneurysm, intracranial neoplasm, or uncal herniation.
  • Paralysis of the third cranial nerve often results in diplopia in multiple directions. Pupillary involvement, as seen in this case, also may be seen in paralysis of the third nerve, but importantly this is not caused by microvascular causes of third nerve palsies such as diabetes and hypertension.
  • The patient also will have a dilated pupil with sluggish or absent reaction to light. This is due to the involvement of parasympathetic fibers originating from the Edinger-Westphal subnucleus.
  • Hypertension and diabetes are microvascular diseases affecting the vasa vasorum. In these instances, the fibers innervating the upper eyelid, and most of the extraocular muscles become ischemic. These microvascular causes of oculomotor nerve palsy are pupil-sparing.
73
Q

A 65-year-old man presents with a pathological lesion of the T11 vertebral body associated with some collapse. Following further investigation, he is diagnosed with metastatic follicular carcinoma of the thyroid. Based on the recommendations of the multidisciplinary tumor board in discussion with the patient, the decision is made to go ahead with surgical treatment of the primary lesion with total thyroidectomy. Non-surgical intervention for the metastatic vertebral lesion is elected with spine stereotactic radiosurgery (SSRS). Which complications are associated more significantly with SSRS when compared with conventional external beam radiation therapy (cEBRT)?

  1. Spinal cord reperfusion edema
  2. Greater toxicity of organs at risk (OAR)
  3. Compression fractures
  4. Spinal cord infarction
A

Compression fractures

  • One of the major concerns following the use of high dose radiation delivery to the involved vertebra during SSRS is the occurrence of vertebral compression fractures.
  • It has been reported that the risk of vertebral compression fractures following SSRS can be as high as 40%, as compared with less than 5% risk with cEBRT.
  • Prophylactic vertebroplasty or kyphoplasty has been recommended in select patients at high risk for vertebral compression fractures before undergoing SSRS. Risk factors include older patients, patients with lytic lesions, vertebral malalignment, or preexisting vertebral compression fractures.
  • There is no enhanced risk for the spinal cord injury (OAR) following an appropriately-planned SSRS.
74
Q

A 26-year-old woman from India presents with a ten-year history of an awkward gait, frequent falls, and progressive thinning of leg and thigh muscles. She reports that for the last three years, she noticed that she became clumsy when doing manual daily activities. The patient also mentions that she always felt slow when trying to do physical activities or sports during childhood. There is a family history of neuropathic illnesses on her father’s side. Electrophysiology shows a median nerve conduction velocity of 38 m/s, uniform and diffuse slowing, lack of dispersion and conduction blocks, and amplitude reduction. Which of the following is correct regarding this patient’s most likely diagnosis?

  1. It is the most common inherited neuromuscular disorder
  2. It is less frequent than inflammatory or paraneoplastic neuropathies
  3. Some forms of the condition have a racial predilection
  4. It is the most serious form of the hereditary neuropathy
A
  1. It is the most common inherited neuromuscular disorder

  • Together, the different forms of Charcot-Marie-Tooth diseases (CMTs) are the most common among the inherited neuromuscular disorders, with an estimated frequency of 1 in 2500 people.
  • Most of the patients have an onset within the first two decades of life.
  • CMT is slightly more prevalent in men than in women.
  • The majority of CMTs are benign and have an insidious onset and slow progression. However, the clinical features and the course vary depending on the underlying genetic variation.
75
Q

A 32-year-old male patient presented with recent onset focal seizures. His CT head revealed frontal lobe hypodense lesion with stippled calcifications in between. The histology from the excised lesion showed characteristic fried egg appearance of the cells. What additional test would be imperative in prognosticating his outcome?

  1. Tumor histologic grade
  2. Periodic follow-up only
  3. Radiation
  4. 1p19q co-deletion status
A

1p19q co-deletion status

  • The description of the presenting features, as well as the pathological findings, are typical of oligodendroglioma.
  • 1p19q co-deletion has a significant role in the prognostication of such lesions.
  • Those with such co-deletion has a good outcome with the adjuvant role of chemotherapy with temozolomide.
  • Near-complete safe resection and periodic follow up are recommended in all brain tumors irrespective of tumor histology.
76
Q

A 35-year-old man comes to the department with complaints of headaches and difficulty walking for the past 6 months. He has no history of blurring of vision or episodes of syncope. He had complaints of flank pain 3 months back but did not seek care as he thought it was “just a kidney stone”. He has a family history of a brain tumor in his father. On examination, his pulse rate is 80 beats/minute and his blood pressure is 130/80 mmHg. There is a palpable flank mass on abdominal examination. Neurologic examination reveals an ataxic gait and abnormal finger-nose testing. Investigations reveal gross hematuria on urinalysis, a cerebellar tumor on head CT and a renal tumor on abdominal CT. The clinician diagnoses him with a genetic syndrome involving a mutation in a tumor suppressor gene. What is the most likely function of the gene product that has undergone the mutation?

  1. Enhances MYC-associated cell proliferation
  2. Inhibit hypoxia-inducible factor 1a
  3. Block the progression of the cell from G1 to S phase
  4. DNA repair protein
A

2. Inhibit hypoxia-inducible factor 1a

  • The patient has von Hippel Lindau disease characterized by hemangioblastomas, renal cell carcinoma, and pheochromocytoma. It has an autosomal dominant inheritance and is caused by a mutation in the VHL gene.
  • The VHL gene normally inhibits the hypoxia-inducible factor 1a. Cancer cells release hypoxia-inducible factors (HIF1 alpha and HIF2 alpha) that exert several physiological effects mediating their survival.
  • HIF1a upregulates glycolytic enzymes, and at lower concentrations of MYC, inhibits MYC-associated cell proliferation. It promotes angiogenesis of surrounding vascular tissue through increased vascular endothelial growth factor (VEGF) expression. It also activates lactate dehydrogenase A (LDHA) and pyruvate dehydrogenase kinase (PDK1), preventing the conversion of pyruvate to acetyl-CoA.
  • HIF2a has been shown to induce epithelial-mesenchymal transition (EMT) in normal cells as well as cancer cells. It stabilizes the MAX/MYC complex overcoming HIF1a’s MYC- inhibiting effect at high MYC concentrations and under hypoxic conditions.
77
Q

A 22-year-old male presents with headaches. He has had headaches occurring every day shortly after dinner for the past year. They last for 0 to 30 minutes. He used to use subcutaneous sumatriptan to abort the headaches, but now his headaches have started becoming increasingly resistant to treatment. What treatment option can be used to prevent these episodes?

  1. Verapamil
  2. Ergotamine
  3. Melatonin
  4. Oral sumatriptan
A

1. Verapamil

  • A thorough history is essential to the diagnosis of cluster headaches. Patients will reveal that they experience ten out of ten unilateral pain, most often located intra or supraorbitally. Patients must have at least one autonomic symptom ipsilateral to the side with pain.
  • The autonomic symptoms include conjunctival injection or lacrimation, nasal congestion or rhinorrhea, eyelid edema or forehead/facial swelling, and miosis or ptosis.
  • Verapamil is the most widely prescribed preventative drug. It is recommended to do regular ECGs to monitor cardiac function when a patient is using this drug.
  • Other pharmacological options include lithium, oral steroids, valproic acid, melatonin, and intranasal capsaicin.
78
Q

A 27-year-old male patient had undergone C5-C6 anterior cervical discectomy and fusion (ACDF) following C5-C6 AO type C injury with ASIA A injury. Overall, he was recuperating fairly well postoperatively, gradually weaned off all respiratory supports, and stepped down to high dependency unit (HDU) care on the 4th postoperative day. However, on the 5th postoperative day, he started complaining of progressive dysphagia. On the 6th postoperative day, he developed a high-grade fever and rigors. There is increasing erythema and tenderness around the surgical wound. His white blood cell counts (WBC), erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) are significantly raised. CT chest reveals pneumomediastinum. Which of the following options describes the most likely underlying pathology leading to the patient’s postoperative deterioration?

  1. Surgical site infection
  2. Pneumonitis
  3. Iatrogenic oesophageal injury
  4. Rupture of a traumatic pseudoaneurysm
A

3. Iatrogenic oesophageal injury

  • Esophageal injury is a rare complication following anterior cervical spine surgeries. This complication is typically reported more often in cervical spine surgeries performed for spinal fractures (especially major fracture-dislocations) than for other pathologies.
  • Esophageal perforation is one of the most dreaded visceral injuries during anterior cervical spine surgeries, and the fatality after the occurrence of posterior mediastinitis is very high. If it is detected intraoperatively, it needs to be immediately repaired to avoid devastating complications.
  • Postoperatively, the patient may present with progressive dysphagia (after being started on oral liquid or solid diets), although progressive evidence of toxic symptoms develops once the patient develops mediastinitis. A high index of suspicion is of utmost importance to diagnose this condition postoperatively.
  • Blood inflammatory markers progressively worsen. Gastrografin study may reveal peri-esophageal dye spillage with the holdup of contrast in the proximal esophagus. CT chest reveals pneumomediastinum, while CT/ MRI cervical spine reveals collection or abscess around the esophagus. The options for management include maintaining the patient nil per-orally (NPO), gastric diversions/nasogastric tube insertion, conservative treatment with antibiotics or surgical exploration, and esophageal repair with or without flap.
79
Q

A patient with disabling seizures for longer than one year despite treatment with adequate doses of two antiepileptic drugs has a video EEG showing three complex partial seizures from the right temporal lobe with right anterior interictal temporal sharp waves. Which one of the following options would be the most likely intervention to stop the seizures?

  1. Addition of a third antiepileptic drug
  2. Right temporal lobe lobectomy
  3. Vagal nerve stimulation
  4. Ketogenic diet
A

2. Right temporal lobe lobectomy

  • In patients with temporal lobe epilepsy with disabling seizures not responsive to multiple medications, temporal lobectomy has the best chance of keeping the patient seizure-free.
  • Patients who do not respond to either their first or second antiepileptic drugs have a less than 10% chance of being seizure-free on a third medication.
  • A ketogenic diet is a palliative measure.
  • Vagal stimulation also is a palliative measure that can lessen seizure burden but won’t make the patient seizure-free. It can be employed in patients who are not a candidate for surgery.
80
Q

A 34-year-old female presents to the office with complaints of hearing loss for one month. She has decreased hearing in bilateral ears. She also reports early morning headaches. She denies fever, ear discharge, numbness/tingling sensation, and trauma. She has a family history of a brain tumor in her father. On examination, the vitals are stable. Weber and Rinne’s tests show bilateral sensorineural hearing loss. MRI of the brain shows mass near the internal acoustic meatus on both sides. Which of the following drugs can be used in the treatment of this condition?

  1. Denosumab
  2. Bevacizumab
  3. Palivizumab
  4. Emicizumab
A

2. Bevacizumab

  • This patient has bilateral sensorineural hearing loss, most likely due to vestibular schwannoma. Bilateral vestibular schwannoma is most likely suggestive of neurofibromatosis 2.
  • Multidisciplinary management is required in patients with NF2, including oncologists, neurologists, neuroradiologists, ophthalmologists, geneticists, and neurosurgeons.
  • Bevacizumab, a monoclonal antibody against vascular endothelial growth factor (VEGF), is new systemic immunotherapy for a wide range of tumors. Some studies have shown tumor regression and hearing improvement in NF2 patients treated with bevacizumab.
  • Small asymptomatic vestibular schwannomas can be managed conservatively with MRI follow-up. Surgery is the primary treatment for large symptomatic vestibular schwannomas.
81
Q

A 6-year-old boy was brought to the emergency department by his parents for evaluation of visual loss on the left side. The parents reported that the patient has been having intermittent frontal headaches over the past few months. On examination, he has difficulty abducting the left eye and has papilledema. His mother reports that he is shorter than his other siblings and friends. Brain imaging revealed a cystic sellar mass measuring 3 cm with calcification. He had trans-sphenoidal surgery. The pathology showed yellow-brown, cholesterol-rich fluid with nodules of “wet keratin,” representing desquamated cells that form large, pale, eosinophilic masses. What is the most likely diagnosis?

  1. Adamantinomatous craniopharyngioma
  2. Papillary craniopharyngioma
  3. Growth hormone-producing pituitary adenoma
  4. Prolactin producing adenoma
A

1. Adamantinomatous craniopharyngioma

  • Craniopharyngiomas are histologically benign grade I tumors. There are two histologic subtypes of craniopharyngiomas: adamantinomatous and papillary.
  • Craniopharyngiomas represent 1.2% to 4% of all childhood intracranial tumors. Craniopharyngioma has a classical bimodal distribution of incidence with increased incidence rates in patients aged 5 to 14 and 50 to 74 years.
  • Adamantinomatous craniopharyngioma (ACP) is primarily seen in pediatric cases but can be seen in adults as well. They have solid and cystic parts.
  • Papillary craniopharyngioma ( PCP) is commonly seen in adults. They are characterized as well-differentiated squamous epithelium lacking surface maturation, with occasional goblet cells and ciliated epithelium. Calcifications are rare in papillary type.
82
Q

A 16-year-old male presented to the emergency department after he was involved in a boating accident. Per his friends, he lost consciousness for 5 seconds at the scene. In the emergency department, he is complaining of a severe headache. He can open his eyes to voice. He is disoriented to place and time. He follows commands appropriately with normal motor function. Due to his severe headache, he has an MRI of his brain which showed small DWI changes in his corpus callosum. What is the diagnosis and Glasgow coma scale (GCS)?

  1. Subdural hematoma; GCS of 14
  2. Subarachnoid hemorrhage; GCS of 12
  3. Diffuse Axonal Injury; GCS of 14
  4. Diffuse Axonal Injury; GCS of 13
A

4. Diffuse Axonal Injury; GCS of 13

  • This patient has a mild TBI with GCS 13.
  • The Glasgow coma scale gives 3 points for opening his eyes, 4 points for the verbal responses, and 6 points for normal motor functions.
  • DAI is a clinical diagnosis suspected in rotational or acceleration-deceleration type injuries.
  • The MRI finding is suggestive of diffuse axonal injury, a type of traumatic brain injury.
83
Q

A 10-year-old unaccompanied boy is brought to the hospital by emergency medical service (EMS) after being hit by a car while crossing a busy street. During the trauma assessment, the patient is moaning, withdraws to painful stimuli only, and does not open his eyes. He is also noted to have a scalp laceration and hemotympanum on secondary assessment. What is the most appropriate management strategy for this patient?

  1. Contact the patient’s parent or legal guardian to obtain consent prior to initiating treatment
  2. Secure the patient’s airway and perform a CT scan of the brain and cervical spine while the healthcare team attempts to contact and obtain consent from the patient’s parent or legal guardian
  3. Stabilize and treat the patient with no attempts to contact and obtain consent from the patient’s parent or legal guardian
  4. Secure the patient’s airway but wait to perform CT scans until the patient’s parent or legal guardian is contacted to give consent to treatment
A

2. Secure the patient’s airway and perform a CT scan of the brain and cervical spine while the healthcare team attempts to contact and obtain consent from the patient’s parent or legal guardian

  • A federal law under the emergency medical treatment and labor act (i.e., EMTALA) mandates that hospitals treat, stabilize, and even transfer minors with emergency conditions to definitive care. At the same time, the staff attempts to contact the family or guardian for consent. This is called the “emergency exception rule” or “doctrine of implied consent,” which assumes that if the parents or guardians were present, they would consent to emergency treatment that is in the best interest of the child.
  • Even though immediately initiating treatment and stabilization of the patient’s emergency medical condition is correct, the hospital team must simultaneously take steps to contact the family or guardian for consent.
  • Once the emergency medical conditions have been stabilized or resolved, the health care team can no longer legally treat the patient until the minor’s parent or legal guardian has provided consent.
  • Performing a CT scan after securing the patient’s airway is still considered part of the evaluation and treatment of an emergency medical condition, which is well within the scope of the “emergency exception rule” or “doctrine of implied consent,” and should not be delayed by waiting for consent.
84
Q

The application of alternating electrical fields to dividing cells result in differential macromolecular localization within the cytoplasm of the cell. Which protein is most likely to be impacted by this effect?

  1. Actin
  2. Septin
  3. Tubulin
  4. Cadherin
A

2. Septin

  • Formation of mitotic spindle involves coordinated localization as well as the orientation of different protein complexes within the cytoplasm.
  • The electric field results in a change in the alignment of the differentially charged proteins (dipoles) like tubulin causing disruption of spindle formation.
  • The electric field gets distributed unevenly within the cell due to the non-spherical shape of the cell during metaphase. Uneven distribution of electric field prevents appropriate localization of protein complex like spectrin due to a process called dielectrophoresis.
  • Septin protein complex is responsible for the coordinated division of parent cell into daughter cells. Disruption of its appropriate localization results in blebbing of the cytoplasm and cell death.
85
Q

A 24-year-old male with a diagnosis of rhabdomyosarcoma of the posterior aspect of the left thigh underwent surgery. Wide excision is performed, and all margins are informed free of the lesion on the frozen sections. During the procedure, a section of the sciatic nerve had to be resected en-block with the specimen leaving a 6 cm nerve gap that was repaired by the use of a sural nerve graft from the same leg. Which of the following factors will have an impact on the nerve regeneration rate across the graft?

  1. Endoscopic nerve harvesting instead open surgical approach
  2. Patient’s age
  3. Patient’s diabetes
  4. Laterality of nerve harvest
A

2. Patient’s age

  • Many factors will have an impact on the potential of nerve regeneration across a nerve graft. These can be separated into patients related factors and other factors.
  • The patient’s age and smoking status are within patient-related factors that will influence the final outcome—the younger the patient, the higher the potential for nerve regeneration.
  • As seen in other tissues, active smoking will have a deleterious effect on tissue regeneration conducting poor results.
  • Other relevant factors that will affect final outcomes are the surgical timing of nerve reconstruction, length of the nerve gap, tension at the site of the suture with the nerve graft. There is no evidence that the open technique of nerve harvesting will have a different outcome compared to the endoscopic harvesting technique. Sural nerve laterality will not have an impact on nerve grafting outcomes, provided that there is no history of traumatic lesions or previous surgeries on the selected donor leg.
86
Q

A 40-year-old man from an Asian country presents with headache and fever for the past week. On examination, he is neurologically intact and has neck stiffness. He undergoes a lumbar puncture, and the cerebrospinal fluid grows acid-fast bacilli. He is started on corresponding medical treatment. Two days later, he develops coughing during food intake. A magnetic resonance imaging scan of the brain shows exudates at the skull base foramen separating the temporal and occipital bones. Which of the following cranial nerves travel through the smaller anteromedial compartment of this foramen?

  1. Vagus and Arnold nerve
  2. Glossopharyngeal and Jacobsen nerve
  3. Spinal accessory nerve
  4. Vagus and spinal accessory nerve
A

2. Glossopharyngeal and Jacobsen nerve

  • The patient is having basal meningitis with exudates secondary to tuberculosis.
  • The jugular foramen is formed by the lateral portion of the occipital bone and petrous portion of the temporal bone. Exudate in this region can involve the cranial nerves passing through the foramen.
  • The anteromedial compartment, the pars nervosa, contains the glossopharyngeal (IX) nerve, Jacobsen nerve, and the inferior petrosal sinus.
  • The pars vascularis contains the jugular bulb, the vagus nerve (X), Arnold nerve, and the spinal accessory (XI) nerve.
87
Q

A 54 year-old-male with a history of pulmonary embolism currently taking Rivaroxaban is brought in to the hospital for severe abdominal pain, diffuse muscle cramping, and headache. The patient is currently on vacation with his family in Hawaii, where he was snorkeling off the coast prior to the onset of his symptoms. The symptoms began with a mild stinging sensation in his left leg, which gradually resolved over the next 5 minutes. However, within the next 30 minutes, he developed severe abdominal pain, back pain, headache, and diffuse muscle cramping. On arrival, his heart rate was 135/min, respiratory rate 25/min, blood pressure 230/115 mmHg, temperature 37 C, and SpO2 99% on room air. Physical examination reveals a diaphoretic male in moderate distress. He is alert, oriented, and follows the command. His pupils are equal and reactive bilaterally. Treatment with intravenous fentanyl, phentolamine, and magnesium is initiated. The patient has some resolution of his pain but remains persistently hypertensive. He suddenly deteriorates and becomes unresponsive. A bedside glucose measurement reveals his blood glucose to be 86 mg/dL. A repeat examination shows a fixed and dilated left pupil. The patient is intubated for airway protection. What is the next best step in the management of this patient?

  1. Intravenous naloxone
  2. CT Imaging of the brain
  3. Intravenous midazolam
  4. Dextrose administration
A

2. CT Imaging of the brain

  • This patient who developed systemic symptoms of severe muscle cramps, headache, hypertension, and diaphoresis after a jellyfish sting, has signs and symptoms consistent with Irukandji syndrome. Up to 25 jellyfish species have been implicated in causing Irukandji syndrome with their stings, but the species Carukia barnesi is the most well-known.
  • The mainstay of treatment for Irukandji syndrome is supportive, as there is no specific antivenom for Irukandji syndrome. Opioid analgesics, nitrates, phentolamine, magnesium, and benzodiazepines are some of the recommended treatment options by consensus guidelines.
  • Although rare, intracranial hemorrhage secondary to persistent severe hypertension in Irukandji syndrome has been documented in the scientific literature. As this patient developed a sudden change in mental status, is exhibiting new asymmetrical pupils, and is taking anticoagulation, there is a high clinical suspicion for intracranial hemorrhage. As such, computed tomography of the brain would be indicated in this scenario.
  • Although the patient received opiates, his sudden change in mental status and asymmetrical pupils rather than the bilateral pinpoint pupils that are classically seen in opiate use suggests an intracranial hemorrhage rather than opiate overdose. Although the patient could have possibly had a seizure and a post-ictal state secondary to intracranial hemorrhage, in the absence of any witnessed seizure-like activity, evaluation for intracranial hemorrhage should take place first. Hypoglycemia is an important consideration for any acute changes in mental status, but this patient’s blood glucose level is within normal limits, so IV dextrose administration would not likely be of any benefit.
88
Q

A patient was brought due to involuntary movements. The movement was noted upon waking up this morning. There was arrhythmic to and fro movement of the left hand with occasional involvement of the elbow. There is no loss of consciousness or alteration in sensorium. The movement has been continuous for the past 3 hours. The patient has a history of hypertension, diabetes, and stroke 1 year ago with left-sided residual deficits. On examination, BP was 135/90 mmHg, HR 89 bpm, and regular; the temperature is normal. Blood glucose was at 190 mg/dl. There are no cranial nerve deficits, the motor examination was 5/5 on the right upper and lower extremity, 4/5 on the left lower extremity, and difficult to assess due to continuous movement on the left upper extremity. He has no sensory deficit. There is hyperreflexia on the left and a positive Babinski on the left. Which of the following is the most likely cause of this patient’s presentation?

  1. Meningitis
  2. Hyperglycemia
  3. Previous stroke
  4. Sepsis
A

3. Previous stroke

  • Patients with epilepsia partialis continua (EPC) experience recurrent seizures over a long period ranging from hours to months and even years.
  • EPC may or may not respond to antiepileptic drugs (AEDs) depending on the underlying pathology.
  • The causes of EPC are very similar to that of any other form of epilepsy as the basic reason for seizures is abnormal electrical activity in the brain brought on by a myriad of pathologies, including architectural damage like a previous stroke.
  • Patients mostly experience Todd paralysis that may last for a varying amount of time.
89
Q

An acute cerebrovascular accident characterized by left-sided hemiparesis but no expressive aphasia is most consistent with occlusion of which of the following?

  1. Left middle cerebral artery
  2. Right middle cerebral artery
  3. Left anterior cerebral artery
  4. Right anterior cerebral artery
A

2. Right middle cerebral artery

  • A stroke in the right middle cerebral distribution would cause left-sided hemiparesis but not expressive aphasia.
  • Expressive aphasia, also known as Broca aphasia, would occur with a left middle cerebral artery stroke.
  • A left anterior cerebral artery stroke would present with right leg weakness and sensory deficit.
  • A right anterior cerebral artery stroke would present with left leg weakness and sensory deficit.
90
Q

A 25-year-old unmarried female presents with the complaint of blurring of vision in both eyes over the past six months. She has had intermittent bilateral frontal mild to moderate headaches for the past two months. She also admits having milk discharge from both breasts over the past three months. On physical examination, she has left lateral rectus palsy, and she is unable to appreciate the peripheral side visions in both eyes. Her serum prolactin is 2433 ng/ml. A magnetic resonance imaging scan of the brain shows a sell-suprasellar lesion with an erosion of the floor of sella and the lesion extending laterally with an invasion of both cavernous sinuses, left more than right. What genetic mutation is most likely in this patient?

  1. PRKAR1A
  2. GNAS1
  3. ADAMTS6
  4. GPR101
A

3. ADAMTS6

  • The patient has an invasive lactotroph adenoma.
  • Tumor recurrence and aggressive behavior in lactotroph adenomas have been associated with the following genes: ADAMTS6, CRMP1, PTTG, ASK, CCNB1, AURKB, and CENPE.
  • Invasion-related genes in lactotroph adenomas include ADAMTS6 and CRMP1.
  • Mutations in PRKAR1A, AIP, GPR101, and GNAS1 are involved in the etiology of somatotroph adenomas.
91
Q

A 83-year-old female presents to acute inpatient rehabilitation and is noted to have weakness to her right foot. She follows up in your clinic after a prolonged hospital course which required prolonged intubation secondary to pneumonia. Her hospital course lasted 4 weeks. Electromyography (EMG) is performed which shows abnormalities to the tibialis anterior and extensor hallucis longus muscles. What is the most likely diagnosis?

  1. Peroneal neuropathy
  2. Tibial neuropathy
  3. Critical illness neuropathy
  4. Peripheral neuropathy
A

1. Peroneal neuropathy

  • The peroneal nerve innervates the muscles in the anterior compartment of the lower leg. This includes the tibialis anterior and extensor hallucis muscles which dorsiflex the foot and extend the great toe, respectively. The peroneal nerve is prone to compression, particularly at the fibular head, which can be seen after prolonged time in one position.
  • The tibial nerve innervates the muscles in the posterior compartment of the lower leg. This includes the gastrocnemius and soleus muscles of the calf, as well as tibialis posterior muscle and toe flexors of the foot. Tibial neuropathy is fairly rare and can often be caused by compression from a prior fracture, osteophytes, or cysts.
  • Critical illness neuropathy commonly presents with distal weakness as well as numbness and tingling. This is frequently seen after a prolonged hospital course and long periods of immobility. Critical illness neuropathy usually presents symmetrically.
  • Peripheral neuropathy causes weakness, numbness, and pain in the extremities. Generally, symptoms are more prevalent distally than proximally and usually develop gradually over time. Common causes of peripheral neuropathy include diabetes, alcohol use, and hereditary causes.
92
Q

A 6-year-old child is brought to the neurology clinic for progressive abnormal movements of her lower limbs. The treating clinician noticed characteristic dancing patterns of her eyes as well as her feet. The abnormal eye movements are observed in all horizontal, vertical as well as diagonal directions. The clinician also found severe ataxia in the child during her neurological assessment. The parents also inform that the child has been developing difficulty in articulating her speech and also has disturbed sleep patterns. What is the recommended first step in the evaluation of the child presenting with the characteristic neurological findings?
1. CT head
2. MRI spine
3. CT abdomen
4. CT chest

A

3. CT abdomen

  • The child presenting with the characteristic dancing eyes and the feet syndrome has most probably an opsoclonus myoclonus syndrome.
  • The most common cause of the opsoclonus myoclonus syndrome in a child is secondary to tumors arising from embryonic nerve cells.
  • The most common tumor-causing opsoclonus in a child is neuroblastoma arising from the adrenal glands. Therefore, the CT abdomen is recommended first during the evaluation of the child.
  • The other imaging modalities are advised only if the adrenal gland imaging is normal, and the possibility of the neuroblastoma from the extrarenal sites is taken into considerations.
93
Q

A 55-year-old male worker came to the clinic with right lower quadrant low back pain for four months after lifting a table during work. X-ray of the lumbar spine had been taken. Which of the following best describes the changes to facet joints in facet arthritis?

  1. The joint capsule starts to have fibrosis and neovascularization in the early course of the disease
  2. Osteophyte will be formed in the early course of the disease
  3. Osteophytes of the facet joints usually form at the middle portion of the joint capsule
  4. There will be thickening of the facet cartilage in facet arthritis
A

1. The joint capsule starts to have fibrosis and neovascularization in the early course of the disease

  • Osteophytes will develop later in the course of the disease.
  • Osteophytes of the facet joints are usually at the border of the capsular insertion.
  • The facet cartilage will be thinner after facet arthritis.
  • Degeneration changes in facet joints will involve the articular cartilage, synovium, and capsule.
94
Q

A 16-year-old patient sustained a severe head injury following a motor vehicle collision. He was being managed in a critical care unit. His laboratory investigation showed persistent hyponatremia. His hourly urine output was also on the lower side. There was also generalized swelling of his extremities. What is the next management plan for the patient in managing his hyponatremia?

  1. Sodium replacement
  2. Fludrocortisone
  3. Fluid restriction
  4. Fluid challenge test
A

3. Fluid restriction

  • The patient has a characteristic presentation of the syndrome of inappropriate antidiuretic hormone (SIADH).
  • Due to the excessive surge of antidiuretic hormone, there is inappropriate retention of water in the body, thereby leading to dilutional hyponatremia, decreased urine output, and the resultant weight gain.
  • The pivotal step in managing SIADH is fluid restriction.
  • Fludrocortisone and sodium replacement are justified only in cases of hyponatremia resulting from excessive renal sodium loss, such as in the cases of cerebral salt wasting.
95
Q

A 20-year-old patient presents to the clinic complaining of chronic muscle weakness. The patient states that he had genetic testing that revealed that he has a CTG trinucleotide repeat expansion located on the DMPK gene. The patient asks if there is any medication that could help his symptoms. What is the most appropriate initial medication choice?

  1. NSAIDs
  2. Lorazepam
  3. Dantrolene
  4. Mexiletine
A

4. Mexiletine

  • CTG trinucleotide repeat expansion located on or near the DMPK gene is the etiology for Myotonic dystrophy type 1.
  • The preferred first-line medication for myotonic dystrophy type 1 is mexiletine with an initial dose of 200mg thrice daily.
  • Providers should check the QT interval with EKG prior to the administration of mexiletine.
  • Myotonic dystrophy type 1 is the most common myotonic disorder.
96
Q

A 52-year-old woman is brought to the emergency department with complaints of severe headache and new vision changes in her right eye. She had a history of systemic lupus erythematosus, poorly controlled hypertension, and nephrectomy two years ago. She has a 30-pack-year history of smoking and drinks a bottle of vodka daily. Her mother died of a brain hemorrhage. On examination, her right eye is blind with a dilated pupil and ptosis. A CT scan done in the emergency department is unremarkable. Which of the following is the next best step in managing this patient?

  1. CT head with contrast
  2. Cerebral angiogram
  3. Lumbar puncture
  4. Slit-lamp examination
A

3. Lumbar puncture

  • The sign and symptoms in this patient are suggestive of subarachnoid hemorrhage. Subarachnoid hemorrhage is defined as blood between the arachnoid membrane and the pia membrane.
  • History is the first clue to where the subarachnoid hemorrhage originated. Spontaneous subarachnoid hemorrhage should raise suspicion for aneurysmal rupture. Most aneurysmal ruptures occur in patients older than 50 years old.
  • Initial evaluation of a patient suspected of having a subarachnoid hemorrhage should include a head computed tomogram (CT). If the initial CT is negative, but a physician has a very high suspicion for hemorrhage, a lumbar puncture should be performed and sent for laboratory evaluation of xanthochromia presence. Sometimes xanthochromia presence can be assessed at the bedside due to visible cerebrospinal fluid color change.
  • As CT angiograms (CTA) have become more readily available, some feel that CTA should be done before lumbar puncture. Cost-effectiveness studies have shown that doing a lumbar puncture is equivalent to or better than doing the CTA.
97
Q

A 65-year-old patient with a left-sided malignant middle cerebral artery infarction was being managed in the neurology intensive care unit (ICU) by placing an intraventricular ICP monitoring catheter. The neurosurgeon was asked for consultation following the appearance of ICP waves rising 50 mm (normal value of 5-15 mm of Hg) above baseline and lasting for 5 to 20 minutes. Which of the following is the most appropriate next management plan for the patient?

  1. keeping the patient in the ventilator and sedated
  2. Barbiturate coma
  3. Hypothermia
  4. Decompressive hemicraniectomy
A

4. Decompressive hemicraniectomy

  • The given clinical scenario is typical of a raised ICP following malignant middle cerebral artery infarction owing to the presence of Lundberg A waves in the ICP waveform.
  • Decompressive hemicraniectomy (DHC) is advisable for life- saving procedures for the same.
  • Timely performed DHC with durotomy allows for the immediate release of the raised ICP and is effective in reducing mortality as well as high dependency among these patients compared to medical management.
  • Other tiers of medical management, such as barbiturate coma, mannitol infusion, and hypothermia, have not been validated for reducing mortality compared to surgical management.
98
Q

A 16-year-old female was brought to the emergency department with a complaint of severe headache and vomiting for the past six hours. The pain is holocranial, and she has never had this much severe headache in her life. Her mother states that she was found to have some organ anomalies when she was born but was not evaluated later. On examination, she is confused. Her pupils are bilaterally equal in size and reacting. There is no focal neurological deficit. She has neck stiffness. She undergoes a computed tomogram scan of the brain, which shows hemorrhage involving the left Sylvian fissure. Which of the following parts of her body needs imaging later so as to identify the associated congenital anomaly?

  1. Heart
  2. Colon
  3. Kidneys
  4. Liver
A

3. Kidneys

  • There are some congenital causes that are associated with subarachnoid hemorrhage (SAH). These congenital causes may reveal a familial occurrence and are associated with certain systemic disorders. Autosomal dominant polycystic kidney disease is strongly associated with berry aneurysms of the cerebral arteries, which can present with SAH.
  • Other possible congenital multi-organ diseases associated with cerebral aneurysms include Ehlers-Danlos and Marfan disease. Marfan and Ehlers-Danlos might be suggested by a chest CT looking for dilation of the aortic root and dural ectasia of the thoracic spine.
  • Risk factors for SAH include family history, smoking, hypertension, heavy alcohol use, and cocaine use.
  • Patients with SAH may report a thunderclap headache or “worst headache I have ever had.” They may present with photophobia, loss of consciousness, intraocular hemorrhage, or third cranial nerve palsy. CT is the initial test of choice. The gold standard is a lumbar puncture. If xanthochromia is seen, then SAH has been present for several hours. It may be negative if present less than 2 hours and is most sensitive 12 hours after SAH. Xanthochromia is a yellowish color and indicates the presence of bilirubin within 9 to 15 hours of SAH.