Section 5 Flashcards
A 24-year-old male patient presents with progressive difficulty with fine motor skills in his right hand. He has a tumor resected from the left frontoparietal region two years prior. Histological analysis from that surgery demonstrated dysmorphic ganglion cells with a separate glial component containing numerous mitoses, nuclear atypica, and invasion of white matter tracts. An MRI demonstrates a recurrent tumor. What is the most appropriate next step for the evaluation and treatment of this patient?
1. Radiation therapy alone
2. Repeat resection with adjuvant radiation therapy
3. Chemotherapy alone
4. Present the patient at a multidisciplinary tumor board conference to discuss further evaluation and treatment recommendations
4. Present the patient at a multidisciplinary tumor board conference to discuss further evaluation and treatment recommendations
- Anaplastic ganglioglioma is an aggressive neuronal brain tumor associated with a poor prognosis despite maximal therapy, including surgery and adjuvant chemoradiation therapy.
- Patients with anaplastic ganglioglioma should be discussed at multidisciplinary tumor board conferences due to the rare nature of the lesion and the lack of standardized treatment paradigms.
- Anaplastic ganglioglioma often shows more aggressive features within the glial component of the tumor.
- Increasing extent of resection is associated with improved survival in anaplastic ganglioglioma patients.
A 32-year-old male was on top of a ladder approximately 15-feet in height. He was painting his house exterior when he lost his balance, fell landing on his right shoulder and right side of his neck on the grass. There was no loss of consciousness and he experienced right shoulder pain and numbness on the lateral aspect of his right upper arm and shoulder. He also immediately recognized that he was unable to flex his right elbow. The initial examination in the emergency department showed that he also could not abduct his right shoulder in addition to impaired pinprick sensation in the same distribution of numbness. The x-ray studies revealed a non-displaced clavicular fracture that required conservative treatment only. The patient was evaluated by neurosurgery and neurology teams in the ER and scheduled for neuroimaging and electrodiagnostic study after three weeks. Magnetic resonance imaging demonstrated no spinal nerve roots avulsion, yet there was a high-intensity signal on one of the brachial plexus trunks with increase contrast enhancement. Somatosensory evoked potentials (SSEPs), electromyography (EMG) and nerve conduction velocity (NCV) showed complete denervation with no voluntary motor units in the right supraspinatus, infraspinatus, biceps and deltoid muscles as well as a loss of SSEP and median nerve sensory conduction. No other significant changes. Given his closed injury, the patient was scheduled for another follow-up appointment. After 4 months, his clinical and electrodiagnostic re-evaluation showed no improvement and no features of re-innervation. Given the lack of recovery, the patient was scheduled for surgical exploration. What type of pre-operative study is mandatory in this case?
1. Repeat magnetic resonance neurography to follow the signal changes in the brachial plexus overtime
2. Repeat clavicular x-ray
3. Spine x-ray for intraoperative patient’s neck positioning for the surgical approach
4. Chest x-ray
4. Chest x-ray
- This is a clinical scenario for a closed brachial plexus injury- traction injury. Ideally, electrodiagnostic studies should be performed three weeks after the injury to allow for denervation changes of the muscle to evolve. Loss of sensation and sensory conduction response are indicative of post-ganglionic nerve injury, which is backed by the absence of pseudo-meningocele and rootlets severing on MRI images. The clinical scenario and the diagnostic studies refer to upper trunk traction injury.
- The upper trunk of brachial plexus is formed from anterior( ventral) rami of mainly C5 and C6 nerve roots. C4 spinal nerve may contribute to the upper trunk innervation. The clinicians should always be vigilant to differentiate between preganglionic and postganglionic injuries. A specific consideration, in this case, is the right phrenic nerve, which originates from C3,4 and 5. Injury of the phrenic nerve is not uncommon in this scenario. Phrenic nerve injury results in diaphragmatic paralysis that can be seen as an elevated diaphragmatic level in Chest x-ray.
- To rule out phrenic nerve injury and diaphragmatic paralysis, a chest x-ray should be done one with full inspiration and followed by another one with full expiration to detect diaphragmatic movement.
- It is of paramount significance to assess phrenic nerve function preoperatively in upper trunk surgical exploration. During the surgical exposure, identification and isolation of the phrenic nerve is an essential step before conducting the neurolysis of scarred nerves. This will help to differentiate between iatrogenic injury of the phrenic nerve or due to the primary injury.
A 17-year-old female with a history of intravenous drug use presents with fever, back pain, and inability to ambulate. She has been experiencing back pain for the past three days. She reports that the weakness has been progressively worsening for the last 6 hours, and she is now unable to walk. On exam, the patient has reduced sensations of her umbilicus and 2/5 strength in her lower extremities bilaterally. Her temperature is 101 F (38.3 C), her heart rate is 89 beats per minute, and her respiratory rate is 17 breaths per minute. Magnetic resonance imaging (MRI) is obtained, and an enhancing lesion is noted from T10-L1. What is the most appropriate next step in the management of this patient?
1. Intravenous antibiotics after culture reports are available
2. Broad-spectrum intravenous antibiotics
3. Surgical incision and drainage and spinal decompression
4. Request an additional MRI with intravenous contrast to confirm the diagnosis
3. Surgical incision and drainage and spinal decompression
- Given that the patient is experiencing symptoms of spinal cord compression, treatment with intravenous antibiotics alone is not appropriate.
- Surgical decompression and drainage with concurrent use of systemic antibiotic therapy is the treatment of choice for many patients with spinal epidural abscess and is essential for those with neurologic deficits, spinal instability, ring-enhancing lesions on MRI, or disease progression on antibiotic therapy.
- Waiting for cultures or the start of surgical drainage is not recommended before starting with empiric intravenous antibiotics when clinical suspicion is high.
- Empiric antibiotics include a combination of vancomycin (to cover methicillin-resistant Staphylococcus aureus), and ceftriaxone, cefotaxime, ceftazidime, cefepime, or meropenem. The choice of the later three antibiotics is especially considered in the suspicion of Pseudomonas.
A 65-year-old female presents to the clinic complaining of low back and buttock pain. She reports the insidious onset of pain without any inciting event. Provocative testing is most consistent with sacroiliac joint pain. Which of the following tests are most reliable in diagnosing sacroiliac joint pain?
1. FABER test
2. Sacral distraction test
3. Ultrasound-guided corticosteroid injection
4. Fortin finger sign
3. Ultrasound-guided corticosteroid injection
- The likelihood of sacroiliac (SI) joint mediated pain increases as the number of positive-provocative tests increase.
- The most informative test for the diagnosis of sacroiliitis is SI joint injection with local anesthetics and corticosteroids. If this procedure relieves the pain, it is likely the SI joint is the cause of the pain.
- Typically range of motion, neurologic, and strength testing are unremarkable in patients with sacroiliitis although the patient may experience pain during some of these tests.
- Sacroiliitis commonly presents as lower back pain but the pain presentation varies greatly, and patients could describe pain in one or both buttocks, hip pain, thigh pain, or even pain more distal.
A 65-year-old male with Parkinson disease has had symptoms for 10 years and is no longer responding to medications. He is unable to do activities of daily living. Electrical stimulation of which areas would most likely improve his symptoms?
1. Hippocampus, globus pallidus interna, and medulla
2. Thalamus, globus pallidus interna, and hippocampus
3. Thalamus, globus pallidus interna, and subthalamic nucleus
4. Medulla, hippocampus, and subthalamic nucleus
3. Thalamus, globus pallidus interna, and subthalamic nucleus
- It is theorized that loss of dopaminergic neurons in Parkinson disease leads to the thalamocortical pathway over inhibition.
- Thalamic deep brain stimulation was used for tremor but other symptoms continued to progress.
- Current treatment includes stimulation of the subthalamic nucleus and globus pallidus interna.
- Criteria for surgery include medically refractory motor complications and the absence of significant mental or physical problems.
A 65-year-old patient with a long-standing history of lower back pain presents with pain in his right leg for the past two days. It radiates from his back to his right thigh, leg, and toes. His pain worsens on lying down, bending, and walking uphill. A magnetic resonance imaging (MRI) of the lumbosacral spine shows disk degeneration with mild bulging at the L3/L4 level. According to the McKenzie classification, the patient’s condition will be classified as which of the following?
1. Postural syndrome
2. Derangement syndrome
3. Dysfunction syndrome
4. Radiculopathy
2. Derangement syndrome
- Derangement syndrome is the most commonly encountered pain syndrome, with a prevalence as high as 78% of patients classified by the McKenzie method.
- In derangement syndrome, pain is caused by a dislocation of articular tissue, causing a disturbance in the normal position of affected joint surfaces. This disturbance will deform the capsule and periarticular supportive ligaments, generating pain and obstructing movement in the direction of the displacement.
- Seven different subsyndromes are classified by the location of pain and the presence or absence of deformities.
- The centralization phenomenon is unique to derangement syndrome, and treatment is targeted in the patient’s directional preference.
A patient developed an ischemic stroke after long periods of sitting. Which of the following is the most likely cause?
1. Atrial septal defect
2. Atrial embolism
3. Coronary atherosclerosis
4. Underlying malignancy
1. Atrial septal defect
- An atrial septal defect results in a shunt between the venous and arterial systems without the pulmonary vasculature to screen out emboli. Initially, the flow is left to right as the left system is higher pressure. Over time, Eisenmenger physiology may develop, leading to a right to left shunt.
- Emboli can bypass the lungs and end up in the arterial system, causing strokes.
- The paradoxical embolus originates in the lower extremities and becomes lodged in the cerebral vasculature.
- An ASD can result in right sided heart failure, pulmonary hypertension, atrial fibrillation, atrial flutter, and Eisenmenger syndrome.
A 50 year old male patient who is referred to your clinic reports a history of a brain vascular malformation in his right frontal lobe which was diagnosed on brain MRI. He states that he was told that it is assocated with a mutation in the CCM1 gene. What is the best estimated annual rate of hemorrhage of this lesion if it has not previously hemorrhaged?
1. 1%
2. 5%
3. 10%
4. 50%
1. 1%
- The annual rate of hemorrhage for a cavernous malformation that has not previously had a hemorrhagic event is approximately 0.7% to 1.1%.
- For a cavernous malformation that has previously hemorrhaged, the annual rate of hemorrhage is 4.5%.
- Infratentorial location is associated with a higher risk of hemorrhage.
- An associated developmental venous anomaly is indicative of a higher risk of hemorrhage.
A 32-year-old male presents to the office with complaints of headache and nausea for the past week. The headache is aggravated by standing or sitting upright, and it is relieved by lying down. Nausea occurs with the episodes of headache. The healthcare professional decides to perform brain and spinal MRI based on his clinical suspicion. Which of the following tests would be best to identify the precipitation cause associated with this disease?
1. MRI of the brain with contrast
2. MR myelography
3. CT scan of the lumbosacral spine
4. CT scan of the brain
2. MR myelography
- Spontaneous intracranial hypotension is secondary to a spinal CSF leak.
- Symptoms include positional headache, nausea, vomiting, neck stiffness, anorexia, diaphoresis, diplopia, photophobia, hiccups, unsteady gait, hyperacusis, tinnitus, blurred vision, and dysgeusia, etc. MRI of the brain shows diffuse pachymeningeal enhancement.
- MR myelography, CT myelography, or radioisotope cisternography can identify the leak.
- MR myelography is noninvasive and does not involve ionizing radiation but the images may be affected by motion, paravertebral veins, fat, or CSF pulsatile flow.
A 3-year-old male child is brought with delayed milestones of development. He has not yet started running, and his speech output is also not normal compared to his peers. On examination, the child is brachycephalic, has an increased distance between his eyes, has a tongue that he struggles to keep in his mouth, and his nose appears small and set-back from the plane of his face. Extremity examination reveals flat, wide thumbs and mild webbing of the toes. There is a history of tracheostomy done in the neonatal period. Which of the following conditions might have necessitated the tracheostomy?
1. Congenital tracheal cartilaginous sleeve
2. Respiratory distress syndrome
3. Cystic fibrosis
4. Vocal cord paralysis
1. Congenital tracheal cartilaginous sleeve
- The child has characteristics of Pfeiffer syndrome (PS) type 1.
- The clinical features of Pfeiffer syndrome include craniosynostosis, midface hypoplasia, and limb anomalies. Broad thumbs and great toes are often associated with partial syndactyly of the second and third fingers, as well as second and third toes in Pfeiffer syndrome.
- Tracheal cartilaginous sleeve (TCS) is an airway malformation, seen in PS, in which distinct tracheal rings cannot be identified.
- Patients having TCS usually requires tracheostomy, mainly to treat obstructive sleep apnea that results from maxillary hypoplasia, choanal stenosis, and macroglossia.
A 49-year-old woman presents to the clinic for follow up. She has had extensive workup for neck pain that radiates to their arm had an MRI of their cervical spine that demonstrated degeneration at the C5/C6 and C6/C7 level. The patient has failed conservative treatment and had a cervical provocation discography, which is positive at the C7 level. Which of the following features is most consistent with the patient’s syndrome?
1. Worsening of pain with forward flexion
2. Worsening of pain from sitting to lying
3. Worsening of pain with palpation
4. Improvement of pain with rest
1. Worsening of pain with forward flexion
- Positive findings in the cervical provocation discography support the diagnosis of cervical discogenic pain syndrome (CDPS). Patients have pain that is especially noticeable with prolonged flexion or with a protruded head posture.
- A common presentation of CDPS is of individuals that use computers for extended periods of time with there head protruded with prolonged flexion.
- A test used to diagnose CDPS is the Lhermitte test. This test utilizes neck flexion with the patient in a sitting position to reproduce pain down the neck and arm, which is present in patients CDPS.
- Cervical discogenic pain syndrome usually presents with pain at rest. Bending can exacerbate this pain. Patients may find relief in frequently changing their position. It may be worse in the morning, secondary to inappropriate postural position while sleeping.
A 16-year-old football player presents to the clinic with complaints of severe headaches, nausea, and vertigo. The patient is oriented to time, place, and person at that time. The patient gives a history that three days ago, during a game, he took off his helmet and appeared confused, and then suddenly fell on his knees and began to vomit. This happened only once. He recalls being hit during the game 2 or 3 times on his head a week back. On neurologic examination, the Glasgow Coma Scale score is 15. Pupils are equally reactive to light. X-rays of the cervical, thoracic, and lumbosacral are normal. A computed tomographic (CT) scan is performed, which shows a hyperdense area, a crescent-shaped mass between the inner table of the skull and the surface of the cerebral hemisphere, which is measured to be 1.3 cm, with an equivalent left-to-right shift. The patient is treated conservatively. Which of the following limits the use of phenytoin in this patient?
1. Phenytoin is not recommended in this age group
2. Phenytoin can worsen the symptoms of this patient
3. Phenytoin has no proven benefit in the prevention of late post- traumatic epilepsy
4. Phenytoin does not reduce the incidence of early post-traumatic seizures
3. Phenytoin has no proven benefit in the prevention of late post- traumatic epilepsy
- Subdural hematomas are very common in athletes.
- Phenytoin is often added as a prophylactic treatment for early post-traumatic seizures, but it is not recommended for the prevention of late post-traumatic seizures.
- NSAIDs are started when the patient is having headaches.
- Surgery is not required until there are severe symptoms or the patient’s GSC is deteriorating.
An indigent patient presents in an emergency room with confusion, bilateral sixth nerve palsies, horizontal nystagmus, and gait ataxia. Which of the following is the most likely diagnosis?
1. A subdural hematoma
2. A brain tumor
3. Hydrocephalus
4. The Wernicke-Korsakoff syndrome
4. The Wernicke-Korsakoff syndrome
- A malnourished patient with confusion, bilateral sixth nerve palsies, horizontal nystagmus, and gait ataxia has the Wernicke- Korsakoff syndrome and should be treated as a medical emergency with intravenous thiamine.
- The individual with Wernicke-Korsakoff Syndrome will usually present with (1) ocular disturbances which may include diplopia, painless vision loss, or strabismus, (2) gait abnormalities that include a wide-based, short-stepped gait and an inability to stand or ambulate without assistance, and (3) mental status changes that include apathy, paucity of speech, and indifference to the environment. As the disorder progresses, other symptoms may develop, including agitation, anger, hallucinations, and confabulations.
- The diagnosis of Wernicke-Korsakoff Syndrome is made by the history and clinical findings that are supplemented with lab studies showing thiamine deficiency. The aim of treatment is to prevent the disorder from getting worse. One should maintain a high level of suspicion for thiamine deficiency to avoid precipitation of severe symptoms of Wernicke-Korsakoff syndrome. Prophylactic thiamine administration is relatively safe and should be started even if the diagnosis has not been confirmed.
- To better understand this disorder, one should think of Wernicke encephalopathy and Wernicke-Korsakoff syndrome as two separate syndromes. Wernicke encephalopathy is characterized by an acute confusional state and often reversible clinical features while Wernicke-Korsakoff syndrome has persistent but often irreversible clinical features, including dementia and gait abnormalities.
An 85-year-old female diagnosed with Alzheimer disease 2 years ago was brought by her daughter to the emergency department due to dysphagia. She started to have difficulty in swallowing solid food and eventually had difficulty even with drinking liquids. She also started bladder and bowel incontinence and had difficulty recognizing her daughter, who takes care of her recently. An MRI of the brain showed bilateral hippocampal atrophy. Which of the following arteries supply the anterior hippocampus?
1. Anterior cerebral artery
2. Posterior cerebral artery
3. Middle cerebral artery
4. Anterior choroidal artery
4. Anterior choroidal artery
- Anterior choroidal arteries supply anterior hippocampus, lower posterior limb/sub retrolentiform portions of the internal capsule, globus pallidus, and tail of caudate nucleus.
- The posterior hippocampus is supplied by PCA branches.
- The corpus striatum (caudate and putamen) and upper parts of anterior limb, genu, and posterior limb of internal capsule are supplied by lenticulostriate branches of MCA.
- It has been seen that patients with early Alzheimer disease have 15-30% of volume loss of hippocampus while those with moderate Alzheimer disease may reach to the extent of 50%.
A 40-year-old woman with glioblastoma undergoes chemoradiation. Three months following treatment, she complains of a worsening headache. An MRI is inconclusive and cannot clearly define if there is any progression of the disease. Pseudoprogression is suspected. Which of the following investigations is most likely confirm the suspected diagnosis in this patient?
1. Amyloid PET scan
2. HMPAO PET scan
3. Tau PET scan
4. FDOPA PET scan
4. FDOPA PET scan
- FDOPA PET is a very sensitive tool to evaluate brain tumor patients at diagnosis or during follow-up. It can be successfully used as a problem-solving tool when the MRI is inconclusive.
- FDOPA PET scans can differentiate glioma recurrence/progression from treatment-induced changes, e.g., pseudoprogression, radionecrosis.
- If a biopsy is contemplated to evaluate for a recurrence site or during initial diagnosis, FDOPA PET scans can define the optimal biopsy site.
- Amyloid and Tau PET scans are used in the evaluation of dementia patients. HMPAO is a SPECT, not a PET radiotracer
A 65-year-old female patient with progressive memory loss for the past six months presents with an inability to recognize faces that should be familiar to her. She is also complaining of headaches for the past month that is worse upon waking up. She has a history of stage 2 breast cancer, post- mastectomy, and underwent chemotherapy and radiation therapy 2 years ago. On examination, she is awake, alert, and able to follow commands. She is not oriented to time but oriented to place and person. Her mini-mental status exam is 18/30. She is unable to name objects presented visually, like a watch and a pen but is able to name them when placed in her hand. She cannot copy simple objects but is able to draw them after their purpose is described. There is no motor or sensory disturbance. What is the location of the lesion in this case?
1. Fronto-parietal cortex
2. Parieto-occipital cortex
3. Temporo-occipital cortex
4. Parieto-temporal cortex
2. Parieto-occipital cortex
- Apperceptive visual agnosia is typically associated with parietal and occipital cortex lesions. This refers to an abnormality in visual perception and discriminative process, despite the absence of elementary visual deficits.
- These people are unable to recognize objects, draw, or copy a figure. They cannot perceive the correct forms of the object, although knowledge of the object is intact.
- Associative visual agnosia refers to difficulty in understanding the meaning of what they see. They can draw or copy but do not know what they have drawn.
- They correctly perceive the form and know the object when tested with verbal or tactile information but cannot identify it.
A 17-year male has been referred to the clinic with severe, sharp pain in his throat for the last two months. He was on a road trip with his friends when he got into an altercation with a gang member. When he woke up the following day, he was found to have a hematoma around his eyes and behind his ears. He was immediately taken to the hospital nearby, where he was found to have a minor fracture in the base of the skull. He was conservatively managed for three days and discharged home. Two months after the incident, he started having pain in his throat. He describes the pain as intermittent with the episode lasting from a few seconds to a minute. His pain is exacerbated by swallowing, chewing, and talking. He was treated by his primary provider with acetaminophen, ibuprofen, gabapentin, and a short course of tramadol, but all these medicines failed to control his pain. On exam, he does not have any tenderness in his mouth or jaw except for his left tonsillar area and the left side of the base of the tongue. Which of the following ganglions should be targetted for a diagnostic block?
1. Cervicothoracic ganglion
2. Meckel’s ganglion
3. Inferior petrous ganglion of Andersch
4. Ganglion cervicale superius
3. Inferior petrous ganglion of Andersch
- The clinical vignette described in the case is a case of glossopharyngeal neuralgia (GN). Per the International Classification of Headache Disorders (ICHD)-3 definition, the pain of GN happens in paroxysmal attacks that last from a few seconds to a minute. The description of the pain is severe, sharp, stabbing, shooting, or electrical in nature. Painful areas are typically below the ear, in the epiglottis, palatine tonsils, the base of the tongue, and/or the posterior pharynx. Paroxysmal pain is precipitated or exacerbated by coughing, yawning, swallowing, or talking.
- The patient developed GN due to the skull base fracture. There are two forms of GN. 1. Idiopathic or essential: caused by vascular compression. 2. Secondary form: caused by traumatic skull base fractures, infections like tonsillitis, pharyngitis, and parapharyngeal abscesses, as well as an elongated styloid process.
- The first line of management of GN pain should be medical management. The most common medications that are used to treat this condition include gabapentin, carbamazepine, duloxetine, valproic acid, and clonazepam.
- The inferior petrous ganglion of Andersch is the target for pain caused by glossopharyngeal neuralgia. The block should be reserved for patients that fail conservation management options and medications. A diagnostic block with a local anesthetic should be tried first in order to confirm the origin of the pain. If diagnostic blocks are successful, chemical neurolysis or thermal radiofrequency ablation can be performed on the nerve. Chemical neurolytic agents such as alcohol, glycerol, or phenol are typically used. Radiofrequency ablation is typically performed at the jugular foramen to target the inferior petrous ganglion of Andersch. Accurate needle placement is extremely important as life-threatening bradycardia and hypotension can occur if the vagus nerve is stimulated during the procedure.
A 78-year-old female presents with neck pain after falling off of the toilet. A CT of the cervical spine shows a fracture through the base of the dens with 3 mm of superior displacement. She is neurologically intact and has numerous medical comorbidities, including uncontrolled diabetes mellitus, anemia, osteoporosis, smoking, chronic obstructive pulmonary disease, congestive heart failure, and two previous heart attacks. Which of the following is the most rational management strategy for this patient?
1. Rigid cervical orthosis
2. Halo vest immobilization
3. Odontoid screw
4. C1-C2 posterior fixation
1. Rigid cervical orthosis
- A type II odontoid fracture can be treated with various modalities depending on the characteristics of the fracture and patient factors.
- The application of a rigid cervical collar is a valid treatment option for this patient.
- Aged patients have increased morbidity with halo vest immobilization.
- Surgery is best avoided owing to multiple medical morbidities in the patient. The patient also has minimal displacement and no angulation of the odontoid fracture.
A 56-year-old patient has been diagnosed with terminal pancreatic cancer. Recently, the tumor has encased upon his left t9-t11 rootlets, thereby causing severe and refractory nocturnal pain in the patient. The patient has been referred to the pain clinic for the management of the same. The treating clinician opts for intrathecal alcohol injection in the appropriate dorsal rootlets of the patient. What is the most appropriate positioning of the patient to ensure maximum efficacy following the procedure in the patient?
1. Prone
2. Supine
3. Left lateral positioning
4. Right lateral positioning
4. Right lateral positioning
- The alcohol (specific gravity of 0.8) is comparatively hypobaric in relation to the cerebral spinal fluid (specific gravity of 1).
- The alcohol, therefore, floats amidst the CSF environment. This necessitates the appropriate positioning of the patient to ensure that the dorsal rootlets are above the presumed site of injection.
- The appropriate lateral positioning, therefore, ensures that the selected dorsal root entry zones (DREZs) are above the level of the corresponding ventral rootlets, thereby facilitating the action of the injected alcohol therapy.
- The prone positioning minimizes the efficacy of the action of the alcohol selectively upon the dorsal rootlets. Moreover, alcohol is also rapidly absorbed. Thereby the large volume of alcohol is required, which increases the risk of adverse neurological events.
A 58-year-old female with a previous history of transsphenoidal surgery for the management of Cushing disease seven years ago and bilateral adrenalectomy due to endocrinological failure despite a complete removal by the postoperative brain magnetic resonance imaging is evaluated by the endocrinologist and the neurosurgeon for the appearance of a new tumor in the pituitary fossa. Her adrenocorticotropic hormone had increased to 780 pmol/L (Normal: 1.3-16.7 pmol/L) and had developed significant skin hyperpigmentation. The magnetic resonance imaging performed last year only suggested a 3 mm abnormality within the pituitary gland. Still, the current study clearly shows an 18 mm lesion with contrast enhancement touching the chiasm. Which is the next best management in this patient?
1. Surgical removal of the tumor
2. Medical treatment with somatostatin-analogs
3. Medical treatment with dopamine agonists
4. Observation until next year magnetic resonance imaging to evaluate the growth pattern
1. Surgical removal of the tumor
- The initial recommended treatment for Nelson syndrome is the surgical removal of the tumor. Risk factors for developing Nelson syndrome have been studied in an attempt to achieve early detection and treatment. High levels of plasma ACTH one year after bilateral adrenalectomy have the strongest predictive capabilities. Fasting ACTH plasma levels above 154-220 pmol/L (700-1000 pg/ml) are predictive of Nelson’s syndrome one year after bilateral adrenalectomy. Screening is done with the help of brain magnetic resonance imaging (MRI) to visualize the sellar area for pituitary tumors. If tumors are not detected, a brain MRI should be performed at regular intervals. MRI may reveal a small tumor forming.
- Surgery can be performed microsurgical or endoscopically by the transsphenoidal route. Extensive tumors with lateral extensions may require a craniotomy. Endocrinological cure is achieved in approximately 50% of the cases. This is due in part to the infiltration of the cavernous sinus and extrasellar extension.
- Surgery can be complicated by postoperative hypopituitarism in 30% of the cases and permanent diabetes insipidus in 20% of the cases.
- Medical treatment is limited, and the results are not curative. Radiotherapy in the form of fractionated radiotherapy or stereotactic radiosurgery can be given to the tumor but is usually used in patients who can not undergo surgical resection. Sometimes, close clinical follow-up is recommended as many of the tumors do not develop clinically meaningful symptoms or have a slow growth pattern of less than 5 mm per year.
A 17-year-old male with a history of C5 incomplete spinal cord injury presents to the clinic with a complaint of increased pain in his left upper extremity and difficulty with activities of daily living. On physical examination, his left arm is in flexion with increased muscle tone. Passive extension of his arm is possible but difficult. When completing his chart, what should be recorded for his modified Ashworth scale score?
1. 1
2. 1+
3. 2
4. 3
4. 3
- The modified Ashworth scale is a quick clinical scale that measures spasticity and increased tone.
- A score of 3 represents a considerable increase in muscle tone with passive movement of the limb being difficult.
- Passive movement is difficult but possible in a modified Ashworth score of 3.
- The modified Ashworth scale score differs from the Ashworth score because it includes the “1+” option. 1+ is a slight increase in muscle tone manifested by a catch, followed by minimal resistance throughout the rest of the range of motion. This must be less than half of the range of motion.
A sonogram of a 20-week gestational age fetus of a 23-year-old female reveals an outpouching of the head with encephalic mass and cerebrospinal fluid protruding from a skull defect at the nasofrontal suture area. The outpouching was noted in an earlier sonogram performed at her 10th weeks of gestation. The mother had a previous normal pregnancy when she was 17. There is no family history of developmental anomalies. Which is the pathophysiologic mechanism for the sonographic imaging finding?
1. Gastrulation abnormality
2. Incomplete primary neurulation
3. Separation of surface ectoderm from neuroectoderm after the closure of the neural folds
4. Failure of regression of dural diverticulum at the fonticulus frontalis
4. Failure of regression of dural diverticulum at the fonticulus frontalis
- A frontal encephalocele is believed to be caused by the failure of the regression of a dural diverticulum that forms at the recess in the anterior fossa called the fonticulus frontalis that is closely associated with the foramen caecum.
- Typically there is a separation of surface ectoderm from neuroectoderm after the closure of the neural folds. Failure of this process has been related to the formation of encephaloceles.
- Sincipital encephaloceles are classified as nasofrontal, nasoethmoidal, or naso-orbital. Nasofrontal encephalocele is the most common type, seen in 46.4% of the patients. It is followed by nasoethmoidal type in 39.2% of the patients. The naso-orbital and the combined type are the least common with 14.2%.
- It was believed that encephaloceles were a problem with primary neurulation, but this idea has been abandoned.
A male neonate born at 31 weeks is undergoing neonatal evaluation. Transcranial ultrasound examination reveals the presence of a cystic space in the pineal region. The color doppler shows the internal cerebral veins adjacent to the cystic space. What is the most likely diagnosis?
1. Cavum veli interposity
2. Cavum septum pellucidum
3. Mega-cistern ambient
4. Cerebral arteriovenous malformation
1. Cavum veli interposity
- The cavum velum interpositum (CVI) is a potential cisternal space between the two layers of the tela choroidea of the third ventricle located between the internal cerebral veins and posterior medial choroidal artery.
- CVI has a triangular shape with a wide base dorsally.
- Its boundaries include superiorly: the fornix and the hippocampal commissure, inferiorly: the tela choroidea of the third ventricle and the internal cerebral veins, laterally: the thalami, anteriorly the roof of the third ventricle and the interventricular foramina, posteriorly: the splenium of the corpus callosum and the habenular commissure.
- The cavum septum pellucidum is a potential liquid space between the two layers of septum pellucidum. It is present in the normal fetus, but over 85% of them fuse by 3-6 months of age meaning that a CSP persists in ~15% of the adult population. Its boundaries include anteriorly: genu of the corpus callosum, superiorly: corpus callosum, posteriorly: fornix, inferiorly: anterior commissure and the rostrum of the corpus callosum, and laterally: leaflets of the septum pellucidum. The ambient cistern is a cerebrospinal fluid-filled space lateral to the cerebral crus. It contains the posterior cerebral artery, superior cerebellar artery, basal vein (Rosenthal’s), and the trochlear nerve. Cerebral arteriovenous malformations (CAVMs) are vascular malformations composed of a nidus of vessels through which arteriovenous shunting occurs. In our clinical scenario the color doppler revealed only normal internal cerebral vein.
A 35-year-old woman is brought to a hospital in Kentucky in an altered state of consciousness. She is accompanied by her husband, who states she fell of stairs. Physical examination shows multiple bruises on the patient’s forehead and arms in different stages of healing. She has a GCS of 7/15 and is intubated. CT head reveals an epidural hematoma. Medical records indicate that the patient has had multiple admission due to domestic violence in the past but had wished it not to be reported to the authorities. While waiting for neurosurgical intervention, the patient suffers brain herniation and passes away. Which of the following needs to be informed of the patient’s history of domestic violence by law at this point?
1. Family
2. Police
3. Hospital ethics committee
4. Adult protection services
2. Police
- In the United States, as many as one in four women and one in nine men are victims of domestic violence. Domestic violence is thought to be underreported.
- Intimate partner violence is not mandated by law to be reported to authorities if the victim wishes it not to be reported.
- However, statutes of certain states require reporting to law enforcement any relevant information on the death of a victim of domestic violence.
- Kentucky is one of three states (with Oklahoma and Arkansas) with a mandatory reporting law specific to domestic violence.
A 32-year-old female patient started 3 weeks ago with mild perianal numbness but did not seek medical attention. This has significantly progressed during the last week, and since last night, she complains of numbness in the saddle area distribution, has urinary retention, and absent bilateral motor function below the knee. There is lax sphincteric tone on the rectal exam. Emergency magnetic resonance imaging shows a very large intraspinal lesion occupying all the spinal canal at the level of L3. Which is the best time-frame to operate this patient?
1. Within the first 48 hours after the presentation of symptoms
2. Within the first 96 hours after the presentation of symptoms
3. Within the first week after the presentation of symptoms
4. Any time as the patient has already bladder dysfunction and absent rectal tone and surgery time will not improve these deficits
1. Within the first 48 hours after the presentation of symptoms
- The timing of surgical decompression is a significant factor in the prognosis and outcome, the best results are obtained when surgery is done within the first 48 hours after the presentation of symptoms.
- Some reports give the benefits until the 72-hour mark.
- Bladder dysfunction has a negative prognostic factor for poor outcome. The return of bladder function is estimated to occur in approximately 50% of patients with cauda equina syndrome.
- Steroids can be useful in patients with tumors, but surgery should be performed before the 72-hour mark.
A 17-year-old male military recruit presents to the emergency room complaining of nausea, fever, and a severe headache. He is currently living in barracks and shares a living space with his fellow recruits. On physical exam, he has a rash on his abdomen and states that it hurts to look into the light and bending his neck downward produces pain as well. It assumed the patient has an infection of the central nervous system. The patient undergoes a lumbar puncture and 10 ml of fluid are removed. How long will the brain take to replenish the lost fluid?
1. 1 hour
2. 12 hours
3. 24 hours
4. 72 hours
1. 1 hour
- Normal cerebrospinal fluid production is about 0.3 ml/min and most of the fluid is produced by the choroid plexus. The total amount of fluid in the brain is 120 ml.
- Most of the fluid removed during a lumbar puncture is easily replaced within one hour.
- The normal route of clearance of cerebrospinal fluid is to the lateral ventricles, then to the interventricular foramen of Monro, the cerebral aqueduct of Sylvius, the subarachnoid space, the dural sinus, and into the venous drainage.
- CSF returns to the vascular system via the dural venous sinuses by first entering the arachnoid granulations.
A 22-year-old male experiences the acute onset of severe painful unilateral headache lasting 15 to 20 minutes. The pain is described as boring and drilling and located primarily around the eye. During the attack, he develops red eyes, rhinorrhea, and a red flushed face. What is the most likely diagnosis?
1. Tension headache
2. Migraine headache
3. Cluster headache
4. Headache due to brain tumor
3. Cluster headache
- The presentation is classic for a cluster headache.
- Migraines sometimes have an aura and last 6-72 hours. It starts as a dull pain getting worse over minutes to an hour. Many times it has been associated with neurological symptoms.
- Cluster headaches are severe, recurrent, periorbital, and unilateral. They fall under the category of trigeminal autonomic cephalgias (TACs). There is often associated autonomic disruption seen as watering eye, periorbital swelling, and nasal congestion, all ipsilateral to the headache.
- Cluster headaches are more common in men (3:1) and affect about 1 in 500 people. Acute attacks are treated with oxygen and triptans. Prophylaxis is with verapamil.
A 3-month-old girl is brought to the clinic for being increasingly irritable. She reports that in the last 3 days, her baby has not been able to keep down her milk. On examination, the anterior fontanelle is open and bulging significantly. There is also evidence of ridging of the cranial suture between the parietal bones and the occipital bone. The baby is also visibly lethargic. Fundoscopy reveals bilateral papilledema. Which of the following conditions is most likely responsible for this patient’s symptoms?
1. Positional plagiocephaly
2. Apert syndrome
3. Bicoronal synostosis
4. Bilambdoid synostosis
4. Bilambdoid synostosis
- 15% of cases of lambdoid synostosis are bilateral. 86% of the cases are associated with raised intracranial pressure.
- Bilateral lambdoid synostosis is usually associated with an elongated vertex.
- In general, lambdoid synostosis is rare. They have a prevalence of only 0.1 per 10 000 live births.
- The presence of suture ridges excludes a positional skull deformity. Apert syndrome is characterized by bicoronal synostosis.
A 16-year-old boy undergoes a repair of his ulnar nerve, following a physical assault with a knife, in his elbow region. Three months later, the patient complains of pain along the course of the nerve during the movement at his elbow joint. Now the patient has severe pain even with the slightest touch in the region. The ultrasound reveals a neuroma formation at the previously operated site along the course of the nerve. The patient did not show any improvement with oral pain medications as well as repeated steroid injections. The pain has hampered his quality of life as well. Which of the following is the next best step in the management of this patient?
1. Direct transcutaneous electrical stimulation
2. Dynamic magnetic flux induced neuro-modulation
3. Repeat steroid injection
4. Excision of the neuroma
2. Dynamic magnetic flux induced neuro-modulation
- The patient has typical features of allodynia following traumatic neuroma formation. The neuroma results following the fascicular escape, leading to sprouting axons recruitment within the epineural space, along with fibromatosis.
- The dynamic magnetic flux induced neuromodulation has shown to improve allodynia in patients with a traumatic neuroma significantly. This also has shown to aid in neuronal regeneration and repair.
- Low-frequency magnetic stimulation results in neuronal inhibition thereby obviating pain. Moreover, since there is no direct device-patient contact, this is more ideal in managing patients with neuronal hypersensitivity.
- The patient has already failed repeated steroid injections. The need for device-patient contact in electrical stimulation is a limiting factor in this patient with allodynia. The excision of neuroma along the ulnar nerve is not justified due to the risk of impending neurological deficits.
A 7-month-old boy is brought to the clinic for evaluation for an abnormal head shape. Premature closure of the metopic suture is suspected. Which of the following sets of findings is most likely to be seen on the patient’s head computed tomographic scan and physical examination?
1. Midline bone ridge at the vertex and hypotelorism only
2. Midline forehead ridge at the forehead, hypotelorism, and widened parietal bones
3. Bilateral harlequin eye and hypertelorism with forehead bone ridge
4. Flattening of the occipital bone with widened temporal bones
2. Midline forehead ridge at the forehead, hypotelorism, and widened parietal bones
- In premature closure of the metopic suture, the most obvious sign for the diagnosis is palpation of a midline bony ridge over the forehead. Craniofacial anomalies like hypotelorism and temporal narrowing with parietal widening are, in most cases, sufficient to accurately diagnose craniosynostosis.
- The premature fusion of the metopic suture produces a narrow forehead, causing the eyes’ position to be closer than usual (hypotelorism) with associated bitemporal narrowing. Early closure before the third month will cause trigonocephaly.
- Metopic craniosynostosis produces a visible midline forehead bone ridge. Hypotelorism is frequently associated. Patients have a narrow bitemporal skull dimension with a compensatory expanded biparietal dimension. Eyebrows are usually raised.
- Early operative treatment is recommended to provide the best possibility for the brain to expand and produce a normal configuration of the skull. Surgery goals are to remove the bony ridge of the metopic suture, advance both orbits and the frontal bones, achieve a rounder forehead contour, and prevent the psychosocial impact and neurodevelopmental delay.
A healthy male is involved in a motor vehicle collision. He loses consciousness but regains it within a few hours and appears normal to friends. He later presents to the emergency department very lethargic, weak, and with a dilated left pupil. Which of the following is most likely to be seen on a head CT scan?
1. Cresent shaped hyperdensity close to the skull
2. Hyperdensity on the basal cisterns and subarachnoid space
3. Biconvex hyperdensity close to the skull
4. Hyperdensity on the brain parenchyma
3. Biconvex hyperdensity close to the skull
- An epidural hematoma is due to an accumulation of blood between the dura and the bone and may occur intracranially or in the spinal cord.
- An epidural hematoma should be suspected in any individual who sustains head trauma. It is associated with a lucid interval followed by an altered level of consciousness.
- Other symptoms of an epidural hematoma may include weakness, numbness, urinary incontinence, and fecal incontinence. Focal neurological deficits may include loss of vision, aphasia, and seizures.
- Findings may include hemotympanum, anisocoria, weakness, ataxia, visual field defects, and weakness of extremities. Bradycardia and hypertension may be indicative of raised intracranial pressure.
A 62-year-old woman is undergoing a right retrosigmoid craniotomy for resection of a cerebellopontine angle tumor. During the surgery, the tumor appears to compress the facial nerve, and resection of the lesion causes some traction on the nerve. Her right facial function was a House Brackman of 4/6 before the procedure but is a 6/6 after the surgery. One year after the procedure, she has not recovered any facial function. What is the most appropriate management strategy for this patient?
1. Conservative management
2. Direct distal facial nerve stump coaptation to the brainstem with the aid of a nerve stimulation probe
3. Sural nerve interneural graft to reconnect the pontine facial nerve with the extratemporal facial nerve
4. Hypoglossal nerve transfer to the extratemporal segment of the facial nerve
4. Hypoglossal nerve transfer to the extratemporal segment of the facial nerve
- Delayed repair of the facial nerve after damage to the cisternal portion of the nerve is best done with a hypoglossal nerve transfer to the intraparotid segment of the facial nerve.
- During surgery, if a nerve is cut, the best repair is direct end-to- end anastomosis if it can be approximated without tension.
- Lack of clinical and electrophysiological signs of spontaneous recovery after 3–6 months indicates surgical intervention. Endoneurial tubes must be in contact with regenerating axons within 18–24 months after injury; otherwise, degeneration will occur.
- Sural nerve interneural graft is used to connect two healthy nerve stumps which cannot be approximated without tension.
Lumbar spinal stenosis may present with which of the following?
1. Back pain that increases upon standing
2. Back pain upon lying in the prone position
3. Numbness in the arms
4. Shooting pain in the legs
1. Back pain that increases upon standing
- Back pain and paresthesias that worsen upon standing but are absent when sitting is indicative of spinal stenosis.
- Severe stenosis is associated with weakness.
- Cervical spinal stenosis can present with decreased hand dexterity and mild proximal lower extremity weakness and progress to neck and arm pain.
- As with standing, one expects lying prone to worsen or exacerbate the symptoms of spinal stenosis (whereas prone positioning may improve the symptoms of HNP).
A 59-year-old woman presents with left-sided weakness. The symptoms started 30 mins ago when she was having lunch in a restaurant. The patient has a past medical history of hypertension and type 2 diabetes mellitus. On examination, the patient is awake, alert, able to follow commands, and is oriented to time, person and place. Cranial nerve examination reveals preferential gaze to the right and dysarthria. The patient is unable to recognize left-sided stimulus on double simultaneous stimulation. The motor examination shows 3/5 strength on the left upper and left lower limb. There is a 20% sensory deficit on the left to pain and light touch. Babinski sign is positive on the left. A head CT is performed. Which of the following do you expect to see on this scan?
1. Encephalomalacic changes in the right periventricular region
2. A small area of hyperdensity in the pons
3. Insular ribbon sign with obscuration of the lentiform nucleus
4. Hyperdensity at the Sylvian fissure and base of the skull
3. Insular ribbon sign with obscuration of the lentiform nucleus
- Ischemic brain tissue stops working in seconds and suffers necrosis in as soon as 5 minutes after complete lack of oxygen and glucose supply, prompting a myriad of potential symptoms and disability.
- Cerebral ischemia can be focal or generalized and cause varying clinical pictures.
- While generalized cerebral ischemia is generally related to syncope and pre-syncope, focal cerebral ischemia usually causes a stroke or transient ischemic attacks.
- As a rule of thumb, acute cerebral ischemia is never painful.
A 68-year-old woman with a history of metastatic breast cancer presents to the office for follow up. She is currently taking hydromorphone/acetaminophen 10/325 mg 4 times a day for severe pelvic pain due to boney metastasis. Despite this medication use, the patient still complains of 9 out of 10 pain. After a careful review of the source of pain, it is suspected to be coming from a neurologic dermatome from the spinal cord. A spinal opioid injection for pain control is then performed. Soon after the injection, the patient describes pain in the frontal and occipital areas that is worse in the upright position and improved in the supine position. She states the pain is also made worse with coughing. Which of the following is the next best step in the management of this patient?
a. Reassurance
b. Head CT scan
c. MRI head
d. Oral hydration
d. Oral hydration
- While serious complications from spinal opioid use in anesthesia are rare, when complications do occur, they are specific to both the medication and the approach utilized in the procedure. In the prototypical example of spinal morphine use, complications include respiratory depression, constipation, altered mentation, and somnolence.
- Complications of spinal opioid injection also include the risk of paresthesias, temporary pain at the injection site, and post-dural puncture headache.
- Rarer procedural complications of spinal opioids also include infection, urinary retention, and epidural hematoma.
- The first step in managing post-dural puncture headache from spinal opioids is using conservative measures with oral rehydration and simple oral analgesia.
A 39-year-old man presents with lower back pain. The patient used to be a semi-professional golf player and says the pain started during a game. This pain did not let him continue with the game. Upon waking up the next morning, he felt the pain now radiates down the right leg. He has no past medical history and takes no medication. Which of the following examinations is the most appropriate to make the diagnosis of this patient’s condition?
1. Ankle jerk reflex
2. Lumbar puncture
3. Straight leg raise
4. Head flexion in a sitting position
3. Straight leg raise
- Straight leg raise is performed with the patient supine and will reproduce the pain if a herniated disc is compressing the nerve root.
- Deep tendon reflexes can be normal, decreased, or absent with nerve root compression. Many pathologies can affect them.
- The crossed straight leg raise is less sensitive but more specific. It is more specific for disc herniation. This maneuver reproduces the pain in the leg when you raise the contralateral leg.
- Head flexion used for Lhermitte’s sign causes a sudden sensation resembling an electric shock that passes down the back of your neck and into your spine and may then radiate out into your arms and legs. This is usually caused by cervical cord compression.
Which of the following is characterized by progressive basal ganglia calcification and encephalopathy?
1. Neuroacanthocytosis
2. Harp syndrome
3. McLeod syndrome
4. Fahr syndrome
4. Fahr syndrome
- Fahr syndrome is also known as idiopathic basal ganglia calcification.
- It is thought to have autosomal dominant inheritance but no specific gene location has been found.
- Age of onset is variable but in children there may be senile appearance, dwarfism, retinitis pigmentosa, ataxia, intellectual disability, seizures, dysarthria, and choreoathetotic movements.
- The calcification usually starts in the pons and dentate followed by the basal ganglia and finally at the corpus callosum.
A 6-year-old boy is brought to the clinic to establish care after recently moving to the city. His mother reports that they needed a new physician to take care of their child due to the condition he has. The parents were told that their child had “premature closure of a skull suture.” The parents deny any symptoms except for the abnormal head shape and mention that he has achieved all milestones adequately for his age but is not doing well in school. He has good attention, but his performance is below most other students. He has good communication with the teacher and interacts well with many students. On examination, there is an elongated, narrow skull with ridging of the sagittal suture with frontal and occipital bossing. Which of the following is the most likely cause for his poor school performance?
1. Hyperactivity-attention deficit
2. Venous occlusion
3. Increased intracranial pressure
4. Adaptive and psycho-social issues
3. Increased intracranial pressure
- In a premature single suture fusion, elevated intracranial pressure occurs in approximately 11% of cases. In sagittal synostosis, this can be as high as 44%.
- Having signs/symptoms of increased intracranial pressure has significant implications and can lead to detrimental outcomes for which management is required.
- Monitorization of intracranial pressure during regular follow-up (i.e., fundoscopic examination) is very important.
- The diagnosis and treatment of craniosynostosis can produce a significant impact on families. Careful explanation of the diagnosis, treatment modalities, postoperative care, and prognosis can help families deal with the initial struggle after the diagnosis is made.
A 12-year-old male presented with acute onset of headache, nausea, vomiting, and diplopia. Ophthalmological examination showed a limited upward gaze. Light reflex was lost, but pupils responded to near stimuli. Nystagmus was noted when he attempted upward gaze. The remaining physical examination was unremarkable. An axial non-contrast computed tomography (CT) scan showed a lobulated, hyperdense mass, pineal calcification, and obstructive hydrocephalus. An external ventricle drain was placed. What is the next best step in the patient’s management?
1. Immediate surgical resection of the tumor
2. Magnetic resonance imaging (MRI)
3. Lumbar puncture
4. Biopsy of the mass
2. Magnetic resonance imaging (MRI)
- Pineal region germinoma usually presents acutely with signs and symptoms of raised intracranial pressure like headache, nausea, vomiting, and diplopia due to obstructive hydrocephalus. Parinaud syndrome is also a common presentation, caused by the compression of the midbrain tectum by the tumor and is characterized by the classical triad of upward gaze palsy, pupillary near-light dissociation, and convergence nystagmus.
- CT and MRI are crucial for the assessment of tumor site and size. However, MRI is the most sensitive imaging modality and is considered a gold standard in detecting central nervous system (CNS) tumors.
- On MRI, germinoma shows a well-circumscribed, lobulated homogenous mass of variable intensity and maybe isointense or hypointense on T1 and isointense or hyperintense on T2 weighted images. In more advanced disease, germinomas may become heterogeneous with a partially cystic appearance and may invade adjacent brain structures with edema of surrounding brain parenchyma.
- Imaging is followed by laboratory evaluation of tumor oncoproteins, biopsy for histological diagnosis, and cerebrospinal fluid (CSF) cytology to rule out disseminated disease. Surgery is not recommended for germinomas. Whole- ventricular radiotherapy or chemotherapy combined with reduced dose ventricular radiotherapy is the standard of care.
A 16-year-old male diagnosed with a unilateral vestibular schwannoma presents with upper limb weakness and tremors. He reports his symptoms started three months ago and have gradually increased in intensity. Computed tomography (CT) of the brain with contrast shows a hyperdense 3x2 cm circumscribed dural based lesion located at the left sphenoid wing. Which is the most common neurocutaneous syndrome associated with this type of lesion?
1. Neurofibromatosis type II
2. Neurofibromatosis type I
3. Ataxia telangiectasia
4. Sturge weber syndrome
1. Neurofibromatosis type II
- Neurofibromatosis type II is one of the most common neurocutaneous syndromes that is usually associated with multiple meningiomas and vestibular schwannoma.
- Unlike neurofibromatosis type II, meningiomas are uncommon in patients with neurofibromatosis type I. Neurofibromatosis type I is frequently associated with optic gliomas, neurofibromas, café- au-lait spots, lisch nodules, and bony lesions.
- Meningiomas are also associated with affected first-degree relatives with meningioma, genetic syndromes such as neurofibromatosis type 2, von Hippel Lindau disease, multiple endocrine neoplasia type 1, Li-Fraumeni, Cowden disease, and Gorlin syndrome.
- Sturge Weber syndrome is a neurocutaneous syndrome that causes leptomeningeal malformations known as angiomas. Patients usually present with a port-wine stain of the face or eyelid and can be associated with intellectual disabilities and seizures.
A 40 years old male complains of severe neck pain and bilateral upper extremity numbness after a car accident 5 hours ago, a flexion-extension mechanism is suspected. His medical history is positive for a rare cervical anomaly called Os Odontoideum. Knowing the physiopathology of this rare condition, which of the following is the primary diagnosis?
1. Subaxial cervical fracture-dislocation
2. C1-C2 instability
3. Atlas fracture (Jefferson fracture)
4. Isolated spinous process fracture
2. C1-C2 instability
- Although cervical fracture-dislocation is a possibility after a car accident, a previous history of Os Odontoideum suggests that the primary diagnosis is cervical instability.
- The patient has a history of Os Odontoideum, and after a cervical trauma experiences symptoms of spinal cord compression, C1-C2 instability must be ruled out. This atlantoaxial instability can lead to cervical spinal stenosis with resultant cervical myelopathy due to vascular compromise, bony compression, and/or stretching of the spinal cord. This instability is almost always anterior; posterior atlantoaxial subluxation has been described; however, it is extremely rare.
- Atlas fracture usually results from axial compression forces; it is not the mechanism described in this case.
- Isolated spinous process fracture has to be considered within the differential diagnosis. However, they are not usually associated with neurological symptoms.
