Neurology Flashcards

1
Q

You are evaluating a 56-year-old woman who had the onset of midthoracic back pain which has progressed to quadriparesis over the last several days. She has also noted the onset of bilateral severe eye pain and is losing vision in the left eye based on your visual acuity exam. Her imaging is demonstrated below. The underlying pathophysiology of this condition is thought to arise due to autoantibodies against what
A. T lymphocytes
B. Aquaporin channel
C. Myelin
D. Presynaptic calcium channel
E. Postsynaptic acetylcholine channel

A

A. T lymphocytes
B. Aquaporin channel
C. Myelin
D. Presynaptic calcium channel
E. Postsynaptic acetylcholine channel

This patient has neuromyelitis optica (Devic’s disease) which is a variant of multiple scleorosis that involves the optic nerves and often presents with longitudinal spinal cord T2 signal change that spans three levels (compared with transverse myelitis which does not span that many segments). Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 110.

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2
Q

You are evaluating a 33-year-old woman with AIDS who has the following imaging. She has headaches, mild vision loss, and ataxia. What is the underlying cause of this patient’s condition?
A. BK virus infection
B. Amoeba infection
C. HIV virus infection
D. JC virus infection
E. Toxoplasmosis

A

A. BK virus infection
B. Amoeba infection
C. HIV virus infection
D. JC virus infection
E. Toxoplasmosis

This patient has classic signs and symptoms of progressive multifocal leukoencephalopathy, caused by JC virus infection that destroys oligodendrocytes in patients with AIDS. It often presents as an asymmetric, parieto-occipital area of demyelination. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 259. Forsting, Jansen. MR Neuroimaging: Brain, Spine, Peripheral Nerves, 2017, page 184.

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3
Q

Ciliary paralysis is seen in what condition listed below?
A. Myasthenia gravis
B. Botulism
C. AIDP
D. CIDP
E. PML

A

A. Myasthenia gravis
B. Botulism
C. AIDP
D. CIDP
E. PML

Both myasthenia gravis and botulism can affect
extraocular muscles but the pupils are spared in
myasthenia and involved in botulism.
Further Reading: Borsody. Comprehensive Board
Review in Neurology, 2007, page 235.

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4
Q

You are evaluating a patient in the emergency department that has an interesting neurologic finding on examination. When you ask the patient to look up, his eyes converge and retract in a bilateral jerk-movement fashion. The lesion is most likely located where?
A. Ventral midbrain
B. Dorsal pons
C. Dorsal midbrain
D. Ventral pons
E. Hypothalamus

A

A. Ventral midbrain
B. Dorsal pons
C. Dorsal midbrain
D. Ventral pons
E. Hypothalamus

This patient has convergence-retraction nystagmus, which can be a form of Perinaud’s syndrome, caused by compression or destruction of dorsal midbrain nuclei. Further Reading: Alberstone, Benzel, Najm, Steinmetz. Anatomic Basis of Neurologic Diagnosis, 2009, page 453

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5
Q

In a patient with a hypertensive hemorrhage of the pons, what exam finding would you expect?
A. Mydriasis
B. Bilateral third nerve palsy
C. Productive aphasia
D. Miosis
E. Expressive aphasia

A

A. Mydriasis
B. Bilateral third nerve palsy
C. Productive aphasia
D. Miosis
E. Expressive aphasia

Pontine hemorrhage leads to bilateral pinpoint pupils. This occurs because the descending sympathetic tracts are disrupted while the parasympathetic tracts to the pupil remain intact. Further Reading: Alberstone, Benzel, Najm, Steinmetz. Anatomic Basis of Neurologic Diagnosis, 2009, pages 502–503.

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6
Q

What neuron must be intact for amphetamine (Paredrine) to affect pupillary size?
A. First order neuron
B. Second order neuron
C. Third order neuron
D. Fourth order neuron
E. Fifth order neuron

A

. First order neuron
B. Second order neuron
C. Third order neuron
D. Fourth order neuron
E. Fifth order neuron

The third order neuron involved in pupillary dilation must be intact for Paredrine to cause dilation of the pupil. Further Reading: Laws, Sheehan. Sellar and Parasellar Tumors, 2012, page 99.

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7
Q

Friedrich’s ataxia is inherited in what fashion?
A. Autosomal recessive
B. Autosomal dominant
C. X-linked recessive
D. Sporadic
E. Autosomal dominant with incomplete
penetrance

A

A. Autosomal recessive
B. Autosomal dominant
C. X-linked recessive
D. Sporadic
E. Autosomal dominant with incomplete
penetrance

Friedrich’s ataxia is caused by a mutation in the frataxin gene, and dysfunction causes failure of iron transport into mitochondria. It often involves a trinucleotide repeat and causes degeneration of the dentate nucleus and spinocerebellar tract. It is inherited in an autosomal recessive fashion. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 199

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8
Q

The following MRI finding is often demonstrated in what inherited condition?
A. Neurofibromatosis type I
B. Neurofibromatosis type II
C. Sturge-Weber disease
D. Tuberous sclerosis
E. VHL

A

A. Neurofibromatosis type I
B. Neurofibromatosis type II
C. Sturge-Weber disease
D. Tuberous sclerosis
E. VHL

Sphenoid hypoplasia is often seen in patients with NF1. Further Reading: Harbaugh, Shaffrey, Couldwell, Berger. Neurosurgery Knowledge Update, 2015, page 431

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9
Q

In many patients with HIV that go on to develop intracranial primary lymphoma, what is thought to be the underlying causative mechanism and type of lymphoma?
A. Epstein-Barr virus/T cell type
B. Epstein-Barr virus/B cell type
C. JC virus/T cell type
D. JC virus/B cell type
E. BK virus/B cell type

A

A. Epstein-Barr virus/T cell type
B. Epstein-Barr virus/B cell type
C. JC virus/T cell type
D. JC virus/B cell type
E. BK virus/B cell type

Lymphoma is thought to develop in up to 5% of patients with HIV. It is associated with Epstein-Barr virus. It is a B cell lymphoma. Treatment involves chemotherapy and dexamethasone as well as whole brain radiation. Survival is short, with the median survival being 3 months. Further Reading: Siddiqi. Neurosurgical Intensive Care, 2017, page 376.

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10
Q

What protein is found in Alzheimer’s-associated neurofibrillary tangles?
A. Amyloid
B. Ubiquitin
C. Tau protein
D. Alpha-synuclein
E. APOE e4

A

A. Amyloid
B. Ubiquitin
C. Tau protein
D. Alpha-synuclein
E. APOE e4

Neurofibrillary tangles are found in patients with Alzheimer’s dementia. They are comprised of tau protein. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 156.

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11
Q

In patients with advanced Alzheimer’s dementia, neurofibrillary tangles and plaques seen in what region are associated with the highest grade of dementia?
A. Substantia nigra
B. CA1 hippocampus
C. Ventral medulla
D. Occipital cortex
E. Corpus callosum

A

A. Substantia nigra
B. CA1 hippocampus
C. Ventral medulla
D. Occipital cortex
E. Corpus callosum

Alzheimer’s dementia is graded pathologically, and when neurofibrillary tangles and plaques are found in the occipital cortex, the highest grade (grade IV disease) is diagnosed. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 155.

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12
Q

You are evaluating a 5-year-old boy who has had difficulty with walking since age 3. He has a waddling gait and has difficulty standing due to proximal muscle weakness. A muscle biopsy is demonstrated below. What gene is affected?
A. Emerin/completely absent
B. Emerin/partial dysfunction
C. Dystrophin/completely absent
D. Dystrophin/partial dysfunction
E. Myotonin/completely absent

A

A. Emerin/completely absent
B. Emerin/partial dysfunction
C. Dystrophin/completely absent
D. Dystrophin/partial dysfunction
E. Myotonin/completely absent

This describes a patient with Duchenne’s muscular dystrophy, a rapidly progressive muscular dystrophy causing wasting of proximal muscles. It is caused in many cases by a frameshift mutation which leads to complete absence of the dystrophin gene. Becker’s muscle dystrophy causes partial dysfunction of the dystrophin gene but has similar symptoms to Duchenne’s, except that it progresses in a much slower fashion. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 238

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13
Q

Which autoantibody is found in patients with limbic encephalitis
A. Anti-Hu
B. Anti-Yo
C. Anti-Ri
D. Anti-glutamic acid decarboxylase
E. Anti-Ma

A

A. Anti-Hu
B. Anti-Yo
C. Anti-Ri
D. Anti-glutamic acid decarboxylase
E. Anti-Ma

Patients with limbic encephalitis can be found to have autoantibodies (Anti-Ma). It is important to rule out herpes encephalitis in these patients. Further Reading: Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, page 223

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14
Q

You are asked to evaluate a high school football player on the sideline of his football game where he was hit hard on the helmet and appears confused. The brain dysfunction seen during the acute post-concussive syndrome is thought to arise due to what process?You are asked to evaluate a high school football player on the sideline of his football game where he was hit hard on the helmet and appears confused. The brain dysfunction seen during the acute post-concussive syndrome is thought to arise due to what process?
A. Axon disruption
B. Subclinical seizures
C. ATP pump failure
D. Neurotransmitter depletion
E. Excitatory toxicity

A

A. Axon disruption
B. Subclinical seizures
C. ATP pump failure
D. Neurotransmitter depletion
E. Excitatory toxicity

The cognitive dysfunction that occurs in the postconcussive syndrome is thought to occur due to ATP pump failure at the cellular level. There are multiple cellular events that are also thought to be associated with this condition. Further Reading: Harbaugh, Shaffrey, Couldwell, Berger. Neurosurgery Knowledge Update, 2015, page 757.

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15
Q

What is the best initial management of post-concussive syndrome?
.A. Immediate return to play
B. Cognitive rest
C. Prophylactic antiepileptic medications
D. Opioid pain medications
E. Intensive blood pressure management

A

A. Immediate return to play
B. Cognitive rest
C. Prophylactic antiepileptic medications
D. Opioid pain medications
E. Intensive blood pressure management

Patients who have experienced a concussion should go through a regimen of cognitive and physical rest until they can progress through stages of increased activity without symptoms. Further Reading: Harbaugh, Shaffrey, Couldwell, Berger. Neurosurgery Knowledge Update, 2015, page 759

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16
Q

You are evaluating a pediatric patient who is thought to have Rasmussen’s encephalitis, resulting from chronic encephalitis with spreading cortical inflammation. This results in epilepsy partialis continua. What is a common treatment technique in these patients?
A. VA nucleus DBS
B. Functional hemispherectomy
C. Medical management
D. Vagal nerve stimulator
E. Motor cortex stimulator

A

A. VA nucleus DBS
B. Functional hemispherectomy
C. Medical management
D. Vagal nerve stimulator
E. Motor cortex stimulator

Rasmussen’s encephalitis is a debilitating disease process that causes epilepsy partialis continua in some patients. Prolonged seizures lead to intellectual disability and significant brain dysfunction. Functional hemispherectomy has been utilized to treat this condition. Further Reading: Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015, page 986.

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17
Q

You are rotating in the EEG department and see a patient with the following EEG. What is the best medication for this patient?
A. Valproic acid
B. Carbamazepine
C. Ethosuxamide
D. Levetiracetam
E. Zonisamide

A

A. Valproic acid
B. Carbamazepine
C. Ethosuxamide
D. Levetiracetam
E. Zonisamide

This EEG demonstrates a burst of generalized 3 Hz spike and wave activity associated with absence seizures. These seizures are best treated with ethosuxamide. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 95.

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18
Q

What percentage of patients with an uncomplicated, simple febrile seizure will go on to develop adult epilepsy?
A. < 5%
B. 15%
C. 25%
D. 35%
E. 50%

A

A. < 5%
B. 15%
C. 25%
D. 35%
E. 50%

In patients who experience a simple febrile seizure, very few (< 5%) will go on to develop any ongoing epilepsy after the initial febrile seizure. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 91

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19
Q

The following MRI demonstrates findings associated with what syndrome
A. Hemimegalencephaly
B. Focal cortical dysplasia
C. Joubert syndrome
D. Lhermitte-Duclos syndrome
E. Rhombencephalosynapsis

A

A. Hemimegalencephaly
B. Focal cortical dysplasia
C. Joubert syndrome
D. Lhermitte-Duclos syndrome
E. Rhombencephalosynapsis

This MRI demonstrates the “molar tooth” malformation commonly seen in Joubert syndrome. There is cerebellar peduncle hypoplasia, a small midbrain and a batwing-shaped fourth ventricle. Further Reading: Forsting, Jansen. MR Neuroimaging: Brain, Spine, Peripheral Nerves, 2017, page 316

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20
Q

How do patients with postoperative brachial neuritis (Parsonage-Turner syndrome) present?
A. Pain before weakness
B. Weakness before pain
C. Weakness alone
D. Pain alone
E. Hyperreflexia alone

A

A. Pain before weakness
B. Weakness before pain
C. Weakness alone
D. Pain alone
E. Hyperreflexia alone

Brachial neuritis can occur after viral infection, and sometimes in the postoperative setting. The full pathophysiology is not well understood, but is thought to be an inflammatory reaction in multiple nerve distributions. It presents with severe shoulder pain followed by resolution and then development of motor weakness of the affected extremity. Further Reading: Mackinnon, Yee. Nerve Surgery, 2015, page 403.

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21
Q

In patients with the following finding, what syndrome should you suspect?
A. Sturge-Weber syndrome
B. Neurofibromatosis type I
C. Blue rubber bleb nevus syndrome
D. Ataxia telangiectasia
E. Friedrich’s ataxia

A

A. Sturge-Weber syndrome
B. Neurofibromatosis type I
C. Blue rubber bleb nevus syndrome
D. Ataxia telangiectasia
E. Friedrich’s ataxia

This patient has a persistent connection between the extracranial veins and the superior sagittal sinus, known as sinus pericranii. This is often seen in patients with blue rubber bleb nevus syndrome. Further Reading: Meyers. Differential Diagnosis in Neuroimaging: Head and Neck, 2017, page 43.

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22
Q

You see a patient on rounds that appears to have transverse white lines on her fingernails, also known as Mees’ lines. What toxic exposure are these signs associated with?
A. Lead
B. Arsenic
C. Mercury
D. Strychnine
E. Botulinum toxin

A

A. Lead
B. Arsenic
C. Mercury
D. Strychnine
E. Botulinum toxin

Mees’ transverse white lines on the fingernails are associated with arsenic exposure. Further Reading: Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, page 296.

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23
Q

A patient with a PTEN mutation may be found to have what underlying process?
A. Dysembryoplastic gangliocytoma of the cerebellum
B. Optic glioma
C. Brainstem cavernous malformation
D. Butterfly glioma
E. Multiple meningiomas

A

A. Dysembryoplastic gangliocytoma of the cerebellum

B. Optic glioma
C. Brainstem cavernous malformation
D. Butterfly glioma
E. Multiple meningiomas

Dysembryoplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is a finding associated with PTEN mutations and Cowden’s syndrome. This syndrome is also associated with multiple trichilemmomas, breast, and endometrial carcinoma. Further Reading: Bernstein, Berger. Neuro-Oncology: The Essentials, 3rd edition, 2015, page

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24
Q

Patients with narcolepsy exhibit onset of what sleep stage immediately upon falling asleep?
A. Stage II sleep
B. Stage IV sleep
C. REM sleep
D. Stage I sleep
E. Stage III sleep

A

A. Stage II sleep
B. Stage IV sleep
C. REM sleep
D. Stage I sleep
E. Stage III sleep

Patients with narcolepsy exhibit sleep-onset REM. This is classic for this condition. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 167.

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25
Q

When reading an EEG, what electrode corresponds to the right frontal region?
A. F1
B. F2
C. C3
D. C4
E. O2

A

A. F1
B. F2
C. C3
D. C4
E. O2

EEG electrodes are placed in a standard fashion, with corresponding letters as follows: F = frontal, C = central, P = parietal, O = occipital. Even numbers correspond with the right side of the head, and odd numbers correspond to the left side of the head. Further Reading: Blume WT, Buza RC, Okazaki H. Anatomic correlates of the ten-twenty electrode placement system in infants. Electroencephalogr Clin Neurophysiol 1974; 36(3):303–307. http://faculty.washington.edu/chudler/1020.html

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26
Q

Cheyne-Stokes respirations are thought to arise from destruction of what brain region?
A. Medullary destruction
B. Pontine destruction
C. Bifrontal destruction
D. Bithalamic destruction
E. Pontomedullary destruction

A

A. Medullary destruction
B. Pontine destruction
C. Bifrontal destruction
D. Bithalamic destruction
E. Pontomedullary destruction

While respiratory patterns are difficult and unreliable for diagnosing lesion locations, CheyneStokes respiratory patterns (waxing and waning respiratory patterns) can be seen in patients with bithalamic injury. Further Reading: Rohkamm. Color Atlas of Neurology, 2007, page 118.

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27
Q

Nelson’s syndrome describes what process after bilateral adrenalectomy?
A. Pituitary adenoma enlargement
B. Panhypopituitarism
C. Pituitary apoplexy
D. Spontaneous CSF leak
E. Optic chiasm compression in nonfunctioning
pituitary adenomas

A

A. Pituitary adenoma enlargement
B. Panhypopituitarism
C. Pituitary apoplexy
D. Spontaneous CSF leak
E. Optic chiasm compression in nonfunctioning
pituitary adenomas

Nelson’s syndrome occurs when a patient with a previously unknown ACTH secreting pituitary adenoma undergoes a bilateral adrenalectomy. Loss of feedback inhibition of ACTH production leads to rapid enlargement of the pituitary adenoma. Further Reading: Gasco, Nader. The Essential Neurosurgery Companion, 2013, page 533.

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28
Q

The patient with the findings depicted in this image would have what findings on laboratory evaluation?
A. Low serum ceruloplasmin, high urine
copper
B. High serum ceruloplasmin, high urine
copper
C. Low serum ceruloplasmin, low urine copper
D. High serum ceruloplasmin, low urine
copper

A

A. Low serum ceruloplasmin, high urine
copper

B. High serum ceruloplasmin, high urine
copper
C. Low serum ceruloplasmin, low urine copper
D. High serum ceruloplasmin, low urine
copper

This image demonstrates Kayser-Fleischer rings and confirms the diagnosis of Wilson’s disease. This patient would be expected to have low serum ceruloplasmin and high urinary excretion of copper. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 195

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29
Q

Which vein does not drain di29. You are preparing to perform a C6-7 ACDF on a patient with a single-level traumatic jumped facet. You elect to utilize MEP and SSEP monitoring for the case. Before making incision, the monitoring technician informs you that there is prolonged latency of the ulnar SSEPs at Erb’s point on the right. What is the most likely cause of this change?rectly into the great cerebral vein of Galen?
A. Spinal cord compression
B. Positioning-related brachial plexus
compression
C. Intracranial hemorrhage, parietal cortex
D. Spinal cord vascular compromise
E. Intracranial hemorrhage, thalamus

A

A. Spinal cord compression
B. Positioning-related brachial plexus compression
C. Intracranial hemorrhage, parietal cortex
D. Spinal cord vascular compromise
E. Intracranial hemorrhage, thalamus

Erb’s point is near the shoulder and when sensory latency is prolonged at Erb’s point, a positioning palsy of the brachial plexus should be considered. In this case, with a low ACDF, pulling on the shoulders to achieve a better X-ray line of sight can lead to brachial plexus traction. Further Reading: Newton, O’Brien, Shufflebarger, Betz, Dickson, Harms. Idiopathic Scoliosis, 2011, page 373.

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30
Q

A diabetic third nerve palsy is often?
A. Painful and permanent
B. Painful and temporary
C. Painless and permanent
D. Painless and temporary

A

A. Painful and permanent
B. Painful and temporary
C. Painless and permanent
D. Painless and temporary

Diabetic third nerve palsies are often pupilsparing (center of the nerve is involved rather than the parasympathetic fibers that travel in the peripheral aspect of the nerve). It is painful and temporary. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 43.

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31
Q

You are evaluating a 32-year-old woman who reports ongoing difficulties with severe, burning pain of the right upper extremity. She has no history of trauma to the extremity. It appears red and warm, and she will not let you touch the extremity due to significant allodynia. She has not had good benefit from medical management. What is another potential treatment for her pain?
A. Limb amputation
B. Sensory neurectomy
C. Percutaneous cordotomy
D. Sympathetic blockade
E. Cervical laminectomy

A

A. Limb amputation
B. Sensory neurectomy
C. Percutaneous cordotomy
D. Sympathetic blockade
E. Cervical laminectomy

This patient has complex regional pain syndrome type I (no nerve injury). Often medications are used to treat this condition, but when these fail, sympathetic blockade can be considered. Neurectomy and cordotomy may worsen the condition. Further Reading: Harbaugh, Shaffrey, Couldwell, Berger. Neurosurgery Knowledge Update, 2015, page 740.

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32
Q

You are evaluating a 44-year-old man who developed a sudden headache and speech difficulty. His imaging is demonstrated below. What condition might this patient have?
A. Neurofibromatosis
B. AIDS
C. Homocystinuria
D. Phenylketonuria
E. Blue rubber bleb nevus syndrome

A

A. Neurofibromatosis
B. AIDS
C. Homocystinuria
D. Phenylketonuria
E. Blue rubber bleb nevus syndrome

This patient has a transverse sinus thrombosis which has resulted in a temporal lobe infarction. Patients with homocystinuria can have prothrombotic states that lead to intracranial sinus thrombosis. Further Reading: Kanekar. Imaging of Neurodegenerative Disorders, 2016, page 213.

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33
Q

You have a patient in burst suppression on pentobarbital for elevated ICP. You decide to turn off the pentobarbital now in order to get a neuro exam. Approximately how long will you have to wait for return of neurological function?
A. 5 hours
B. 24 hours
C. 48 hours
D. 72 hours
E. 100+ hours

A

A. 5 hours
B. 24 hours
C. 48 hours
D. 72 hours
E. 100+ hours

Pentobarbital is a long-acting sedative that can be used for refractory ICP elevation. When therapy is ceased, it can take 48 hours for neurologic function to return. Further Reading: Siddiqi. Neurosurgical Intensive Care, 2017, page 162.

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34
Q

You are evaluating the EMG of a patient in whom there is an intact F wave, but the H-reflex is absent. Where is the injury most likely located?
A. Motor endplate
B. Distal motor nerve
C. Dorsal root ganglion
D. Upper cervical spine
E. Anterior horn cells

A

A. Motor endplate
B. Distal motor nerve
C. Dorsal root ganglion
D. Upper cervical spine
E. Anterior horn cells

The H-reflex is used in the S1 nerve and approximates the reflex arc of the spinal cord. Signal is sent through the peripheral sensory nerves and motor response is recorded. The F-wave involves supramaximal stimulation of the peripheral motor nerves and the wave propagates proximally through the nerve root into the spinal canal, also firing several other nerve roots as well in the process. It is a way to determine the integrity of the motor roots. If the H-reflex is absent but the F-wave is normal, the problem is likely to be in the DRG. Further Reading: Fehlings, Boakye, Ditunno, Vaccaro, Rossignol, Burns. Essentials of Spinal Cord Injury, 2013, page 449.

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35
Q

Which of the following findings would help you to determine whether a patient has zoster oticus or Bell’s palsy?
A. Upper facial weakness
B. Lower facial weakness
C. Ear vesicles
D. Facial pain
E. Corneal abrasion

A

A. Upper facial weakness
B. Lower facial weakness
C. Ear vesicles
D. Facial pain
E. Corneal abrasion

Ramsay Hunt syndrome (zoster oticus) can present similar to Bell’s palsy with facial weakness, but attention should be paid to the development of vesicular rashes on the ear, as this leads to the diagnosis of zoster oticus. Further Reading: Di Ieva, Lee, Cusimano. Handbook of Skull Base Surgery, 2016, page 194.

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36
Q

You are asked to see an 86-year-old woman who reports dizziness. She says she hasn’t really had this before the last 2 days and it seemed to start all of a sudden. She has had difficulty standing and walking due to the dizziness. On exam she has spontaneous, direction changing nystagmus and skew deviation. She reports minimal nausea and no vomiting since onset. What is the next best step in management?
A. Scopolamine patch
B. Otolith repositioning
C. Antibiotics
D. Brain MRI
E. Dexamethasone

A

A. Scopolamine patch
B. Otolith repositioning
C. Antibiotics
D. Brain MRI
E. Dexamethasone

This patient has vertigo and there are several signs that would make you think this is central in origin rather than peripheral. She is having difficulty standing and walking, it was a fairly acute onset, there is little nausea, and she has both skew deviation and spontaneous direction changing nystagmus. MRI will likely demonstrate a cerebellar stroke. Further Reading: Adunka, Buchman. Otology, Neurotology, and Lateral Skull Base Surgery: An Illustrated Handbook, 2011, page 70

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37
Q

What structure of the auditory system is most sensitive to high volum
A. Tympanic membrane
B. Inner hair cells
C. Outer hair cells
D. Semicircular canals
E. Spiral ganglion

A

A. Tympanic membrane
B. Inner hair cells
C. Outer hair cells
D. Semicircular canals
E. Spiral ganglion

The inner hair cells of the ear are extremely sensitive to high volume and repeated exposure to high volume can lead to loss of inner hair cells. Further Reading: Greenstein. Greenstein Color Atlas of Neuroscience, 2000, page 258.

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38
Q

Internuclear ophthalmoplegia affects what brainstem tract?
A. Medial longitudinal fasciculus
B. Paramedian pontine reticular formation
C. Corticospinal tract
D. Rubrospinal tract
E. Optic tract

A

A. Medial longitudinal fasciculus
B. Paramedian pontine reticular formation
C. Corticospinal tract
D. Rubrospinal tract
E. Optic tract

Internuclear ophthalmoplegia can be seen in patients with MS. It is caused by disruption of the medial longitudinal fasciculus, which connects the abducens nucleus to the contralateral oculomotor nucleus in order to preserve conjugate eye movements. Further Reading: Alberstone, Benzel, Najm, Steinmetz. Anatomic Basis of Neurologic Diagnosis, 2009, page 225.

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39
Q

Bilateral carpal tunnel syndrome would be classified as what?
A. Polyradiculopathy
B. Mononeuropathy
C. Mononeuropathy multiplex
D. Polyneuropathy
E. Peripheral neuropathy

A

A. Polyradiculopathy
B. Mononeuropathy
C. Mononeuropathy multiplex
D. Polyneuropathy
E. Peripheral neuropathy

Involvement of multiple, distinct nerves is considered a mononeuropathy multiplex. Further Reading: Rohkamm. Color Atlas of Neurology, 2007, Page 316.

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40
Q

What would you expect to see on EMG of a patient with Lambert-Eaton syndrome?
A. Decremental response
B. Incremental response
C. Steady response
D. No response

A

A. Decremental response
B. Incremental response
C. Steady response
D. No response

Lambert-Eaton syndrome involves autoantibodies directed against calcium channels on the presynaptic membrane. This decreases neurotransmitter release due to lack of calcium. EMG will initially be flat, but with repetitive actions there will be an incremental response as calcium levels increase. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 237.

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41
Q

This MRI demonstrates lesions discovered in a 29-year-old man with known AIDS. What is the diagnosis?
A. HIV encephalopathy
B. Staph aureus abscess
C. Listeria abscess
D. Toxoplasmosis
E. PML

A

A. HIV encephalopathy
B. Staph aureus abscess
C. Listeria abscess
D. Toxoplasmosis
E. PML

This MRI demonstrates classic signs of toxoplasmosis. Toxo is the most common mass lesion in patients with known AIDS. Further Reading: Hall, Kim. Neurosurgical Infectious Disease, 2014, page 255.

42
Q

What is the most common neurologic complication in patients with AIDS
A. Toxoplasmosis
B. Primary lymphoma
C. Leukoencephalopathy
D. Bacterial abscess
E. Glioma

A

A. Toxoplasmosis
B. Primary lymphoma
C. Leukoencephalopathy
D. Bacterial abscess
E. Glioma

The most common neurological complication of HIV infection is HIV leukoencephalopathy. Further Reading: Kanekar. Imaging of Neurodegenerative Disorders, 2016, page 22.

43
Q

Charcot joints are thought to be due to what process?
A. Obesity
B. Peripheral neuropathy
C. Complex regional pain syndrome
D. Infection
E. Tumor

A

A. Obesity
B. Peripheral neuropathy
C. Complex regional pain syndrome
D. Infection
E. Tumor

Charcot joints (neuropathic osteoarthropathy) are commonly seen in patients with diabetes who have peripheral neuropathy. The neuropathy leads to destruction of the joint over time. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, Page 293.

44
Q

A patient who demonstrates the opsoclonusmyoclonus reaction (rapid, involuntary conjugate eye movements in multiple directions associated with myoclonic jerks) may have which of the following tumors?
A. Glioblastoma
B. Hemangioblastoma
C. Neuroblastoma
D. Pineoblastoma
E. Choroid plexus carcinoma

A

A. Glioblastoma
B. Hemangioblastoma
C. Neuroblastoma
D. Pineoblastoma
E. Choroid plexus carcinoma

Opsoclonus-myoclonus syndrome is a rare disease seen in some patients with neuroblastoma. It is thought to be mediated by an autoimmune phenomenon. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 136

45
Q

You see a pediatric patient who suffers from intractable epilepsy that manifests as drop attacks. He has had several injuries related to his seizures. What surgical procedure might provide him some relief from his condition?
A. Functional hemispherectomy
B. Corpus callosotomy
C. Temporal lobectomy
D. Selective amygdalohippocampectomy

A

A. Functional hemispherectomy
**B. Corpus callosotomy **
C. Temporal lobectomy
D. Selective amygdalohippocampectomy

Corpus callosotomy is a palliative surgical procedure in patients with intractable drop attacks. It can significantly decrease the frequency of drop attacks in these patients. Further Reading: Harbaugh, Shaffrey, Couldwell, Berger. Neurosurgery Knowledge Update, 2015, page 409

46
Q

You are evaluating a child on the pediatric neurology service that is currently hospitalized for a subdural hematoma. He appears to have kinked hair, and laboratory studies have demonstrated low levels of ceruloplasmin. What is the inheritance pattern of this disorder?
A. X-linked
B. Autosomal dominant
C. Sporadic
D. Autosomal recessive

A

A. X-linked
B. Autosomal dominant
C. Sporadic
D. Autosomal recessive

This child has Menkes kinky hair syndrome which is a deficiency of copper transport and metabolism, causing copper deficiency. It is inherited in an X-linked fashion. Patients develop subdural hematomas and are found to have tortuous vasculature. Further Reading: Choudhri. Pediatric Neuroradiology: Clinical Practice Essentials, 2017, page 121

47
Q

The term “palinopsia” refers to what symptom?
A. Color blindness
B. Inability to recognize faces
C. Burned in images when eyes are closed
D. Cortical blindness
E. Visual field cut

A

A. Color blindness
B. Inability to recognize faces
C. Burned in images when eyes are closed
D. Cortical blindness
E. Visual field cut

Palinopsia refers to visual preservation, or burned in images when the eyes are closed. Further Reading: Tsementzis. Differential Diagnosis in Neurology and Neurosurgery, 2000, page 16

48
Q

The following findings are often seen in patients with what genetic condition?
A. NF1
B. NF2
C. Sturge-Weber syndrome
D. Blue rubber bleb nevus syndrome
E. Tuberous sclerosis

A

A. NF1
B. NF2
C. Sturge-Weber syndrome
D. Blue rubber bleb nevus syndrome
E. Tuberous sclerosis

This image demonstrates ungula fibromas, which can be seen in patients with tuberous sclerosis. Further Reading: Borsody. Comprehensive Board Review in Neurology, 2007, page 277

49
Q

You evaluate a patient with macrocephaly, developmental delay, and seizures. He is an infant. MR imaging demonstrates bifrontal symmetric T2 hyperintensities. There is concern for Alexander disease. If a brain biopsy were to be performed, what would you expect to see on pathology?
A. Hirano bodies
B. Rosenthal fibers
C. Lewy bodies
D. Neurofibrillary tangles
E. Eosinophilic cytoplasmic inclusion bodies

A

A. Hirano bodies
B. Rosenthal fibers
C. Lewy bodies
D. Neurofibrillary tangles
E. Eosinophilic cytoplasmic inclusion bodies

Alexander disease is a leukodystrophy that can cause significant deficits in infants. It involves a defect of GFAP and on pathologic specimen Rosenthal fibers are commonly seen. Further Reading: Forsting, Jansen. MR Neuroimaging: Brain, Spine, Peripheral Nerves, 2017, page 2

50
Q

Which of the following is not a part of Wernicke’s triad?
A. Ataxia
B. Ophthalmoplegia
C. Confusion
D. Aphasia

A

A. Ataxia
B. Ophthalmoplegia
C. Confusion
D. Aphasia

Wernicke’s encephalopathy can occur in patients with severe thiamine deficiency and the classic triad includes ataxia, ophthalmoplegia, and confusion. Aphasia is not a component of the triad. Further Reading: Rohkamm. Color Atlas of Neurology, 2007, page 312.

51
Q

Elevated alpha fetoprotein is a primary marker utilized for diagnosis of a condition that results from what embryologic defect?
A. Gastrulation defect
B. Failure of closure of anterior neuropore
C. Primary neurulation defect
D. Secondary neurulation defect

A

A. Gastrulation defect
B. Failure of closure of anterior neuropore
C. Primary neurulation defect
D. Secondary neurulation defect

Spina bifida is one type of spinal dysraphism that is often discovered on imaging or utilizing serum alpha fetoprotein levels. It is caused by errors in primary neurulation. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 64. Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015.

52
Q

Diastematomyelia results from what embryologic deficit?
A. Gastrulation defect
B. Failure of closure of anterior neuropore
C. Primary neurulation defect
D. Secondary neurulation defect

A

A. Gastrulation defect
B. Failure of closure of anterior neuropore
C. Primary neurulation defect
D. Secondary neurulation defect

Diastematomyelia (split cord malformation) results from an error in gastrulation. There are two subtypes; Type I, when there are two separate dural tubes divided by a fibrous band, and Type II, where there are two hemicords but one continuous dural covering. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 65. Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015.

53
Q

Myelomeningocele is associated with what condition?
A. Arnold-Chiari type I malformation
B. Arnold-Chiari type II malformation
C. Klippel-Feil anomaly
D. Caudal regression syndrome

A

A. Arnold-Chiari type I malformation
B. Arnold-Chiari type II malformation
C. Klippel-Feil anomaly
D. Caudal regression syndrome

Myelomeningocele (herniation of the dura and neural elements through a spinal defect posteriorly) is often associated with Chiari II malformations. Other associations include hydrocephlus, microgyria, basilar invagination, and bending or “beaking” of the brainstem can be seen. Further Reading: Psarros. The Definitive Neurosurgical Board Review, Page 66. Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015.

54
Q

Agenesis of the corpus callosum is associated with trisomy of all chromosomes listed below except?
A. 13
B. 15
C. 18
D. 21

A

A. 13
B. 15
C. 18
D. 21

Agenesis of the corpus callosum is associated with trisomy 13, 15, and 18, but not trisomy 21. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 67. Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015.

55
Q

Premature closure of the metopic suture leads to what named craniosynostosis?
A. Scaphocephaly
B. Oxycephaly
C. Trigonocephaly
D. Posterior plagiocephaly

A

A. Scaphocephaly
B. Oxycephaly
C. Trigonocephaly
D. Posterior plagiocephaly

The metopic suture is located in the midline of the forehead and regresses as normal aging occurs. When craniosynostosis involves the metopic suture, trigonocephaly (wedge-shaped or triangle-shaped head) occurs. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 67. Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015.

56
Q

A 17-year-old adolescent boy is referred to you due to gait imbalance. He has a positive Romberg sign on testing. You also note several cherry red spots on his skin and face. What other manifestation is associated with this autosomal recessive condition?
A. Fused cervical vertebrae
B. Hypotelorism
C. Pes cavus
D. Thymic atrophy

A

A. Fused cervical vertebrae
B. Hypotelorism
C. Pes cavus
D. Thymic atrophy

This patient has ataxia-telangiectasia, an autosomal recessive condition associated with cutaneous telangiectasias and ataxia, dysarthria, and chorea. These patients have difficulty with DNA repair and can be very sensitive to CT scans. They also exhibit atrophy of the thymus and can be susceptible to infections and malignancies (especially lymphoma). Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 72. Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015

57
Q

You are evaluating a 3-week-old infant with marked hypotonia and respiratory problems. If the underlying disease is a lower motor neuron disorder, what is the most likely diagnosis
A. Spinal muscle atrophy type I
B. Spinal muscle atrophy type II
C. Amyotrophic lateral sclerosis
D. Charcot-Marie-Tooth disorder

A

A. Spinal muscle atrophy type I
B. Spinal muscle atrophy type II
C. Amyotrophic lateral sclerosis
D. Charcot-Marie-Tooth disorder

This child has spinal muscle atrophy type I (Werdnig-Hoffman disease), a progressive lower motor neuron disease that leads to advanced hypotonia and muscle atrophy in infants. Ultimately these children die after 4 to 6 weeks of respiratory failure. There are 4 spinal muscle atrophies based on age and symptoms. SMA type I is quickly progressive and presents in infants, while type IV presents in adults and is more slowly progressive. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 72. Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015.

58
Q

You are evaluating a 38-year-old man who has multiple cutaneous telangiectasias of the skin and mucosa, as well as recurrent epistaxis. He has evidence of a brain arteriovenous malformation. This condition runs in his family in an autosomal dominant fashion. What receptor is mutated in this condition?
A. EGFR
B. VEGF
C. TGF-B
D. MuSK

A

A. EGFR
B. VEGF
C. TGF-B
D. MuSK

This patient has hereditary-hemorrhagic telangiectasia, or Osler-Weber-Rendu disease. It is associated with AVMs of the brain and other organs. TGF-B receptors are mutated in this condition. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 74. Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015

59
Q

You have been consulted on a 40-year-old woman who is found to have multiple cutaneous facial nevi and is currently hospitalized for subarachnoid hemorrhage that appears to be coming from an orbital arteriovenous malformation that extends posteriorly along the optic tract. What is the most likely diagnosis?
A. Hereditary hemorrhagic telangiectasia
B. Wyburg-Mason syndrome
C. Sturge-Weber syndrome
D. Klippel-Feil anomaly

A

A. Hereditary hemorrhagic telangiectasia
B. Wyburg-Mason syndrome
C. Sturge-Weber syndrome
D. Klippel-Feil anomaly

This patient has Wyburg-Mason syndrome, a disorder that presents with facial nevi and orbital/optic pathway AVMs. The underlying cause of this disorder is currently unknown. Patients often present with SAH and seizures. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 74. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.

60
Q

You are in Rome on vacation and you overhear a neighboring conversation from a man who is describing a medical illness that his friend is suffering from. Despite his inability to properly pronounce the anatomic structures, it seems his friend is suffering from necrosis and demyelination of the corpus callosum. If his friend consumes large quantities of wine, what is the diagnosis?
A. Hereditary hemorrhagic telangiectasia
B. Wyburg-Mason syndrome
C. Sturge-Weber syndrome
D. Marchiafava-Bignami disease

A

A. Hereditary hemorrhagic telangiectasia
B. Wyburg-Mason syndrome
C. Sturge-Weber syndrome
D. Marchiafava-Bignami disease

This is the classic “Italian-man” disorder originally described in Italian men who consume large quantities of wine. These patients are found to have demyelination and necrosis of the corpus callosum. The onset often leads to initially nonspecific symptoms including depression, memory loss, and confusion. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 77. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.

61
Q

You are evaluating an infant with evidence of dwarfism, coarse facial features, corneal clouding, cardiac disease, and deafness. This condition results from defects in what process?
A. Mucopolysaccharide degradation
B. Oxidation of very long chain fatty acids
C. Deficiency in glycogen storage
D. Mitochondrial DNA abnormalities

A

A. Mucopolysaccharide degradation
B. Oxidation of very long chain fatty acids
C. Deficiency in glycogen storage
D. Mitochondrial DNA abnormalities

These symptoms describe Hurler’s syndrome (as compared to Hunter’s syndrome without corneal clouding), which is caused by a defect in α-L-iduronidase, one of the mucopolysaccharidoses. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 78. Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015.

62
Q

You have been asked to see a 6-year-old boy with mental retardation, spasticity, and evidence that he has been chewing on his fingers. What enzyme deficiency would you expect?
A. Alpha-L-iduronidase
B. Hypoxanthine-guanine-phosphoribosyltransferase
C. Sphingomyelinase
D. Glucocerebrosidase

A

A. Alpha-L-iduronidase
B. Hypoxanthine-guanine-phosphoribosyl transferase
C. Sphingomyelinase
D. Glucocerebrosidase

This child has the classic symptoms of LeschNyhan syndrome, with mental retardation and self-mutilation often presenting with chewing of the fingertips. This is caused by a deficiency in the enzyme HPRT and leading to an increase in levels of uric acid. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 80. Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015

63
Q

Which h63. You are consulted on a patient admitted to the medicine service with the acute onset of seizures, abdominal pain, diarrhea, myalgias, and peripheral neuropathy. The patient has also demonstrated evidence of psychosis. What is the most likely diagnosis?ypothalamic nucleus controls satiety?
A. Halloverden-Spatz disease
B. Acute intermittent porphyria
C. Hurler’s syndrome
D. Wilson’s disease

A

A. Halloverden-Spatz disease
B. Acute intermittent porphyria
C. Hurler’s syndrome
D. Wilson’s disease

This patient has the classic symptoms of acute intermittent porphyria, an unfortunately often tested disorder on the neurosurgery boards. It results from abnormal metabolism of heme, and there is evidence of urinary excretion of aminolevulinic acid, porphobilinogen, and several porphyrins. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 81. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.

64
Q

You are evaluating an 8-year-old girl who exhibits hyperreflexia, stiffness, and frozen facial expressions that make it very difficult for her to eat. MRI is shown below. What is the most likely diagnosis?
A. Halloverden-Spatz disease
B. Acute intermittent porphyria
C. Hurler’s syndrome
D. Wilson’s disease

A

A. Halloverden-Spatz disease
B. Acute intermittent porphyria
C. Hurler’s syndrome
D. Wilson’s disease

This child has symptoms consistent with Halloverden-Spatz disorder, and the MRI confirms the diagnosis. These patients have movement disorders, and MRI demonstrates the classic “eye of the tiger” phenomenon in the globus pallidus bilaterally. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 81. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.

65
Q

You performed an L3-5 laminectomy on a patient 2 years ago that was complicated by a CSF leak. A pseudomeningocele is evident on MR imaging. The patient presents with sensorineural hearing loss and cerebellar ataxia. There is some evidence of myelopathy. MR imaging of the brain demonstrates some GRE signal along the surface of the cerebellum. What is the most likely diagnosis?
A. CSF hypotension
B. Superficial siderosis
C. Arachnoiditis
D. Spinal cord herniation

A

A. CSF hypotension
B. Superficial siderosis
C. Arachnoiditis
D. Spinal cord herniation

This patient has evidence of superficial siderosis, as evident by the clinical symptoms (hearing loss, ataxia) and the imaging findings (GRE) positivity on the surface of the cerebellum). These signs and symptoms, taken with evidence of a chronic CSF leak lead to a diagnosis of superficial siderosis. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 81. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.

66
Q

This syndrome is characterized by uncontrolled seizures, mental retardation, and spike and wave complexes at 1 to 2 Hz on EEG. What is the most likely diagnosis?
A. West syndrome
B. Apert syndrome
C. Pfeiffer syndrome
D. Lennox-Gastaut syndrome

A

A. West syndrome
B. Apert syndrome
C. Pfeiffer syndrome
D. Lennox-Gastaut syndrome

Lennox-Gastaut syndrome is a chronic epilepsy condition that is characterized but multiple, uncontrolled daily seizures, mental retardation, and spike and wave complexes at 1 to 2 Hz on EEG. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 92. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.

67
Q

You are asked to evaluate a 5-month-old infant with a history of congenital CMV infection who has developed flexor/extensor spasms of the head, trunk, and limbs. EEG demonstrates hypsarrhythmia. What is the most likely diagnosis?
A. West syndrome
B. Apert syndrome
C. Pfeiffer syndrome
D. Lennox-Gastaut syndrome

A

A. West syndrome
B. Apert syndrome
C. Pfeiffer syndrome
D. Lennox-Gastaut syndrome

West syndrome, also known as infantile spasms, presents with flexor/extensor spasms that are persistent and demonstrate hypsarrhythmia on EEG (chaotic appearance). West syndrome can present after CNS infections/hypoxic events or be sporadic. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 92. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.

68
Q

This syndrome involves craniosynostosis, deafness, hypertelorism, and dental problems. An image is shown below. What is the most likely diagnosis?
A. West syndrome
B. Apert syndrome
C. Pfeiffer syndrome
D. Lennox-Gastaut syndrome

A

A. West syndrome
B. Apert syndrome
C. Pfeiffer syndrome
D. Lennox-Gastaut syndrome

This patient has Pfeiffer syndrome, characterized by craniosynotosis, deafness, hypertelorism, and dental problems. It is caused by mutations in the FGFR gene and is inherited in an autosomal dominant fashion. Further Reading: Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015, page 252.

69
Q

This syndrome involves craniosynostosis, hypertelorism, dental problems, as well as syndactyly of both hands and feet. An image is shown below. What is the most likely diagnosis?
A. West syndrome
B. Apert syndrome
C. Pfeiffer syndrome
D. Lennox-Gastaut syndrome

A

A. West syndrome
B. Apert syndrome
C. Pfeiffer syndrome
D. Lennox-Gastaut syndrome

This patient has Apert syndrome, characterized by craniosynostosis, hypertelorism, dental problems, and syndactyly of at least 3 fingers on each hand and at least 3 toes on each foot. This is a helpful way to distinguish from Pfeiffer syndrome, which can also have syndactyly, but does not require it on each hand/foot for the diagnosis Further Reading: Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015, page 252.

70
Q

This syndrome involves craniosynostosis, hypertelorism, dental problems, and cleft palate. An image is shown below. What is the most likely diagnosis?
A. West syndrome
B. Apert syndrome
C. Pfeiffer syndrome
D. Crouzon syndrome

A

A. West syndrome
B. Apert syndrome
C. Pfeiffer syndrome
D. Crouzon syndrome

This patient has evidence of Crouzon syndrome, characterized by craniosynostosis, deafness, hypertelorism, and cleft palate. The cleft lip/ palate is characteristic for this syndrome and helps to distinguish it from Apert syndrome and Pfeiffer syndrome. Further Reading: Albright, Pollack, Adelson. Principles and Practice of Pediatric Neurosurgery, 3rd edition, 2015, page 252.

71
Q

Loss of dopaminergic neurons in the substantia nigra pars compacta leads to what manifestations within the basal ganglia?
A. Atrophy of the putamen
B. Decreased activity of the indirect pathway
C. Increased activity of the indirect pathway
D. Increased activity of the direct pathway

A

A. Atrophy of the putamen
B. Decreased activity of the indirect pathway
C. Increased activity of the indirect pathway
D. Increased activity of the direct pathway

Loss of dopaminergic neurons in the SNR leads to Parkinsonism, characterized by 3 to 5 Hz resting tremor, bradykinesia, cog-wheel rigidity, postural imbalance, and masked facies. The loss of dopaminergic input into the striatum leads to overactivity of the indirect pathway and decreased activity of the direct pathway, leading to the classic symptoms. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 68. Borsody. Comprehensive Board Review in Neurology, 2007, movement disorders sectio

72
Q

What process is thought to result in the increase in α-synuclein and ubiquitin in the neurons of Parkinson patients?
A. Mitochondrial dysfunction
B. Loss of dopamine
C. Persistence of the Gs phase in the cell cycle
D. Silent infarcts

A

A. Mitochondrial dysfunction
B. Loss of dopamine
C. Persistence of the Gs phase in the cell cycle
D. Silent infarcts

Mitochondrial dysfunction is thought to play a role in Parkinsonism. Specifically, loss of mitochondrial complex I activity is thought to result in the increased levels of α-synuclein and ubiquitin within neurons. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 68. Borsody. Comprehensive Board Review in Neurology, 2007, movement disorders section.

73
Q

You are evaluating a 62-year-old man who is presenting with unexplained falls, axial and neck rigidity, downward gaze paralysis, masked facies, and dysarthria. L-DOPA has had little effect. What is the most likely diagnosis?
A. Parkinsonism
B. Progressive supranuclear palsy
C. Huntington’s disease
D. Multiple system atrophy

A

A. Parkinsonism
B. Progressive supranuclear palsy
C. Huntington’s disease
D. Multiple system atrophy

This patient has progressive supranuclear palsy, which can present like Parkinson patients and is considered a Parkinson plus disorder. It has the characteristic findings of Parkinsonism, but patients lack the tremor and have downward gaze paralysis. The disease course is rapidly progressive. Further Reading; Psarros. The Definitive Neurosurgical Board Review, page 68. Borsody. Comprehensive Board Review in Neurology, 2007, movement disorders sectio

74
Q

You are evaluating a 62-year-old man who is presenting with unexplained falls, axial and appendicular instability, nystagmus, resting tremor, and rigidity. What is the most likely diagnosis?
A. Huntington’s disease
B. Striatonigral degeneration
C. Shy-Drager syndrome
D. Olivopontocerebellar atrophy

A

A. Huntington’s disease
B. Striatonigral degeneration
C. Shy-Drager syndrome
D. Olivopontocerebellar atrophy

This patient has signs and symptoms of Parkinsonism; however, there are multiple findings concerning for cerebellar involvement. This makes one of the multiple system atrophies more likely, specifically olivopontocerebellar atrophy, which presents predominantly with cerebellar findings. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 68. Borsody. Comprehensive Board Review in Neurology, 2007, movement disorders section.

75
Q

You are evaluating a 75-year-old man presenting with asymmetric and marked dyspraxia of his left arm. He occasionally experiences movements of the arm that he feels he is not in control of. There is also bradykinesia, tremor, and rigidity within the arm. Deep tendon reflexes within the arm are elevated as well. What is the most likely diagnosis?
A. Corticobasal degeneration
B. Striatonigral degeneration
C. Shy-Drager syndrome
D. Olivopontocerebellar atrophy

A

A. Corticobasal degeneration
B. Striatonigral degeneration
C. Shy-Drager syndrome
D. Olivopontocerebellar atrophy

This patient has symptoms consistent with corticobasal degeneration, specifically the involvement of only one limb as well as the “alienlimb” phenomenon. These patients also exhibit Parkinson-like symptoms of the limb and have increased deep tendon reflexes. Dementia is not often a part of this disorder. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 69. Borsody. Comprehensive Board Review in Neurology, 2007, movement disorders section.

76
Q

Huntington’s disease affects what aspect of the basal ganglia primarily?
A. Dopaminergic projections from the substantia
nigra
B. GABA projections from the striatum to the GPe
C. Glutamatergic projections from the striatum
to the GPe
D. GABA projections from the GPI to the thalamus

A

A. Dopaminergic projections from the substantia nigra
B. GABA projections from the striatum to the GPe
C. Glutamatergic projections from the striatum
to the GPe
D. GABA projections from the GPI to the thalamus

Huntington’s disease is known to cause caudate atrophy, but also greatly affects the GABA projections from the striatum to the GPe (indirect pathway). Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 69. Borsody. Comprehensive Board Review in Neurology, 2007, movement disorders section.

77
Q

Hemiballismus is caused by damage or degeneration of what structure?
A. Ipsilateral caudate
B. Contralateral subthalamic nucleus
C. Ipsilateral subthalamic nucleus
D. Contralateral substantia nigra

A

A. Ipsilateral caudate
B. Contralateral subthalamic nucleus
C. Ipsilateral subthalamic nucleus
D. Contralateral substantia nigra

Hemiballisumus (the sudden, brief movement of a limb from the resting position without continued contraction) results from damage or degeneration of the contralalteral subthalamic nucleus. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 69. Borsody. Comprehensive Board Review in Neurology, 2007, movement disorders section.

78
Q

Essential tremor often occurs at what frequency?
A. 2 to 3 Hz
B. 3 to 5 Hz
C. 6 to 8 Hz
D. 8 to 13 Hz

A

A. 2 to 3 Hz
B. 3 to 5 Hz
C. 6 to 8 Hz
D. 8 to 13 Hz

Essential tremor is often seen at a frequency of 6 to 8 Hz. Intention tremor occurs at 2 to 3 Hz, 3 to 5 Hz describes the resting tremor of Parkinsonism, and physiologic or postural tremor occurs at 8 to 13 Hz. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 70. Borsody. Comprehensive Board Review in Neurology, 2007, movement disorders section.

79
Q

Alzheimer’s dementia is associated with what chromosomal disorder?
A. Trisomy 13
B. Trisomy 18
C. Trisomy 21
D. Trisomy X

A

A. Trisomy 13
B. Trisomy 18
C. Trisomy 21
D. Trisomy X

Alzheimer’s dementia is associated with Down’s syndrome (Trisomy 21). Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 70. Borsody. Comprehensive Board Review in Neurology, 2007, behavioral neurology section.

80
Q

This genetic finding is associated with 25 to 40% of all cases of Alzheimer’s disease?
A. Presenilin 1
B. Presenilin 2
C. E4 of apolipoprotein E
D. Amyloid precursor protein

A

A. Presenilin 1
B. Presenilin 2
C. E4 of apolipoprotein E
D. Amyloid precursor protein

The E4 allele of apolipoprotein E has been found to be associated with 25 to 40% of cases of Alzheimer’s disease. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 70. Borsody. Comprehensive Board Review in Neurology, 2007, behavioral neurology section

81
Q

You have been following a patient who has had the onset of personality changes, hyperorality, hypersexuality, and has now developed sucking and rooting reflexes. There are associated speech deficits and urinary incontinence. What is the most likely diagnosis?
A. Alzheimer’s disease
B. Multiple system atrophy
C. Progressive supranuclear palsy
D. Pick’s disease

A

A. Alzheimer’s disease
B. Multiple system atrophy
C. Progressive supranuclear palsy
D. Pick’s disease

Pick’s disease is one of the frontotemporal dementias, characterized by symptoms of frontal and temporal lobe degeneration as well as progressive dementia. This patient has Klüver-Bucy syndrome as well as frontal release signs (sucking and rooting reflexes). This constellation of symptoms leads to the diagnosis of a FTD, in this case Pick’s disease. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 71. Borsody. Comprehensive Board Review in Neurology, 2007, behavioral neurology section

82
Q

You are evaluating a 78-year-old woman who has had the onset of progressive cognitive difficulties, memory problems, urinary incontinence, and recurrent hallucinations. On exam there is mild rigidity present. What is the most likely diagnosis?
A. Normal pressure hydrocephalus
B. Dementia with Lewy bodies
C. Corticobasal degeneration
D. Pick’s disease

A

A. Normal pressure hydrocephalus
B. Dementia with Lewy bodies
C. Corticobasal degeneration
D. Pick’s disease

This patient has symptoms classic for dementia with Lewy bodies. Progressive cognitive difficulties as well as mild Parkinsonism and recurrent hallucinations lead to the diagnosis. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 71. Borsody. Comprehensive Board Review in Neurology, 2007, behavioral neurology se

83
Q

You see a 64-year-old man in your office who has had the onset of progressive cognitive difficulties and memory problems as well as mood changes. He has a long standing history of poorly controlled hypertension. MRI is demonstrated below. What is the most likely diagnosis?
A. Normal pressure hydrocephalus
B. Dementia with Lewy bodies
C. Binswanger’s disease
D. Alzheimer’s disease

A

A. Normal pressure hydrocephalus
B. Dementia with Lewy bodies
C. Binswanger’s disease
D. Alzheimer’s disease

This patient has cognitive decline at a fairly young age in the setting of chronic hypertension and evidence of small vessel white matter ischemic disease on MRI. This can lead to a diagnosis of Binswanger’s disease. Further Reading: Kanekar. Imaging of Neurodegenerative Disorders, 2016, page 3.

84
Q

What dementia classically presents with a stepwise decline in cognitive function
A. Normal pressure hydrocephalus
B. Alzheimer’s disease
C. Binswanger’s disease
D. Multi-infarct dementia

A

A. Normal pressure hydrocephalus
B. Alzheimer’s disease
C. Binswanger’s disease
D. Multi-infarct dementia

Multi-infarct dementia classically presents with stepwise decline in function. It is considered degeneration of cortical structures while Binswanger’s disease is a subcortical infarct-based dementia. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 71. Borsody. Comprehensive Board Review in Neurology, 2007, behavioral neurology section.

85
Q

Carbidopa is administered with L-DOPA in order to decrease the peripheral metabolism of L-DOPA by what enzyme?
A. Tyrosine hydroxylase
B. Catechol-O-methyltransferase
C. Aromatic amino acid decarboxylase
D. Acetylcholinesterase

A

A. Tyrosine hydroxylase
B. Catechol-O-methyltransferase
C. Aromatic amino acid decarboxylase
D. Acetylcholinesterase

Carbidopa is an inhibitor of aromatic amino acid decarboxylase in the periphery in order to decrease degradation of L-DOPA before it reaches the brain in sufficient levels. Carbidopa does not cross the blood brain barrier. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 68. Borsody. Comprehensive Board Review in Neurology, 2007, movement disorders section.

86
Q

You are asked to evaluate a patient who has evidence of peripheral neuropathy, progressive night blindness and hearing loss that seems to improve when phytol is avoided in the diet. What is the most likely diagnosis?
A. Refsum disease
B. Dejerine-Sottas disease
C. Charcot-Marie-Tooth disease
D. Parsonage-Turner syndrome

A

A. Refsum disease
B. Dejerine-Sottas disease
C. Charcot-Marie-Tooth disease
D. Parsonage-Turner syndrome

This patient has Refsum disease, a progressive peripheral neuropathy that is due to increased levels of phytanic acid in tissues. The findings are reversible if patients are able to cut phytol (phytanic acid precursor) from their diets. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 82. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, pathology section.

87
Q

Guillain-Barre syndrome has been shown to be associated with what pathogen?
A. E. Coli
B. Staphylococcus aureus
C. Campylobacter jejuni
D. Klebsiella pneumoniae

A

A. E. Coli
B. Staphylococcus aureus
C. Campylobacter jejuni
D. Klebsiella pneumoniae

Guillain-Barre syndrome can be associated with bacterial infections of the GI tract, specifically campylobacter infections. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 83. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, pathology section.

88
Q

You are evaluating a patient with a progressive peripheral neuropathy causing weakness that started in the legs and has progressed up the trunk to involve the arms. Sensation remains intact. What would you expect to see on analysis of the CSF?
A. High protein, low cellularity
B. High protein, high cellularity
C. Low protein, high cellularity
D. Low protein, low cellularity

A

A. High protein, low cellularity
B. High protein, high cellularity
C. Low protein, high cellularity
D. Low protein, low cellularity

This patient has Guillain-Barre syndrome and the classic findings include albuminocytologic dissociation, leading to high protein and low cellularity within the CSF. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 83. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, pathology section

89
Q

What is the most common major motor nerve affected by diabetic amyotrophy?
A. Sciatic nerve
B. Femoral nerve
C. Tibial nerve
D. Common peroneal nerve

A

A. Sciatic nerve
B. Femoral nerve
C. Tibial nerve
D. Common peroneal nerve

Diabetic amyotrophy usually involves the femoral nerve, and presents with asymmetric muscle weakness, atrophy of iliopsoas, quadriceps, and thigh adductor muscles with burning pain. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 83. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, pathology section

90
Q

What is the most common cause of infectious peripheral neuropathy worldwide?
A. Mycobacterium tuberculosis
B. Mycobacterium leprae
C. Corynebacterium diphtheriae
D. Human immunodeficiency virus

A

A. Mycobacterium tuberculosis
B. Mycobacterium leprae
C. Corynebacterium diphtheriae
D. Human immunodeficiency virus

Several pathogens can cause the development of peripheral neuropathy, but the most common worldwide is Mycobacterium leprae. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 84. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, pathology section

91
Q

You are evaluating a 40-year-old woman who has noticed the onset of proximal leg weakness. On further examination, you note a rash on extensor surfaces, but most notably involving the nose, cheeks, and eyelids. She also has scaly macules on the extensor surfaces of the hand. What is the most likely diagnosis?
A. Polymyositis
B. Dermatomyositis
C. Blue rubber bleb nevus syndrome
D. Hand-foot-mouth disease

A

A. Polymyositis
B. Dermatomyositis
C. Blue rubber bleb nevus syndrome
D. Hand-foot-mouth disease

This patient presents with the classic heliotrope rash of the face, proximal leg weakness (myositis), as well as scaly macules of the hand (Gottran’s sign). These findings lead to the diagnosis of dermatomyositis. Further Reading: Psarros. The Definitive Neurosurgical Board Review, Page 86. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, pathology section.

92
Q

You see a patient with mental retardation, microcephaly, delayed speech, and inappropriate laughter. This named disorder results from what genetic error?
A. Paternal imprinting of chromosome 15
B. Maternal imprinting of chromosome 15
C. Trinucleotide repeat on chromosome 15
D. Trisomy 15

A

A. Paternal imprinting of chromosome 15
B. Maternal imprinting of chromosome 15
C. Trinucleotide repeat on chromosome 15
D. Trisomy 15

This patient has Angelman syndrome, and inappropriate laughter is a helpful key to the diagnosis. This condition results from maternal imprinting on chromosome 15. Prader-Willi syndrome results from paternal imprinting. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 87. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, pathology section.

93
Q

Sleep spindles and K-complexes are seen during what stage of sleep on EEG?
A. Stage 1
B. Stage 2
C. Stage 3
D. Stage 4

A

A. Stage 1
B. Stage 2
C. Stage 3
D. Stage 4

Sleep spindles (12–14 Hz sinusoidal waves) and K-complexes (brief high voltage discharges) are EEG phenomena seen during stage 2 of sleep. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 87. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section

94
Q

Patients with narcolepsy exhibit what diagnostic phenomenon on EEG?
A. Lack of K-complexes
B. Lack of REM sleep
C. Sleep-onset REM
D. Increased theta rhythm

A

A. Lack of K-complexes
B. Lack of REM sleep
C. Sleep-onset REM
D. Increased theta rhythm

Patients with narcolepsy exhibit REM sleep very early during their sleep cycles, the so called “sleep-onset REM” which is diagnostic of the condition. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 88. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.

95
Q

Narcolepsy is thought to arise from abnormalities in what neurotransmitter system?
A. Hypocretin/orexin
B. Serotonin
C. Norepinephrine
D. Histamine

A

A. Hypocretin/orexin
B. Serotonin
C. Norepinephrine
D. Histamine

Narcolepsy is thought to arise from abnormalities within the hypocretin/orexin signaling pathway. These neuropeptides are involved in sleep regulation. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 88. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.

96
Q

You are performing a microvascular decompression on a patient with V3 distribution facial pain. While you are retracting, the monitoring tech alerts you that Wave V of the brainstem auditory-evoked potentials is increasing. Wave V represents what aspect of the auditory pathwa
A. Cochlear nuclei
B. Superior olive
C. Lateral lemniscus
D. Inferior colliculus

A

A. Cochlear nuclei
B. Superior olive
C. Lateral lemniscus
D. Inferior colliculus

BAER monitoring is utilized during many MVD procedures. The waves and corresponding structures are listed as follows: Wave I (auditory nerve), Wave II (cochlear nuclei), Wave III (trapezoid body), Wave IV (lateral lemniscus), Wave V (inferior colliculus), Wave VI (medial geniculate body), and Wave VII (auditory radiations). Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 89. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.

97
Q

You are evaluating a patient admitted to the neuro intensive care unit for respiratory compromise. She has fatigable weakness of the face and ocular muscles and profound neck weakness. This condition is caused by what?
A. Antibodies against presynaptic voltage gated
calcium channels
B. Antibodies against postsynaptic acetylcholine
receptors
C. Antibodies against neuronal sodium channels
D. Exotoxin production inhibiting presynaptic
release of acetylcholine

A

A. Antibodies against presynaptic voltage gated calcium channels
B. Antibodies against postsynaptic acetylcholine receptors
C. Antibodies against neuronal sodium channels
D. Exotoxin production inhibiting presynaptic release of acetylcholine

This patient has symptoms of myasthenia gravis, a condition that arises due to development of antibodies against postsynaptic acetylcholine receptors. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 91. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section. 9

98
Q

You are asked to evaluate a patient currently being worked up for presence of a lung mass. You are consulted because she appears to have proximal muscle weakness early in the morning that seems to improve as the day goes on. This disorder is caused by what?
A. Antibodies against presynaptic voltage gated
calcium channels
B. Antibodies against postsynaptic acetylcholine
receptors
C. Antibodies against neuronal sodium
channels
D. Exotoxin production inhibiting presynaptic
release of acetylcholine

A

A. Antibodies against presynaptic voltage gated calcium channels
B. Antibodies against postsynaptic acetylcholine
receptors
C. Antibodies against neuronal sodium
channels
D. Exotoxin production inhibiting presynaptic release of acetylcholine

This patient has Lambert-Eaton syndrome, caused by antibodies to presynaptic calcium channels. This condition is associated with paraneoplastic disorders, specifically small cell lung cancer. The weakness is overcome with exercise. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 91. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.

99
Q

You are asked to evaluate a patient currently admitted to the neuro intensive care unit for respiratory weakness. She initially presented with nausea, vomiting, constipation, severe ptosis, diplopia, and unreactive pupils. This was followed by progressive weakness of the limbs and face. This condition is caused by what mechanism?
A. Antibodies against presynaptic voltage gated
calcium channels
B. Antibodies against postsynaptic acetylcholine
receptors
C. Antibodies against neuronal sodium channels
D. Exotoxin production inhibiting presynaptic
release of acetylcholine

A

A. Antibodies against presynaptic voltage gated calcium channels
B. Antibodies against postsynaptic acetylcholine receptors
C. Antibodies against neuronal sodium channels
D. Exotoxin production inhibiting presynaptic release of acetylcholine

This patient has botulism, caused by exotoxin release that ultimately inhibits release of acetylcholine at the NMJ. Botulism can cause respiratory weakness similar to myasthenia gravis, but presents with nausea/vomiting, GI symptoms, and bilateral pupillary paralysis. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 91. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section

100
Q

Myasthenia gravis is associated with tumors of what organ?
A. Lung
B. Liver
C. Thymus
D. GI tract

A

A. Lung
B. Liver
C. Thymus
D. GI tract

Around 15% of patients with MG have an encapsulated tumor of the thymus. Further Reading: Psarros. The Definitive Neurosurgical Board Review, page 90. Citow, Macdonald, Refai. Comprehensive Neurosurgery Board Review, 2nd edition, 2010, neurology section.