Section 10 Flashcards
Comprehensive
Which of the following irnroluntary movements is
seen most commonly after injury to the caudate
and putamen?
A. Tumor
B. Hemiballismus
C. Athetosis
D. Dystonia
E. Chorea
E. Chorea
Chorea is a brisk arrhythmic movement associated with Huntington’s disease, rheumatic heart disease, and haloperidol (3Hs). It can be associated with hypotonia and pendular reflexes. Hemiballismus is due to a lesion in the contralateral STN, athetosis in the globus pallidus, and Parkinson’s disease in SN.
lnjury to which part of the cerebellum results in
dysmetria?
A. Flocculonodular lobe
B. Anterior lobe
C. Interposed nuclei (globose and embolifonn)
D. Fastigial nucleus
E. Dentate nucleus
E. Dentate nucleus
The flocculonodular lobe (archicerebellum) is involved with equilibrium and nystagmus. Injury to the fastigial nucleus (vermis) results in trunc.al ataxia (inability to sit, stand, or walk) and scanning speech. Injury to the anterior lobe (paleocerebelIum) disrupts spinocerebellar tracts from muscle
tendons. This connects with the interposed nuclei and functions with muscle tone and posture, thereby resulting in increased extensor tone (in dogs) and gait ataxia (tandem walk-heel-to-toe test) when injured. The dentate nucleus ( neocerebellum) input is corticopontine, it connects with the cerebellar hemispheres (posterior lobe), and injury causes
intention tremor, ipsilateral ipsilateral dysmetria (finger-to-nose test), dysarthria, hypotonia, and poor voluntary movement planning and coordination ( dysdiadokokinesia: inability to perform rapid alternating movements).
Which of the following symptoms is not associated with cluster headache?
A. Female predominance
B. Unilateral
C. Rhinorrhea
D. Sympathetic dysfunction
E. Parasympathetic discharge
A. Female predominance
Unlike most other types of headache, cluster headache has a male predominance.
Besides the ophthalmic arteries, which other
arteries can be thrombosed in temporal arteritis
resulting in blindness and ophthalmoplegia?
A. Superficial temporal
B. Facial
C. Vertebral
D. Posterior ciliary
E. Internal maxillary
D. Posterior ciliary
In temporal arteritis, thrombosis of the posterior ciliary and ophthalmic aa is associated with amaurosis fugax and ophthalmoplegia due to granulomatous inflammation. Giant cells are seen on temporal a biopsy and patients typically present with temporal headaches, jaw pain, and shoulder
pain. Treatment includes corticosteroids until ESR normalizes.
Which of the following cranial n pain syndromes is
associated with syncope and bradycanlia?
A. Trigeminal neuralgia
B. Occipital neuralgia
C. Glossopharyngeal neuralgia
D. Hemifacial spasm
E. Ramsay Hunt syndrome
C. Glossopharyngeal neuralgia
Glossopharyngeal neuralgia is characterized by pain in the tonsillar fossa elicited by swallowing and is associated with bradycardia and syncope due to irritation and stimulation of the nerve of the carotid body and sinus (Hering’s n).
Which of the following anticonvulsants has the
longest half-life?
A. Carbamazepine
B. Phenytoin
C. Levetiracetam
D. Phenobarbital
E. Ethosuximide
D. Phenobarbital
Side effects of phenobarbital can be lethargy and nystagmus. The half-life is 96 hours which is the longest out of all the anticonvulsants. Valproic acid has the shortest half-life of 8 hours.
Which vessel is depicted by the arrow in the following cerebral angiogram?
A. Anterior chornidal a
B. PCOM
C. Persistent trigeminal a
D. Hypoglossal a
E. Proatlantal a
C. Persistent trigeminal a
Persistent trigeminal a (arrow) connects the ICA anteriorly (precavernous or proximal cavernous) to the basilar a posteriorly. It is observed in 0.1-0.5% of cerebral angiograms.
A 39-year-old woman presents to the ER after having a transient episode of aphasia and right arm weakness. She recently had fever, joint pain, muscle aches, fatigue, and a malar rash. Antinuclear and anti-Smith antibodies were positive. Which of the following human leulmcyte antigen (HLA) alleles is associated with this disease?
A. B47
B. B27
C. DQ2
D. DR3
E. DR4
D. DR3
Gilliatt-Sumner hand is seen in neurogenic thoracic outlet syndrome with involvement of the lower trunk of the brachial plexus. There is involvement of the median and ulnar motor with ulnar sensory both clinically and by EMG/NCS. Wartenberg’s, Jeanne’s (hyperextension of the metacarpophalangeal joint with flexion of the interphalangealjoint of the thumb), and Froment’s signs are tests for ulnar nerve weakness whereas Gamekeeper’s is a test for ulnar collateral ligament tears at the thumb.
A 68-year-old man is seen in the clinic with complaints of right-hand weakness. On examination, he has weakness and atrophy of the thenar, hypothenar, and interossei as well as decreased sensation on the ulnar aspect of the hand. Compound muscle action potentials are decreased for the median and ulnar nerves while sensory latencies are prolonged for the ulnar n only. The constellation of clinical features is characteristic of which neurologic pattern?
A. Wartenberg’s sign
B. Gilliatt-Sumner hand
C. Froment’s sign
D. Jeanne’s sign
E. Gamekeeper’s sign
B. Gilliatt-Sumner hand
Gilliatt-Sumner hand is seen in neurogenic thoracic outlet syndrome with involvement of the lower trunk of the brachial plexus. There is involvement of the median and ulnar motor with ulnar sensory both clinically and by EMG/NCS.
Wartenberg’s, Jeanne’s (hyperextension of the metacarpophalangeal joint with flexion of the
interphalangealjoint of the thumb), and Froment’s signs are tests for ulnar nerve weakness whereas Gamekeeper’s is a test for ulnar collateral ligament tears at the thumb.
A 29-year-old woman presents with a 6-month
history of relapsing and remitting difficulties with
gait, muscle weakness, and double vision. On
examination, she has nystagmus, intention tremor, and scanning speech. Which of the following evoked potentials are most useful for diagnosis?
A. Brainstem auditory evoked potentials
B. Somatosensory evoked potentials
C. Visual evoked potentials
D. Motor evoked potentials
E. Laser evoked potentials
C. Visual evoked potentials
Visual evoked potentials are the most useful in the diagnosis of multiple sclerosis; they identify impaired transmission along the optic pathways which is an early finding in MS.
Stimulation of which gaze center results in ipsilateral horizontal conjugate eye movements?
A. Superior colliculus
B. Inferior colliculus
C. Rostral interstitial nucleus (riMLF)
D. Paramedian pontine reticular formation (PPRF)
E. Frontal eye fields
D. Paramedian pontine reticular formation (PPRF)
PPRF stimulates the ipsilateral VI n and contralateral III n. Stimulation of the frontal eye fields or the superior colliculus results in contralateral horizontal conjugate gaze. The riMLF is the vertical gaze center.
Which of the following eye movements present
rhythmically with the same speed in both directions and are associated with head nodding in infants?
A. See-saw nystagmus
B. Convergence nystagmus
C. Downbeat nystagmus
D. Ocular bobbing
E. Spasmus nutans
E. Spasmus nutans
Spasmus nutans occurs in the first 2 years of life and is characterized by ocular oscillations, head nodding, and abnormal head position. See-saw nystagmus is seen with parasellar lesions, convergence nystagmus is caused by indudes pineal lesions, downbeat nystagmus is caused by cervicomedullary junction tumors, and ocular bobbing is seen with pontine masses.
An 8-year-old boy has been frequently sent to
detention by the teacher because of failure to
answer questions when prompted in class. The
teacher notes the student is unresponsive when
addressed and acts weird when he does respond.
He will often stare blankly into space instead of
paying attention in class. If you suspect a seizure
disorder, which anticonvulsant is the first-line
treatment?
A. Carbamazepine
B. Phenytoin
C. Levetiracetam
D. Phenobarbital
E. Ethosuximide
E. Ethosuximide
Ethosuximide and valproic acid are first-line medications to treat absence seizures.
Which of the following will result in mid-sized
fixed pupils?
A. Third cranial n palsy
B. Pontine lesion
C. Death
D. Atropine toxicity
E. Opiates
C. Death
Brain death is associated with mid-size fixed pupils due to loss of both sympathetic and parasympathetic output to the eye. Atropine and III n palsy result in fixed and dilated pupils, while pontine lesions and opiates result in pinpoint pupils.
A 5-year-old boy is being evaluated for sleepwalking. He frequently wakes up with bruises on his knees and elbows. During which stage of sleep does this typically occur?
A. REM
B. Stage 4 and REM
C. Stage 3 and 4
D. Stage 2
E. Stage 1
C. Stage 3 and 4
Sleepwalking (somnambulism) and night terrors occur during stage 3 or 4 sleep, nightmares occur during REM sleep, and nocturnal epilepsy occurs in stage 4 and REM.
A 65-year-old man undergoes emergent endovascular thrombectomy and thrombolysis for an acute basilar a thrombosis. Postoperatively he was found to have bilateral parieto-occlpital cortical infarcts but malces a good recovery. On examination, he is unable to look voluntarily at a peripheral field while his extraocular muscle function is intact during involuntary gaze. He has difficulty fixating the eyes and is unable to move his hand to a specific object using vision. Which syndrome is he likely to be suffering from?
A. Balint’s syndrome
B. Disconnection syndrome
C. Gerstmann’s syndrome
D. Anton’s syndrome
E. aaude’s syndrome
A. Balint’s syndrome
Balint’s syndrome is a rare manifestation of parietal lobe lesions. It manifests with optic ataxia (inability to move the hand toward an object that is seen), oculomotor apraxia (difficulty fixating the eyes), and simultagnosia (inability to perceive more than one object at a time in the visual field). Disconnection syndrome presents with apraxia of the left hand. Gerstmann’s syndrome is characterized by agraphia, acalculia, finger agnosia, and left-right dissociation. Anton’s syndrome (also called Anton-Babinski) is cortical blindness (visual anosognosia) with denial due to lesions of bilateral occipital lobes. Oaude’s syndrome is oculomotor palsy with contralateral ataxia due to a midbrain lesion.
A 64-year-old female presents with speech difficulty and memory problems. She has a history of anal squamous cell carcinoma. She does not smoke and does not drink alcohol. On examination, she has expressive aphasia. MRI is shown below. Left temporal open biopsy was performed, the histology is shown below. What is the diagnosis?
A. Toxoplasmosis
B. Lymphoma
C. Gliomatosis cerebri
D. Herpes encephalitis
E. Progressive multifocal leukoencephalopathy
(PML)
B. Lymphoma
Pathology shows loosely arranged B-lymphocytes with prominent nudeoli confirming the diagnosis of lymphoma. Note the multiple periventricular lesions on MRI: left temporal (a) (the one biopsied) and bilateral on coronal (b ).
Which of the following enzymes is responsible for
catalyzing the conversion of L-DOPA to dopamine?
A. Aldehyde dehydrogenase
B. Monoamine oxidase
C. Phenylalanine hydroxylase
D. Tyrosine hydroxylase
E. Tryptophan decarboxylase
E. Tryptophan decarboxylase
Tryptophan decarboxylase (also known as DOPA decarboxylase or aromatic L-aminoacid decarboxylase) converts L-DOPA to dopamine. Tyrosine hydroxylase converts tyrosine to L-DOPA and is the first and rate-limiting step.
A 21-year-old man presents with progressive
myelopathy. MRI of the cervical spine reveals a
ventral cystic intradural extramedullary mass. You
suspect a neurenteric cyst. What is the origin of
this type of lesion?
A. Neural crest
B. Notochord
C. Endoderm
D. Mesoderm
E. Ectoderm
C. Endoderm
Neurenteric cysts are composed of heterotopic endodermal tissue. Histopathology reveals columnar or cuboidal epithelium characteristic of respiratory or gastrointestinal tissue. These are benign lesions.
Which of the following is not characteristic of cisplatin toxicity?
A. Nephrotoxicity
B. Leukoencephalopathy
C. Ototoxicity
D. Visual loss
E. Nausea and Vomiting
B. Leukoencephalopathy
Subacute necrotizing leukoencephalitis (SNLE) is frequently seen in methotrexate toxicity whereas cisplatin affects microtubules and typically involves the peripheral nervous system.
A 5-month old infant presents with an abnormally
shaped head. The parents note he is developing normally and had an uncomplicated delivery.
On examination, he has scaphocephaly (boat-shaped head). Which of the following is associated with the development of secondary sagittal craniosynostosis?
A. Rickets
B. Vitamin A deficiency
C. Hypothyroidism
D. Hyperphosphatemia
E. Hypercortisolemia
A. Rickets
Vitamin D deficiency (rickets), hyperthyroidism, hypophosphatemia, and sickle cell disease are associated with secondary craniosynostosis.
Carotid sinus and body input are transmitted
through which of the following?
A. Scarpa’s ganglion
B. Petrosal ganglion
C. Superior ganglion of cranial n IX
D. Nodosal ganglion
E. jugular ganglion
B. Petrosal ganglion
The petrosal (inferior) ganglion of IX receives taste and carotid sinus and body (Hering’s n) input. The superior ganglion of IX as well as superior (jugular) ganglion of X receive ear sensation. The nodose (inferior) ganglion of X controls taste and visceral sensation. Scarpa’s ganglion encompasses the superior and inferior vestibular ganglia and controls vestibular function with the utride to the superior ganglion and saccule to the inferior ganglion.
Parasympathetic input to the genitals, bowel, and
bladder is transmitted through which nerves?
A. Nervi erigentes
B. Hypogastric plexus
C. Pudendal n
D. Genitofemoral n
E. Iliohypogastric n
A. Nervi erigentes
The pelvic splanchnic nn (nervi erigentes) are the preganglionic parasympathetic fibers from S2, S3, and S4 to the pelvic organs and hindgut. The hypogastric plexus (superior and inferior) provides sympathetic input while the pudendal, genitofemoral, and iliohypogastric nn are somati
Localization of sound is regulated by which of the
following structures?
A. Inferior oolliculus
B. Superior olivary nucleus
C. Lateral lemniscus
D. Medial geniculate body
E. Heschl’s gyrus
B. Superior olivary nucleus
Sound localization is achieved by the superior olivary nucleus where the medial nuclei detect the time lag between the ears while the lateral ones detect the difference in intensity.
A 62-year-old male is brought to the ER for ataxia,
slurred speech, and cognitive changes. The patient has a history of hypertension, type 2 diabetes mellitus, and renal failure. He does not smoke cigarettes, but consumes four alcoholic drinks per week. On examination, he was noticed to have dysarthria, nystagmus, dysmetria, myodonus, and ataxic gait. Diffusion-weighted MRI is shown below. The patient’s mental status continued to deteriorate rapidly and he succumbed to his disease after 5 days. Brain autopsy findings are shown below. What is the most likely diagnosis?
A. lschemic stroke
B. Lymphoma
C. Prion disease
D. Wemicke’s encephalopathy
E. Hypertensive encephalopathy
C. Prion disease
The patient has subacute spongiform encephalopathy (Prion disease, Creutzfeldt-Jakob disease, CJD). fypical clinical findings are rapidly progressive dementia, myoclonus, and ataxia. Diffusion weighted MRI revealed cortical ribboning in the
right temporoparietal cortex (a, b, c) and diffusion restriction in the right corpus striatum (b, c). Involvement of the dorsomedial thalamus and pulvinar can give the hockey-stick sign. Gross autopsy can reveal brain atrophy. Histology shows vacuoles (d) (spongiform changes). Work-up can include EEG, typically showing periodic sharp wave complexes. CSF findings include 14-3-3 protein and RT-QulC.
The following picture was taken intra-operatively
during an endoscopic third ventriculostomy. What
anatomic structure represents the fornix?
A. 1
B. 2
C. 3
D. 4
E. 5
A.1
Fornix (1) forms the superior and anterior border of the foramen of Monro (4). The choroid plexus lies between the thalamus (2) laterally and the septum pellucidum (3) medially. The latter has the septal v coursing anteriorly from the foramen of Monro.
The following picture was taken intra-operatively
during an endoscopic third ventriculostomy. The
location of the third ventriculostomy (arrow) is in the
A. Tuber cinereum
B. Basilar artery
C. Mammillary bodies
D. Infundibular recess
E. Chiasmatic recess
A. Tuber cinereum
The tuber cinereum of the hypothalamus is the site of a third ventriculostomy which lies between the mammillary bodies and the infundibular recess.
A 24-year-old male presents with headache and
diplopia. MRI is shown below. Upon removal of the tumor, the following histology was revealed. What is the diagnosis?
A. SEGA
B. Subependymoma
C. Ependymoma
D. PNET
E. Central neurocytoma
E. Central neurocytoma
The mammillary bodies are identified on either side just posterior to the tuber cinereum which is the site of third ventriculostomy.
A 28-year-old Asian woman presents to the
ER with transient episodes of aphasia. A non-contrast head CT is negative. Based on the following angiogram findings, what associated disease could worsen the patient’s prognosis?
A. Diabetes
B. Hypertension
C. Grave’s disease
D. Polycystic kidney disease
E. Hypercholesterolemia
C. Grave’s disease
Grave’s disease with Moyamoya disease (shown here on
the angiogram} and precipitate cerebral ischemic events. Thyroid hormones may augment vascular sensitivity to sympathetic outflow, induce pathological changes in the arterial walls, and increase cerebral metabolism as well as oxygen consumption.
A 16-year-old girl presents with a two-day history of severe headaches assodatecl with nausea
and vomiting, and now complains of diplopia. The
only medications she takes ls for binb control. MR
venogram is shown below. The most likely associated cranial nerve palsy is
A. Oculomotor n
B. Trigeminal n
C. Abducens n
D. Vagus n
E. Hypoglossal n
C. Abducens n
The patient has sagittal sinus thrombosis with increased venous congestion and increased ICP. The most common cranial neuropathy with increased ICP is the abducens (VI) nerve.
A 3-month-old infant presents with a scalp mass.
On examination, it is red, non-tender, but soft and Huctuant and on palpation there appears to be a
bony defect surrounding this lesion. There Is no
evidence of a dimple or cutaneous tract. An MRI is
performed. What is the diagnosis?
A. Epidermoid cyst
B. Eosinophilic granuloma
C. Dermal sinus tract
D. Sinus pericranii
E. Atretic cephalocele
E. Atretic cephalocele
Atretic cephaloceles are small subcutaneous scalp lesions that consist of dura, fibrous tissue, and dysplastic brain tissue presenting in infants and young children. They are palpable midline soft tissue masses thought to represent involution of a true cephalocele connected to the dura mater via a fibrous stalk. Radiographically there is a CSF tract and vertical falcine vein pointing toward the subcutaneous scalp mass. Sinus pericranii is a venous extracranial lesion that communicates with a dural sinus and gets smaller with erect posture (unless
thrombosed). Eosinophilic granuloma (Langerhans cell histiocytosis) present as lytic skull lesions in children, with more involvement of the inner table (beveled edges). Epidermoid cysts have sclerotic margins on X-ray and restricted diffusion on MRI.
The parents of a 7-year-old girl note the patient
has been increasingly clumsy over the past few
months. She has been running into the furniture
while walking and has multiple bruises on her right side. On examination, she has over 10 pigmented, flat spots on various areas of the skin. In addition to the lesion shown in the MRI. what is an additional major criterion to confirm the diagnosis?
A. Second-degree relative with NFl
B. First-degree relative with NF2
C. One plexiform neurofibroma
D. One Lisch nodule
E. Three cafe-au-lait spots
C. One plexiform neurofibroma
The clinical diagnosis of NF-1 is made if two or more of the following criteria are present: six or more cafe-au-lait spots, axillary or inguinal freckling, two or more neurofibromas or one plexiform neurofibroma, optic nerve glioma (seen on
MRI), two or more iris hamartomas (Lisch nodules), sphenoid dysplasia or long bone abnormalities, and a first-degree relative with NF-1.
A 4-year-old girl presents to the ER after being
involved in a high-speed motor vehicle accident.
She complains of neck pain but is otherwise
newologically intact. The following image was
obtained. which shows a synchondrosis fracture
of the axis. How many primary ossification centers are normally seen in the axis?
A. 1
B. 2
C. 3
D. 4
E. 5
E. 5
There are five primary ossification centers of the axis: two in the vertebral arch, one in the vertebral body, and two along the dens.
