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1
Q

+ romberg means problems in …

A

cerebellar, vestibulopathy, and posterior column

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2
Q

resting tremor in parkinsons is

A

asymetric

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3
Q

resting tremor vs intentional tremor anatomy

A

Resting: substaintia nigra

Intentional: cerebellum

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4
Q

sensory disturbances is likely due to

A

thalamus

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5
Q

what happens to pill rolling tremor during sleep

A

disappears

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6
Q

phenytoin can cause what side effect

A

gaze evoked nystagmus

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7
Q

problem w convergence is due to

A

midbrain

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8
Q

what is the weber test and what does it inidcate

A

Vibration in the middle:

louder in the affected side: conductive hearing loss as outside voice is blocked and you hear lower on affected side

louder in unaffected side: sensisinoral hearing loss due to loss of cochlear nerve

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9
Q

ocular bobbing

A

up and down of eyes even in sleep or open eyes; pons

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10
Q

triad of parkinsonism

A

is asymmetric resting

tremor, rigidity, and bradykinesia.

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11
Q

post viral cases are

A

ADEM or demylenating in nature

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12
Q

Hyperacusis is an indication that

A

the
damage to the facial nerve is close to its origin from the brainstem, because
the nerve to the stapedius muscle is one of the first branches of the facial
nerve. Non functional stapedius muscle means undampened transmission of acustic sound

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13
Q

Inappropriate slack to tympanic membrane means

A

damage to tensor tympani controlled by motor nerve of CN5

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14
Q

atrophy and fasciculation of tongue means

A

denervation of 12th hypoglossal never which provides motor to tongue;
hypersensitivity to acetylcholine acting at the dener-
vated neuromuscular junction;

—brainstem disease, such as stroke or bulbar
amyotrophic lateral sclerosis (ALS), or with transection of the hypoglossal nerve.

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15
Q

caloric testing tests which organ

A

medulla

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16
Q

how does caloric testing normally work

A

Cold: turns off singnal in that ear, acts as if head turns in the opposite direction=> eyes towards stimulated ear but nystagmus away

warm: turns on signal in that ear, acts as if head turns in the stimulated direction=> eyes away from stimulation but nystagmus towards stimulated

Nystagmus: COWS

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17
Q

how does caloric testing normally work

A

Cold: turns off singnal in that ear, acts as if head turns in the opposite direction=> eyes towards stimulated ear but nystagmus away

warm: turns on signal in that ear, acts as if head turns in the stimulated direction=> eyes away from stimulation but nystagmus towards stimulated

Nystagmus: COWS

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18
Q

horner syndrome has

A

ptosis and myosis

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19
Q

horner syndrome, the problem is in..

A

superior cervical ganglion

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20
Q

sympathetic pathway

A

begins in the hypothala-
mus, travels down through the lateral aspect of the brainstem, synapses in the intermediolateral cell column of the spinal cord, exits the spinal cord at
the level of T1, and synapses again in the superior cervical ganglion.

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21
Q

painful horner without anhidrosis after vigorous activity is a sign of what? and why

A

– Aortic dissection
- postganglionic fibers travel along the surface of the common carotid
and internal carotid arteries until branches leave along the ophthalmic artery
to the eye. Fibers of the sympathetic nervous system, which are destined
to serve the sudomotor function of the forehead, travel with the external carotid artery. Thus diseases affecting the internal carotid artery and the overlying sympathetic plexus do not produce anhidrosis, the third ele-
ment of Horner syndrome.

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22
Q

how to distinguish CNS3 pstosis w horner syndrome.

How to distinguish superior cervical ganglion from T1 nerve root lesion

A

Lesions of cranial nerve (CN) III do cause ptosis, but they would also be expected to cause ipsilateral mydriasis, or pupillary enlargement, not miosis. The degree of ptosis is usually much more severe in third nerve palsy than in Horner syndrome; this is because CN III supplies the levator palpebrae, the primary levator of the lid, whereas the sympathetics supply Müller muscle, which plays an accessory role.

The sympathetic pathway does exit the spinal cord at T1, but injury at this location would not cause orbital pain, which is typical of carotid arterial dissection.

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23
Q

most dense on CT

A

bone>blood

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24
Q

hx of bells palsy now treated, pt he has noticed invol-

untary twitching at the left corner of the mouth each time he tries to blink the left eye. why

A

Aberrant regeneration is possible only if the nerve cell bodies survive the injury and produce axons
that find their way to neuromuscular junctions. Fibers intended for the periorbital muscles end up at the perioral muscles, and signals for eye closure induce mouth retraction.

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25
Q

hx of bells palsy now treated, pt he has noticed invol-

untary twitching at the left corner of the mouth each time he tries to blink the left eye. why

A

Aberrant regeneration is possible only if the nerve cell bodies survive the injury and produce axons
that find their way to neuromuscular junctions. Fibers intended for the periorbital muscles end up at the perioral muscles, and signals for eye closure induce mouth retraction.

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26
Q

subarchnoid hemorrage can present w headache and

A

neck stiffness

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27
Q

suspect subarachnoid hemorrage but ct non con is neg. what next

A

LP to rule out SAH

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28
Q

Which of the follow-

ing is the most definitive test for identifying intracranial aneurysms?

A

cerebral angiography

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29
Q

what happens to RBC in traumatic LP

A

RBC numbers decrease from tube 1 to 4. If not, then increased RBC

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30
Q

how to know if wbc is elevated in CSF

A

1 wbc: 500 RBC is appropirate. Any more WBC means infection

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31
Q

site most likely to produce non communicating hydrocephalus is…

A

aqueduct

of Sylvius, which connects the third ventricle with the fourth ventricle.

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32
Q

communicating hydrocephalus vs non comm

A

com: problem is production vs absorption of csf

non com: obstrcution of ventricle

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33
Q

Chiari MF 1 vs 2 vs dandywalker

A

1: tonsilar herniation
2: tonsilar + vermis herniation
dandywalker: enlargement of 4th ventricle w mega cisterna magna

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34
Q

Tumors common in posterior fossa

A

a) Meningiomas
b. Ependymomas
c. Hemangioblastomas
d. Medulloblastomas

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35
Q

tentorium cerebelli is

A

part of the meninges that separates the superior cerebellum from the cerebrum

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36
Q

tentorium cerebelli is the common site for

A

meningiomas

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37
Q

enpendymomas orgin is

A

choroid plexus

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38
Q

astrocytoma orignate in

A

glial cells + healthy brain tissues

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39
Q

distinguish vascular vs calcemic mass on CT

A

Calcemic: hyperdense wo contrast

vascular: hyperdense after contrast

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40
Q

posterior fossa mass without involvement of the eighth CN, seems to arise from bone, the most
probable neoplasm is

A

meningioma

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41
Q

tx for calcified mass in right frontal

A

meningioma=> resection

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42
Q

NF1 vs NF2

1) chromosomal involvement
2) chracteristics

A

1) NF1: deletion of chrm 17
NF2: deletion of long arm of chr 22
2) NF1: cutaneous manifestations
NF2: Meningiomas and bilateral acoustic neuromas occur

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43
Q

eeg in normal relaxed person

A

8-13 hertz aka alpha wave => becomes less obvious w drowsy or when eye opening and concentration on math

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44
Q

how to test wernicke aphasia

A

(impaired naming, com-

prehension, and repetition with fluent speech).

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45
Q

Neglect and left hemiparesis

would be more likely with

A

right brain lesion

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46
Q

Alexia without agraphia is a disconnection syndrome associated with lesions involving

A

left occipital lobe or splenium of corpus colosum

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47
Q

window for tpa

A

3 hours

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48
Q

absence seizure on eeg

A

3-Hz spike-

and-wave pattern

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49
Q

elevated ESR and periungual telangiectasias in pt w proximal weakness probably has …

A

dermatomyositis

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50
Q

muscle biopsies of myopahties

A

polymyositis: extensive necrosis of mus-
cle fiber segments is seen with macrophage and lymphocyte infiltration.

dermatomyositis: perifascicular muscle fiber atrophy, and the inflammatory infiltrate occurs in the perimy-
sial connective tissue rather than throughout the muscle fibers themselves.

Inclusion body myositis: same as polymyositis except that rimmed vacuoles are also seen.

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51
Q

muscle biopsies of myopahties

A

polymyositis: extensive necrosis of mus-
cle fiber segments is seen with macrophage and lymphocyte infiltration.

dermatomyositis: perifascicular muscle fiber atrophy, and the inflammatory infiltrate occurs in the perimy-
sial connective tissue rather than throughout the muscle fibers themselves.

Inclusion body myositis: same as polymyositis except that rimmed vacuoles are also seen.

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52
Q

CSF in seizure is..

A

normal

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53
Q

classic guimbre csf

A

high protein due to albumin with normal everything else

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54
Q

what is elevated in LP of SAH

A

RBC, WBC, opening pressure, xanthocromia (aka yellow)

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55
Q

signs of spinal cord involvment, ataxia, incontience, transient impotence CSF is

A

likely MS showing elevated IgG and oligoclonal bands

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56
Q

obese woman w headache csf

A

elevated opening pressure => pseudotumor cerebri

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57
Q

pure motor function deficit without any sensory involvement is

A

lacunar infarct with involvement of posterior limb of internal capsule being the site of injury

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58
Q

loss of ONLY sensation involving the entire left side of his

body (face, arm, and leg).

A

right posteroventral nucleus of the lateral thalamus

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59
Q

Thalamic pain syndrome

A

During recovery from this thalamic stroke, paradoxical
pain may develop in the area of sensory impairment. This paradoxical pain associated with decreased pain sensitivity is referred to as the thalamic pain
syndrome.

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60
Q

Wallenberg syndrome has

A

ipsilateral ataxia and ipsilateral Horner syndrome. The trigeminal tract damage may produce ipsilateral loss of facial pain and temperature perception and ipsilateral impairment of the corneal reflex.

The lateral spinothalamic damage produces pain and temperature disturbances contralateral to the
injury in the limbs and trunk.

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61
Q

Wallenberg syndrome is due to interurption of which vessel

A

vertebral artery

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62
Q

most common cause of hemosideran deposit

in elderly patients without hypertension.

A

hemosiderin deposit = sign of lobar hemorrage => most common sign is
cerebral B amyloid angiopathy

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63
Q

hoarseness and dysphagia in an pt w vertebral artery dissection is due to…

A

The nucleus ambiguus, located in the ventrolateral medulla, contains the motor neurons that contrib-
ute to the ninth (glossopharyngeal) and tenth (vagus) cranial nerves.

motor neurons of the nucleus ambiguus innervate the striated muscles of the larynx and pharynx + preganglionic parasympa-
thetic supply to thoracic organs, including the esophagus, heart, and lungs.

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64
Q

nucleus solitarius

A

combines afferents from CNs VII, IX, and X responsible for visceral sensation.

Its projections are primarily to parasym-
pathetic and sympathetic preganglionic neurons in the medulla and spinal cord.

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65
Q

headache during sex or defaction

A

aneurysm rupture

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66
Q

lesion not enchanced by contrast on ct

A

bleeding

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67
Q

IV drugs + aortic valve lesion + several hemoragic lesion in the brain

A

endocarditis => septic emboli or mycotic aneurysm (shape) => bleeds a lot so xanthochromic

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68
Q

mycotic aneurysm

A
  • bleeds a lot but not easily detected on imaging

- present in gram pos or neg infection of the brain ie endocarditic septic embolism

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69
Q

todd’s paralysis may last

A

many hours, or even days.

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70
Q

encephalofacial angiomatosis (Sturge-Weber syndrome),is associated w

A

leptomeningeal angiomas and seizures

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71
Q

PICA stroke produces what…

A

a variety

of brainstem and cerebellar signs,

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72
Q

Anterior cerebral artery

stroke would be expected to cause

A

lower extremity weakness and would

not affect vision, although it could result in decreased speech production.

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73
Q

mca stroke

A

contralateral motor weakness, speech, vision (optic radiata loops arounds temporal lobe)

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74
Q

Vertebral or basilar artery occlusion would primarily affect

A

brainstem or cerbellar structure

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75
Q

If either posterior cerebral artery had its blood supply

compromised, there could be

A

visual loss or posterior (fluent) aphasia

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76
Q

Choroidal artery occlusions might produce

A

focal weakness wo speech problems

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77
Q

problems w pupil dilation + oculomotor muscles dysfunction is most likely due to

A

compression by saccualar aneurysm; transfemoral angiogram is diagnositic

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78
Q

Hippel Lindau

A

Hemangioblastomas are vascular tumors seen in association with polycystic disease of the kidney

and telangiectasias of the retina

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79
Q

Charcot Buchard aneurysm ASSOCIATED W

A

chronic hypertension and most commonly appear in perforating

arteries of the brain like lenticulostrial arteries

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80
Q

is especially susceptible to the formation of Charcot-

Bouchard aneurysms.

A

dentate nucleus of cerebellus

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81
Q

61

A

k

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82
Q

TIA or amourosis fugax is due to

A

carotid artery disease occluding central retinal artery

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83
Q

symptomatic carotid stenois by 70%. tx is

A

carotid endarterectomy

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84
Q

Patients with the rare syndrome of
conduction aphasia have problems with repetition that are more obvious
than their problems with comprehension. Their speech usually does not
sound very fluent.

A

conduction aphasia

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85
Q

type of speech loss in brocas aphasia

A

Speech becomes telegraphic (ie, consisting of short phrases
with omission of small connecting words such as articles and conjunctions)

permanent loss of all ability to produce meaning-
ful language is unlikely if the area of infarction is less than a few centimeters across.

The most persistent difficulty usually exhibited by patients with this type of stroke is a permanent loss of syntax.

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86
Q

Mixed transcortical aphasia

A

can repeat but cannot spontaneously speak or comprehension aka broca and wernicke but can repeat

hypotension => watershed areas => affects brocas and wernecke

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87
Q

Anomic aphasia

A

Naming is impaired. Comprehension, repetition, and

fluency are relatively maintained.

–common in patients with diffuse brain dysfunction.

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88
Q

Transcortical motor aphasia

A

similar to Broca aphasia with the exception of preserved repetition.

-poor naming ability and is nonfluent.

Comprehension and repetition are relatively preserved.

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89
Q

Transcortical sensory aphasia

A

similar to Wernicke aphasia with the exception of preserved repetition.
lesion generally occupies the white matter underlying the
cortex of Wernicke area. In most cases, the prognosis for improvement is
better than that for Wernicke aphasia.

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90
Q

1-2 vs 2-3 hertz spike on EEG

A

3 Hz spike-and-wave pattern would confirm absence.

1-2 Hz would be consistent with severe neurological dysfunction and symptomatic generalized epilepsy.

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91
Q

preferred benzo for status

A

1) lorazepam: fast acting and remains in brain for long time

Past: diazapam- fast acting to reach brain, but cleared from brain in 20 min thus would need another medicine after 20 mninutes

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92
Q

side effects of

b. Diazepam
c. Phenobarbital
d. Clonazepam
e. Phenytoin

A

Phenytoin: if infused at greater than 50mg/min, then cardioarrythmia

Fospheytoid: if infused at greater than 150 mg/min then cardioarrythmia; IM possible

phenobarbital: hypotension and resp distress
pam: hypotension and autonomic dysfunction

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93
Q

pt comes to hospital after simple compelx seizure. now normal. What next

A

MRI to figure the cause of seizure.

EEg is useless as already know its seizure and it adds nothing new to information

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94
Q

timeline for alcoholic withdrawl

A

seizures: 1-2 days
delirious tremens: 2-4 days
symtoms resolve within 7-14 days

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95
Q

Landau-Kleffner syndrome

A

associated with loss of

language function and an abnormal EEG during sleep.

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96
Q

Juvenile myoclonic epilepsy

A

benign epilepsy syndrome with onset in late ado-

lescence or early adulthood.

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97
Q

LGS eeg

A

by mental dysfunction, multiple seizure types, and 1-to 2-Hz generalized spike-wave discharges on EEG.

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98
Q

oldfactory aura in seizure - lesion?

A

the mesial temporal lobe, particularly the hippocam-

pus or parahippocampal gyrus.

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99
Q

what is given for seizure prophalaxis in traumatic head? Why?

A

Phenytoin - to prevent post traumatic EARLY seizure. they are not been shown to be helpful against longterm or late seizures. Preventing early seizure decreases mortality. so phenytoin given for short amount of time.

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100
Q

most common complication of temporal lobectomy

A

visual field defect caused by interruption of
fibers from the optic tracts passing over the temporal horn of the lateral
ventricles. Superior quadrantanopsia

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101
Q

history of febrile seizure => now intractable seizure in the last year. MRI shows

A

sclerotic hippocampus aka mesial temporal sclerosis

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102
Q

the most common cause of intractable complex

partial seizures in adults.

A

mesial temporal sclerosis

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103
Q

tx of mesial temporal sclerosis

A

anterior temporal lobe may produce seizure freedom in up to 80% of cases.

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104
Q

jacksonian march

A

1) sequential seizure, the patient develops focal seizure activity that is primarily motor and spreads.
2) starts w hand and then generalizes w loss of consciousness
3) The face may be involved early because the thumb and the mouth are situated near each other on the motor strip of the cerebral cortex.

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105
Q

myoclonic jerks is …

associated w….

A

invountary muscle jerking, no loss of consciousness

benign juvenile myoclonic epilepsy (BJME) when mycolonic jerks occur after waking up

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106
Q

general clonic tonic seizures

A

high risk for a variety of injuries, such as dislocated shoulders, broken bones, and head trauma.

Patients with this type of seizure always lose consciousness during the attack.

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107
Q

—- may

be mistaken for a psychiatric problem,

A

complex partial seizure– esp if uncinate (from temporal lobes uncus) features like unpleasant smell or taste

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108
Q

complications of status

A

include respiratory failure,

aspiration, acidosis, hypotension, rhabdomyolysis, renal failure, and cogni-
tive impairment.

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109
Q

def of status

A

generalized clonic tonic seizure that lasts continuously for 30 minutes or a series of

seizures over a 30-minute period without the patient regaining full con-
sciousness between them.

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110
Q

Epilepsia partialis continua

A

aka focal motor status elipticus
- persistent focal seizures for hours or for months. The
response to therapy is often poor.
- alert, able to follow commands, and has no gaze deviation.

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111
Q

tx for having complex

partial seizures without secondary generalization.

A

levicitaram

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112
Q

tx for seizure localized to temporal lobe

A

levicitaram

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113
Q

west syndrome characterisitics

A

generalized seizure disorder of infants characterized by recurrent spasms,

the EEG pattern of hypsarrhythmia (diffuse, high-voltage, polyspike-
and-slow-wave discharges between spasms and suppression of these bursts

during the spasms.) and retardation.

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114
Q

tx of seizures in someone whose sibling died with a brainstem glioma, and the father
has several large areas of hypopigmented skin in the shape of ash leaves.

The infant had obvious psychomotor retardation even before the appear-
ance of the spasms.

A

its tuberous sclerosis thus ACTH

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115
Q

seizures due to west sydnrome

A

ACTH

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116
Q

tx for absence seizure

A

1) Ethusuximide is first choice
2) If side effect of GI probs in children, seizures not under control on ethusximide, then Divalproex sodium
3) If the absence seizures
are associated with generalized tonic-clonic seizure ====> divalproex sodium

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117
Q

eclampsia seizure

A

that magnesium sulfate (MgSO4)

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118
Q

what is unique about classic migraine

A

visual aura

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119
Q

classic migraine vs bibasilar migrane

A

Similarity: women > men, vision changes, the aura usually resolves within 10-to-30 minutes,
and the headache invariably follows, rather than precedes, the neurological deficits;

DIfference: the character and severity of neurological deficits associated with basilar migraine are distinct. The visual change may evolve to complete blindness. Irritability may develop into psychosis. Rather than
a mild hemiparesis, the patient may have a transient quadriplegia. Stupor, syncope, and even coma may appear and persist for hours.

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120
Q

trigenmnal neuralgia vs atypical facial pain

A

Trigeminal: praoxysmal, lancinating pains

atypical facial pain: constant, deep pain, uni or bilateral,

  • – responds to SSRI indicating may be related to depression
  • –Progressive loss of sensation in the distribution of the fifth cranial nerve (CN) should prompt search for malignancy

BOTH: can be unilateral

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121
Q

trigeminal neuralgia is assocaited w

A
  • multiple sclerosis
  • basilar artery aneurysms, acoustic schwanno-
    mas, and posterior fossa meningiomas => all of which cause injury to 5th nerve compression
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122
Q

Tolosa-Hunt syndrome

A

inflammatory disorder that produces ophthalmoplegia associated with headache and loss of sensation over the forehead.
–pathologic site: superior orbital fissure or the cavernous sinus

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123
Q

prophalactic meds for migraine

A

amitriptyline hydrochloride, propranolol, verapamil, and valproate.

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124
Q

acute tx for migraine

A

Metoclopramide hydrochloride,

sumatriptan, and ergotamine tartrate

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125
Q

presentation of giant cell arteritis aka temporal arteritis

A

persistent fevers and progres-

sive weight loss.

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126
Q

tx for postherpetic neuralgia

A

imipramine hydrochloride,

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127
Q

sudden headache that prompts CT non con and LP is..

A

thunderclap headache

  • —even if ct and LP negative, consider angiogram as changes in aneurysm can cause that type of headache
  • ——classic occurs during sex
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128
Q

MRI and LP in pseudotumor cerebri

A

normal MRI w increased LP opening pressure + papiledema; presents w morning headaches w vision changes and tinnitus

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129
Q

tx for pseudo tumor cerebri

A

include lumbar
puncture, diuretics, ventriculoperitoneal shunting, and optic nerve sheath
fenestration.

130
Q

causes of headache worsened by standing but relieved w laying down

A

Intracranial hypotension due to following causes:
1) LP
2) continued leak of cerebrospinal fluid (CSF) from the subarachnoid space after head trauma, neurosurgery, or even pneumonectomy (thoracoarachnoid fistula)
3) occult pituitary tumor
4)a leak from a dural tear in the spinal root sleeves; traumatic nerve root avulsion
5) systemic illness
such as dehydration, diabetic coma, uremia, or meningoencephalitis.

131
Q

what happens when elbow is injured

A

ulnar nerve runs superficially and is at high risk of damage:

  • –claw hand w failure of last two fingers to extend
  • –lumbricals weakness
132
Q

gunshot wound to upper arm causing partial damage to median nerve may result in what?

A

Even though median nerve ws not directly hit, it can be affected through passing of energy or force
Trauma to nerves in the extremities may give rise to causalgia, a disturbance in sensory perception
characterized by hypesthesia, dysesthesia, and allodynia.

Hypesthesia: decrease in the accurate perception of stimuli.
dysesthesia: persistent discomfort, which in the situation described is likely to be an unremitting burning pain.
Allodynia: perception of pain with the application of
nonpainful stimuli.

133
Q

Acoustic trauma may produce

severe— in persons who have relatively little hearing loss.

A

tinnitus

134
Q

nerve damage in humerus fracture

A
  • musculocutenaous nerve injury
    ——–supplies the
    biceps brachii, brachialis, and coracobrachialis muscles
    ——–Flexion at the elbow with damage to this nerve is most impaired with the forearm supinated.
135
Q

saturday nigth palsy is related to

A

radial injury: at radial groove, wrist drop and impaired extension

136
Q

why is ct bad at dectecting bleeding several days old

A

because within few days, the blood will be broken down into less dense material equal in density as brain so difficult to assess at that point

137
Q

what to do w respiratory setting in ICP

A

hypervenlation

138
Q

head trauma => consciousness in and out => then coma

A

epidural hematoma: middle meningeal artery

139
Q

Although subdural hemato-

mas are often bilateral, epidural hematomas are invariably unilateral.

A

j

140
Q

Chronic subdural hematoma is rel-

atively common in

A

elderly and renal dialysis patients

  • –often times not picked on CT as blood turns isodense after several days but MRI should pick up
  • —should not cause a shift as this is a chronic process and brain has a chance to adjust
141
Q

most commonly involved part of brain in trauma such as car accident

A

temporal lobes and infe-

rior frontal lobes

142
Q

more common effects of TBI occurs due to..

A

1)anosmia –occurs due to Avulsion of olfactory rootlets. Taste sense also affected simultaneously

143
Q

He is awake and alert, has intact cranial nerves (CNs),
and is able to move his shoulders, but he cannot move his arms or legs. He
is flaccid and has a sensory level at C5. Management?

A

spinal cord injury is initially flaccid then takes some time for spasticity to develop

High-dose intravenous
methylprednisolone improves outcomes in SCI

144
Q

On examina-

tion the abductor pollicus brevis is weak bilaterally,. think..

A

carpal tunnel

145
Q

(encephalitis lethargica) aka post viral encephalitis presents w features of

A

features of parknsonism

146
Q

sarcoidosis affects which cranial nerve

A

facial weakness aka facial paresis from CN 7 injury

147
Q

Polio vs guimbre

A

Both: ascending paralysis
Polio: preceded by fever + affects anterior horn of motor neuron + elevated protein and white count on LP
Guimbre: preceded by diarrhea

148
Q

most common causes of fungal meningitis in immunocompromised

A

cryptococcos and india stain

149
Q

subacutely evolving paraparesis due to parasite

A

Schistosoma mansoni: The fluke deposits eggs in the valveless veins of Batson, which drain the intestines and communicate with the drainage from the lumbosacral spinal cord. The patient develops granulomas around the ova that lodge in the spinal cord, and these granulomatous lesions crush the cord.

150
Q

The parasitic brain

lesion most likely to have a large cyst containing numerous daughter cysts
is that associated with

A

Echinococcus granulosa: acquired
by ingesting material contaminated with fecal matter from sheep or dogs.
children more likely than adults to have these hydatic cyts

151
Q

82 yo w bacterial meningitis. what first

A

intravenous ceftriaxone plus ampicillin=> then LP

152
Q

LP of prions disease

A

normal typically: if (14-3-3 proteinase

inhibitor) it is specific to prion but not common

153
Q

recurrent meningitis often occurs in person w

A

csf leak — can have rhinohrea secondary to trauma

154
Q

tb in brain has

A

caseating granuloma that makes them act like mass

155
Q

in HIV pt w potential of bleed, abscess or mass, what is the best first test when someone presents w hemiparesis

A

keep it broad so ct w contrast

156
Q

diagnosis of most common etiologies

of rim-enhancing brain lesions in AIDS patients are

A

Primary CNS lymphoma: PCR EBV of CSF
Toxo: CSF
thus..LP

157
Q

tx of toxoplasmosis

A

combination of sulfadiaz-

ine and pyrimethamine

158
Q

tx for pml

A

HAART is effective against JC virus

159
Q

most common form of acute encephalitis

A

herpes: high lymphocytes w elveated opening pressure

160
Q

Lyme disease affects which neurological problems?

A

facial weakness aka CNS 7

161
Q

why does the mass in cerebellum not cause seziure

A

because you need cerebrocortical (or at least

cerebral) lesion.

162
Q

herpes encephalitis shows what in eeg

A

Bilateral, periodic epileptiform discharges

163
Q

HIV and cyto-

megalovirus infections in the brain characteristically produce what

A

microglial nodules

HIV: the microglial nodules are distributed around blood vessels throughout the brain; microglial nodules associated with syncytial cells in the brain and spinal cord,
CMV: nodules are more char-
acteristically subpial and subependymal.

164
Q

primary amebic meningoencephalitis.

1) caused by
2) introduced to body through

A

1) caused by organisms from the genera Hartmanella or
Acanthamoeba.
2) fresh water swimming: parasites enter the nervous system through: the cribriform plate at the perforations for the olfactory nerves.

165
Q

tx of meningieal involvement of lyme disease

A

high-dose penicillin or ceftriaxone must be given intravenously for 10-to-14 days. Tetracycline qid for 30 days should be used for patients who are
allergic to the intravenous treatments.

166
Q

Abscesses in the brain most often develop from

A

Hematogenous spread of infection

167
Q

known infection + a rim-enhancing lesion in the left
frontal lobe. Which of the following is the most common site for formation
of this type of lesion?

A

Abscess; starts at grey white junction

168
Q

symphylis affects brain in creating

A

A gumma is a largely or entirely

avascular granuloma that grows and masks picture as brain tumor

169
Q

rabies goes and resides in

A

animal saliva

170
Q

cause of malignant external otitis and osteomyelitis of the

base of the skull in HIV pt

A

aspergillus

171
Q

the most common symptom in patients with

brain abscess?

A

headache

172
Q

Which of the following is the most common cause of brain abscess
in patients with AIDS?

A

toxo&raquo_space;> fungal (crypto, aspergillus, etc)

173
Q

General paresis eg due to symphilis

tx

A

early symptoms are a subtle dementia, characterized by memory loss
and impaired reasoning, with later development of dysarthria, myoclonus,
tremor, seizures, and upper motor neuron signs, leading to a bedridden
state. CHRONIC process

penecillin

174
Q

—- of the brain are involved

in general paresis infection.

A

Both the meninges and the parenchyma

175
Q

bacterial cause of absess

A

1 cause is strep; if stab wound or surgery causing abscess then staph most common

176
Q

spongiform disease is transmitted through..

A

infected nervous system tissue, including dura mater grafts, and occasionally via growth hormone preparations acquired from cadaver
pituitary glands.

177
Q

listerial meningtis tx

A

ampicillin and gentamicin are recommended therapy.

178
Q

cause of meningtis in 90 yo w pleacytosis, no organism,

A

listeria

179
Q

162,

A

x

180
Q

Biopsy of this lesion reveals oli-
godendrocytes with abnormally large nuclei that contain darkly staining

inclusions. There is extensive demyelination, and there are giant astrocytes
in the lesion.

A

PML w JC virus in the large nuclei

181
Q

tabes dorsalis is associated with

A

neurosyphyllis; exhibit

abnormal (Argyll Robertson) pupils and optic atrophy.

182
Q

SSPE produces a CSF pattern

A

similar to that seen with multiple sclerosis, whose features include an increase in the γ-globulin fraction and the presence of oligoclonal bands.

183
Q

seizure. A neurologist exam-
ining the child discovers chorioretinitis, ataxia, hyperactive reflexes, and

bilateral Babinski signs. With

A

EEG exhibits periodic bursts of high-voltage
slow waves followed by recurrent low-voltage stretches (burst suppression
pattern).

SSPE

184
Q

bartonella in HIV pt causes … MRI shows

A

more virulent encephalitis associated with status epilepticus.
—(bacillary angiomato-
sis).
—-MRI may show a characteristic increased signal intensity in the
pulvinar, suggesting a tropism of the organism or immune response to this
particular structure in the posterior thalamus.

185
Q

Cysticercosis causes…

Brain lesion looks like…

A

Cysticercal infection of
muscles produces a nonspecific myositis. Brain involvement may lead to
seizures. The lesions in the brain may calcify and often appear as multiple small cysts spread throughout the cerebrum.

186
Q

Cysticercosis is produced by

A 
the
larval form (cysticercus) of the pork tapeworm, Taenia solium=> invades gut
187
Q

In adults, the most common primary brain tumor is the

A

gial cell tumor called astrocytoma

188
Q

is the usual

location for brain tumors in children.

A

posterior fossa aka infratentorial : Medulloblastoma, ependymomas, and cerebellar (or brainstem) gliomas

189
Q

left hemisphere mass with an overlying hyperostosis of the skull.

A

meningioma

190
Q

Several different types of germ cell tumors arise from the

tissues in this region,

A

this region is called pineal gland

191
Q

the most common source of meta-

static tumors to the brain in patients without a known primary tumor?

A

lung tumor

192
Q

brain mets usually lie in

A

gray-white matter junction

193
Q

The shortest life expectancy with metastatic disease to the brain will
be found in the patient with which of the following metastatic cancers?

A

Malignant melanoma

194
Q

compliation of colloid cyst

A

hydrocephalus: Colloid cysts may produce

transient or persistent obstruction of the flow of cerebrospinal fluid

195
Q

bitemporal hemianopsia. Which of the following tumors is most
likely responsible for this finding?

A

1) only the temporal quadrants of the field in each eye are affected.
2) Pituitary adenoma => sella turcia presses on optic chaiasm causing the #1 visual defect

196
Q

Which of the following tumor types is common in the brain of
patients with acquired immune deficiency syndrome (AIDS), but otherwise
extremely rare?

A

primary CNS lymphoma

197
Q

delayed girl has precocious puberty or excess growth harmone
and poorly controlled seizures. Her seizures are typically preceded by
episodes of uncontrollable laughter. Which of the following mass lesions
might explain her symptoms?

A

hypothalamic hamartomas => non malignant => surgical resection successful

198
Q

an ependymoma. This patient is potentially at risk of dying because of

A

transforminal herniation. Ependymoma is in posterior fossa.

199
Q

A 4-year-old boy presents with ataxia, lethargy, and obstructive
hydrocephalus.

A

Medulloblastomas (lies in posterior fossa) and are one

of the most common CNS tumors of childhood.

200
Q

A 16-year-old boy with café au lait spots and cutaneous nodules has
a gradual decrease of vision in his left eye.

A

optic glioma -NF1

201
Q

A 55-year-old woman presents with mild unsteadiness, tinnitus, and
hearing loss.

A

Schwannomas usually develop
on the vestibular division of CN VIII and are pathologically derived from Schwann cells rather than nerve tissue. Although this is not the division of the nerve that carries information from the cochlea, the cochlear division is crushed as the tumor expands.
NF2

202
Q

Pineocytomas

A

This patient’s symptoms and signs
constitute Parinaud syndrome, which may include loss of vertical gaze, loss of pupillary light reflex, lid retraction, and convergence–retraction nystag-
mus, in which the eyes appear to jerk back into the orbit on attempted upgaze.

203
Q

lesion for pineocytomas

A

dorsal midbrain in the region of the superior colliculus.

204
Q

paraneoplastic cerebellar dysfunction

A

harbor anti-Purkinje cell antibodies (called anti-Yo antibod-
ies), and these are especially commonly found in women with breast cancer

or gynecologic malignancies.

205
Q

70-year-old man with a history of lung cancer develops nausea
and vomiting and then becomes lethargic. On examination, he is lethargic
but arousable, disoriented, and inattentive. He is weak proximally and has
diminished reflexes.

A

hypercalcemia

206
Q

Tay-Sachs disease,- deficiency in ..

A

Hexosaminidase A

207
Q

An 8-month-old boy develops spasticity, head retraction, and diffi-
culty swallowing.
His physician discovers an abnormal accumulation of
glucosylceramide and tells the parents their child will continue to deteriorate and likely die within 3 years. This child has which of the following?

A

β-glucosidase deficiency

208
Q

The cerebrospinal fluid (CSF) protein content of pt w hypertensive stroke is likely to be which of the following?

A

elevatedelevated but less than 100

209
Q

A 42-year-old man presents to the emergency room with seizures,

mental status change, and vision difficulties. A magnetic resonance imag-
ing (MRI) reveals an abnormally high T2 signal in the posterior cerebral

white matter. There is proteinuria, and blood pressure is 210/120 mm Hg.
The cerebrospinal fluid (CSF) protein content of this patient is likely to be
which of the following?

A

elevated but less than 100

210
Q

A patient has had progressive, chronic liver failure for the past 5 years.
At the time of death, he would be expected to exhibit changes in which
type of brain cells?

A

increase in Alzheimer type II astrocytes. These astrocytes are
relatively large cells.

211
Q

An 8-month-old boy develops spasticity, head retraction, and diffi-
culty swallowing. His physician discovers an abnormal accumulation of

glucosylceramide and tells the parents their child will continue to deterio-
rate and likely die within 3 years. This child has which of the following?

A

1) The disease responsible for
the accumulation of glucosylceramide is Gaucher disease.
2) deficient glucocerebrosidase in fibroblasts or leukocytes.

212
Q

the most common neurological

complication of chronic renal failure?

A

Peripheral neuropathy

213
Q

what helps restless leg syndrome

A

clonazepam, gabapentin, L-dopa,

dopamine agonists (eg, pramipexole or ropinirole), and opiates.

214
Q

visual manifestation of B12 def, alcoholism -thimine def, tobacco

A

Centrocecal scotoma: blind spot enlarges

215
Q

hx of alcoholism and benzodiazepine use, including diazepam, loraze-
pam, and clonazepam. Before long surgery, what should have bene given

A

thaimine

216
Q

tx of alcohol and benzo withdrawl with

A

benxo like chlordiazepoxide

217
Q

Her diet is strictly

vegetarian and limited almost entirely to grains, such as corn. what deficiency

A

niacin or tryptophan def => causes pallegra

218
Q

vitamin D def causes what

A
  • rare but usually during early childhood.

- spinocerebellar degeneration (ATAXIA), polyneuropathy, and pigmentary retinopathy.

219
Q

korsikoff can also cause neurolgoic problems like slow mentation

A

k

220
Q

hx of blood transfusions, don’t forget

A

AIDS

221
Q

pickwickian syndrome

A

obesity associated with hypersomnelence in someone with OSA.

222
Q

trauma to brain => pt disoriented after 4 days

A

post ictal —significant trauma can cause seizures esp during sleep

223
Q

Carbon tetrachloride can cause

A

hepatic encephalopathy as it is a hepatic toxin=> high NH3

224
Q

triphasic waves

A

hepatic encepathopathy

225
Q

path of alzhiemers

A

neuronal loss, fibrillary tangles, loss of

synapses, and amyloid (or neuritic) plaque formation in hipocampus

226
Q

eeg on alzhiemers

A

Generalized background slowing

227
Q

disturbance, dementia, and incontinence.

A

normal pressure hydrocephalus

228
Q

complication of VP shunts for NPH

A

Subdural hema-
toma occurs because the reduction in intracranial pressure brought on

by the reduction in CSF volume may cause the brain to pull away from
the covering meninges, stretching and potentially rupturing the bridging
veins.

229
Q

Language testing is most likely to uncover which of the following
deficits in a patient with Alzheimer disease?

A

Transcortical sensory aphasia:which refers to a reduction in the ability to understand complex linguistic structures. Repetition of verbal material is intact.

230
Q

transient global amnesia

A

TGA refers to an episode of
complete and reversible anterograde and retrograde memory loss lasting
up to 24 hours.

231
Q

A 50-year-old woman began having double vision and blurry vision
3 months ago and has since had diminishing interaction with her family,
a paucity of thought and expression, and unsteadiness of gait. Her whole

body appears to jump in the presence of a loud noise. A magnetic reso-
nance imaging (MRI) scan and routine cerebrospinal fluid (CSF) examina-
tion are unremarkable.

A

CJD

232
Q

normal development untile 2 but LOSS OF language and stereotyped movement

A

rhett sydnrome

233
Q

—- is one of the manifestations of neurosyphilis. It is a chronic, often insidious meningoencephalitis that may be delayed up to 20 years after the original spirochetal infection.

A

General paresis

234
Q

t whippei on neurology

A

seizures, myoclonus, ataxia, supra-
nuclear gaze disturbances, hypothalamic dysfunction, and dementia.
Oculomasticatory myorhythmia (pendular convergence movements of the eyes in association with contractions of the masticatory muscles) may occur
and is considered pathognomonic.

235
Q

one hand buttons shirt while other unbuttons is called….

occurs due to lesion in…

this is seen in…

A

alien hand syndrome

parietal lobe

subcortical white matter, particularly in the occipital or parietal regions= > JC virus causing PML

236
Q

dementia, delusions, dysar-

thria, tremor, myoclonus, seizures, spasticity, and Argyll Robertson pupils.

A

general paresis assocaited with neurosyphillis

237
Q

– designates an
involuntary movement disorder that occurs during pregnancy and involves
relatively rapid and fluid, but not rhythmic, limb and trunk movements.

A

chorea gravidarus or estrogen use in non prego

238
Q

A mag-
netic resonance imaging (MRI) indicates atrophy in the head of the caudate

nucleus. This MRI finding would be expected to affect the shape of which
of the following?

A

lateral ventricle rhomboid in shape

239
Q

dopaminergic drugs in huntington causes

A

unmasking of chorea

240
Q

1-methyl-4-phenyl-1,2,3,
6-tetrahydropyridine (MPTP). The neurological syndrome for which he is
at risk is clinically indistinguishable from which of the following?

A

MTP caues degenration of substantia nigra => parkinsonism symptoms

241
Q

what is dysfunctional in parkinson?

A

Brainstem nuclei

242
Q

252

A

ü

243
Q

Postmortem

study of the substantia nigra of a patient with Parkinson disease is likely to
exhibit which of the following?

A

Intracytoplasmic inclusion bodies- w eosinophils

244
Q

Trihexyphenidyl

A

aniticholinergic: it reduces parkinsonism when given antidopa meds like haloperidol

245
Q

L dopa ___ haloperidol induced parkinsonism

A

worsens

246
Q

in parkinsons’s the decrement in

speech that would be expected would result in

A

Progressively inaudible speech: low volume and speed

247
Q

why L dopa but not dopamine as a tx for parkinson

A

Dopamine cannot cross the blood–brain barrier and therefore has no therapeu-
tic effect in the central nervous system (CNS).

248
Q

why add carbidopa to l-dopa

A

L-dopa can be converted to dopamine in variety of tissues peripherally. Dopamine cna’ tcross to brain. Carbidopa inhbits the conversion of L dopa into dopa peripherally.
Once in brain, L dopa converts to dopa.

249
Q

tx for tourette

A

haloperidol

250
Q

After several years of successful antiparkinsonian treatment, a patient
abruptly develops acute episodes of profound bradykinesia and rigidity.

Remission of these signs occurs as abruptly as the onset. Which of the fol-
lowing is the most likely etiology?

A

on-off phenomenon

251
Q

Meige sydnrome

tx

A

blepharospasm, forceful jaw opening,
lip retraction, neck contractions, and tongue thrusting.
—also elicited by phenothiazine or butyrophenone use,

tx: botulinum injectiion

252
Q

He breathes independently and even
swallows food when it is placed in his mouth, but he remains mute. With
painful stimuli, he exhibits semipurposeful withdrawal of his limbs. His
clinical status remains unchanged for several more months.

A

The vegetative state is a clini-

cal condition in which autonomic activity is sustained with little evidence of cognitive function.

253
Q

cause of : Consciousness is preserved in the

locked-in syndrome, but the patient is paralyzed from the eyes down.

A

ischemic or hemorrhagic damage to the pons, such as that occurring with
basilar artery occlusion.

254
Q

Hepatolenticular degeneration

Wilson disease

A
  • Renal tubular acidosis develops along with hepatic fibrosis=> brain and liver disease w progressive dimentia
  • atrophy of putamen and globus pallidus
255
Q

oligoclonal bands are

A
  • limited number of bands of excess immunoglobulin indicates that the
    species of IgG produced by the disease
  • syphilis, Lyme, and sub-
    acute sclerosis panencephalitis may also produce oligoclonal bands.
256
Q

MS neck exam

A

peculiar sensory phenomenon
in which the patient feels an electrical sensation radiating down the spine
when the neck is passively flexed is called Lhermitte sign and is believed to
signify spinal cord disease.

257
Q

Which of the following evoked response

patterns is most often abnormal in patients with early MS?

A

Optic neuritis occurs early
and often in many patients with MS. This involves inflammation and
demyelination of the optic nerve and slows conduction along the optic
nerve.====> slow Visual evoked response (VER)

258
Q

pt on corticosteriod should be given what

A

Ranitidine: Gastric disturbances are a

possible side effect of corticosteroid use.

259
Q 
A papovavirus infection of the central ner-
vous system (CNS) in this person would be most likely to produce which

of the following?

A

also known as jC virus => PML

260
Q

Canavan disease

A

-produce
developmental regression at about 6 months age

  • extensor posturing and rigidity.
  • Myoclonic seizures may develop.
  • Underlying the disease is a defect in N-acetylaspartic acid metabolism.

-Elevated levels of this material can be detected in the blood and urine, but elevated
levels in the brain establish the diagnosis. Changes in brain white matter
are widespread and may result in a spongiform appearance. There is an
increase in brain volume and weight. =>MACROENCEPHALY

261
Q

how does ms affect bladder

A

more spasticity => thus urinary incontinence aka inappropirate emptying of bladder

262
Q

tx of leg spasm in ms

A

Baclofen is an antispasmodic

agent that may be used in MS.

263
Q

what worsens ms symtpoms

A

hot weather

264
Q

young woman with bilateral leg weakness and

numbness, urinary retention, and impaired bowel control.

A

transverse myelitis due to nmo

265
Q

Two weeks after recovering from a febrile illness associated with a
productive cough, a 19-year-old man complains of headache and neck
stiffness. These complaints are associated with fever and are soon followed
by deteriorating cognitive function. He becomes disoriented, lethargic, and
increasingly unresponsive. MRI reveals widespread damage to the white
matter of the cerebral hemispheres.

A

Acute disseminated encephalomyelitis aka post viral autoimmune encephalitis affecting white matter

266
Q

a mutation of mito-

chondrial DNA.

A

A young man presenting
with centrocecal scotoma pattern of visual loss is much more likely to have Leber optic
atrophy or another cause of optic atrophy (eg, tobacco-alcohol amblyopia,
tertiary syphilis, or vitamin deficiencies)

267
Q

ATP-binding transporter in the peroxisomal system

responsible for long-chain fatty acid metabolism.

A

X lined adrenal leukodystrophy
exhibit limb ataxia, nystagmus,
and mental retardation. MRI of their brains reveals areas of abnormal signal
in the white matter. Cerebellar involvement is substantial.

268
Q

A 3-month-old boy exhibits nystagmus and limb tremors unassoci-
ated with seizures. Over the next few years, he develops optic atrophy, choreoathetotic limb movements, seizures, and gait ataxia. He dies dur-
ing status epilepticus and at autopsy is found to have widespread myelin breakdown with myelin preservation in islands about the blood vessels.
The pathologist diagnoses a sudanophilic leukodystrophy to describe
the pattern of staining observed on slides prepared to look for myelin
breakdown products.

A

Pelizaeus-Merzbacher

demyelinating disorder that belongs to a group of degenerative diseases known as sudanophilic leukodystrophies.

269
Q

rapid increase of sodium causes

A

Central pontine myelinolysis. quadriplegic and unre-

sponsive

270
Q

a mutation in the gene that encodes a subunit of the
——, which results in impaired VLDL
formation and consequent decreased vitamin E delivery to the peripheral
and central nervous system.

A

microsomal triglyceride transfer protein => causes ataxia

271
Q

spinal bifida oculta
meningocele
meningomyelocele

A

1) spine protuding out
2) dura protuding out
3) dura+spinal cord protuding out

272
Q

best menagement for hemangioblastoma found in von Hippel-Lindau syndrome

A

surgical resection

273
Q

c1 fused to skull increases risk of

A

chiari malformation

274
Q

second cervical vertebra extends above the

level of the foramen magnum and places the patient at high risk of having

A

brainstem compression

275
Q

agenesis of the corpus callosum causes what on MRI

A

1) Abnormally shaped lateral and third ventricles

agenesis of the corpus callosum,

276
Q

divalproex sodium dur-

ing the first trimester of pregnancy.

A

ntd

277
Q

A 17-month-old boy had developed normally until approximately 13

months of age, when he began having progressive gait problems. On exam-
ination, the patient is spastic, yet nerve conduction studies (NCS) reveal

slowed motor and sensory conduction velocities. Cerebrospinal fluid (CSF)

protein is elevated. MRI reveals white matter abnormalities. Leukocyte test-
ing reveals deficient arylsulfatase A activity. Which of the following tests

may also provide useful diagnostic information in this condition?

A

this is
metach-
romatic leukodystrophy.

DO: nerve biopsy

278
Q

phenylketonuria (PKU) have high levels of

A

phenelalanine

279
Q

fragile x in men vs women

A

Men with the fragile X syn-
drome have hyperextensible joints and prominent thumbs, seizure but carrier women may appear quite normal.
women mostly normal but may have retardation in half of case

280
Q

16 month old; dysarthric and mental functioning decreases, low reflexes, deficiency of arylsulfatase

A

metrachormatic leukodystrophy

281
Q

Hexosaminidase deficiencies

produce

A

Sandhoff and Tay-Sachs diseases.

282
Q

Glucocerebrosidase is deficient in

A

gaucher

283
Q

4-year-old previously healthy girl develops an intermittent red, scaly rash over her face, neck, hands, and legs.
+
devleopmental delay + emotional lability + episodic cerebellar ataxia.

A

Hartnup disease

284
Q

tx of hartnup disease

A

its is inability to absorb tryptophan and other neutral aa so giving the prodruct of trpytophan aka nicotinamide provides relief

285
Q

CT reveals calcifications in the cerebral cortex in a railroad track pattern.

A

Sturge-Weber syndrome

286
Q

association of erythrocytosis with cerebellar signs, microscopic hematuria, and hepatospleno-
megaly suggests

A

von Hippel-Lindau syndrome.
- polycystic liver disease, polycystic kidney disease, reti-
nal angiomas (telangiectasia), and cerebellar tumors.

287
Q

causes of retinitis pigmentosa

A

abetalipoproteinemai, mitochondrial diseases, Bardet-Biedl syndrome, Laurence-
Moon syndrome, Friedreich ataxia, and Refsum disease.

288
Q

features of Abetalipoproteinemia

what helps

A

abnormally shaped
erythrocytes (acanthocytes), low/absent cholesterol and triglyceride, ataxia, reveals posterior column
and spinocerebellar tract degeneration, retinits pigmentosa
—–vitamin E

289
Q

inheritance pattern of tuberous scleorsis

A

autosomal dominant but variable penetrance

290
Q

Congenital hydrocepha-
lus may develop as a consequence of which of the following first-trimester

maternal disorders?

A

A maternal infection with

mumps or rubella virus may produce aqueductal stenosis and, as a con-
sequence, hydrocephalus.

291
Q

uncorrected hydroencephalus leads to

A

macroencephaly

292
Q

type 2 Chiari malformation. Which of

the following defects would this child be likely to have?

A

spina bifida

293
Q

Adenoma sebaceum occurs

in about 90% of patients with

A

tuberous sclerosis.

294
Q

metastatic cncer in von hippau is likely from what organ

A

VHL has high rate of renal cell carcinoma=> causing mets

—the cerebellar hemangioblastomas can bleed but does not have mets

295
Q

eye finding in tuberous sclerosis

A

Retinal phakomas are gliomatous
tumors that require no treatment and are a principal criterion for making the
diagnosis of tuberous sclerosis.

296
Q

Calcifications evident on the skull x-ray or CT scan of a patient with
tuberous sclerosis usually represent

A

subependymal glial nod-
ules that have calcified.
—–not malignant but can obstruct

297
Q

Tay-Sachs disease develop blindness before they
die, with retinal accumulation of gangliosides that produces which of the
following?

A

cherry red spots

298
Q

sign of upper motor neuron disease in child

A

congeni-

tal weakness, hypotonia, and muscle atrophy

299
Q

hyperpigmentation in 9 yo boy could be

A

cafe aulit sign present in NF1>2

300
Q

a cyst occupying 50% of his posterior fossa and incomplete fusion of the cerebel-
lar elements inferiorly. Lifespan?

A

This is Dandy walker malformation => if obstructive hydrocephalus, death soon after birth

If no obstructive hydrocephalus, then pt will be asymtpomatic

301
Q

having hundreds of seizures per day. The clini-
cal manifestations are somewhat subtle and consist of sit-up like movements. Interictal EEG shows multifocal, high-amplitude spikes (hypsarrhythmia) and slowing.

A

Adrenocorticotropic hor-

mone is usually given as a gel intramuscularly to control infantile spasms in children with tuberous sclerosis;

302
Q

Cerebral palsy is a static encephalopathy because

A

the injury to the brain does

not progress.

303
Q

DS brain

A

Smaller than normal for age and body size

304
Q

right parietal parenchymal defect that

is continuous with the ventricle and does not appear to be lined with gray
matter. This type of lesion usually develops as a consequence

A

Vascular or other destructive injuries to the fetal brain

305
Q

fetal alcohol on brain

A

Impaired neuronal migration causing heterotopias (collections of cortical neurons in abnormal locations),

306
Q

type 1 and 2 Chiari malformation usually becomes symptomatic as which of the following in adults?

A

ataxia

307
Q

proximal weakess in cancer pt

A

lung:Les=repetition helps
thymus: mg= weak w repetition
all= dermatomyositis

308
Q

ALS: cough becomes totally ineffective for clearing his

airway- tx?

A

Chest physical therapy

309
Q

A high — in a woman

with male relatives affected by Duchenne dystrophy indicates a high prob-
ability that she is a carrier of the abnormal dystrophin gene.

A

cpk

310
Q

The spontaneous mutation rate for the dystrophin gene is presumed
to be high for which of the following reasons?

A

Men with Duchenne dystrophy do not reproduce.

311
Q

intellectual in dystrophy

A

Slightly impaired. Beckers better intelllectual than duchenne

312
Q

pseudohypertorphy in dystrophy occurs in

A

limited to calves

313
Q

exhibit problems with relaxing their grip, hypersom-

nolence, premature baldness, testicular atrophy, and cataracts.

A

myotonic dystrophy

314
Q

myotonic dystrophy eeg

A

Repetitive discharges with minor stimulation

315
Q

EMG of anterior horn damage

A

abnormal fibrillation: conduction time normal even with extensive motor neuron disease, but the pattern of spon-
taneous and evoked muscle potentials would be abnormal.

316
Q

The pathologist reports that there

are numerous abnormally small muscle fibers intermingled with hypertro-
phied muscle fibers. THis is due to

A

denervation atrophy: some groups supplied by certain nerves atropy due to denervation while other still innervated muscles do not atrophy

317
Q

Which of the following is the

most common manifestation of muscle weakness with myasthenia gravis?

A

ocular weakness

318
Q

shortest life expectancy is associated with which clinical sign in
amyotrophic lateral sclerosis?

A

Fasciculations in the tongue—bulbar involvement is poor prognosis

319
Q

alcohol affects what of cerebellum

A

superior vermis

320
Q

Triorthocresyl phos-
phate is an organophosphate that may cause lethal neurological complica-
tions by which of the following means?

A

Causing a severe motor polyneuropathy

321
Q

lead effect in brain

A

small: ataxia and tremor
large: edema=>herniation and encephalopahty

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