Boards facts Flashcards

1
Q

Targets for DBS in Parkinsons

A

GPi and STN

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2
Q

Mechanism of dabigatran

A

Direct thrombin inhibitor

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3
Q

Mechanism of rivaroxiban, apixiban

A

Factor Xa inhibitors

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4
Q

Chronic progressive ophthalmoplegia with cardiac involvement

A

Kearns-sayres

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5
Q

PComm aneurysm CN effects

A

CN III lesions

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6
Q

sensory innervation to the shoulder as well as the teres minor and deltoid muscles

A

Axillary nerve (posterior cord, C5-C6)

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7
Q

responsible for stabilizing the scapula against the thorax

A

Serrates anterior (long thoracic nerve, C5,6,7 right off brachial plexus)

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8
Q

responsible for the first 15 degrees of shoulder abduction

A

Supraspinatus (suprascapular nerve, c5,6)

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9
Q

CSF analysis will show an elevated protein, low glucose, and an elevated lymphocytic-predominant white cell count

A

Cryptococcus, CD4<200!

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10
Q

Lasmiditan

A

Migraine abortive that targets 5HT-1F receptors , safe in CAD

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11
Q

Looks like Rathke’s cleft cyst, but calcified

A

Craniopharyngioma

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12
Q

acute denervation to the tibial (gastrocnemius) and deep peroneal (tibialis anterior) innervated muscles

A

Sciatic nerve proximal to knee

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13
Q

The only CMT with axonal (not demylenating) polyneuropathy

A

CMT 2

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14
Q

CIDP, Charcot-Marie-Tooth type 1A, and Refsum disease can have this finding appreciated on a sural nerve biopsy.

A

Onion bulbs

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15
Q

superior gluteal (gluteus medius) tibial (gastrocnemius), and tibialis anterior nerves

A

L5 radiculopathy, the most common radiculopathy

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16
Q

VPL

A

relay center for sensory information of the body

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17
Q

VPM

A

relay center for sensory information of the face as well as taste

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18
Q

superior colliculi, located in the midbrain, actually play a prominent role in

A

Visual system

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19
Q

Auditory pathway

A

Hair cells > cochlear nucleus > trapezoid body > ventral pons > superior olivary complex > inferior colliculi (midbrain) > thalamus

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20
Q

thalamic astasia

A

Unilateral thalami stroke imitating cerebellar stroke (full strength but can’t stand)

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21
Q

Thalamic aphasia

A

presents with a fluency-predominant deficit and less impressive difficulties with comprehension

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22
Q

inferior olivary nucleus in the medulla, the red nucleus in the tegmentum of the midbrain, and the contralateral dentate nucleus in the cerebellum

A

Mollart’s triangle, lesions cause palatial myoclonus

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23
Q

ipsilateral third nerve palsy with contralateral hemiparesis

A

Weber’s sydrome, medial midbrain

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24
Q

Lesion for Alexia without agraphia

A

Splenium of the corpus callous

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25
Q

change in heart rate, blood pressure, respiratory rate during a seizure

A

Increase > R insula, decrease > l insula

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26
Q

Forearm pronation

A

median or radial nerve

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27
Q

Wrist flexion

A

Median nerve

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28
Q

elbow flexion

A

Musculocutaneous nerve

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29
Q

Musculocutaneous nerve and C6-7 portion of median nerve

A

Lateral cord

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30
Q

Radial nerve and axillary nerve

A

Posterior cord C5-C8

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31
Q

Ulnar nerve and C8-T1 portion of median nerve

A

Medial cord

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32
Q

Deltoid abduction plus triceps and wrist extension

A

Axillary AND radial nerves = posterior cord

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33
Q

Scapular winging

A

long thoracic nerve (C5-C7) damage (serratus anterior)

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34
Q

SCM (contralateral head turn) and trapezius (assists deltoid in lifting arm >90 degrees)

A

Spinal accessory nerve

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35
Q

ascending tract that carries information about pain and temperature

A

Spinothalamic (C shaped)

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36
Q

descending tract that controls skeletal muscle motor movement of the contralateral limbs

A

Paternal corticospinal (oval shaped)

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37
Q

Rhomboids

A

Dorsal scapular nerve

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38
Q

pufferfish dishes. Tetrodotoxin mechanism

A

preferentially block sodium channels

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39
Q

Looks like radial neuropathy at the axilla, plus weak deltoid and latissimus dorsi

A

Posterior cord plexopathy

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40
Q

Tongue deviation from a stroke–vascular supply

A

middle medulla is primarily supplied by the anterior spinal artery (ASA)

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41
Q

lacunar infarct to the ventral posterior nuclei

A

Pure sensory stroke

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42
Q

developmental regression, failure to thrive, and ataxia; tiger without eyes

A

Leigh syndrome

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43
Q

Osteolytic lesion in a child

A

Langerhans cell histiocytosis

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44
Q

anti-Hu paraneoplastic syndrome, Sjogren syndrome, pyridoxine toxicity, and exposure to certain chemotherapeutic agents

A

Common causes of sensory neuron-patchy (like isolated loss of proprioception)

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45
Q

type-II topoisomerase inhibitor that disrupts DNA synthesis and repair

A

Mitoxantrone

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46
Q

selectively targets the CD20-positive B-cells causing antibody-dependent cellular cytolysis

A

Ocrelizomab

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47
Q

α4-integrin antagonist that inhibits the transmigration of T-cells across the blood-brain barrier

A

Natalizomab

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48
Q

inhibits pyrimidine de novo synthesis thus inhibiting rapidly dividing cells, including t-cells

A

Teriflunomide

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49
Q

prolonged exposure to colchicine, amiodarone, chloroquine, or hydroxychloroquine

A

Myopathy and neuropathy!

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50
Q

The myelencephalon is responsible for the formation of the

A

Medulla

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51
Q

The telencephalon develops into

A

cerebral hemispheres, the basal ganglia, the hippocampus, and the amygdala

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52
Q

The diencephalon develops into the

A

thalamus, hypothalamus, and retina

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53
Q

The mesencephalon develops into the

A

Midbrain

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54
Q

The metencephalon develops into

A

the pons and cerebellum.

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55
Q

posterior predominant demyelination

A

X-linked luekodystrophy

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56
Q

Diffuse demyelination sparing U fibers

A

Krabbe

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57
Q

Frontal predominant demyelination

A

Alexander’s disease

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58
Q

Diffuse demyelination including U fibers

A

Canavan (Krabbe and metachromatic spare U)

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59
Q

Accumulates in Krabbe

A

galactosylceramide (space crab, U sparing)

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60
Q

Sphingomyelin accumulates in

A

Neimann-Pick disease

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61
Q

Glucosylceramide accumulates in

A

Gaucher disease

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62
Q

Globotriasylceramide accumulates in

A

Fabry disease (global fabric)

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63
Q

café-au-lait spots, Lisch nodules, and axillary freckling

A

NF1

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64
Q

Schwannomas

A

NF2

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65
Q

Ash-leaf spots and facial angiofibromas

A

Tuberous sclerosis

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66
Q

Hemangioblastomas

A

Von-Hippel Landau disease

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67
Q

can cause first-dose bradycardia, atrioventricular block, and QT interval prolongation

A

fingolimod

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68
Q

Looks like ALS but with conduction block

A

multifocal motor neuron (MMN) disease! Treatment is IVIG

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69
Q

deletion, mutation, or inappropriate methylation of the paternal copy of the q11-q13 segment of chromosome 15

A

Prader-Willi

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70
Q

deletion, mutation, or inappropriate methylation of the maternal copy of the q11-q13 segment of chromosome 15

A

Angelman

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71
Q

Infantile spasms and ash leaf spots, dx and tx

A

Tuberous sclerosis, vigabatrin (hipsarthymia, but NOT treated with ACTH)

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72
Q

Lacosamide mechanism

A

Selective enhancement of slow inactivation of SODIUM channels

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73
Q

Topiramate mechanism

A

SODIUM channels, GABA, glutamate and carbonic anhydrase inhibition

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74
Q

Entacapone

A

COMT inhibitor, reduces dopa breakdown

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75
Q

Exacerbates myoclonic seizures (5)

A

Gabapentin, pregabalin, vigabatrin, lamotrigine, carbamazepine

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76
Q

Enzyme inducing AEDs

A

phenytoin, phenobarb, oxcarb, carb, topiramate >200mg

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77
Q

Riluzole mechanism

A

inhibition of glutamine release (ALS)

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78
Q

irresistible attacks of sleep

A

ropinirole, pramipexole

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79
Q

Alemtuzumab

A

monoclonal antibody that targets the CD-52 receptor on activated T- and B-lymphocytes

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80
Q

Targets VEGF receptors

A

Avastin

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81
Q

Galactosyloceramidase deficiency

A

Krabbe

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82
Q

Alpha-galactosidase A deficiency

A

Fabry

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83
Q

Alpha-glucosidase deficiency

A

Pompe

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84
Q

myophosphorylase deficiency.

A

McArdle

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85
Q

Eteplirsen

A

Treats Duchenne’s with out-of-frame mutations on exon 51 of the dystrophin gene

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86
Q

Most cases present in children under the age of 10 and are located in the 4th ventricle

A

Ependymoma if (perivascular rosettes)

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87
Q

adenosine diphosphate (ADP) receptor inhibitor that must be activated by p450

A

Clopidogrel

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88
Q

Cardiomegaly, hepatomegaly, progressive muscle weakness, macroglossia, and hypotonia, and vacuolar myopathy can be seen on a muscle biopsy.

A

pompe, tx is alglucosidase alfa

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89
Q

Typical MR spectroscopy findings associated with glioma (choline, lactate, choline creatinine ratio)

A

levations in choline and lactate, reduction in NAA, and relatively unchanged creatine. The higher the ratio of choline to creatine, the high probability the lesion is a high-grade glioma

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90
Q

the only FDA-approved medication for primary progressive multiple sclerosis (PPMS

A

Ocrelizumab

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91
Q

NF1 stuff besides neurofibromas

A

optic nerve/chiasm glioma, pilocytic astrocytoma; cafe au lait spots, lisch nodules and axillary freckling

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92
Q

Assymetric finger weakness

A

Inclusion body myositis

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93
Q

SCLC and myotonia

A

Isaac’s syndrome, potassium channel antibodies

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94
Q

Perimysial inflammation

A

Dermatomyocytis

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95
Q

dysmorphic facies and elevated very-long-chain fatty acids

A

Zellwegger syndrome, peroxisome disfunction

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96
Q

Bilateral thalamic strokes

A

Artery of percheron

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97
Q

wrist extension

A

Radial nerver

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98
Q

Radial nerve injury: axilla, spiral groove or posterior interosseous

A

Wrist drop only at spiral groove, posterior interosseous is motor only

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99
Q

TX syddeham chorea

A

steroids

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100
Q

PD degeneration leading to tremor

A

substantia nigra pars compacta

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101
Q

lead poisoning leads to

A

wrist and finger peripheral neuropathy

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102
Q

from paint, causes CNS effects: AMS, cerebellar, basal ganglia

A

toulene poisoning

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103
Q

warfarin reversal

A

PCC is faster than vit K or FFP

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104
Q

Phenelezine

A

MAO-i, causes wt gain, sedation, constipation, urinary retention

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105
Q

ICP A waves

A

increased ICP, 30-50mmHg, >20min

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106
Q

ICp B waves

A

normal, respiratory cycle

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107
Q

ICP C waves

A

normal, cardiac cycle (c=cardiac)

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108
Q

forearm supination

A

biceps brachii (musculocutaneous) and brachioradialis (radial)

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109
Q

familial ALS inheritance

A

AD

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110
Q

manganese vs PD

A

manganese causes palladial degeneration (spares SN) and motor symptoms are symmetric at onset

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111
Q

Neomycin streptomycin gentamycin

A

Neo is cochleo-toxic, others are vestibulotoxic (so can treat meneire’s)

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112
Q

critical illness neuropathy: SNAP, CMAP, conduction velocity

A

decreased, decreased, does not change (Axonal not demyleinating)

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113
Q

Low vs High NMDA antagonism

A

Low: Memantine, High: Ketamine

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114
Q

Tx for lithium tremor

A

propranolol

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115
Q

Amitriptyline contraidication

A

Cardiac, but also BPH! (use nortriptyline, despramine)

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116
Q

endocrine abnormality not associated with proximal myopathy

A

HYPOparathyroidism

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117
Q

Tx for chorea gravidum

A

PLEX (if anti-phospholipid); haldol or chlorpromazine

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118
Q

latex allergy

A

spina bifida

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119
Q

brain areas involved in smell

A

frontal, temporal and limbic

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120
Q

DHE vs prochlorperazine in status migrainousis

A

No DHE if triptan within 4 hours

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121
Q

FHM and episodic ataxia genetic overlap (and 2 other conditions)

A

FHM1 and episodic ataxia 2 are both CACNA1A, calcium channel (same as SMA6 and childhood absence epilepsy)

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122
Q

motor function // origin of tibial nerve

A

tibialis posterior and plantar interossei // sciatic, L4-S3

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123
Q

motor function // origin of common peroneal nerve

A

short head of biceps femoris // sciatic, L4-S2

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124
Q

motor function // origin of sciatic nerve

A

long head of biceps femoris, semitendinous, semimembranous (“hamstrings”) // L4-S3

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125
Q

motor function // origin of saphenous nerve

A

no motor! sensory to medial lower leg // femoral, L3-L4

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126
Q

motor function // origin of sural nerve

A

no motor! sensory to lateral ankle and foot // L4-S1

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127
Q

optic neuritis refractory to steroids

A

PLEX

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128
Q

Are NF1 fibromas pre-malignant?

A

No, but patients do get malignant nerve sheath tumors and soft tissue sarcomas

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129
Q

Does optic neuritis count toward McDonald criteria?

A

No, at least 2 lesions in brain or spine separated by time

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130
Q

Vercay bodies

A

schwannoma (NF2)

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131
Q

Negri bodies

A

in pyramidal cells in rabies

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132
Q

Cowdry bodies

A

HSV, VZV, CMV

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133
Q

Pick bodies

A

FTD (tau fibrils, ubiquitin and tubulin)

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134
Q

psammoma bodies

A

balls of calcium seen in meningioma

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135
Q

weakness of hamstrings only

A

sciatic nerve (L5-S2)

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136
Q

weakness of hamstrings and dorsiflexors

A

S1-S2

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137
Q

weakness of hip flexors

A

femoral (L2-L4)

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138
Q

weakness of quadriceps (and numbness of medial calf)

A

L4

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139
Q

calcium release channel of sarcoplastic reticulum

A

RYR! mech of malignant hyperthermia and central core myopathy

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140
Q

paroxismal fast activity

A

infantile spasms

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141
Q

frontal intermittent rhythmic delta

A

toxic/metabolic encephalopathy

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142
Q

continuous spike-wave in slow sleep

A

Landau-Kleffner

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143
Q

Slow spike and wave

A

Lennox Gastaut

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144
Q

IV nimodipine

A

kills you? (calcium channel blocker for vasospasm prevention in SAH, give enterally)

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145
Q

Impairment in only one domain

A

Can still be major cognitive disorder! Alzheimers requires 2 domains, one being memory

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146
Q

strokes in Fabry’s

A

ischemic due to fatty deposits

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147
Q

intention tremor more likely in stroke or MS?

A

MS

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148
Q

lithium and endocrine

A

hyperparathyroidism

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149
Q

clozapine side effects

A

eosinophilic colitis, neutropenia (rare), drooling (common)

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150
Q

depakote and pancreatitis

A

yes! rarely

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151
Q

child who snores

A

has OSA

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152
Q

cell bodies of CNV arise from

A

semilunar ganglion

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153
Q

vasculitis with antibodies for PR3 (proteinase 3) or myeloperoxidase (MPO)

A

ANCA! GPA, EGPA and microscopic polyamgiitis

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154
Q

vasculitis and small vessel predominant in a child

A

Kawasaki

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155
Q

vasculitis with HBV

A

polyarteritis nodosa

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156
Q

vasculitis with HCV

A

cryoglobenemia

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157
Q

polyarteritis nodosa usually causes

A

mononeuritis multiplex, not strokes!

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158
Q

Whipple disease tx

A

Ceftriaxone

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159
Q

Stage 3 sleep

A

deep/delta, alernates with REM, first half of night

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160
Q

mania in pregnancy tx

A

Haldol

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161
Q

chorea, hemiballism, tongue biting

A

neuroacanthocytosis (not Leish-Nyhan–normal uric acid and tongue biting is accidental)

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162
Q

palpable vertebrae

A

C7

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163
Q

ocular bobbing

A

pontine lesion

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164
Q

number of vertebrae

A

24 articulated and 9 fused

165
Q

last baby reflex

A

Landau (4m-1y)

166
Q

tympanic nerve and internal carotid plexus

A

tympanic plexus

167
Q

injury at the coup

A

contusion

168
Q

CNS behcets

A

rarely focal parenchymal lesions + vascular thrombosis, most commonly aseptic meningitis

169
Q

abort a cluster

A

not verapamil! sumatriptan/zolmitriptan and O2

170
Q

anti-LG1-1

A

potassium channel antibody; memory issues, myoclonus, faciobrachial seizures

171
Q

anti Yo

A

cerebellar signs in breast/pelvic malignancy

172
Q

Depakote and CYP450

A

Inhibition!

173
Q

What scores 2 on ABCD

A

unilateral weakness and duration greater than 60min

174
Q

ECCOOLI

A

mnemonic for auditory brainstem response

175
Q

EMG in sub clinical diabetic neuropathy

A

prolonged F wave latency

176
Q

Facial nerve anatomy

A
177
Q

p53 and ATRX

A

70% of astrocytomas

178
Q

tuberomammillary nucleus

A

histamine! Makes you itchy

179
Q

prevalence of insomnia

A

30-40%

180
Q

first nerves to go in peripheral neuropathy

A

unmyleinated C fibers (pain, temp, itch), myleinated A-delta fibers (acute pain)

181
Q

MG - most frequent and severely involved eye muscle

A

medial rectus

182
Q

Myleination perinatally

A

starts at 14 weeks (CNS, oligodendrocytes) and continues post-natally (optic nerve, others)

183
Q

fevers before hearing loss

A

ruptured epidermoid cyst

184
Q

Rassmussen’s tx

A

hemispherectomy

185
Q

what are neurofibrillary tangles made of?

A

hyperphosphorylated tau

186
Q

facial angiomas

A

tuberous sclerosis

187
Q

numbness over dorsal thumb and dorsolateral hand

A

superficial sensory radial neuropathy

188
Q

elevated serum glutamine and alanine

A

arginase deficiency (also vomiting, IDD and seizures)

189
Q

damage to these causes spasticity

A

corticoreticular fibers

190
Q

diabetic plexopathies are actually caused by

A

microvasculitis

191
Q

runs in the foramen lacerum

A

ICA!

192
Q

runs in the foramen spinosum

A

middle meningeal artery

193
Q

benzos safe in liver disease

A

oxazolam and lorazepam (inactive metabolites)

194
Q

painless altitudinal vision loss

A

anterior ischemic optic neuropathy

195
Q

AEs include headache, diplopia and dizziness

A

Lacosamide (slow sodium channels)

196
Q

blocks sodium and calcium channels to decrease glutamate relsease

A

lamotrigine

197
Q

gabapentin mechanism

A

calcium channe blocker

198
Q

clozapine side effects besides neutropenia

A

eosinophilic colitis and myocarditis

199
Q

pancreatitis from an AED

A

depakote

200
Q

constant amount eliminated over time (rather than constant proprotion)

A

zero order kinetics (phenytoin)

201
Q

bubbly on T2, presents with temporal lobe epilepsy

A

DNET

202
Q

antibiotic causing severe encephalopathy

A

cefipime

203
Q

CRPS: which is worse, cold or hot?

A

cold

204
Q

CMT 4 inheritance

A

AR! The rest are AD

205
Q

alpha adrenergic agonists guanfacine and clonidine

A

Tourette’s! NOT FDA APPROVED but fewer side effects than neuroleptics

206
Q

prevent strokes in SCD

A

exchange transfusion

207
Q

severity of thoracolumbar fractures (low to high)

A

compression, burst, translation/rotation, distraction

208
Q

MS tx in pregnancy

A

Glatimer acetate, if indicated

209
Q

meningitis with petechial rash and DIC

A

neisseria menigitidis (without rash most common cause is strep pneumo)

210
Q

Lumbosacral plexus roots

A

T12-S3

211
Q

prolactinomas arise from

A

surface ectoderm

212
Q

age range where women have more strokes

A

25-34

213
Q

“raised oval shaped pigmented plaques”

A

tuberous sclerosis

214
Q

forehead plaques

A

tuberous sclerosis

215
Q

most common CP angle tumor in children

A

vestibular schwannoma

216
Q

500x in delirium

A

dopamine

217
Q

dyphasic psychic aura

A

left parasylvian area

218
Q

Early onset MG 20-50

A

anti-striated muscle antibodies

219
Q

superior gluteal nerve innervates

A

glut medius and minimus (first sciatic branch nerve, L4-S3)

220
Q

mifipristone

A

glucocrticoid antagonist that may prevent steroid myopathy

221
Q

steady pressure on skin surface

A

Merkel disk (Pancini corpuscles also do this, plus vibration)

222
Q

skin temp sensors

A

Krause endings

223
Q

Abnormal H reflex

A

high specificity low sensitivity for S1 radiclopathy

224
Q

post-infectious shoulder pain

A

parsonage turner, neuralgic amyotrophy

225
Q

most common congenital facial palsy

A

unilateral lower lip

226
Q

sympathetic preganglionic cell bodies are located

A

intermediolateral cell column

227
Q

Popeye arms

A

fascioscapularhumeral dystrophy, foot drop, retinal degeneration, no cardiac involvement, chr 4

228
Q

TX conduct disorder

A

multisystemic therapy

229
Q

chancre and then AMS after travel to African

A

Trypanosoma brucie rhodesine (african sleeping sickness)

230
Q

tX for stiff person syndrome

A

benzos

231
Q

dopamine reuptake inhibitors

A

cocaine and methamphetamines. meth also increases presynaptic dopamine release

232
Q

thalamic nuclei affected in fatal familial insomnia

A

mediodorsal

233
Q

SMA is caused by

A

impaired mRNA splicing on chr 5

234
Q

fast axonal anterograde transport

A

kinesin

235
Q

cobblestone lissencephaly

A

impaired neuronal migration, usually with hydrocpehalas: usually AR, walker-warburg, fukuyama, and muscle eye brain disease

236
Q

duration for hypersomnolence disorder

A

3 months

237
Q

CSF in Varicella Zoster vasculopathy

A

elevated protein and mononuclear pleocytosis

238
Q

Wound botulism

A

most abrupt than ingestion botulism, plus CN involvement

239
Q

auras required for migraine with aura

A

2

240
Q

cyclopsia

A

sonic hedgehog

241
Q

familial ftd

A

9

242
Q

MMA acidemia

A

unable to metabolize valine

243
Q

nucleus solitarius: caudal vs rostral

A

caudal is baroreceptors via glossopharyngeal, rostral is sensory fibers from face tongue and epiglottis

244
Q

presense of IDH1 mutation

A

means GBM used to be a lower-grade glioma (IDH mutated has better prognosis)

245
Q

mechanism of lasmitidan (migraine tx in CAD)

A

5HT-1F (triptans do 5-HT1B and D) AE: dizziness and sedation

246
Q

organic mercury (seafood)

A

ataxia, parasesias and visual feild deficits

247
Q

CSF glucose

A

lower than plasma

248
Q

temperature control

A

hypothalamus (AC, cooling–anterior cools, posterior warms)

249
Q

CMT with hands affected early in the course

A

CMT 2D

250
Q

adrenoleukodystrophy

A

defect in beta-oxisidation leaing to a build up of very long fatty chain acids

251
Q

PARK2

A

genetic PD, prominent dystonia, no lewy bodies, younger onset

252
Q

nystagmus in CP angle tumor like schwannoma

A

Bruns nystagmus: slow toward lesion with fast correctiong

253
Q

haldol and fluphenazine

A

first gen antipsychotics, cause more parkinsonism

254
Q

confirms L5 and rules out s1 or common peroneal

A

tensor fascia late

255
Q

fast axon retrograde transport

A

dynein

256
Q

short half-life for short term insmonia

A

zaleplon

257
Q

spinous processes serve as

A

attachement for muscles and ligaments

258
Q

SLCO1B1

A

changes susceptibility to statin myopathy

259
Q

Rinne and Weber in sensorineural hearing loss

A

Rinne is normal, Weber lateralizes to good ear

260
Q

convergence retraction nystagmus

A

is not nystagmus

261
Q

alcoholic brains have

A

fewer GABA receptors

262
Q

DM1 vs 2

A

DM! is a trinucleotide repeat, more severe, grip mytonia; DM2 is a tetranucleotide repeat, with insulin resistance, cataracts and stiffness that improves throughout the day

263
Q

Allay lamp

A

only green light, improves photophobia

264
Q

AMS/personality changes, hearing loss and BRAO

A

Susac disease, microangiopathy affecting corpus callosum

265
Q

19p1q co-deletion

A

oligodendroglioma

266
Q

sleepwalking occurs

A

in N3 sleep, in the first third of the night

267
Q

PMA versus PLS

A

PMA is muscles/LMN only, PLS is UMN only

268
Q

AEs: ataxia, dizziness and double vision

A

carbamazepine

269
Q

most common primary lesion for brain mets

A

NSCLC
non-small cell lung cance

270
Q

brittle hair with shaft nodules

A

argininosuccinate lyase deficiency

271
Q

visual seizures, resting and active myoclonus

A

EPM2, la fora body disease

272
Q

PHOX2B trinucleutide repeats

A

congenital central hypoventilation syndrome

273
Q

soft palate rises when good side touched only

A

contralateral glossopharyngeal (sensory) and vagus (motor) are out

274
Q

soft palate on good side rises no matter which side you touch

A

contralateral vagus (motor) is out

275
Q

soft palate doesn’t move when bad side is touched, rises symmetrically when good side is touched

A

contralateral glossopharyngeal (sensory) is out

276
Q

opening pressure in IIH

A

> 25mmH2O

277
Q

EEG frequency with benzo use

A

20-25 hz

278
Q

acute arsenic poisoning

A

tachycardia, then shock (diffuse capillary leak)

279
Q

dalfampridine mechanism, indication and AE

A

calcium channel blocker, increase walking speed in MS, siezures

280
Q

MMA and homocysteine in b12 deficiency

A

both are elevated

281
Q

Ubrogepant AE and mech

A

nausea, CGRP modulation

282
Q

what is this and how does it spread?

A

mucormycosis, local invasion and abscess formation

283
Q

sensory and motor NCS in myasthenia

A

normal

284
Q

MG decrement

A

> 10%

285
Q

complications from temporal epilepsy surgery

A

emotional dysregulation and quadrantopia

286
Q

levels remain unchanged during pregnancy

A

carbemazepine

287
Q

forced blinking and tongue/jaw contractions

A

meige syndrome

288
Q

PCP mechanism

A

NMDA antagonist, it also inhibits the reuptake of dopamine, serotonin and norepinephrine– you get this confused with meth, which inhibits dopamine reuptake

289
Q

melatonin in hypoactive delirium

A

increased

290
Q

isolated hoarseness

A

recurrent laryngeal nerve injury

291
Q

PMP22 deletion

A

Heriditary pressure palsy

292
Q

PMP22 duplication

A

CMT1A

293
Q

ECT and memory

A

anterograde amnesia

294
Q

Tx malignant hyperthermia

A

Dantrolene or bromocriptine

295
Q

can alter the appearance of acute SDH

A

anemia

296
Q

lesion at facial motor nucleus

A

unilateral face paralysis

297
Q

lesion at geniculate ganglion

A

unilateral face paralysis, hyperacusis, loss of taste from anterior tongue, loss of salivation and loss of tearing of the eye

298
Q

lesion immeadiately distal to geniculate ganglion

A

unilateral face paralysis, hyperacusis, loss of taste from anterior tongue, loss of salivation (spares the eye)

299
Q

lesion immeadiately proximal to chorda typani

A

unilateral face paralysis, loss of taste from anterior tongue, loss of salivation (spares the eye and the eye)

300
Q

global persistent hypotonia since birth, Japanese

A

Fukuyama congenital MD (or get weak, but have early contractures of hip, knee and ankle and skull asymmetry)

301
Q

global persistent hypotonia since birth, eye issues

A

Walker Warburg

302
Q

anti-ENA

A

negative risk of malignancy in polymyositis

303
Q

ferritin level to supplement in RLS

A

<75

304
Q

fragile X genetics

A

unstable expansion of CGG repeat on chr X

305
Q

Cadasil chromosome

A

19! the gene is NOTCH 3

306
Q

the only CGRP antagonist that acts against the receptor

A

Erenumab (AImovig) which also causes constipation!!

307
Q

upper motor neuron of the nervous system

A

Betz cells, motor cortex

308
Q

Tx Serotonin syndrome

A

cyproheptidine

309
Q

SREDA is maximal

A

in parietal and posterior temporal regions

310
Q

thalamus blood supply

A

peforating arteries from PCA

311
Q

abducens + hypoglossal nerve palsy

A

Gottfredson’s syndrome (metastatic tumor to the clivus)

312
Q

MG tx in pregnancy

A

pyridostigmine (and prednisone, but C)

313
Q

tx dykinesias from carbodopa-levodopa

A

amantidine (DBS if severe)

314
Q

pure motor stroke

A

internal capsule

315
Q

pure sensory stroke

A

thalamus

316
Q

C6, C7, C8 dermatomes

A

6 is thumb, 7 is middle, 8 is pinkie

317
Q

type 1 muscles

A

low and slow, more vascular, higher oxidative demand, Krebs cycle

318
Q

upper extremity adduction and pronation, legs extended

A

decerberate (below red nucleus)

319
Q

upper extremity adduction and flexion, legs extended with feet inward

A

decorticate (above red nucleus)

320
Q

recovery from sleep deprivation

A

first recovery night has long N3 and short R

321
Q

hemangioblastomas in eyes and cerebellum

A

von hippel lindau, AD, VHL gene on chr 3; also RCC and pheochromocytomas

322
Q

spinocerebellar degeneration from a vitamin deficiency

A

Always E!

323
Q

definitive Alzheimer’s dx requires

A

biopsy

324
Q

buspirone site of action

A

5HT-1A

325
Q

macular edema

A

fingolimod

326
Q

spinal nucleus of CN V

A

pain and temperature to all of the head

327
Q

mesenceaphalic nucleus of CN V

A

jaw jerk reflex

328
Q

chief nucleus of CN V

A

vibration, proprioception and light touch to face

329
Q

intracellular reticulin deposition

A

pleomorphic xanthroastrocytoma

330
Q

chicken wire vasculature

A

oligodendroglioma

331
Q

bipolar hairlike astrocytes

A

pilocytic astrocytoma

332
Q

shopping cart sign

A

L4/L5 + enlarged ligamentum flavum

333
Q

vetrolateral preoptic nucleus

A

hypothalamic egulation of sleep cycles

334
Q

holoprosencephaly most associated chr abnormality

A

Trisomy 13

335
Q

distal greater than proximal weakness, sensory loss, axonal NCS in ICU

A

critical illness polyneuropathy

335
Q

proximal weakness, decreased reflexes and normal sensory exam in ICU

A

critical illness myopathy

335
Q

repetition deficits and phonemic paraphasic errors

A

conduction aphasia

335
Q

predominantly proximal weakness, with sensory invovlement in ICU

A

critical illness polymyoneuropathy

336
Q

pilocytic astrocytomas, WHO grade and prognosis

A

WHO grade 1, slow growing with good prognosis

337
Q

cancer most commonly associated with opspclonus myoclonus

A

SCLC

338
Q

adrenoleukodystrophy mechanism and treatment

A

inflammatory demyleination; steroids and bone marrow transplant

339
Q

scapular winging, rectus abdominus weakness, child or young adult

A

calpain-3 deficiency

340
Q

wilson’s inheritance

A

AR

341
Q

wilson’s in pregnancy

A

treat with zinc acetate

342
Q

palpable liver, reducing substances in the urine

A

galactosemia

343
Q

erections

A

parasympathetics s2-s4

344
Q

urine smells like sweaty feet

A

isovaleric acidemia

345
Q

a group of normal neurons that did not migrate correctly

A

heterotopia

346
Q

basal ganglia calicifications from HIV

A

more common in children

347
Q

vertical gaze palsy

A

pineal tumors, compression of the rostral interstitial nucleus of the MLF

348
Q

second generation antipsychotics

A

antangonize D2 and 5HT 2A

349
Q

Homer-Wright Rosettes

A

medulloblastoma

349
Q

CMT with acute attacks of ataxia, dysarthria, and weakness

A

CMT1X

350
Q

symmetric Parkinsonism, lacking tremor, plus OSA or laryngeal stridor

A

Multi-system atrophy P (striatonigral degeneration)

351
Q

most common neuro Behcets

A

relapsing remitting aseptic meningitis (5-20%)

352
Q

first line tx for JME in girls

A

Keppra

353
Q

proximal contractures, distal hyperlaxity, global hypotonia, no reflexes, first months of life

A

Ullrich’s congenital MD

354
Q

BPPV: otoliths separate from

A

Utrical and saccule

355
Q

Iris hamaratomas

A

Lisch nodules! NF1

356
Q

GABA B agonist

A

baclofen

357
Q

seizures in sleep

A

commonly stage 2, never REM

358
Q

bone marrow suppression, bleeding and bruising

A

carbamazepine

359
Q

chorea and basal ganglia lesions in neonate

A

kernicterus, from bilirubin

360
Q

colchicine vs statin myopathy

A

colchicine also causes axonal neuropathy!

361
Q

steroid vs statin myopathy

A

steroid has normal CK, EMG doesn’t show fibrillation

362
Q

damaged by carbon monoxide toxicity

A

globus pallidus

363
Q

topiramate in pregnancy

A

low birth weight, 1.4% facial cleft

364
Q

is mesial temporal sclerosis a focal cortical dysplasia?

A

No! neuronal loss/gliosis

365
Q

Neiman Pick C

A

cannot metabolize cholesterol or lipids; diagnose with Filipin test

366
Q

Niemann-Pick A and B

A

Spingomylelinase deficiency in white blood cells.

367
Q

tx atonic neurogenic bladder

A

cholinergics like Bethanechol

368
Q

a farmer with diarrhea, miosis, diaphoresis

A

Acetylcholinesterase inhibition (organophosphate poisoning)

369
Q

deep brain damage in a seizing baby, pyruvate dehydrogenase

A

Leigh disease, fatal; can also be mitochondrial

370
Q

ASA ischemia

A

anterior cord: loss of pain and temperature, motor function

371
Q

PML cells affected, median survival

A

Glial cells, 3 months

372
Q

vestibular schwannomas arise within

A

internal acoustic meatus (ie the auditory canal)

373
Q

CN IX-XI deficits

A

uvula deviation, weak head turn, taste loss, dysphagia: jugular foramen! (all originate in the medulla)

374
Q

deficient branched chain amino acids and sweet smelling urine

A

maple syrup urine disease , tx with diet and HD

375
Q

basophilic stippling of RBCs

A

lead poisoning

376
Q

sniffing glue

A

n-Hexane, facial numbness and vision loss

377
Q

glucocerebroside

A

Gaucher, lysosomal storage disorder, stores in liver and bones, AR

378
Q

most common genetic disorder in Ashkenazi Jews

A

Gaucher, glucocerebroside deficiency

379
Q

D-methylmalonyl-Co A deficiency

A

Methylmalonic acidemia, lethargy and FTF in first week of life, methylmalonic acid and propinionyl Co A accumulate (and can’t metabolize VALINE)

380
Q

MECP2

A

Rett syndrome, X linked, 6-18 month old girl with hand wringing

381
Q

GFAP mutation

A

Alexander, frontal, Rosenthal fibers, NOT U-SPARING,

382
Q

PKU

A

musty odor, can’t convert phenylalalanine to tyronisine, AR

383
Q

Frederich’s ataxia transmission

A

AR

384
Q

lissencephaly, low ear, upturned nose

A

Miller Deiker

385
Q

big baby, low tone, clumsy, IDD, sometimes seizures

A

Soto

386
Q

increase valine, leucine and isoleucine in urine

A

maple syrup urine disease, first week of life

387
Q

Alpha galatosidase A deficiency

A

metachromatic leukodystrophy, diffuse, SPARES U

388
Q

optic atrophy, exaggerated startle, FTF

A

Krabbe (galactoceramide )

389
Q

U sparing, galactoceramide

A

Krabbe

390
Q

cardiomegaly, hepatomegaly, macroglossia, muscle weakness

A

Pompe (Alglucosidase alpha)

391
Q

Alglucosidase alpha

A

Pompe

392
Q

ataxia, poor vision, mild proximal muscle weakness

A

NARP

393
Q

hearing loss, gradual vision loss, no ataxia

A

Ushers

394
Q

Eyelid/eye movement issues, deafness, ataxia, retinopathy, cardiac

A

Kearn-Sayres (can be mitochondrial)

395
Q

macroorchidism

A

and tall, with IDD? FMR1, fragile X sydrome, it’s a CGG repeat!

396
Q

cherry red spot, hepatomegaly, normal til 3-5 months

A

Neiman Pick (1 and 2 are spingomylenase) … foma cells?

397
Q

cherry red spot, color blindness and convulsions

A

sialidosis (skin fibroblast alpha neuramidase)

398
Q

cherry red spot, “startle seizures”

A

Tay Sachs, hexoamindase A

399
Q

walks, scribbles if you do, stacks 2 cubes

A

12 month old

400
Q

Age 2-12 with scoliosis, ataxia arms > legs, dysarthria and heart problesm

A

Fredrich’s ataxia, AR

401
Q

Sinusitis and ataxia

A

and telangectasias! its ataxia telangectasia. (AFP and CEA elevated)

402
Q

marfanoid, pigeon chest, retinal detachments,

A

homocysteiuria, tx is pyridoxine

403
Q

infantile spasms, oral rash, IDD

A

biotinidase deficiency

404
Q
A