Boards facts Flashcards
1
Q
Targets for DBS in Parkinsons
A
GPi and STN
2
Q
Mechanism of dabigatran
A
Direct thrombin inhibitor
3
Q
Mechanism of rivaroxiban, apixiban
A
Factor Xa inhibitors
4
Q
Chronic progressive ophthalmoplegia with cardiac involvement
A
Kearns-sayres
5
Q
PComm aneurysm CN effects
A
CN III lesions
6
Q
sensory innervation to the shoulder as well as the teres minor and deltoid muscles
A
Axillary nerve (posterior cord, C5-C6)
7
Q
responsible for stabilizing the scapula against the thorax
A
Serrates anterior (long thoracic nerve, C5,6,7 right off brachial plexus)
8
Q
responsible for the first 15 degrees of shoulder abduction
A
Supraspinatus (suprascapular nerve, c5,6)
9
Q
CSF analysis will show an elevated protein, low glucose, and an elevated lymphocytic-predominant white cell count
A
Cryptococcus, CD4<200!
10
Q
Lasmiditan
A
Migraine abortive that targets 5HT-1F receptors , safe in CAD
11
Q
Looks like Rathke’s cleft cyst, but calcified
A
Craniopharyngioma
12
Q
acute denervation to the tibial (gastrocnemius) and deep peroneal (tibialis anterior) innervated muscles
A
Sciatic nerve proximal to knee
13
Q
The only CMT with axonal (not demylenating) polyneuropathy
A
CMT 2
14
Q
CIDP, Charcot-Marie-Tooth type 1A, and Refsum disease can have this finding appreciated on a sural nerve biopsy.
A
Onion bulbs
15
Q
superior gluteal (gluteus medius) tibial (gastrocnemius), and tibialis anterior nerves
A
L5 radiculopathy, the most common radiculopathy
16
Q
VPL
A
relay center for sensory information of the body
17
Q
VPM
A
relay center for sensory information of the face as well as taste
18
Q
superior colliculi, located in the midbrain, actually play a prominent role in
A
Visual system
19
Q
Auditory pathway
A
Hair cells > cochlear nucleus > trapezoid body > ventral pons > superior olivary complex > inferior colliculi (midbrain) > thalamus
20
Q
thalamic astasia
A
Unilateral thalami stroke imitating cerebellar stroke (full strength but can’t stand)
21
Q
Thalamic aphasia
A
presents with a fluency-predominant deficit and less impressive difficulties with comprehension
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
23
Q
ipsilateral third nerve palsy with contralateral hemiparesis
A
Weber’s sydrome, medial midbrain
24
Q
Lesion for Alexia without agraphia
A
Splenium of the corpus callous
25
change in heart rate, blood pressure, respiratory rate during a seizure
Increase > R insula, decrease > l insula
26
Forearm pronation
median or radial nerve
27
Wrist flexion
Median nerve
28
elbow flexion
Musculocutaneous nerve
29
Musculocutaneous nerve and C6-7 portion of median nerve
Lateral cord
30
Radial nerve and axillary nerve
Posterior cord C5-C8
31
Ulnar nerve and C8-T1 portion of median nerve
Medial cord
32
Deltoid abduction plus triceps and wrist extension
Axillary AND radial nerves = posterior cord
33
Scapular winging
long thoracic nerve (C5-C7) damage (serratus anterior)
34
SCM (contralateral head turn) and trapezius (assists deltoid in lifting arm >90 degrees)
Spinal accessory nerve
35
ascending tract that carries information about pain and temperature
Spinothalamic (C shaped)
36
descending tract that controls skeletal muscle motor movement of the contralateral limbs
Paternal corticospinal (oval shaped)
37
Rhomboids
Dorsal scapular nerve
38
pufferfish dishes. Tetrodotoxin mechanism
preferentially block sodium channels
39
Looks like radial neuropathy at the axilla, plus weak deltoid and latissimus dorsi
Posterior cord plexopathy
40
Tongue deviation from a stroke--vascular supply
middle medulla is primarily supplied by the anterior spinal artery (ASA)
41
lacunar infarct to the ventral posterior nuclei
Pure sensory stroke
42
developmental regression, failure to thrive, and ataxia; tiger without eyes
Leigh syndrome
43
Osteolytic lesion in a child
Langerhans cell histiocytosis
44
anti-Hu paraneoplastic syndrome, Sjogren syndrome, pyridoxine toxicity, and exposure to certain chemotherapeutic agents
Common causes of sensory neuron-patchy (like isolated loss of proprioception)
45
type-II topoisomerase inhibitor that disrupts DNA synthesis and repair
Mitoxantrone
46
selectively targets the CD20-positive B-cells causing antibody-dependent cellular cytolysis
Ocrelizomab
47
α4-integrin antagonist that inhibits the transmigration of T-cells across the blood-brain barrier
Natalizomab
48
inhibits pyrimidine de novo synthesis thus inhibiting rapidly dividing cells, including t-cells
Teriflunomide
49
prolonged exposure to colchicine, amiodarone, chloroquine, or hydroxychloroquine
Myopathy and neuropathy!
50
The myelencephalon is responsible for the formation of the
Medulla
51
The telencephalon develops into
cerebral hemispheres, the basal ganglia, the hippocampus, and the amygdala
52
The diencephalon develops into the
thalamus, hypothalamus, and retina
53
The mesencephalon develops into the
Midbrain
54
The metencephalon develops into
the pons and cerebellum.
55
posterior predominant demyelination
X-linked luekodystrophy
56
Diffuse demyelination sparing U fibers
Krabbe
57
Frontal predominant demyelination
Alexander’s disease
58
Diffuse demyelination including U fibers
Canavan (Krabbe and metachromatic spare U)
59
Accumulates in Krabbe
galactosylceramide (space crab, U sparing)
60
Sphingomyelin accumulates in
Neimann-Pick disease
61
Glucosylceramide accumulates in
Gaucher disease
62
Globotriasylceramide accumulates in
Fabry disease (global fabric)
63
café-au-lait spots, Lisch nodules, and axillary freckling
NF1
64
Schwannomas
NF2
65
Ash-leaf spots and facial angiofibromas
Tuberous sclerosis
66
Hemangioblastomas
Von-Hippel Landau disease
67
can cause first-dose bradycardia, atrioventricular block, and QT interval prolongation
fingolimod
68
Looks like ALS but with conduction block
multifocal motor neuron (MMN) disease! Treatment is IVIG
69
deletion, mutation, or inappropriate methylation of the paternal copy of the q11-q13 segment of chromosome 15
Prader-Willi
70
deletion, mutation, or inappropriate methylation of the maternal copy of the q11-q13 segment of chromosome 15
Angelman
71
Infantile spasms and ash leaf spots, dx and tx
Tuberous sclerosis, vigabatrin (hipsarthymia, but NOT treated with ACTH)
72
Lacosamide mechanism
Selective enhancement of slow inactivation of SODIUM channels
73
Topiramate mechanism
SODIUM channels, GABA, glutamate and carbonic anhydrase inhibition
74
Entacapone
COMT inhibitor, reduces dopa breakdown
75
Exacerbates myoclonic seizures (5)
Gabapentin, pregabalin, vigabatrin, lamotrigine, carbamazepine
76
Enzyme inducing AEDs
phenytoin, phenobarb, oxcarb, carb, topiramate >200mg
77
Riluzole mechanism
inhibition of glutamine release (ALS)
78
irresistible attacks of sleep
ropinirole, pramipexole
79
Alemtuzumab
monoclonal antibody that targets the CD-52 receptor on activated T- and B-lymphocytes
80
Targets VEGF receptors
Avastin
81
Galactosyloceramidase deficiency
Krabbe
82
Alpha-galactosidase A deficiency
Fabry
83
Alpha-glucosidase deficiency
Pompe
84
myophosphorylase deficiency.
McArdle
85
Eteplirsen
Treats Duchenne’s with out-of-frame mutations on exon 51 of the dystrophin gene
86
Most cases present in children under the age of 10 and are located in the 4th ventricle
Ependymoma if (perivascular rosettes)
87
adenosine diphosphate (ADP) receptor inhibitor that must be activated by p450
Clopidogrel
88
Cardiomegaly, hepatomegaly, progressive muscle weakness, macroglossia, and hypotonia, and vacuolar myopathy can be seen on a muscle biopsy.
pompe, tx is alglucosidase alfa
89
Typical MR spectroscopy findings associated with glioma (choline, lactate, choline creatinine ratio)
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
90
the only FDA-approved medication for primary progressive multiple sclerosis (PPMS
Ocrelizumab
91
NF1 stuff besides neurofibromas
optic nerve/chiasm glioma, pilocytic astrocytoma; cafe au lait spots, lisch nodules and axillary freckling
92
Assymetric finger weakness
Inclusion body myositis
93
SCLC and myotonia
Isaac’s syndrome, potassium channel antibodies
94
Perimysial inflammation
Dermatomyocytis
95
dysmorphic facies and elevated very-long-chain fatty acids
Zellwegger syndrome, peroxisome disfunction
96
Bilateral thalamic strokes
Artery of percheron
97
wrist extension
Radial nerver
98
Radial nerve injury: axilla, spiral groove or posterior interosseous
Wrist drop only at spiral groove, posterior interosseous is motor only
99
TX syddeham chorea
steroids
100
PD degeneration leading to tremor
substantia nigra pars compacta
101
lead poisoning leads to
wrist and finger peripheral neuropathy
102
from paint, causes CNS effects: AMS, cerebellar, basal ganglia
toulene poisoning
103
warfarin reversal
PCC is faster than vit K or FFP
104
Phenelezine
MAO-i, causes wt gain, sedation, constipation, urinary retention
105
ICP A waves
increased ICP, 30-50mmHg, >20min
106
ICp B waves
normal, respiratory cycle
107
ICP C waves
normal, cardiac cycle (c=cardiac)
108
forearm supination
biceps brachii (musculocutaneous) and brachioradialis (radial)
109
familial ALS inheritance
AD
110
manganese vs PD
manganese causes palladial degeneration (spares SN) and motor symptoms are symmetric at onset
111
Neomycin streptomycin gentamycin
Neo is cochleo-toxic, others are vestibulotoxic (so can treat meneire's)
112
critical illness neuropathy: SNAP, CMAP, conduction velocity
decreased, decreased, does not change (Axonal not demyleinating)
113
Low vs High NMDA antagonism
Low: Memantine, High: Ketamine
114
Tx for lithium tremor
propranolol
115
Amitriptyline contraidication
Cardiac, but also BPH! (use nortriptyline, despramine)
116
endocrine abnormality not associated with proximal myopathy
HYPOparathyroidism
117
Tx for chorea gravidum
PLEX (if anti-phospholipid); haldol or chlorpromazine
118
latex allergy
spina bifida
119
brain areas involved in smell
frontal, temporal and limbic
120
DHE vs prochlorperazine in status migrainousis
No DHE if triptan within 4 hours
121
FHM and episodic ataxia genetic overlap (and 2 other conditions)
FHM1 and episodic ataxia 2 are both CACNA1A, calcium channel (same as SMA6 and childhood absence epilepsy)
122
motor function // origin of tibial nerve
tibialis posterior and plantar interossei // sciatic, L4-S3
123
motor function // origin of common peroneal nerve
short head of biceps femoris // sciatic, L4-S2
124
motor function // origin of sciatic nerve
long head of biceps femoris, semitendinous, semimembranous ("hamstrings") // L4-S3
125
motor function // origin of saphenous nerve
no motor! sensory to medial lower leg // femoral, L3-L4
126
motor function // origin of sural nerve
no motor! sensory to lateral ankle and foot // L4-S1
127
optic neuritis refractory to steroids
PLEX
128
Are NF1 fibromas pre-malignant?
No, but patients do get malignant nerve sheath tumors and soft tissue sarcomas
129
Does optic neuritis count toward McDonald criteria?
No, at least 2 lesions in brain or spine separated by time
130
Vercay bodies
schwannoma (NF2)
131
Negri bodies
in pyramidal cells in rabies
132
Cowdry bodies
HSV, VZV, CMV
133
Pick bodies
FTD (tau fibrils, ubiquitin and tubulin)
134
psammoma bodies
balls of calcium seen in meningioma
135
weakness of hamstrings only
sciatic nerve (L5-S2)
136
weakness of hamstrings and dorsiflexors
S1-S2
137
weakness of hip flexors
femoral (L2-L4)
138
weakness of quadriceps (and numbness of medial calf)
L4
139
calcium release channel of sarcoplastic reticulum
RYR! mech of malignant hyperthermia and central core myopathy
140
paroxismal fast activity
infantile spasms
141
frontal intermittent rhythmic delta
toxic/metabolic encephalopathy
142
continuous spike-wave in slow sleep
Landau-Kleffner
143
Slow spike and wave
Lennox Gastaut
144
IV nimodipine
kills you? (calcium channel blocker for vasospasm prevention in SAH, give enterally)
145
Impairment in only one domain
Can still be major cognitive disorder! Alzheimers requires 2 domains, one being memory
146
strokes in Fabry's
ischemic due to fatty deposits
147
intention tremor more likely in stroke or MS?
MS
148
lithium and endocrine
hyperparathyroidism
149
clozapine side effects
eosinophilic colitis, neutropenia (rare), drooling (common)
150
depakote and pancreatitis
yes! rarely
151
child who snores
has OSA
152
cell bodies of CNV arise from
semilunar ganglion
153
vasculitis with antibodies for PR3 (proteinase 3) or myeloperoxidase (MPO)
ANCA! GPA, EGPA and microscopic polyamgiitis
154
vasculitis and small vessel predominant in a child
Kawasaki
155
vasculitis with HBV
polyarteritis nodosa
156
vasculitis with HCV
cryoglobenemia
157
polyarteritis nodosa usually causes
mononeuritis multiplex, not strokes!
158
Whipple disease tx
Ceftriaxone
159
Stage 3 sleep
deep/delta, alernates with REM, first half of night
160
mania in pregnancy tx
Haldol
161
chorea, hemiballism, tongue biting
neuroacanthocytosis (not Leish-Nyhan--normal uric acid and tongue biting is accidental)
162
palpable vertebrae
C7
163
ocular bobbing
pontine lesion
164
number of vertebrae
24 articulated and 9 fused
165
last baby reflex
Landau (4m-1y)
166
tympanic nerve and internal carotid plexus
tympanic plexus
167
injury at the coup
contusion
168
CNS behcets
rarely focal parenchymal lesions + vascular thrombosis, most commonly aseptic meningitis
169
abort a cluster
not verapamil! sumatriptan/zolmitriptan and O2
170
anti-LG1-1
potassium channel antibody; memory issues, myoclonus, faciobrachial seizures
171
anti Yo
cerebellar signs in breast/pelvic malignancy
172
Depakote and CYP450
Inhibition!
173
What scores 2 on ABCD
unilateral weakness and duration greater than 60min
174
ECCOOLI
mnemonic for auditory brainstem response
175
EMG in sub clinical diabetic neuropathy
prolonged F wave latency
176
Facial nerve anatomy
177
p53 and ATRX
70% of astrocytomas
178
tuberomammillary nucleus
histamine! Makes you itchy
179
prevalence of insomnia
30-40%
180
first nerves to go in peripheral neuropathy
unmyleinated C fibers (pain, temp, itch), myleinated A-delta fibers (acute pain)
181
MG - most frequent and severely involved eye muscle
medial rectus
182
Myleination perinatally
starts at 14 weeks (CNS, oligodendrocytes) and continues post-natally (optic nerve, others)
183
fevers before hearing loss
ruptured epidermoid cyst
184
Rassmussen's tx
hemispherectomy
185
what are neurofibrillary tangles made of?
hyperphosphorylated tau
186
facial angiomas
tuberous sclerosis
187
numbness over dorsal thumb and dorsolateral hand
superficial sensory radial neuropathy
188
elevated serum glutamine and alanine
arginase deficiency (also vomiting, IDD and seizures)
189
damage to these causes spasticity
corticoreticular fibers
190
diabetic plexopathies are actually caused by
microvasculitis
191
runs in the foramen lacerum
ICA!
192
runs in the foramen spinosum
middle meningeal artery
193
benzos safe in liver disease
oxazolam and lorazepam (inactive metabolites)
194
painless altitudinal vision loss
anterior ischemic optic neuropathy
195
AEs include headache, diplopia and dizziness
Lacosamide (slow sodium channels)
196
blocks sodium and calcium channels to decrease glutamate relsease
lamotrigine
197
gabapentin mechanism
calcium channe blocker
198
clozapine side effects besides neutropenia
eosinophilic colitis and myocarditis
199
pancreatitis from an AED
depakote
200
constant amount eliminated over time (rather than constant proprotion)
zero order kinetics (phenytoin)
201
bubbly on T2, presents with temporal lobe epilepsy
DNET
202
antibiotic causing severe encephalopathy
cefipime
203
CRPS: which is worse, cold or hot?
cold
204
CMT 4 inheritance
AR! The rest are AD
205
alpha adrenergic agonists guanfacine and clonidine
Tourette's! NOT FDA APPROVED but fewer side effects than neuroleptics
206
prevent strokes in SCD
exchange transfusion
207
severity of thoracolumbar fractures (low to high)
compression, burst, translation/rotation, distraction
208
MS tx in pregnancy
Glatimer acetate, if indicated
209
meningitis with petechial rash and DIC
neisseria menigitidis (without rash most common cause is strep pneumo)
210
Lumbosacral plexus roots
T12-S3
211
prolactinomas arise from
surface ectoderm
212
age range where women have more strokes
25-34
213
"raised oval shaped pigmented plaques"
tuberous sclerosis
214
forehead plaques
tuberous sclerosis
215
most common CP angle tumor in children
vestibular schwannoma
216
500x in delirium
dopamine
217
dyphasic psychic aura
left parasylvian area
218
Early onset MG 20-50
anti-striated muscle antibodies
219
superior gluteal nerve innervates
glut medius and minimus (first sciatic branch nerve, L4-S3)
220
mifipristone
glucocrticoid antagonist that may prevent steroid myopathy
221
steady pressure on skin surface
Merkel disk (Pancini corpuscles also do this, plus vibration)
222
skin temp sensors
Krause endings
223
Abnormal H reflex
high specificity low sensitivity for S1 radiclopathy
224
post-infectious shoulder pain
parsonage turner, neuralgic amyotrophy
225
most common congenital facial palsy
unilateral lower lip
226
sympathetic preganglionic cell bodies are located
intermediolateral cell column
227
Popeye arms
fascioscapularhumeral dystrophy, foot drop, retinal degeneration, no cardiac involvement, chr 4
228
TX conduct disorder
multisystemic therapy
229
chancre and then AMS after travel to African
Trypanosoma brucie rhodesine (african sleeping sickness)
230
tX for stiff person syndrome
benzos
231
dopamine reuptake inhibitors
cocaine and methamphetamines. meth also increases presynaptic dopamine release
232
thalamic nuclei affected in fatal familial insomnia
mediodorsal
233
SMA is caused by
impaired mRNA splicing on chr 5
234
fast axonal anterograde transport
kinesin
235
cobblestone lissencephaly
impaired neuronal migration, usually with hydrocpehalas: usually AR, walker-warburg, fukuyama, and muscle eye brain disease
236
duration for hypersomnolence disorder
3 months
237
CSF in Varicella Zoster vasculopathy
elevated protein and mononuclear pleocytosis
238
Wound botulism
most abrupt than ingestion botulism, plus CN involvement
239
auras required for migraine with aura
2
240
cyclopsia
sonic hedgehog
241
familial ftd
9
242
MMA acidemia
unable to metabolize valine
243
nucleus solitarius: caudal vs rostral
caudal is baroreceptors via glossopharyngeal, rostral is sensory fibers from face tongue and epiglottis
244
presense of IDH1 mutation
means GBM used to be a lower-grade glioma (IDH mutated has better prognosis)
245
mechanism of lasmitidan (migraine tx in CAD)
5HT-1F (triptans do 5-HT1B and D) AE: dizziness and sedation
246
organic mercury (seafood)
ataxia, parasesias and visual feild deficits
247
CSF glucose
lower than plasma
248
temperature control
hypothalamus (AC, cooling--anterior cools, posterior warms)
249
CMT with hands affected early in the course
CMT 2D
250
adrenoleukodystrophy
defect in beta-oxisidation leaing to a build up of very long fatty chain acids
251
PARK2
genetic PD, prominent dystonia, no lewy bodies, younger onset
252
nystagmus in CP angle tumor like schwannoma
Bruns nystagmus: slow toward lesion with fast correctiong
253
haldol and fluphenazine
first gen antipsychotics, cause more parkinsonism
254
confirms L5 and rules out s1 or common peroneal
tensor fascia late
255
fast axon retrograde transport
dynein
256
short half-life for short term insmonia
zaleplon
257
spinous processes serve as
attachement for muscles and ligaments
258
SLCO1B1
changes susceptibility to statin myopathy
259
Rinne and Weber in sensorineural hearing loss
Rinne is normal, Weber lateralizes to good ear
260
convergence retraction nystagmus
is not nystagmus
261
alcoholic brains have
fewer GABA receptors
262
DM1 vs 2
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
Allay lamp
only green light, improves photophobia
264
AMS/personality changes, hearing loss and BRAO
Susac disease, microangiopathy affecting corpus callosum
265
19p1q co-deletion
oligodendroglioma
266
sleepwalking occurs
in N3 sleep, in the first third of the night
267
PMA versus PLS
PMA is muscles/LMN only, PLS is UMN only
268
AEs: ataxia, dizziness and double vision
carbamazepine
269
most common primary lesion for brain mets
NSCLC
non-small cell lung cance
270
brittle hair with shaft nodules
argininosuccinate lyase deficiency
271
visual seizures, resting and active myoclonus
EPM2, la fora body disease
272
PHOX2B trinucleutide repeats
congenital central hypoventilation syndrome
273
soft palate rises when good side touched only
contralateral glossopharyngeal (sensory) and vagus (motor) are out
274
soft palate on good side rises no matter which side you touch
contralateral vagus (motor) is out
275
soft palate doesn't move when bad side is touched, rises symmetrically when good side is touched
contralateral glossopharyngeal (sensory) is out
276
opening pressure in IIH
>25mmH2O
277
EEG frequency with benzo use
20-25 hz
278
acute arsenic poisoning
tachycardia, then shock (diffuse capillary leak)
279
dalfampridine mechanism, indication and AE
calcium channel blocker, increase walking speed in MS, siezures
280
MMA and homocysteine in b12 deficiency
both are elevated
281
Ubrogepant AE and mech
nausea, CGRP modulation
282
what is this and how does it spread?
mucormycosis, local invasion and abscess formation
283
sensory and motor NCS in myasthenia
normal
284
MG decrement
> 10%
285
complications from temporal epilepsy surgery
emotional dysregulation and quadrantopia
286
levels remain unchanged during pregnancy
carbemazepine
287
forced blinking and tongue/jaw contractions
meige syndrome
288
PCP mechanism
NMDA antagonist, it also inhibits the reuptake of dopamine, serotonin and norepinephrine-- you get this confused with meth, which inhibits dopamine reuptake
289
melatonin in hypoactive delirium
increased
290
isolated hoarseness
recurrent laryngeal nerve injury
291
PMP22 deletion
Heriditary pressure palsy
292
PMP22 duplication
CMT1A
293
ECT and memory
anterograde amnesia
294
Tx malignant hyperthermia
Dantrolene or bromocriptine
295
can alter the appearance of acute SDH
anemia
296
lesion at facial motor nucleus
unilateral face paralysis
297
lesion at geniculate ganglion
unilateral face paralysis, hyperacusis, loss of taste from anterior tongue, loss of salivation and loss of tearing of the eye
298
lesion immeadiately distal to geniculate ganglion
unilateral face paralysis, hyperacusis, loss of taste from anterior tongue, loss of salivation (spares the eye)
299
lesion immeadiately proximal to chorda typani
unilateral face paralysis, loss of taste from anterior tongue, loss of salivation (spares the eye and the eye)
300
global persistent hypotonia since birth, Japanese
Fukuyama congenital MD (or get weak, but have early contractures of hip, knee and ankle and skull asymmetry)
301
global persistent hypotonia since birth, eye issues
Walker Warburg
302
anti-ENA
negative risk of malignancy in polymyositis
303
ferritin level to supplement in RLS
<75
304
fragile X genetics
unstable expansion of CGG repeat on chr X
305
Cadasil chromosome
19! the gene is NOTCH 3
306
the only CGRP antagonist that acts against the receptor
Erenumab (AImovig) which also causes constipation!!
307
upper motor neuron of the nervous system
Betz cells, motor cortex
308
Tx Serotonin syndrome
cyproheptidine
309
SREDA is maximal
in parietal and posterior temporal regions
310
thalamus blood supply
peforating arteries from PCA
311
abducens + hypoglossal nerve palsy
Gottfredson's syndrome (metastatic tumor to the clivus)
312
MG tx in pregnancy
pyridostigmine (and prednisone, but C)
313
tx dykinesias from carbodopa-levodopa
amantidine (DBS if severe)
314
pure motor stroke
internal capsule
315
pure sensory stroke
thalamus
316
C6, C7, C8 dermatomes
6 is thumb, 7 is middle, 8 is pinkie
317
type 1 muscles
low and slow, more vascular, higher oxidative demand, Krebs cycle
318
upper extremity adduction and pronation, legs extended
decerberate (below red nucleus)
319
upper extremity adduction and flexion, legs extended with feet inward
decorticate (above red nucleus)
320
recovery from sleep deprivation
first recovery night has long N3 and short R
321
hemangioblastomas in eyes and cerebellum
von hippel lindau, AD, VHL gene on chr 3; also RCC and pheochromocytomas
322
spinocerebellar degeneration from a vitamin deficiency
Always E!
323
definitive Alzheimer's dx requires
biopsy
324
buspirone site of action
5HT-1A
325
macular edema
fingolimod
326
spinal nucleus of CN V
pain and temperature to all of the head
327
mesenceaphalic nucleus of CN V
jaw jerk reflex
328
chief nucleus of CN V
vibration, proprioception and light touch to face
329
intracellular reticulin deposition
pleomorphic xanthroastrocytoma
330
chicken wire vasculature
oligodendroglioma
331
bipolar hairlike astrocytes
pilocytic astrocytoma
332
shopping cart sign
L4/L5 + enlarged ligamentum flavum
333
vetrolateral preoptic nucleus
hypothalamic egulation of sleep cycles
334
holoprosencephaly most associated chr abnormality
Trisomy 13
335
distal greater than proximal weakness, sensory loss, axonal NCS in ICU
critical illness polyneuropathy
335
proximal weakness, decreased reflexes and normal sensory exam in ICU
critical illness myopathy
335
repetition deficits and phonemic paraphasic errors
conduction aphasia
335
predominantly proximal weakness, with sensory invovlement in ICU
critical illness polymyoneuropathy
336
pilocytic astrocytomas, WHO grade and prognosis
WHO grade 1, slow growing with good prognosis
337
cancer most commonly associated with opspclonus myoclonus
SCLC
338
adrenoleukodystrophy mechanism and treatment
inflammatory demyleination; steroids and bone marrow transplant
339
scapular winging, rectus abdominus weakness, child or young adult
calpain-3 deficiency
340
wilson's inheritance
AR
341
wilson's in pregnancy
treat with zinc acetate
342
palpable liver, reducing substances in the urine
galactosemia
343
erections
parasympathetics s2-s4
344
urine smells like sweaty feet
isovaleric acidemia
345
a group of normal neurons that did not migrate correctly
heterotopia
346
basal ganglia calicifications from HIV
more common in children
347
vertical gaze palsy
pineal tumors, compression of the rostral interstitial nucleus of the MLF
348
second generation antipsychotics
antangonize D2 and 5HT 2A
349
Homer-Wright Rosettes
medulloblastoma
349
CMT with acute attacks of ataxia, dysarthria, and weakness
CMT1X
350
symmetric Parkinsonism, lacking tremor, plus OSA or laryngeal stridor
Multi-system atrophy P (striatonigral degeneration)
351
most common neuro Behcets
relapsing remitting aseptic meningitis (5-20%)
352
first line tx for JME in girls
Keppra
353
proximal contractures, distal hyperlaxity, global hypotonia, no reflexes, first months of life
Ullrich's congenital MD
354
BPPV: otoliths separate from
Utrical and saccule
355
Iris hamaratomas
Lisch nodules! NF1
356
GABA B agonist
baclofen
357
seizures in sleep
commonly stage 2, never REM
358
bone marrow suppression, bleeding and bruising
carbamazepine
359
chorea and basal ganglia lesions in neonate
kernicterus, from bilirubin
360
colchicine vs statin myopathy
colchicine also causes axonal neuropathy!
361
steroid vs statin myopathy
steroid has normal CK, EMG doesn't show fibrillation
362
damaged by carbon monoxide toxicity
globus pallidus
363
topiramate in pregnancy
low birth weight, 1.4% facial cleft
364
is mesial temporal sclerosis a focal cortical dysplasia?
No! neuronal loss/gliosis
365
Neiman Pick C
cannot metabolize cholesterol or lipids; diagnose with Filipin test
366
Niemann-Pick A and B
Spingomylelinase deficiency in white blood cells.
367
tx atonic neurogenic bladder
cholinergics like Bethanechol
368
a farmer with diarrhea, miosis, diaphoresis
Acetylcholinesterase inhibition (organophosphate poisoning)
369
deep brain damage in a seizing baby, pyruvate dehydrogenase
Leigh disease, fatal; can also be mitochondrial
370
ASA ischemia
anterior cord: loss of pain and temperature, motor function
371
PML cells affected, median survival
Glial cells, 3 months
372
vestibular schwannomas arise within
internal acoustic meatus (ie the auditory canal)
373
CN IX-XI deficits
uvula deviation, weak head turn, taste loss, dysphagia: jugular foramen! (all originate in the medulla)
374
deficient branched chain amino acids and sweet smelling urine
maple syrup urine disease , tx with diet and HD
375
basophilic stippling of RBCs
lead poisoning
376
sniffing glue
n-Hexane, facial numbness and vision loss
377
glucocerebroside
Gaucher, lysosomal storage disorder, stores in liver and bones, AR
378
most common genetic disorder in Ashkenazi Jews
Gaucher, glucocerebroside deficiency
379
D-methylmalonyl-Co A deficiency
Methylmalonic acidemia, lethargy and FTF in first week of life, methylmalonic acid and propinionyl Co A accumulate (and can't metabolize VALINE)
380
MECP2
Rett syndrome, X linked, 6-18 month old girl with hand wringing
381
GFAP mutation
Alexander, frontal, Rosenthal fibers, NOT U-SPARING,
382
PKU
musty odor, can't convert phenylalalanine to tyronisine, AR
383
Frederich's ataxia transmission
AR
384
lissencephaly, low ear, upturned nose
Miller Deiker
385
big baby, low tone, clumsy, IDD, sometimes seizures
Soto
386
increase valine, leucine and isoleucine in urine
maple syrup urine disease, first week of life
387
Alpha galatosidase A deficiency
metachromatic leukodystrophy, diffuse, SPARES U
388
optic atrophy, exaggerated startle, FTF
Krabbe (galactoceramide )
389
U sparing, galactoceramide
Krabbe
390
cardiomegaly, hepatomegaly, macroglossia, muscle weakness
Pompe (Alglucosidase alpha)
391
Alglucosidase alpha
Pompe
392
ataxia, poor vision, mild proximal muscle weakness
NARP
393
hearing loss, gradual vision loss, no ataxia
Ushers
394
Eyelid/eye movement issues, deafness, ataxia, retinopathy, cardiac
Kearn-Sayres (can be mitochondrial)
395
macroorchidism
and tall, with IDD? FMR1, fragile X sydrome, it's a CGG repeat!
396
cherry red spot, hepatomegaly, normal til 3-5 months
Neiman Pick (1 and 2 are spingomylenase) ... foma cells?
397
cherry red spot, color blindness and convulsions
sialidosis (skin fibroblast alpha neuramidase)
398
cherry red spot, "startle seizures"
Tay Sachs, hexoamindase A
399
walks, scribbles if you do, stacks 2 cubes
12 month old
400
Age 2-12 with scoliosis, ataxia arms > legs, dysarthria and heart problesm
Fredrich's ataxia, AR
401
Sinusitis and ataxia
and telangectasias! its ataxia telangectasia. (AFP and CEA elevated)
402
marfanoid, pigeon chest, retinal detachments,
homocysteiuria, tx is pyridoxine
403
infantile spasms, oral rash, IDD
biotinidase deficiency
404
