1
Q
Balint syndrome lesion location
A
bilateral parieto-occipital lobes
2
Q
Balint Syndrome characterized by
A
optic ataxia, ocular apraxia, and simultagnosia
3
Q
optic ataxia
A
deficit of reaching objects under visual guidance
4
Q
ocular apraxia
A
inability to voluntarily move the eyes when oculomotor function is in tact
5
Q
simultagnosia
A
inability to visually perceive more than 1 object at a time
6
Q
prosopagnosia lesion location
A
bilateral fusiform gyri
7
Q
Anton syndrome localization
A
bilateral medial occipital lobes
8
Q
Anton syndrome characterized by
A
visual anosognosia characterized by cortical blindness, denial of such blindness, and confabulation
9
Q
myoclonus-dystonia genetics
A
autosomal dominant movement disorder associated with epsilon-sarcoglycan (SGCE) or DYT11 gene on chromosome 7q21
10
Q
myoclonus-dystonia characterized by
A
brief involuntary jerks mainly affecting necks, arm, and trunk as well as sustained twisting resulting from abnormal postures usually of the neck and/or hand.
begins in adolescence.
assoc with psych conditions.
alcohol improves
11
Q
central cord syndrome caused by
A
low-velocity, low-impact trauma in setting of pre-existing spondylotic disease
12
Q
MRI findings in corticobasal degeneration
A
midcallosal atrophy and asymmetric atrophy of the parieto-frontal cortex
13
Q
corticobasal degeneration histology
A
neocortical ballooned neurons, referred as achromatic neurons, and astrocytic plaques containing tau
14
Q
corticobasal degeneration characterized by
A
histologic findings of tauopathy.
strongly asymmetric limb rigidity/hemi-dystonia, akinesia or myoclonus, alien limb phenomenon, apraxia, and cortical sensory loss.
early gait instability and cognitive decline
15
Q
neurodegenerative conditions associated with REM sleep behavior disorder
A
parkinson disease, multiple system atrophy (MSA), and lewy body disease (LBD)
16
Q
neurodegenerative condition associated with ALS
A
FTD has an autosomal dominant inheritance pattern in up to 20% of cases and can be associated with ALS in up to 15% of patients
Path: ubiquitin or tau inclusions. Pick cells described, which are tau protein deposits within neurons
17
Q
Pick cells
A
tau protein deposits within neurons, can be seen in FTD
18
Q
AD pathology
A
extracellular deposition of amyloid and intracellulary neurofibrillary tangle depositions.
19
Q
alpha-synuclein deposition in cortical layers V and VI
A
LBD.
20
Q
MRI in neurosarcoidosis
A
patchy and thick leptomeningeal enhancement, hypothalamic and pituitary involvement, and other less specific white matter lesions
21
Q
lateral medullary syndrome
A
ipsilateral horner syndrome, sensory loss of ipsilateral hemiface, dysarthria and dysphagia, ipsilateral ataxia.
22
Q
third order neuron lesion of the oculosympathetic pathway
A
partial Horner with mi0osis, anisocoria more apparent in dim light (impaired sympathetic response) and ptosis. contralateral hemineglect can also be a feature
23
Q
first order sympathetic neurons
A
arise from posterolateral hypothalamus
24
Q
what can confirm Horner syndrome
A
cocaine and apraclonidine
25
cocaine MOA
monoamine neurotransmitter reuptake in hibitor
26
apraclonidine MOA
direct adrenergic agonist
27
differentiate Horner syndrome third order from first/second order
hydroxyamphetamine
third order lesion: eye will not dilate
first or second order lesion: eye will dilate
28
GEFS+
SCN1A most commonly.
hereditary epilepsy characterized by febrile seizures plus febrile seizures out of the age limit, and nonfebrile seizures
29
foot drop with L5 radiculopathy
additional weakness of hip abduction and internal rotation and plantar inversion and eversion, as well as tibialis anterior
30
L5 root supplies what muscles
gluteus maximus, internal hamstring, biceps femoris, gltueus medius, TFL, peronei, TA, EHL, EDL, EDB, TP, FDL, FHL
31
deep peroneal nerve lesion
dorsiflexion and first web space sensory loss
32
common peroneal nerve lesion
weak dorsiflexion/eversion and loss of anterolateral leg, dorsum of foot sensory
33
sciatic nerve lesion
weak dorsiflexion, plantarflexion, inversion, eversion, and knee flexion; abnormal sense posterior thigh, anterolateral and dorsum of leg
34
L5 radiculopathy lesion
weak dorsiflexion, plantarflexion, inversion, eversion, hip abduction, internal rotation, hip extension, sometimes isolated toe extensor weakness; variable sensory, back pain radiating down the leg
35
effective management for major depressive disorder in parkinsons
bupropion
36
sumatriptan MOA
agonist of 5-HT1B and 5-HT1D
37
targeted ALS treatment with SOD1 pathogenic variant
toferson, gene silencing therapy
38
edaravone
free-radical scavenger that slows functional deterioration in ALS which is thought to reduce oxidative stress, recommended for all patients with ALS
39
most common neuropsych symptom in patients with Huntington
depression
40
LBD
presence of Lewy Body in the midbrain and cortex and formed by alpha synuclein protein aggregates
41
beta amyloid
neuritic plaques characteristic of Alzheimer
42
tau-opathy
FTD, AD with secondary tau pathology
43
TDP-43
ALS with dementia, FTD
44
LBD characterized by
dementia plus 2/4 core features: cognitive fluctuations, visual hallucinations, rapid eye movement sleep behavior disorder, and parkinsonism
45
PET in LBD
glucose hypometabolism in the occipital lobe
46
PET in AD
glucose hypometabolism in the temporal and parietal lobes
47
PET in Huntington's
glucose hypometabolism in the head of the caudate
48
PET in FTD
glucose hypometabolism in the frontal and temporal lobes
49
best antipsychotic with LBD
avoid antipsychotic due to sensitivity. reactions can be severe. Quetiapine and clozapine have shown better tolerability
50
meningocele
sac protrusion with no spinal cord
51
anosodiaphoria
preserved awareness of a neurologic deficit despite emotional indifference and/or lack of insight into functional impairment
R frontal lesions
52
frontal lesions stroke
anosodiaphoria, gaze preference, abulia, apraxia, executive dysfunction, incontinence, unilateral motor weakness, and disinhibition
53
most anterior tract of the spinal cord
anterior corticospinal tract
54
Barrow classification
used to differentiate types of carotid-cavernous fistulas
Type A: high flow - direct connection between ICA and cavernous sinus
Type B: low flow - indirect connection between the dural branches of the ICA and the cavernous sinus
Type C: low flow - indirect connection between the dural branches of the ECA and the cavernous sinus
Type D: low flow - indirect connection between the dural branches of the ICA and ECA and the cavernous sinus
55
oculomotor nerve palsy
unreactive, dilated pupil, ptosis, and inability of the eye to elevate or adduct
56
atherosclerosis on histology
foam cells and atheroma formation
57
LIBERTY study
long-term safety and efficacy of erenumab monotherapy through 112 weeks.
58
erenumab
calcitonin gene-related peptide (CGRP) receptor antagonist for prevention of migraine
59
phencyclidine MOA
NMDA-R antagonist
60
cocaine MOA
inhibits re-uptake of dopamine and norepinephrine
61
ethanol MOA
GABA-A receptor agonist
62
amphetamine MOA
direct release of dopamine, serotonin, and norepinephrine
63
phencyclidine intoxication
bizarre behaviors, belligerence, superhuman-appearing strength, and hallucinations
64
polymyositis
inflammatory myopathy characterized by the presence of inflammatory infiltrates in striated muscle
65
muscle biopsy in polymyositis
endomysial inflammatory cells
66
dermatomyositis
acute or insidiously progressive proximal weakness accompanied by or preceded by a characteristic skin rash
67
dermatomyositis histology
perifascicular atrophy with increased endomysial connective tissue and inflammatory infiltrates
68
inclusion body myositis
over 40 with insidious onset involving both the proximal and distal musculature in a symmetric or asymmetric fashion, starting in the flexor forearm muscles, particularly deep finger flexors
69
inclusion body myositis histology
atrophic and hypertrophic fibers with internal, rimmed vacuoles
70
necrotizing autoimmune myopathy
subacute, progressive, proximal muscle weakness without a rash, generally more rapidly than polymyositis
71
necrotizing autoimmune myopathy histology
scattered necrotic muscle fibers surrounded by sparse inflammatory cells, predominantly lymphocytes and occasionally some CD4+ and CD8+ T cells
72
McArdle disease presentation
exercise intolerance, contractures, myoglobinuria, and rhabdo
73
McArdle disease deficiency
myophosphorylase
74
myophosphorylase
enzyme found in muscle that breaks down glycogen, thereby giving substrates for glycolysis
deficient in McArdle disease
75
McArdle disease diagnosis
four features: exercise intolerance, high CK levels, 1 or more episodes of elevated CK after exercise, and the "second wind" phenomenon
76
Pompe disease
presents in infants as hypotonia and cardiac failure and in adults as proximal muscle weakness and atrophy
77
Pompe disease deficiency
lysosomal acid alpha-glucosidase
78
Von Gierke disease
hepatomegaly, hypoglycemia, and growth retardation.
79
Von Gierke disease deficiency
glucose-6-phosphatase
80
Von Gierke disease lab findings
elevated lactate and uric acid
81
Galactosemia
presents with failure to thrive, intellectual disability, and infantile cataracts
81
Galactosemia deficiency
galactose-1-phosphate uridyltransferase
82
Galactosemia lab findings
aminoaciduria
83
Cori disease
hepatomegaly, hypoglycemia, weakness, atrophy, and cardiomyopathy
84
Cori disease deficiency
liver and muscle debranching enzyme
85
internal capsule parts
anterior limb, genu, and posterior limb
86
anterior limb of internal capsule fibers
frontopontine fibers and anterior thalamic radiation fibers
87
genu of internal capsule fibers
corticobulbar tract
88
posterior limb of internal capsule fibers
sensory fibers from the thalamus int he most anterior portion and corticospinal tract fibers in the most posterior portion
89
corticobulbar tract
carries upper motor neurons from the motor cortex to cranial nerve nuclei that govern motion of striated muscle in the head and face
90
pure motor syndrome stroke
small vessel stroke in the internal capsule
91
corticospinal tract
white matter motor pathway connecting the cortex and the medullary pyramids and are primarily involved in carrying the motor function of the spinal nerves
92
dystonic storm first line treatments
trihexyphenidyl and tetrabenazine (most often), benzodiazepines, baclofen, and gabapentin
93
dystonic storm breakthrough dystonia treatment
propofol
94
refractory dystonic storm
botox for focal or segmental dystonia, intrathecal baclofen, pallidotomy, and DBS of GPi may be considered
95
status dystonicus/dystonic storm
increasingly frequent or continuous severe generalized dystonic contractions which may be refractory to medical treatment
generally occurs in acquired dystonia but can occur in genetic causes of dystonia
96
risk factors of critical illness neuropathy
prolonged mechanical ventilation, multiorgan failure, persistent systemic inflammation. hyperglycemia.
97
critical illness myopathy
symmetric flaccid weakness of extremities that usually spares the facial and extraocular muscles
caused by loss of myofibrillar proteins and loss of myosin
98
critical illness polyneuropathy caused by
caused by severe axonal dysfunction without any major demyelination
99
medically refractory essential tremor
adaptive device with TENS unit for noninvasive treatment
100
trofinetide
DMT for Rett syndrome
most common adverse effect is diarrhea
101
Rett syndrome genetics
MECP2 on X chromosome
102
manganese toxicity
parkinsonism
103
cyanide toxicity
encephalopathy, headaches, nausea, vomiting
complaint of a bitter almond odor is characteristic
104
mercury exposure
personality changes, psychosis, and cerebellar dysfunction; also inflammed gingiva
mining and thermometer factories
105
organophosphates toxicity
encephalopathy, salivation, lacrimation, bradycardia, and other parasympathetic manifestations
insecticides
106
arsenic toxicity
symmetric sensorimotor neuropathy
garlic breath highly suggestive of arsenic poisoning
107
urea cycle defect presentation
vomiting, decreased feeding, hyperventilation. hyperammonemia and respiratory alkalosis
108
urea cycle defect with decreased serum citrulline and arginine levels and increased urinary orotic acid level
ornithine transcarbamoylase deficiency
109
ornithine transcarbamoylase deficiency genetics
X-linked
most common urea cycle disorder
110
urea cycle defect with decreased serum citrulline and arginine levels and normal orotidine/orotic acid level
carbamoylphosphate synthetase I deficiency
111
urea cycle defect with increased citrulline level, decreased arginine level, and absent arginosuccinate aciduria
argininosuccinate synthetase deficiency
112
urea cycle defect with increased serum citrulline level, decreased/normal serum arginine level, with arginosuccinate aciduria
argininosuccinate lyase deficiency
113
findings in argininosuccinate lyase deficiency
hepatomegaly and trichorrhexis nodosa in late-onset form
114
increased arginine level
arginase deficiency
115
urea cycle
116
meds that cause drug-induced parkinsonism
block dopamine signaling
risperidone, metoclopramide, quetiapine, valproic acid
117
dialectal behavior therapy
used to treat intense emotional reactions and relationship difficulties
118
Mcdonald Criteria for MS diagnosis
>2 attacks, >2 lesions = dx
>2 attacks, 1 lesion = demonstrate DIS with MRI or clinical attack in different site
1 attack, >2 lesions = demonstrate DIT with MRI, second clinical attack, or OCBs in CSF
1 attack, 1 lesion = demonstrate DIS and DIT
119
primary progressive MS diagnosis
1 year of disease progression (retrospective or prospective) and 2 of the following:
- >/= 1 T2 lesions, either periventricular, juxtacortical, cortical, or infratentorial
- >/= 2 T2 lesions in the spinal cord
- OCBs in CSF
120
most common finding in uremia
encephalopathy with myoclonus
121
three layers of supportive tissue for peripheral nerves from outer to inner
epineurium, perineurium, and endoneurium
122
Isaacs syndrome
VGKC complex autoantibodies
acquired neuromyotonia
123
Isaacs syndrome presentation
painful muscle cramps, slow muscle relaxation after contraction and hyperhidrosis
124
monozygotic twins and MS
concordance rate of 20-30%
125
dizygotic twins and MS
concordance rate of 2-5%
126
strongest genetic factor in MS
HLA*DRB1*15:01 allele
127
fingolimod MOA
blocks sphingosine 1-phosphate receptor (S1PR) keeping lymphocytes in lymph nodes
128
ocrelizumab MOA
depletion of B-cells with antigen CD20
129
glatiramer acetate MOA
induces and activates T-lymphocyte suppressor cells specific for myelin antigen
130
natalizumab MOA
blocks integrin-adhesion molecule interactions and inhibits migration of T-lymphocyte migration into the CNS
131
dimethyl fumarate MOA
anti-inflammatory and cytoprotective properties via activation of the Nrf2 pathway
132
TOR1A genetics
autosomal dominant dystonia with incomplete penetrance
133
TOR1A presentation
lower body dystonia progressing to other limbs and torso with onset in 2nd decade
134
dopa-responsive dystonia (DYT-GCH1) genetics
autosomal dominant mutation in GCH1 -> deficient GTP cyclohydrase
135
dopa-responsive dystonia features
dystonia in legs with features of parkinsonism, dramatic response to levodopa/carbidopa
onset 1st decade
136
GNAL features
focal-onset dystonia of the cranial and cervical musculature in an adult
137
paroxysmal exertional dyskinesia
recurrent attacks of chorea, athetosis and dystonia followed by prolonged physical exercise
138
cause of paroxysmal exertional dyskinesia
mutation in SLC2A1 -> deficient GLUT-1 transporter
139
paroxysmal kinesigenic dyskinesia
attacks triggered by sudden movement (not sustained activity)
140
SLC2A1/GLUT-1 deficiency phenotypes
paroxysmal exertional dyskinesia OR infantile encephalopathy and epilepsy
141
Westphal variant
Huntington disease with unique juvenile onset presentation
142
Westphal variant of Huntington disease presentation
rigidity, dystonia, and bradykinesia more so than choreoathetosis in typical presentation. cognitive impairment and seizures.
143
imaging of Toxo
multiple ring-enhancing lesions, commonly in basal ganglia and gray-white junction
144
Kufor-Rekab Syndrome (KRS)
rare, autosomal recessive subtype of juvenile Parkinson disease caused by genetic mutation of ATP13A2
symptom onset younger than 21 years old
145
early onset parkinsons disease age
< 60
146
young-onset parkinsons disease age
21-40
147
juvenile parkinsons disease age
<21
148
genetic mutations associated with early onset PD
SCNA (PARK1, PARK4)
PARK7
PINK1
149
genetic mutations associated with late-onset PD
LRRK2
150
genetic mutations associated with juvenile onset PD
ATP13A2
151
genetic mutations associated with young-onset PD with dystonia
PARK2
152
genetic mutations associated with adult-onset PD with dystonia
PARK14
153
genetic mutations associated with early-onset atypical parkinsonism
PARK9
154
lacunar infarcts on autopsy
liphyalinosis and microatheroma formation in cerebral blood vessels
155
classic presentations of lacunar infarcts
- pure hemiparesis
- pure hemianesthesia
- mixed sensory-motor hemiparesis/hemianesthesia
- ataxia hemiparesis
- dysarthria-clumsy hand syndrome
156
carbamazepine MOA
extends inactivated phase of voltage-dependent sodium channels
157
carbamazepine common side effects
nausea, vomiting, ataxia, dizziness, drowsiness, confusion, constipation, diarrhea, hyponatremia, rash, pruritus, and fluid retention
158
carbamazepine significant drug interactions
cytochrome P450 inducer, auto-induces metabolism
159
lacosamide MOA
enhances slow inactivation of voltage-dependent sodium channels
160
lacosamide common side effects
dizziness, nausea, vertigo, abnormal coordination, ataxia, and PR prolongation
161
lacosamide drug interactions
none
162
lamotrigine MOA
inactivating voltage-dependent sodium channels; decreased synthesis of glutamate/aspartate
163
lamotrigine common side effects
rash, hypersentivity reactions, Stevens Johnson syndrome, and cardiac arrhythmias
164
lamotrigine drug interactrions
clearance increased by estrogen-containing hormone replacement; decreased clearance with valproate; increased clearance with cytochrome P450 inducer
165
phenytoin MOA
blocks voltage-dependent neuronal sodium channels
166
phenytoin common side effects
gingival hypertrophy, body hair increase, rash, folic acid depletion, decreased bone density, confusion, slurred speech, double vision, ataxia, and neuropathy
167
phenytoin drug interactions
cytochrome P450 inducer, enhances glucuronidation
168
zonisamide MOA
blocks both voltage-dependent sodium and T-type calcium channels; weak carbonic anhydrase inhibitor
169
zonisamide common side effects
somnolence, ataxia, anorexia, confusion, abnormal thinking, nervousness, fatigue, and dizziness
170
zonisamide drug interactions
none
171
isolated benzo poisoning
CNS depression and normal vital signs from increased GABA activity in CNS
172
Lafora body
fatal autosomal-recessive condition leading to intracytoplasmic inclusions found in heart, liver, muscle, and skin
aggregate of polyglucosans
173
Lewy body
composed of alpha-synuclein
LBD, PD, MSA
174
Bunina bodies
found in ALS
abnormal aggregates of TDP-43 found in motor cortex, brainstem, and spinal cord
175
Pick body
found in Pick disease
silver aggregates of taue protein
176
neurofibrillary tangles
Alzheimer disease
hyperphosphorylated tau protein
177
Tuberous sclerosis genetics
TSC1 - ch9
TSC2 - ch16
178
VHL genetics
ch3
autosomal dominant
179
NF2 genetics
ch22
autosomal dominant
180
neuropsych manifestations of juvenile SLE
headache, cognitive disorder, and seizure
181
cause of left superior homonymous quadrantanopsia
right temporal lobe tumor
182
pre-chiasmatic lesions
monocular vision loss
183
chiasmatic lesions
bitemporal anopsias
184
post-chiasmatic lesions
homonymous anopsias
185
occipital cortex lesions
homonymous anopsias with macular sparing
186
cause of center sector sparing sectoranopsia
anterior choroidal artery infarct
187
junctional scotoma
lateral optic chiasm compresses ipsilateral optic nerve and contralateral inferonasal nerve fiber -> ipsilateral monocular visual loss and contralateral temporal visual field defect
caused by skull base meningiomas and ACA aneurysms
188
center involving sectoranopsia
posterior choroidal artery infarct
189
FTD clinical manifestations
abnormal social conduct, apathy, compulsions, and eating disorders
190
lowest risk of drug induced parkinsonism
quetiapine and clozapine
191
ropinirole side effect
sleep attack
avoid dopamine agonists if these occur
192
RLS first line treatments
gabapentin
ripinirole
193
risk of infective endocarditis
congenital cyanotic heart defect
194
histopathology of Wallerian degeneration
neurofibril degradation distal to the lesion
195
recent travel to central Africa with subsequent development of confusion, sleep disturbance, and arthralgias
African sleeping sickness
196
treatment for African sleeping sickness
fexinidazole for Trypanosoma brucei gambiense (more indolent) and melarsoprol for trypanosoma brucei rhodesiense (more aggressive)
197
fingolimod main side effect
AV block
- also HA, elev LFTs, macular edema, abdominal distress, leukopenia, inc risk of infections
198
ocrelizumab main side effect
URI
- also infusion reaction, edema, neutropenia
199
dimethyl fumarate common side effect
flushing
- also nausea and diarrhea, angioedema, lymphopenia, rare cases of PML
200
alemtuzumab common side effect
thyroid disease
- also headache, rash, abdominal distress, infusion reaction, fever
201
alemtuzumab MOA
CD52 monoclonal antibody
202
natalizumab side effect
PML
- also hepatotoxicity, fatigue, allergic reaction
203
glatiramer acetate side effect
injection site pain
- also hypersensitivity reaction, nausea, edema
204
teriflunomide side effect
teratogenicity
- also hypersensitivity reactions, nausea, headache, hepatotoxicity
205
teriflunomide MOA
pyrimidine synthesis inhibitor
206
pantothenate kinase-associated neurodegeneration
rare genetic movement disorder with progressive movement disorder, subcortical dementia (impaired executive function, processing speed, and attention), and "eye of the tiger" sign on brain MRI
207
PKAN genetics
autosomal recessive cause of neurodegeneration with brain iron accumulation secondary to mutation in the PANK2 gene on chromosome 20
208
classic PKAN
rapidly progressive choreoathetosis in 1st decade of life
209
atypical PKAN
slowly progressive, leading to movement disorder in the 2nd-3rd ecade of life
210
Kufor-Rakeb syndrome
PARK9, ATP12A2 gene mutation. AR.
parkinsonism without tremor, spasticity, supranuclear upgaze paresis, and dementia. brain imaging may demonstrate marked atrophy of the globus pallidus
211
Wilson disease
ATP7B gene mutation. AR.
hepatic, neurologic, and psychiatric symptoms. 5-35y. Neuro: dysarthria, gait abnormalities, dystonia, tremor, and parkinsonism. Typical brain MRI include hyperintensities in the tectal plate and central pons
212
Huntington's disease
juvenile onset HD: CAG expansion. dystonia, ataxia, seizures, akinetic-rigid parkinsonism syndrome, minority
213
PLA2G6-associated neurodegeneration
PLA2G6 gene mutation, aka infantile neuroaxonal dystrophy. 2-3y with loss of distal sensation, hypotonia, and hypoactive DTRs. central symptoms include optic atrophy, hypothalamic dysfunction, hypothyroidism, cognitive decline, and spasticity
214
sleep spindles
short bursts of bilateral and synchronous, waxing and waning, rhythmic waves with frequency of 12-16Hz and maximum in frontal and central regions. associated with K complexes
N2
215
K complexes
high amplitude diphasic waves maximum in the frontocentral region.
N2
216
N2 sleep features
spindles, K complexes, POSTS, vertex waves, <20% delta waves
217
N1 sleep features
decrease of alpha rhythm and admixed theta activity, slow rolling eye movements, POSTS, and vertex waves
218
N3 sleep features
less-pronouned K complex and sleep spindles, >20% delta
219
REM sleep features
muscle atonia with low-voltage background and REMs
220
vestibular schwannoma pathology
Antony A and B fibers
221
Alexander disease pathology
rosenthal fibers in chronic gliosis
also seen in pilocytic astrocytoma
222
medulloblastomas pathology
small round blue cells
incl neuroblastomas, medulloblastomas
223
CJD pathology
spongiform changes
224
malignant tumors pathology
atypical cells and nuclear hyperchromasia, necrosis, increased mitotic figures, and angiogenesis
225
migraine-associated triggers
emotional stress, menstrual-induced, sleep disturbance, food and alcoholic beverages, and weather changes.
79% report stress as trigger
226
behavioral variant of FTD
most common subtype
personality and behavioral changes, such as disinhibition, apathy, hyperorality with increased carbohydrate cravings, and compulsive behaviors
227
myalgic encephalomyelitis
chronic fatigue syndrome characterized by >/= 6 months of persistent fatigue despite adequate rest and exclusion of another cause that would otherwise explain fatigue symptoms
3 main symptoms (fatigue, post-exertional malaise, unrefreshing sleep) + 1 of the following (cognitive impairment, orthostatic intolerance)
228
Global Cortical Atrophy Scale score 0
normal volume/no ventricular enlargement
229
global cortical atrophy scale score 1
opening of sulci/mild ventricular enlargement
230
global cortical atrophy scale score 2
volume loss of gyri/moderate ventricular enlargement
231
global cortical atrophy scale score 3
severe (knife-blade) atrophy/severe ventricular enlargement
232
brain regions evaluated for global cortical atrophy scale
sulcal dilation: frontal (L&R), parieto-occipital (L&R), temporal (L&R)
ventricular dilation: frontal (L&R), parieto-occipital (L&R), temporal (L&R), third ventricle
233
signs/symptoms of chiari 1
headache that is worse with valsalva maneuvers, usually occipital or nuchal and paroxysmal.
other symptoms: balance problems, neck pain, dizziness, vision problems, poor hand coordination, numbness and tingling of hands and feet, change in bladder/bowel habits, trouble swallowing, hoarseness
234
iliohypogastric nerve
numbness of hypogastric and lateral gluteal areas and abdominal wall weakness
can be injured in abdominal surgeries
235
X-ALD etiology and genetics
ABCD1 gene on chromosome Xq28
236
X-ALD childhood cerebral phenotype signs and symptoms
age of onset: 3-10y. ADHD, behavior problems, loss of speech, vision, and ambulation
237
X-ALD adolescent cerebral phenotype signs and symptoms
age of onset: 11-21y. similar symptoms to childhood phenotype, but slower progression
238
X-ALD adult cerebral phenotype signs and symptoms
dementia, behavior problems, focal deficits
239
X-ALD adrenomyeloneuropathy signs and symptoms
age of onset: young adult/adult. spinal cord (eg walking), adrenal insufficiency
240
X-ALD adrenal insufficiency signs and symptoms
age of onset: school-aged children. isolated adrenal insufficiency. most later develop adrenomyeloneuropathy
241
X-ALD diagnosis
elevated VLCFA levels
genetic testing
newborn screen
242
X-ALD imaging findings
T2 hyperintense white matter most often in parietal and occipital region and involves the posterior corpus callosum
243
X-ALD treatment
treatment of adrenal insufficiency
HSCT with or without gene therapy
244
globoid cell leukodystrophy (Krabbe disease) etiology and genetics
galactosylceramidase enzyme deficiency
GALC on chromosome 14q31
245
globoid cell leukodystrophy (Krabbe disease) early-onset infantile signs and symptoms
most common. age of onset <6mo. rapid progression through 3 stages
Stage 1: irritable, failure to thrive, siff and clenched fists
Stage 2: rapid progression of stiffness, opisthotonos, optic atrophy, seizure-like episodes
Stage 3: burnt out decerebrate posturing, loss of vision, loss of hearing, no movement due to spasticity
246
globoid cell leukodystrophy (Krabbe disease) late-onset infantile, juvenile, and adult signs and symptoms
spasticity, paraparesis, visual impairment, ataxia
247
globoid cell leukodystrophy (Krabbe disease) diagnosis
low galactosylceramidase levels
genetic testing
newborn screen
248
globoid cell leukodystrophy imaging
T2 hyperintense white matter periventricular or parietal occipital
newborn may be normal
249
globoid cell leukodystrophy treatment
HSCT
250
metachromatic leukodystrophy etiology and genetics
arylsulfatase A enzyme deficiency
ARSA gene on 22q13.3
251
metachromatic leukodystrophy all phenotypes signs/symptoms
peripheral neuropathy
gallbladder polyps
252
MLD late infantile signs/symptoms
most common. Age of onset <30 months
developmental regression, hypotonia then hypertonia, spasms
253
MLD juvenile signs/symptoms
age of onset: before puberty.
behavior problems, personality changes, gait abnormalities, upper motor neuron signs
254
MLD adult signs/symptoms
age of onset: after puberty
dementia, behavior problems
255
MLD diagnosis
low arylsulfatase A activity + urinary excretion of sulfatides
newborn screening
256
MLD imaging
T2 hyperintense white matter that is periventricular with a tigroid appearance
257
MLD treatment
HSCT
trials of gene therapy ongoing
258
Aicardi-Goutieres syndrome etiology and genetics
Nucleic acid metabolism and signaling from 1 of 7 genes TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR, or IFIH1
259
Aicardi-Goutieres syndrome prenatal signs/symptoms
abnormal neurologic symptoms from birth, hepatomegaly, thrombocytopenia
260
Aicardi-Goutieres syndrome perinatal signs/symptoms
abnormal neurologically from birth
261
Aicardi-Goutieres syndrome infant form signs/symptoms
most common. developmental regression, spasticity, dystonia, acquired microcephaly, glaucoma
262
Aicardi-Goutieres syndrome childhood form signs/symptoms
variable motor presentation, glaucoma
263
Aicardi-Goutieres syndrome diagnosis
clinical picture + radiologic findings are diagnostic
CSF findings of lymphocytic pleocytosis, and elevated neopterin and biopterin may be helpful
264
Aicardi-Goutieres syndrome treatment
none
265
Aicardi-Goutieres syndrome imaging
calcifications in the basal ganglia, deep white matter, thalamus, and dentate nuclei of the cerebellum
cerebral atrophy, temporal lobe swelling and cyst, temporal horn dilation
266
cerebrotendinous xanthomatosis etiology and genetics
sterol 27-hydroxylase deficiency on CYP27A1 gene
267
cerebrotendinous xanthomatosis signs/symptoms
neonatal jaundice, infantile chronic diarrhea and elevated liver transaminase and bilirubin levels, juvenile cataracts and Achilles xanthoma, xanthomas in other organs
268
cerebrotendinous xanthomatosis diagnosis
elevated serum cholestanol, bile alcohols in the urine
newborn screen
269
cerebrotendinous xanthomatosis imaging
T2 hyperintense white matter in cerebellum and dentate nuclei
270
cerebrotendinous xanthomatosis treatment
chenodeoxycholic acid or cholic acid
271
Microglia on histo
CD68-expressing cells
involved in pathogenesis of MS
272
temporal lobe epilepsy auras
psychic phenomena (cogntiive, deja vu, fear)
olfactory or gustatory hallucinations
autonomic features (lightheadedness, flushing)
vertigo, epigastric rising sensation
273
temporal lobe epilepsy ictal features
restlessness or agitation
speech arrest or aphasia
confusion
oral or manual automatisms (chewing, lip smacking, picking with hands [usually ipsilateral])
dystonic posturing of the contralateral limb
early head turning is ipsilateral and forceful; late head turning is contralateral
274
temporal lobe epilepsy postictal features
the confusion usually resolves within minutes
psychosis (not immediate, lasts days to weeks)
ipsilateral nose wiping
aphasia
Todd paralysis (contralateral hemiplegia)
275
MRI findings in temporal lobe epilepsy
hippocampal sclerosis with increased hippocampal T2 signal, hippocampal atrophy, and changes in hippocampal morphology or architecture
276
when do sleep terrors occur
in NREM during first third of the night in stage 3 of sleep with low frequency delta waves
277
common peroneal vs L5 radic
in common peroneal will have intact hamstrings reflex, sparing of ankle inversion, well demarcated borders of sensory loss on lateral calf and dorsum of foot
symptoms may be provoked by forced inversion of foot as opposed to straight leg raise
278
SPECT
single photon emission computed tomography is used to localize area of seizure onset, most useful in the ictal phase during which areas of increased cerebral blood flow are seen in the seizure onset zone
more sensitive when tracer is injected within 20 seconds of seizure onset and if seizures last for more than 30 seconds
interictal SPECT can sometimes show areas of decreased perfusion, but ictal is more sensitive
279
most commonly seen feature in Wilson disease
Kayser-Fleischer rings (abnormal color in the limbus of the cornea)
280
treatment for severe generalized spasticity
intrathecal baclofen
281
brainstem auditory evoked potentials
objective measures of the auditory pathway from the auditory nerve to the brainstem performed via surface electrodes placed on the scalp and forehead and measure the electrical activity after an acoustic stimulus. there are 7 peaks named 'waves' corresponding to a specific location along the auditory pathway
282
brainstem auditory evoked potential wave 1 anatomic site & clinical relevance
distal auditory nerve (near cochlea)
- delayed latency in conductive loss
reduced amplitude or absent in sensory (cochlear) or neural (auditory nerve) sensorineural loss
283
brainstem auditory evoked potential wave 2 anatomic site & clinical relevance
proximal auditory nerve/cochlear nucleus
- affected in severe auditory nerve dysfunction
284
brainstem auditory evoked potential wave 3 anatomic site & clinical relevance
superior olivary complex (caudal pons)
prolonged I-III interpeak interval in neural sensorineural loss
normal in conductive loss
285
brainstem auditory evoked potential wave 4 anatomic site & clinical relevance
lateral lemniscus (rostral pons)
affected in central lesions
286
brainstem auditory evoked potential wave 5 anatomic site & clinical relevance
inferior colliculus (midbrain)
delayed latency in central brainstem pathology
normal in conductive loss
287
brainstem auditory evoked potential wave 6 anatomic site & clinical relevance
medial geniculate nucleus
affected in supratentorial lesions involving the internal capsule and basal ganglia
288
brainstem auditory evoked potential wave 7 anatomic site & clinical relevance
auditory cortex
frequently absent in normal individuals
preserved in the bilateral auditory cortex or radiation lesions. literature indicates that this wave may have a subcortical (rather than cortical) origin; therefore, abnormalities are less reliable than with wave 6
289
presentation of spinal epidural abscess
fever, severe pain, and neurologic deficits
most often MSSA
290
visual-evoked potential in MS
unilaterally prolonged P100 waveform with preserved morphology due to demyelination of the ipsilateral optic nerve
291
split-hand syndrome
characterized by thenar weakness and atrophy and hypothenar preservation. NCS can show diminished amplitudes in opponens pollicis and abductor pollicis brevis with normal amplitudes in the abductor digiti minimi
supportive of diagnosis of ALS
292
when to admin tetanus vaccine iso of MS relapse
immediately after deep wound if it has been 5 years or longer since the last dose of vaccine, even in the setting of active multiple sclerosis relapse
293
myokymic discharges
radiation-induced neuropathy
regular or irregular discharge of a group of motor units, which can sound like soldiers marching in the snow
294
myotonic discharges
classic "dive-bomber" sound, common in muscle fiber membrane channelopathies, congenital myotonias, myotonic dystrophy, and hyperkalemic periodic paralysis
295
National Alzheimer's Project Act
requires the Secretary of the US DHS to establish the National Alzheimer's Project to achieve goals toward research, diagnosis, and treatment of Alzheimer's disease
296
2021 update to the National Alzheimer's Project Act
accelerate the action to promote healthy aging and reduce risk factors for Alzheimer's disease
297
medications recommended in treatment of Sydenham chorea
penicillin - antibiotic prophylaxis to prevent group A strep infections leading to recurrent acute rheumatic fever and rheumatic heart disease
298
myoclonic seizures on EEG
time-locked polyspike and wave complexes
299
key features of semantic primary progressive aphasia
fluent speech with single-word deficits
object deficits
surface dyslexia and dysgraphia
no speech apraxia, grammar deficits, or repetition deficits
300
localization of semantic primary progressive aphasia
anterior and ventral left temporal lobes
301
key clincopathologic correlation of semantic primary progressive aphasia
predominantly frontotemporal lobar degeneration with TDP-43 pathology
302
key features of nonfluent/agrammatic primary progressive aphasia
nonfluent speech with speech apraxia
grammar deficits
no single-word comprehension, object identification, or repetition deficits
303
localization of nonfluent/agrammatic primary progressive aphasia
left inferior frontal lobe
304
key clinicopathologic correlation of nonfluent/agrammatic primary progressive aphasia
predominantly frontotemporal lobar degeneration with tau pathology
305
key features of logopenic primary progressive aphasia
relatively fluent speech with repetition deficits
no speech apraxia, single-word comprehension, object identification, or grammar deficits
306
localization of logopenic primary progressive aphasia
left inferior parietal and posterior temporal lobes
307
key clinicopathologic correlation of logopenic primary progressive aphasia
predominantly Alzheimer's disease
308
retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations
due to TREX1 gene mutation leading to progressive retinal vasculopathy, focal and global CNS manifestations, psychiatric disturbances, mild liver and renal impairments, hypertension, and Raynaud phenomenon
309
order of needle traversing in LP
skin, subQ tissue, supraspinous ligament, interspinous ligament, ligamentum flavum, epidural space, dura, arachnoid, and subarachnoid space
310
DVA
abnormal venous drainage pathways that drain healthy, functional brain tissue and can be seen on contrast-enhanced MRIs as a medusa head or palm tree sign
311
POEMS syndrome
polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes is a rare paraneoplastic syndrome with ascending painful peripheral neuropathies with monoclonal gammopathy and kappa light chains
312
opioid-associated amnestic syndrome
leads to acute anterograde amnesia and bilateral symmetric hippocampi hyperintensity with restricted diffusion
313
prognosis in concussion
most consistent prognostic indicator is severity of symptoms within the first few days after a head injury
314
treatment for prolactinoma
ergot derivate dopamine agonists such as bromocriptine and cabergoline
315
lamotrigine added to VPA adjunct
dose should be titrated slowly, but can lead to drug rash because VPA inhibits LAM metabolism by blocking hepatic glucuronidation -> increased serum levels
316
secondary progressive MS vs primary progressive MS
relapsing-remitting course at beginning of their disease before they develop progressive disability vs primary progressive occurring at the onset
317
treatment for active secondary progressive MS
siponimod
318
treatment for moderate to severe Alzheimer's disease
memantine
319
memantine MOA
noncompetitive NMDA receptor antagonist that inhibits glutamate stimulation
320
donepezil MOA
pure acetylcholinesterase inhibitor
321
galantamine MOA
combined acetylcholinesterase inhibitor and allosteric nicotinic modulator
322
rivastigmine MOA
combined acetylcholinesterase and butyrylcholinesterase antagonist
323
selegiline MOA
potent irreversible inhibitor of MAO with greater affinity for MAO-B playing a major role in catabolism of dopamine, serotonin, norepinephrine, and epinephrine
324
memantine SE
fewer GI side effects, dizziness most common
325
Lasegue sign
positive when the patient has worsening radicular pain during the straight leg maneuver
326
pineal parenchymal tumor of intermediate differentiation
moderate cellularity and moderate MIB1 indexes
327
pineocytoma
form pineocytic rosettes with low MIB1 indexes indicating low mitoses
328
pineoblastoma
highly malignant. show small, round, blue cells on histopathology that are often tightly packed together
329
pineal papillary tumors
papillary changes and form ependymal rosettes and pseudorosettes
330
germinoma
most common pineal region tumors with sheets of cells with large round nuclei
331
most likely damaged in anoxic event with difficulty in short term recall
CA1 of the hippocampus - the most hypoxia-sensitive cells in the brain
332
mesencephalic nucleus
located within the pons and is one of the trigeminal nuclei responsible or proprioception of the jaw and muscles of mastication (V3)
333
principal sensory nucleus
located within the pons and is one of the trigeminal nuclei involved in the sensation of light touch from the face
334
spinal trigeminal nuclei
extends from the pons to the upper cervical spinal cord and is a trigeminal nuclei involved in pain, temperature and deep-pressure sensation
335
nucleus ambiguus
located within the medulla, shared between glossopharyngeal and vagus nerve involving swallowing and speaking
336
nucleus solitarius
located in the medulla and is responsible for the regulation of internal homeostasis
337
Cefaly device
involves electrical stimulation applied to the forehead using a device targeting the supraorbital nerve
338
toluene
inhalant common drug of abuse causing euphoria involved in manufacturing of paints, chemicals, pharmaceuticals, and rubber
mood changes, nausea, slurred speech, diplopia, dizziness, confusion
339
inhaled heroin
CNS depression, abulia, psychomotor dysfunction and can result in a leukoencephalopathy called chasing the dragon
340
ketamine
induces trancelike state while providing pain relief, sedation, and memory loss
341
nitrous oxide
euphoria, spatial disorientation, temporal disorientation, and reduced pain sensitivity
can accelerate B12 deficiency
342
melanocytoma
benign, often dural-based tumors characteristically hyperintense on T1 with homogenous contrast enhancement
often occur within the cervical spine, posterior fossa, and supratentorial space
stain positive S100 and vimentin, and negative for glial markers such as GFAP
343
meningiomas
various locations, isointense on T1 and homogenous enhancement.
negative for S100 and positive for epithelial membrane antigen and vimentin
344
hemangiopericytoma
solitary fibrous tumors that appear similar to meningiomas, risk of extracranial mets
stain positive for CD34 and STAT6 and are negative for S100
345
lymphoma
hyperdense on CT due to high cellularity, hypointense on T1
positive for CD19, CD20, and CD22
346
glioblastoma
ring-enhancing with contrast administration.
347
bilateral absence of what potentials on median nerve somatosensory evoked potentials is a strong predictor of poor neurologic outcome in comatose patients.
N20
348
tectal plate gliomas
low grade, isointense on T1, hyperintense on T2, rarely enhance. often present with hydrocephalus.
349
tracts in the middle cerebellar peduncle
pontocerebellar tract
afferent tract composed of second order neurons of the corticopontocerebellar tract/pathway
DIPGs can be centered within pons and affect this area
350
tract in the inferior cerebellar peduncle - vestibulocerebellar tract
afferent tract that is involved with equilibrium and monitoring head position and eye movements
351
tract in the inferior cerebellar peduncle - cuneocerebellar tract
afferent tract that relays ipsilateral proprioceptive information from the upper extremities to the cerebellum
352
Weber test in sensorineural hearing loss
produces a louder sound in the contralateral ear
353
vertebral hemangioma
venous malformations that characteristically have a "polka dot" appearance due to vertical trabeculations within the lesion
354
distinguish myopathy from motor neuropathy
fasciculations can be present in motor neuropathies, but are not present in myopathies.
motor neuropathy = low amplitude CMAP, mild slowing of CV, mild prolong DL, reduced recruitment, LARGE MUPs
myopathy = typically normal CMAPs, early recruitment, SMALL complex MUPs, full interference pattern
355
carbon monoxide poisoning affects where
bilateral globus pallidus
356
pyridoxine toxicity affects
DRG
357
chronic alcohol use disorder affects
mammillary bodies, affected by thiamine deficiency
358
methanol toxicity affects
bilateral putamen
359
zinc toxicity affects
provokes copper deficiency and affects dorsal columns and corticospinal tract in a syndrome similar to subacute combined degeneration
360
erenumab warning
new-onset or worsening hypertension, constipation, and injection site reactions
361
IgLON5-associated encephalitis
most commonly presents with sleep disorders (apnea and REM sleep behavior disorder), brainstem syndromes (dysphagia, dysarthria), ataxia, and cognitive decline.
screen for malignancy
362
anti ANNA-1
aka anti-Hu Ab associated with limbic encephalitis and peripheral sensory and autonomic neuropathies
highly associated with small cell lung cancer
MRI: temporal lobe T2 hyperintensities
363
LGI-1 encephalitis
faciobrachial dystonic seizures and ictal-associated autonomic symptoms
thymoma, breast cancer, or other types of cancer
364
MA-1
hypersomnia and new onset narcolepsy and cataplecy
testicular germ cell tumors, lung cancer, or breast cancer
365
MS drug therapies
366
dural AV fistula presentation
pulsatile tinnitus, chemosis, proptosis, or focal deficits
risk of hemorrhage includes prior history of hemorrhage, venous ectasia, and presence of cortical venous reflux
367
arachnoiditis
inflammation of arachnoid from variety of causes leading to radicular pain, numbness, and weakness
MRI demonstrates thickened enhancing nerve roots that clump together
Treatment focuses on symptom control
368
treatment of hemifacial spasm
botox
369
where is the compression of a third nerve palsy from PCA aneurysm
interpeduncular cistern
370
favorable predictors of survival and functional outcomes in natalizumab-associated PML
younger age, lower EDSS score, higher Karnofsky Performance Scale, lower CSF JC viral load, and less extensive disease on MRI at the time of PML diagnosis
371
most common location of spinal cord meningioma
thoracic cord
372
what to do if MS patient experiences relapse on DMT
switch to higher efficacy medication, including:
- anti CD20: ublituximab and ofatumamab as well as natalizumab, fingolimod, alemtuzumab, and cladribine
373
Ublituximab approval
relapsing MS, including relapsing-remitting, secondary progressive, and clinically isolated syndrome
374
environmental risk factors for Parkinson
TBI (particularly at young age), long-term trichloroethylene exposure, pesticide exposure, herbicide exposure, area of residence
375
tumor marker in choriocarcinoma
beta HCG
376
tumor marker in endodermal sinus tumor
alpha fetoprotein
377
tumor marker in germinoma
placental alkaline phosphatase
378
the stalk effect
direct compression of pituitary stalk leads to a decrease in dopamine release from the hypothalamus and a modest rise in the prolactin level
379
pleomorphic xanthoastrocytoma
histo with large pleomorphic cells with eosinophilic granular bodies and lymphocytic infiltration
380
pleomorphic xanthroastrocytoma molecular genetics
mutations in BRAF V600E and co-existing deletions of CDKN2A/B
381
pleomorphic xanthoastrocytoma imaging characteristics
cystic mass with an enhancing nodule that can sometimes be seen with a dural tail, making it resemble meningioma. can involve the leptomeninges and cause scalloping of the adjacent skull
382
polymorphous low-grade neuroepithelial tumor of the young histo
oligodendroglial-like components with calcifications and infiltrative growth
383
polymorphous low-grade neuroepithelial tumor of the young molecular
strong expression of CD34 (diff from oligodendroglioma) BRAF V600E mutations and fusions of fibroblast growth factor receptor genes
384
polymorphous low-grade neuroepithelial tumor of the young imaging
cortical and subcortical regions with predilection for temporal lobe
solid with peripheral cysts and have a dense central calcification
hyperintense on T2 and little to no contrast enhancement
385
DNETs histo
round, monotonous, oligodendroglia-like cells arranged in columns. the presence of "floating neurons" is seen with an abundance mucinous matrix
386
DNETs imaging
most often temporal lobe
"bubbly" in appearance on MRI because they can have a multicystic structure.
hyperintense ring sign seen on FLAIR and T2. rarely cause mass effect or have surrounding edema
387
ganglioglioma histo
neoplastic glial cells in combination with dysplastic neurons. glial components often resemble astrocytomas.
388
ganglioglioma molecular
presence of BRAF V600E mutations
389
ganglioglioma imaging
can be cystic or solid tumors and typically expand the underlying cortex. edema does not usually surround the tumor. has calcifications (vs DNETs which do not)
390
angiocentric glioma histo
thin bipolar cells surrounding the perivascular spasics
391
angiocentric glioma molecular
MYB:QKI fusion
392
angiocentric glioma imaging
most often in supratentorial space and are well-defined on imaging. nonenhancing with T1 and T2 hyperintensities. characteristic that may be seen on imaging is a stalk-like extension of the tumor to the adjacent lateral ventricle
393
gene mutation associated with CAA
apolipoprotein E
394
mutation associated with CADASIL
NOTCH3
395
mutation associated with hereditary cavernomatosis
KRIT1
396
mutation associated with Fabry disease
GLA gene -> alpha-galactosidase A
397
Fabry disease neurologic manifestations
peripheral and CNS Involvement, with globotriaosylceramide accumulation found in Schwann cells and DRG together with deposits in CNS neurons. cerebrovasculopathy with inc incidence of stroke.
398
mutation associated with pseudoxanthoma elasticum
ATP-binding cassette transporter
399
pseudoxanthoma elasticum
progressive disorder characterized by the accumulation of deposits of calcium and other minerals in elastic fibers
400
imaging in NPH
ventriculomegaly out of proportion to atrophy, enlarged sylvian fissures, and crowding of the sulci at the vertex
401
pathology of NPH
impaired glymphatic drainage
402
imaging in corticobasal degeneration
asymmetric atrophy of the perirolandic cortices, particularly the premotor cortex or supplemental motor area
403
pathology of corticobasal degeneration
astrocytic plaques and four-repeat tau inclusions
404
corticobasal degeneration characterized by
parkinsonism, unilateral dystonia/dyspraxia/myoclonus, alien limb phenomenon, and behavioral changes
405
pathology of CAA
beta-amyloid deposition in leptomeningeal vessels
406
imaging in CAA
superficial siderosis and/or lobar, cortical, or cortical-subcortical hemorrhages
407
pathology of dementia with lewy bodies
cortical alpha-synuclein aggregation in neurons
408
imaging in dementia with lewy bodies
reduced dopamine transporter uptake, as measured by SPECT
409
embryonal carcinoma molecular
pathology is CD30 positive
410
embryonal carcinoma on pathology
prominent nucleoli and vesicular nuclei
411
Schiller-Duvall bodies
associated with yolk sac tumors described as "central blood vessel surrounded by epithelial-like cells"
412
V2 segment of vertebral artery
most at risk of injury in cervical spine fracture or dislocation
foraminal segment
originates as the artery enters the transverse foramen of the lower cervical vertebrae, most often C6, and terminates as it exits the transverse foramen of C2
413
V1 segment of the vertebral artery
preforaminal segment
extends from its origin at the subclavian artery and ends at the entrance of the transverse foramen of the lower cervical vertebrae
414
V3 segment of the vertebral artery
atlantic or extradural segment
begins as the artery exits the transverse foramen of C2 and ends as it penetrates the dura and enters the skull via the foramen magnum
most likely to be injured in subaxial cervical spine injuries
415
V4 segment of the vertebral artery
intradural or intracranial segment
as it penetrates the dura and conjoins the contralateral VA to form the basilar artery
416
polyarteritis nodosa
medium-vessel vasculitis often presenting with difficult to control HTN, postprandial pain, cutaneous symptoms such as erythematous nodules and livedo reticularis, secondary CNS vasculitis, fatigue, and weight loss
can be triggered by hepatitis B or C infection
417
treatment of symptomatic noncommunicating hydrocephalus
EVD placement
418
myokymia apperance
undulating muscle contractions over a broad area
EMG: soldiers marching out of sync
MC cause: radiation induced plexopathy or nerve root damage
419
Pott disease
result of tuberculous osteomyelitis of the spine can lead to significant kyphotic deformity
420
imaging features of TB spondylitis
thoracic spine involvement, skip lesions, paraspinal abscess in 75% of cases, intraosseous abscess present, posterior-element involved, frequent destruction of vertebral bodies
421
imaging features of pyogenic spondylitis
absent thoracic spine involvement, contiguous involving <2 vertebrae, paraspinal abscess in 40% of cases, no intraosseous abscess, posterior element, or destruction of vertebral bodies
422
apneustic breathing
pontine lesion
end inspiratory pause and short, inadequate exhalations
often trauamtic injuries or stroke
423
Cheyne-stokes respirations
periodic waxing then waning hyperpnea followed by brief apnea
impaired cardiac output or bihemispheric dysfunction; diencephalic lesions
424
central neurogenic hyperventilation
rapid respiratory rate of 40-70 breaths per minute
midbrain dysfunction
425
ataxic respirations
irregular respiratory frequency and amplitude
medullary dysfunction with impending respiratory arrest
426
Herring bodies
enlarged endings of terminal axons in the posterior pituitary that store oxytocin and vasopressin
427
cell types in anterior pituitary
acidophils, basophils, and chromophobes that appear red, blue, and pale respectively on H&E
428
acidophil cells secrete
growth hormone and prolactin
429
basophil cells secrete
ACTH, FSH, LH, and TSH
430
chromophobe cells secrete
do not contain significant amounts of hormone
431
mononeuropathy multiplex pathophysiology
immune complex deposition within vessel walls of nerves
432
mononeuropathy multiplex presentation
acute-onset mononeuropathies in stepwise manner
433
AIDP pathophysiology
autoantibody response to peripheral nerve epitopes
434
diabetic amyotrophy pathophysiology
microvasculitis of multiple nerve roots
435
diabetic amyotrophy presentation
weakness of pelvifemoral muscles, either bilaterally or unilaterally with associated pain
436
diabetic polyneuropathy presentation
slowly progressive peripheral neuropathy with a stocking-glove distribution rather than acute-onset multifocal neuropathy
437
CMT 1A genetics
duplication in PMP22 gene
438
hereditary neuropathy with liability to pressure palsies genetics
deletion in PMP22 gene
439
CMT 1A presentation
within the first 2 decades of life with a progressive peripheral neuropathy and associated foot deformities
NCS with significant demyelination
440
adult with OMAS
small cell lung cancer
441
familial FTD and ALS genetics
repeat expansion in the C9ORF72 gene on chromosome 9p21
442
Jacobsen syndrome
partial deletion of chromosome 11
intellectual disability, dysmorphic face, head, hands, feet, and ears, and thrombocytopenia
443
Williams syndrome
chromosome 7 partial deletion characterized by distinct facial features such as broad forehead, short nose, full cheeks, and wide mouth with moderate intellectual disability and difficulty with visuo-spatial tasks, such as drawing and assembling puzzles
444
astroblastoma
MN1-altered
are well-circumscribed tumors predominantly found in the frontal and parietal lobes in children and adolescents. strong female predominance
pathology with pseudopapillary or perivascular growth
445
ependymoma pathology
perivascular pseudorosettes, true ependymal rosettes, and ependymal canals
446
Neuro Boards
flashcards
Decks in class (16)
# Cards
-
Anatomic Syndromes80
-
Basal Ganglia and Cerebellum61
-
Basic Neuroanatomy71
-
Biostatistics132
-
CNP Course - EMG Anatomy142
-
CNP Course - NCS217
-
CNP Course - EMG Waveforms56
-
Cortex and Connections67
-
Thalamus and Hypothalamus42
-
Cranial Nerves73
-
Demyelinating Diseases145
-
Inherited Leukodystrophies38
-
Vascular Neurology141
-
Tumors & Cysts359
-
Movement Disorders158
-
RITE 2026447
