Neurology Flashcards
(281 cards)
1
Q
Primitive reflexes should not persist beyong _______.
A
3-5 months
2
Q
What is the Galant reflex?
A
stroking one side of the back of the infant causes lateral curvature of the
3
Q
What is the Moro reflex?
A
when support is lost, the arms will abduct (swing out) and extend and hands will open followed by adduction and flexion
4
Q
Stuttering affects what percent of children?
A
2%
5
Q
What improves fluency in stuttering?
A
ethanol and singing
6
Q
Mental retardation affects what percent of births?
A
3%
7
Q
What generally separates delirium from other confusional states?
A
typically has autonomic symptoms (dilated pupils, tachycardia, hyperthermia, increased sweating)
8
Q
What percent of Alzheimer’s disease is sporadic? What is the life expectancy following the diagnoses of Alzheimer/s?
A
95% usually progress to death in 7 to 10 years
9
Q
What type of cortical atrophy is seen in Alzheimer’s?
A
general cortical atrophy, most pronounced in parietal and temporal lobes
10
Q
What histologic features are seen in Alzheimer’s?
A
- Neurofibrilary tangles- Senile Plaques-Hirano’s bodies
11
Q
What are neurofibrilary tangles?
A
intraneuronal inclusion
Paired helical filaments formed by hyperphosphorylation of microtubule- associated protein tau
12
Q
What are senile plaques?
A
extracellular accumulation
Comprised of amyloid β peptides, proteolytic product of amyloid precursor protein (APP)
13
Q
What are two scenarios that lead to increased accumulation of amyloid?
A
Trisomy 21 (Down’s syndrome)
ApoE4 mutation
14
Q
APP is on what chromosome?
A
Ch 21
15
Q
What are Hirano’s bodies?
A
intraneuronal inclusion
eosinophilic intracellular aggregates of actin and associated proteins in neurons
16
Q
What neurotransmitter changes are seen in Alzheimer’s disease?
A
decreased ACh and increased glutamate
17
Q
Name three AChEi.
A
donepizil, galantamine, rivastigmine
18
Q
What histologic features are seen in Pick’s disease?
A
Pick’s bodies and Pick’s cells
19
Q
What are Pick’s bodies?
A
intracellular inclusions of tau protein
20
Q
What are Pick’s cells?
A
swollen neurons
21
Q
What clinical picture typifies Lewy Body Disease?
A
alzheimer like patient with early onset hallucinations and parkinsonian features
22
Q
What neurotransmitter changes are seen in Lewy Body Disease?
A
Loss of ACh and dopamine producing neurons
23
Q
What histologic feature is characteristic of Lewy Body Disease?
A
a-synuclein cytoplasmic inclusions (Lewy Bodies)
24
Q
What cerebral structure is affected in Wernicke-Korsakoff Syndrome?
A
atrophy of the mammillary bodies
25
What is apraxia? What cortical lesion can cause apraxia?
inability to perform complex movements in the absence of paralysis
premotor lesion (lateral portion of BA 6)
26
Frontal Eye fields are located in BA ___. Stimulation causes _____ whereas lesioning causes ______.
BA 8
contralateral eye movement
impaired contralateral gaze
27
Broca's speech is associated with what BA and what anatomical structures?
BA 44 and 45pars opercularis and pars triangularis
28
What is abulia? What cortical lesion can cause it?
decrease in thought, movement, speech, will, or initiative
b/l frontal lobe lesions can cause it
29
Akinetic mutism can be seen in adults with what cortical lesion?
b/l cingulate lesions
30
What are the symptoms of pseudobulbar palsy?
unable to close their eyes or move their mouth ortongue, but are able to yawn, cry, cough, etc., which are reflexes of the pons and medulla
31
What leads to pseudobulbar palsy?
damage to cortical motor fibers projecting to lower brainstem
32
Stimulation to supplementary motor cortex leads to _______.
gross b/l movements
33
What is the function of the ventromedial pathway? What composes it?
controls axial movements and posture
tectum, vestibular nuclei, and pontine and medullary reticular nuclei to the interneurons in the ventromedial spinal cord
34
Dominant parietal lobe is involved in ________ and non-dominant parietal lobe is involved in ________.
mathematical calculations and language
visuospatial relationships and geographic memory
35
The secondary somatosensory area is located on ______ and is BA ______.
supramarginal gyrus
area 40
36
Primary gustatory cortex is located on ______ and is BA _______.
anterior portion of parietal operculum
43
37
What causes Gerstmann's syndrome?
dominant parietal lobe lesion at angular gyrus (area 39)
38
Gerstmann's syndrome is characterized by what symptoms?
right/left dissociation, finger agnosia, acalculia, and agraphia
39
The uncinate fasciculus connects the ______ to the ______,
anterior temporal lobe to the orbitofrontal gyrus
40
Arcuate fasciculus connects ______ and ________.
Wernicke's area and Broca's area
41
Heschl's gyrus is the _______, located in ______, and is BA ________.
primary auditory cortex
posterosuperior temporal lobe deep to Sylvian fissure
41, 42
42
Kluver Bucy syndrome results from ________.
b/l amygdala injuries
43
Kluver Bucy syndrome is characterized by _______.
Absence of emotional response, compulsion to explore all objects visually, tactilely, and orally; hypersexuality and visual agnosia
44
The Band of Gennari is located in ________.
the fourth layer of the occipital lobes
45
Cortical blindness is characterized by what on EEG?
absence of a-waves on EEG in the occipital waves
46
What is Anton's syndrome?
an anosognosia; cortical blindness with an affected association area such that the patient denies that he or she is blind
47
What is Balint's syndrome?
caused by a bilateral lesion in the parietooccipital cortices
Ocular ataxia and apraxia with normal extraocular function. Associated with inattention to the peripheral visual field and simultanagnosia
48
What connects the receptive and expressive speech areas of the brain?
arcuate fasciculus
49
What is the concept of kindling in regards to seizures?
seizures beget seizures
50
What is the irritative zone?
area of cortex that generates interictal spikes
51
What is the ictal onset zone?
area of cortex where seizures are generated
52
What is an epileptogenic lesion?
structural abnormality that is direct cause of epileptic seizures
53
What is a symptomatogenic lesion?
area of the brain that first manifests seizure symptoms
54
What is the functional deficit zone?
cortical area of nonepileptic dysfunction
55
What is the epileptogenic zone?
area of brain necessary and sufficient for initiating seizures; removal or disconnection required for amelioration of seizures
56
What is Todd's paralysis? What causes it?
post ictal paralysis, could be due to decreased glucose or increased lactate
57
What EEG findings are seen on Absence seizures?
3 Hz Spike and Wave
58
What does pathology show on mesial temporal lobe sclerosis?
hippocampal atrophy with gliosis and neural loss in CA1, CA4, and dentate gyrus
59
What is West's syndrome?
infantile spasms; repeated extension and flexion of the neck, trunk, and extremities
60
What is characterizes West's syndrome (infantile spasms) on EEG?
hypsarrhythmia (chaotic background with random high voltage, slow spike and wave)
61
What is the treatment for infantile spasms (West syndrome)?
ACTH, vigabatrin, and ketogenic diet
62
What are Benign epilepsy of childhood with Rolandic spikes? What is the typical clinical course?
focal motor seizures typically of the face during sleep-wake transitions; conscious but aphasic post ictally
remits spontaneously by adolescences
63
What is Juvenile myclonic epilepsy?
myoclonus in the morning without loss of consciousness, affects upper > lower
64
What is the inheritance pattern of Juvenile myoclonic epilepsy?
Autosomal dominant
65
What is the treatment of juvenile myoclonic epilepsy?
valproic acid for life
66
What characterizes Lennox-Gastaut syndrome?
multiple seizure types with mental retardation
67
What surgical options are available for patients with Lennox Gastaut syndrome?
VNS and corpus callosotomy
68
What is the mechanism of action of phenytoin?
Na Channel blockade
69
There are many side effects to phenytoin. What are some of the most serious ones?
Steven Johnson syndrome, cerebellar degeneration
70
What is the mechanism of action of Carbamazepine/Oxacarbazepine?
Na channel blockade
71
What are the side effects of Carbemazepine/Oxacarbazepine?
Pancytopenia and SIADH
72
What is the MOA of Lacosamide?
Na channel blocker
73
What is the MOA of Ethosuximide?
Ca Channel blocker
74
What is the MOA of acetazolamide?
carbonic anhydrase inhibitor
75
What is the MOA of lamotrigine?
inhibit presynaptic glutamate release
76
What is the MOA of Topiramate?
affects Na channels, GABA, and Glutamate receptors
77
What is the MOA of Valproic acid?
inhibits Na and Ca channels
increases GABA synthesis and activity
78
What are the side effects of Valproic acid?
Thrombocytopenia, hyperammonemia (hepatotoxicity in < 2 years)
79
What is the MOA of Felbamate?
NMDA receptor antagonist
80
What are the side effects of Felbamate?
aplastic anemia and hepatic failure
81
Absence seizures are thought to originate/involve what cerebral structure?
thalamus
82
What is the go to treatment for absence seizures?
Ethosuximide
83
WADA testing determines _______.
laterality of speech and memory
84
What is injected in a WADA test?
sodium amobarbital
85
Corpus callosotomy can be used for _______ and _______.
atonic seizures (drop attacks)
seizures with secondary generalization
86
What HLA type is associated with MS?
HLA-DR2
87
What causes an oligoclonal band pattern in MS?
elevated IgG in the CSF
88
What is Schilder's disease?
massive demyelination in children and adolescents, affecting bilateral hemispheres, brainstem, and cerebellum
Malignant course
89
What is Balo's concentric sclerosis?
alternating spared and damaged white matter progressing in concentric ring like fashion from the ventricles outward
typically fatal
90
What is the pathophysiology of neuromyelitis optica (Devic's disease)?
IgG autoantibodies versus aquaporin 4 on astrocytes
91
What are the imaging findings for neuromyelitis optica?
MRI shows optic nerve/chiasm enhancement and/or spinal cord lesion affecting more than three segments in length
92
Describe the tremor seen in Parkinson's disease.
resting tremor of 4-5 per second in the lips, head, and fingers (pill rolling)
93
Where is the mutation in primary dystonia?
DYT1 TOR1A gene
94
What is the other name for Wilson's Disease?
hepatolenticular degeneration
95
What tests are indicative of Wilson's disease?
LFTs, slit lampe test (Kayser-Fleischer rings), decreased ceruloplasmin, elevated urine copper
96
What defines Tourette's syndrome?
multiple motor tics with at least one vocal tic
97
Name two COMT inhibitors.
entacapone, tolcapone
98
Name two Monoamine oxidase B inhibitors.
selegiline, rasagiline
99
What is olivopontocerebellar degeneration?
multiple system atrophy associated with ataxia
100
What is Striatonigral degneration?
form of MSA in which Parkinsonian features predominate although autonomic and cerebellar symptoms can be seen
101
What is Shy-Drager syndrome?
MSA with autonomic dysfunction predominating; Orthostatic hypotension is the key finding
102
What is Progressive Supranuclear Palsy?
triad of :
- progressive supranuclear ophthalmoplegia (impaired voluntary vertical gaze, but preserved doll’s eyes)
- pseudobulbar palsy
- axial rigidity
103
What is the inheritance of Huntington's disease?
AD
104
What is the genetic abnormality seen in Huntington's disease?
great than forty tri-nucleotide CAG repeat on Ch 4
105
Where is degeneration seen in Huntington's disease?
Frontal lobe and caudate
106
Autonomic dysfunction is associated with what type of headache?
cluster headaches
107
What procedure can be used to treat refractory cluster headaches?
sphenopalatine ganglion lesioning
108
What is Tolosa-Hunt syndrome?
nonspecific inflammation of the cavernous sinus or superior orbital fissure
ocular and retro-orbital pain, ocular motor paralysis (pupil may be involved), and possibly sensory loss over the forehead (CNs III, IV, V1, and VI, sympathetics)
109
Where is the area of compression/demyelination of the Trigeminal nerve in Trigeminal neuralgia?
Obersteiner-Redlich zone
110
Steroids help prevent what complication of temporal arteritis?
Blindness
111
What is Ramsay Hunt syndrome?
geniculate ganglion (CN VII) herpes infection with ear pain and vesicles (in the external auditory meatus), facial weakness, possible hearing deterioration, vertigo, and tinnitus
112
Climbing fibers enter via the ________ and mossy fibers enter via the _______.
inferior cerebellar peduncle
middle cerebellar peduncle
113
What is the main function of the flocculonodular lobe?
vestibular function (inputs from vestibular apparatus and nuclei)
114
What is the main function/input of the anterior lobe of the cerebellum?
posture, muscle tone, processing information from muscle tendons
input from spinocerebellar tracts
115
What does the anterior lobe of the cerebellum arise from?
dorsal nucleus of Clarke
116
The output of the vermis is via the __________.
fastigial nucleus
117
Output of the intermediate zone is via ______.
globose and emboliform nuclei
118
Output of the latera zone is via ________.
dentate nucleus
119
Anterior lobe of the cerebellum degeneration occurs from _______ or _______.
ethanol and malnutrition
120
What is titubation? If present, where is the pathology?
3 Hz anterior/posterior head bob
cerebellar
121
What causes slurred speech?
corticobulbar tract interruption
122
What is the inheritance pattern of Friedrich's ataxia?
AR
123
What is the genetic cause of Friedrich's ataxia?
GAA repeat on Frataxin gene on Ch 9
124
Pathologically what occurs in Friedrich's ataxia?
Demyelination of posterior columns, corticospinal, ventral, and lateral spinocerebellar tracts, leading to gait ataxia and clumsiness
125
The most frequent visual disorder in children is ______. In adults, it's ________.
Myopia (nearsigtedness)
Hyperopia (farsightedness)
126
On CN III, why does a fixed and dilated pupil present earlier than a "down and out" motor palsy with a compressive lesion?
parasympathetics are located peripherally on the nerve, motor is central
127
What causes pinpoint pupils?
pontine lesion causing loss of sympathetic input (from the hypothalamus to the superior cervical ganglion) resulting in unapposed sympathetic tone
128
What defines an Argyll Robertson pupil?
reacts to accomodation and not light
129
What is Adie's tonic pupil? What causes it? What's the treatment?
mydriasis and blurry vision that occurs at 20 to 40 years with female predominance from degeneration of the ciliary ganglion (parasympathetics)
eyes respond to accommodation and not light
no treatment needed
130
What nerve brings sympathetic fibers to Muller's muscle in the eyelid? What does this cause?
long ciliary nerve
ptosis (such as in Horner's syndrome)
131
What is pilocarpine? How does it affect pupils?
ACh agonist
should constrict any pupil unless dilated by atropine
132
The region in the retina responsible for high acuity vision is called the ________ and the central portion of it is called ________.
macula
fovea
133
The macula contains (rods/cones) while the fovea contains (rods/cones).
rods and cones
rods
134
What is Terson's syndrome?
vitreous hemorrhage associated with SAH/ICHis associated with more severe SAH/ICH
135
What are Roth's spots? What are they associated with?
a pale spot in the retina from the accumulation of white blood cells and fibrin
Associated with subacute bacterial endocarditis or embolic plaques
136
What percent of people with optic neuritis develop MS?
75%
137
What percent of optic neuritis cases are bilateral?
10%
138
What percentage of optic neuritis patients completely recover their vision? What symptoms often lingers?
30%
color blindness (dyschromatopsia)
139
What can be seen on fundoscopic exam with ischemic optic neuropathy?
flame hemorrhages and edema w/ disc atrophy
140
Toxic and nutritional optic neuropathies cause what kind of visual loss?
bilateral, symmetric central, or centrocecal scotomas(unlike demyelinating disease) with normal peripheral fields
141
What is the most common type of glaucoma?
open angle glaucoma
142
What is the visual loss in gluacoma?
arcuate defect in the upper and lower nasal fields
143
What causes floaters?
opacities in the vitreous humor
144
A sudden increase in floaters with a bright flash of light is a concern for what?
retinal detachment
145
Uveitis accounts for what percent of blindness in the US?
10%
146
What is the inheritance of Leber's hereditary optic neuropathy?
mitochondrial
147
What is the clinical picture of Lever's hereditary optic neuropathy?
optic atrophy with painless vision loss in one eye before the other
148
What is the affected chromosome in Retinitis pigmentosa?
Ch 3
149
What occurs in Retinitis pigmentosa?
b/l degeneration of all layers of the retina with foveal sparing
150
What occurs in Stargardst disease?
b/l degneration of the macula and fovea (opposite of retinitis pigmentosa)
151
What is a junctional scotoma? What causes it?
ipsilateral monocular blindness and contralateral superotemporal quadrantanopia
optic nerve/chiasm junction (think von Willebrand's knee)
152
What ischemic stroke causes macular sparing? Why?
PCA strokes
macula represented by occipital poles which is supplied by MCA collaterals
153
What and where is the vertical gaze center?
rostral interstitial nucleus of the medial longitudinal fasciculas (riMLF) located at the junction of the midbrain and thalamus
154
What nucleus assists in maintaining vertical gaze?
interstitial nucleus of Cajal
155
What comprises the horizontal gaze center?
paramedian pontine reticular formation (PPRF) and CN VI nuclei
156
Damage to one CN VI nucleus causes what clinical findings? Why?
failure of abduction of the ipsilateral eye and adduction (medial rectus) of the contralateral eye
CN VI nerve fibers cross to the contralateral CN III to innervate the medial rectus
157
What midbrain nucleus is responsible for the accommodation reflex?
Perlia's nucleus
158
Superior colliculus is responsible for coordinating what eye movement?
contralateral horizontal
159
What composes Parinaud's syndrome?
- impaired gaze
- impaired convergence
- mydriasis
- convergence nystagmus
- lid retraction (Collier's sign)
160
Decreased downgaze is seen in what pathology?
progressive supranuclear palsy
161
Downward eye deviation can by caused by what hemorrhage location?
thalamic hemorrhages
162
What causes intranuclear opthalmoplegia?
lesion of the ipsilateral MLF rostral to the abducens nerve
163
What is the clinical picture of patients with INO?
ipsilateral eye cannot adduct completely while contralateral eye has nystagmus as it abducts
164
What is the most common cause of INO in the young?
MS
165
Neurons in what CNS structures undergo turnover?
Olfactory receptor cells, subventricular zone, dentate gyrus
166
What symptoms characterize Kallman's syndrome? What causes it?
absent or delayed puberty with impaired smell due to hypothalamic hypogonadism and failure of olfactory bulbs to form
167
Seizures in what location cause olfactory hallucinations?
temporal
168
Conductive hearing loss is ______ hearing loss while sensorineural hearing loss is ______ hearing loss.
uniform
high frequency
169
Vestibular schwannomas cause ________ hearing loss.
high tone
170
What is the symptomatology of Meniere's disease?
recurrent vertigo with fluctuating unilateral tinnitus and low tone sensironeural hearing loss
171
On which side is the nystagmus in Meniere's disease?
contralateral to the affected side
172
On to which side do patient's generally fall in Meniere's disease?
ipsilateral to the affected side
173
What is the physiologic cause of Meniere's disease?
distention of the endolymphatic duct with rupture into the perilymph
174
What medications can be used for prophylaxis in Meniere's disease?
diuretics
175
What are Kussmaul respirations associated with?
rapid breathing associated with acidosis
176
MIdbrain lesions cause ______ pupils.
fixed and dilated
177
Pontine lesions cause _______ pupils.
pinpoint
178
Opiates cause ______ pupils.
pinpoint
179
Anesthesia generally causes _______ pupils.
mid sized fixed
180
Atropine toxicity causes ______ pupils.
dilated
181
What is decorticate posturing?
upper extremity flexion and adduction with lower extremity posturing
182
What are Lundberg A waves?
plateau waves that cover every `15-30 minutes
sustained, reach levels around 50 mm Hg, may cause death
183
What are Lunderg B waves?
pressure pulses that last 30 seconds to 2 minutes, 10-20 mmHg less than A waves
184
What are Lunberg C waves?
low amplitude waves that occur every 4-8 minutes, can occur on top of A waves
185
In what percent of pseudotumor patient's dose unilateral optic nerve sheath fenestration cause bilateral vision improvement?
66%
186
In a normal ICP waveform, what do P1, P2, and P3 correlate with? What are their typical relative amplitudes?
P1 > P2 > P3
P1: percussion wave - arterial pulsation
P2: tidal wave - compliance
P3: dicrotic wave - closure of aortic valve
187
Hemorrhage accounts for what percent of stroke?
10%
188
What is the stroke mortality at 1 month? 1 year? 3 years? 7 years?
19%, 23%, 46%, 60%
189
What is the angiogram risk of stroke in the general population? In patient's with atherosclerosis?
1-3%up to 5%
190
How common do seizures occur in cortical embolic strokes?
20%
191
What is the risk of stroke following TIA? What is the risk of MI?
10% at 3 months, 26% over 2 years
21% over 5 years
192
When is the highest stroke risk following TIA?
first 48 hours
193
What is the risk of ICH with tPA?
6%
194
Endovascular clot retrieval, angioplasty, or intra-arterial tPA may be performed up to ________ after onset of stroke deficit.
4.5 to 6 hours
195
Decompressive hemicraniectomy in stroke has been show to _______ and ________.
decrease ICP, increase survival
196
Why are thrombotic strokes generally slower onset than embolic strokes?
more time for collaterals to form in thrombotic strokes
197
tPA can be given to patients when stroke deficit symptoms onset within _______.
3 hours
198
What histologic finding is seen with lacunar strokes?
lipohyalin degeneration of small vessels (lipohyalinosis)
199
What were the conclusions of the NASCET trial?
North American Symptomatic Endarterectomy Trial
- CEA in symptomatic patients w/ >70% stenosis
- reduces stroke risk from 26% to 9% over 2 years
200
What were the conclusions of the ACAS trial?
Asymptomatic Carotid Atherosclerosis Study
- CEA in asymptomatic patients w/ >60% stenosis
- reduces stroke risk from 11% to 5% over 5 years
201
Stroke of the anterior choroidal artery territory results in what sympotmology?
Contralateral hemiplegia, hemianesthesia, and homonymous hemianopsia
202
What is Weber's syndrome?
midbrain stroke syndrome from occlusion of interpeduncular branches to the midbrain
ipsilateral CN III palsy w/ contralateral hemiplegia
203
Occlusion of what posterior circulation artery can cause horner's syndrome?
AICA
204
Ventral pontine syndrome is caused by occlusion of what vessel?
basilar artery brances (circumfrential/paramedian arteries)
205
What's another name for ventral pontine syndrome?
Millard-Gubler syndrome
206
What are the symptoms in Ventral pontine syndrome?
Ipsilateral facial weakness
Contralateral hemiplegia
Horizontal diplopia
207
What is another name for lateral pontine syndrome?
Marie-Foix syndrome
208
What vessel is occluded in lateral pontine syndrome?
basilar artery circumferential brance or AICA
209
What are the symptoms in lateral pontine syndrome?
Contralateral hemiplegia
Contralateral decreased pain and temperature
Ipsilateral ataxia
210
What is another name for medial medullary syndrome?
Dejerine's syndrome
211
What vessel is occluded in medial medullary syndrome?
Vertebral artery
212
What are the symptoms in medial medullary syndrome?
Contralateral hemiplegia
Contralateral impaired posterior column function
Ipsilateral tongue weakness
213
What is another name for lateral medullary syndrome?
Wallenberg's syndrome
214
What vessel is occluded in lateral medullary syndrome?
PICA
215
What are the symptoms in lateral medullary syndrome?
Decreased contralateral pain and temperature
Ipsilateral Horner’s syndrome
Ipsilateral decreased facial pain and temperature(descending fibers and spinal nucleus of V)
NystagmusNauseaVertigo (vestibular nuclei)
Decreased gag
HoarsenessDysphagiaCord paralysis (IX and X)
Decreased taste (solitary tract)
Ipsilateral falling
Ataxia (restiform body)
Hiccups
216
What nerve innervates the ligaments, periosteum, outer annulus, and facet capsule of the spine?
sinuvertebral nerve
217
As we age, glycosaminoglycans in the nucleus pulposes is replaced by ___________.
collagen and elastin
218
Lumbar disc disease is exacerbated by what movement?
flexion (increasing disc protrusion)
219
Spondylosis and lumbar stenosis are exacerbated by what movement?
Extension (buckling of the ligamentum flavum)
220
Where are the most common sites for disc disease in the lumbar spine?
L5-S1 (45-50%)L4-L5 (40-45%)
221
Where are the most common sites for disc disease in the cervical spine?
C6-C7 (70%)C5-C6 (20%)
222
Ankylosing is associated with HLA ____?
HLA B27
223
Ankylosing spondylitis is associated with what other conditions?
Reiter's syndrome, psoriasis, iritis, and inflammation of the intestines
224
Ankylosing spondylitis leads to ______ deformity of the spine.
kyphotic
225
What are the most common origins of spinal metastases?
breast, lung, thyroid, prostate, kidney, and multiple myeloma
226
What is the genetic cause of Charcot-Marie-Tooth syndrome?
mutation of peripheral myelin protein 22
227
What is seen histologically with Charcot-Marie-Tooth syndrome?
onion bulb appearance of nerves
228
What is the symptomolgy progression of Charcot-Marie-Tooth syndrome?
Distal muscle weakness with atrophy starting in the legs and often develop lower extremity deformities
(toe walking, foot eversion, hammertoes)
229
What is Guillain-Barre syndrome?
acute inflammatory demyelinating polyneuropathy. that results in ascending weakness, paralysis, and hyporeflexia ± sensory or autonomic involvement
230
Forty percent of GBS patients are seropositive for __________.
Campylobacter Jejuni
231
How long does it take to recover from GBS?
1-3 months (depends on remyelination)
232
What is seen in nerve conduction studies in GBS?
delay of F waves
233
What is seen in CSF in GBS?
elevated protein without pleocytosis
234
What is the treatment of GBS?
plasma exchange; no role for steroids
235
What is Chonic Inflammatory Demyelinating Polyneuropathy?
chronic form of GBS
236
What is ALS?
Degeneration of anterior horn motor neurons resulting in a mixed upper and lower motor neuron disease:
- Affects motor system (extremity weakness, bulbar symptoms, diaphragm, etc.) with ocular and
bladder sparing. There are no sensory symptoms.
- Tongue atrophy and fasciculations are classic
237
What is Martin-Gruber anastamosis?
median to ulnar nerve crossover in the forearm; important to identify to prevent confusion with NCV
238
What causes the compression in thoracic outlet syndrome?
anterior and medial scalene muscles
239
What part of the brachial plexus is affected in neurogenic thoracic outlet syndrome?
lower trunk
240
What are visual evoked potentials?
potentials evoked from the occipital lobe from retinal stimulation
241
What are SSEPs?
somatosensory evoked potentials
monitor the posterior columb pathway of the sensory system
242
Axonal disease causes ______ on NCV.
decreases wave amplitude
243
Myelin disease causes _______ on NCV.
increases latency and decreases velocity.
244
What is an H reflex on NCV?
submaximal stimulation of a mixed somatosensory nerve that is not strong enough to obtain a direct motor response
245
What is an M wave on NCV?
direct motor response caused by stimulation of a motor nerve
246
What is myokymia?
spontaneous firing of a motor unit
247
What causes myesthenia gravis syndrome?
antibodies to post synaptic ACh receptors on striated muscle
autoimmune or paraneoplastic (thymoma)
248
What is the treatment for myesthenia gravis?
AChE inhibitor (pyridostigmine), immunosuppresion, thymus removal
249
What causes Lambert Eaton syndrome?
paraneoplastic syndrome to presynaptic Ca channels, thus reducing ACh release
250
Fasciculations are associated with (UMN/LMN) injury.
LMN
251
What causes Clasp Knight phenomenom?
reticulospinal lesions
252
Spasmodic toticollis predominantly affects what muscle groups?
sternocleidomastoid, trapezius, posterior cervical muscles
253
What surgical treatment can be done for spasmodic torticollis?
sectioning of the ipsilateral CN XI and bilateral cervical roots C1-C3
254
What causes tactile agnosias?
dominant parietal lesions (affects both hands)
255
What is allochiria?
perceiving a stimulus that is on one side of the body that is thought to be on the other side
256
What is tabes dorsalis?
involves posterior column and roots
Lower extremity numbness, pain, decreased vibration, proprioception, reflexes and tone, abnormal gait, atonic bladder, and NORMAL STRENGTH
257
What are the classic sensory findings with syringomyelia?
sensory loss of pain and temperature with preservation of proprioception and touch
258
A festinating gait is associated with what disease?
Parkinons's disease
259
What regulates the circadian rhythm?
suprachiasmatic nucleus
260
K complexes and sleep spindles are seen in what stage of sleep?
Stage II
261
What medication can suppress REM sleep?
Monoamine oxidase inhibitors
262
Narcolepsy is associate with HLA ______.
DR2
263
What is the classic tetrad of narcolepsy?
Excessive daytime sleepiness, hallucinations on going to sleep or waking up, cataplexy, sleep paralysis
264
What is the cause of narcolepsy?
destruction of the hypothalamic hypocretin/orexin neurons which stimulates ARAS
265
All patients with ________ have narcolepsy.
cataplexy
266
There are ______ cervical, ______ thoracic, and ______ lumbar sympathetic ganglia.
3 cervical
11 thoracic
4-6 lumbar
267
What is the stellate ganglion?
sympathetic ganglion formed from fusion of inferior cervical and upper thoracic ganglia
supplies UE
268
Postganglionic fibers are (myelinated/unmyelinated).
unmyelinated
269
What is atropine's MoA?
muscarinic receptor blocker (parasympathetic post ganglionic synapses are affected)
270
What is the MoA of curare?
blocks nicotinic receptors (affects autonomic ganglia and NMJ)
271
Which andrenergic alpha receptors are presynatpic and which ones are post synatpic?
a-1 are postsynaptic
a-2 are presynaptic
272
What are crocodile tears? What causes it?
lacrimation from gustatory stimulation
if the CN VII parasympathetic fibers are injured, they may undergo aberrant regeneration with connections of the fibers of the chorda tympani reaching the sphenopalatine ganglion
273
What is the treatment for hyperhydrosis?
ablating T2-T3 symapthetic ganglia (leave T1 to prevent Horner's)
274
Sympathetic output to the bladder is through the _______ ganglion and ______ nerve.
inferior mesenteric ganglion and hypogastric nerve
275
The external sphincter of the bladder and the anus is innervated by _________.
pudendal nerve
276
The mictruition center is located in ________.
locus ceruleus
277
LMN lesions to the bladder cause _______ and can be treated with _______.
atonic bladder, urinary retention, poor bladder contraction
bethanechol
278
UMN lesions to the bladder cause _____ and can be treated with _______.
spastic bladder
ACh antagonists, propantheline, oxybutynin
279
Rapid correction of hyponatremia can cause _____.
central pontine myelinolysis
280
Pituitary adenomas causing Cushing's have a ________ dexamethasone suppression test.
positive
281
Ectopic ACTH secretion leading to Cushing's has a _________ dexamethasone suppression test.
negative
