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neuro16 Flashcards

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1
Q

genetics of parkinsons disease

A

mutations I the alpha synuclein, E3 ubiquitin ligase, ubiquitin carboxyterminal esterase L1, PTEN-induced putative kinase 1, and leucine-rich repeat kinase 2

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2
Q

pathology of PD

A

dopaminergic neuronal loss in the ventral tier of the substantia nigra pars compacta with accum of Lewy bodies; neuronal loss other places as well

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3
Q

four cardinal features of PD

A

tremor, rigitiy, bradykinesia, and postural instability

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4
Q

tremor in PD

A

slow (3 to 5 per sec), and most prominent when the limb is at rest; affects the distal arm more than leg; pill rolling; tremor may also affect the lips, chin and tongue

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5
Q

dementia in PD

A

about 25 percent of patient

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6
Q

treatment for PD

A

L-dopa, given with a peripheral decarboxylase inhib

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7
Q

why L-dopa and not dopamine

A

dopamine does not cross the BBB

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8
Q

why the peripheral decarboxylase inhib

A

to prevent the conversion ot L-dopa to dopamine in the periphery, which could cause n/v, and hypertension

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9
Q

other agents besides L-dopa used in early PD

A

anticholinergics, amantadine, MAO-B inhib, COMT transferase inhib

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10
Q

deep brain stimulation for PD

A

motor thalamus, the internal segment of the globus pallidus, or the subthalamic nuc; use when patients are medially refractory

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11
Q

progressive supranuc palsy

A

a parkinsonian-like disorder with supranuc ophthalmoplegia with limited vertical more than horizontal gaze; axial rigidity and neck extension; frequent falls

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12
Q

corticobasal ganglionic degeneration

A

parkinsonian-like disorder; apraxia, cortical sensory impairment, and alien-limb phenomenon; severe unilateral rigidity; stimulus-sensitive myoclonus

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13
Q

Diffuse Lewy body disease

A

parkinsonian-like; early dementia; priminent visual hallucinations, extreme sens to extrapyramidal side effects of antidopaminergic neuroleptic drugs

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14
Q

Vascuar parkinsonism

A

“Lower half” parkinsonism with rigidity in the legs and marked gait impairment; other evidence of diffuse vascular disease (corticospinal tract dysfunction, pseudobulbar palsy)

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15
Q

Multiple system atrophy

A

parkinson-like sydnrome; early and prominent features of autonomic disfunction; evidence of corticospinal tract dysfunction; cerebellar signs; stimulus-sens myoclonus vocal cord paresis

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16
Q

Neuroleptic malignant syndrome

A

muscle rigidity, fever, autonomic lability, altered level of consciousness, elevated CK, and leukocytosis; caused by drugs

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17
Q

Akathisia

A

caused by drugs; dysphoric state characterized by need to be in constant motion; inability to sit still

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18
Q

treatment of akathisia

A

anti-cholinergics and beta blockers

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19
Q

tardive dyskinesia

A

orolinguomasticatory dyskinesia (like lipsmacking) that occurs as the most common movement disorder complicating neuroleptic use (use of dopamine receptor blockers)

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20
Q

the propensity of a drug to cause movement disorders

A

is related to its D2-receptor blocking activity

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21
Q

most common causes of drug-induced movement disorders

A

antipsychotics like haloperidol

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22
Q

therpeutic strategies in parkinson disease

A

start with levodopa/decarb inhib or dopamine agonist; if no response, increase dose and consider alternative diagnoses

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23
Q

for tremor-predominent PD

A

anticholinergic or amantadine

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24
Q

for early morning stiffness PD

A

overnight controlled-release prep of L-dopa

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25
when patient gets L-dopa induced hallucinations
decrease dose of L-dopa; can consider giving low dose atypical antipsychotic
26
what to do when patient has dyskinesia and PD
reduce dose of L-dopa, add or increase dose of dopamine agonist
27
stiff-man sydnrome
fluctuating and progressive muscle rigidity with spasms; may be autoimmune or paraneoplastic process; lumbar hyperlordosis; painful spasms
28
diagnosis of stiff man syndrome
characteristic clinical pic and demonstration of continuou motor unit activity without evidence of neuromyotonia, pyramidal or extrapyramidal dysfunction, or structural SC diseaes
29
may see what kind of antibodies in stiff man syndrome/
antibodies directed against glutamic acid decarboxylase (GAD) or amphiphysin
30
antispacity agents used in stiff man syndrome
benzos and baclofen
31
baclofen
GABA agonist
32
what is intention tremor?
action tremor that increases as the target is approached
33
essential tremor
postural tremor but no resting or action tremor
34
essential tremor affects which body parts
hands most commonly; but also the head, face, voice, trunk, or legs; bilateral;
35
people with essential tremor get improvement with what
small amounts of alcohol
36
treatment of ET
primidone and propranolol
37
action or intenton tremor localizes to where?
cerebellum or its connection
38
typical cause of resting tremor
parkinsons
39
typicla cause of postural tremor
ET
40
typical cause of action tremor
crbellum and cerbellar outflow tract dysfunction
41
causes o fchorea
huntingtons, neuroacanthocytosis, Wilson disease, neuroleptics, antiparkinsonian meds, alcohol, anoxia, carbon monoxide, metabolic causes, SLE, post-strep
42
ballism
large amplitude and poorly paterned flinging or flailing movements or a limb; frequently unilateral (hemiballismus)
43
cause of ballism
contralateral lesion in the caudate putamen or subthal nuc; stroke is most common; hyperglycemia may also cause
44
treatment for ballism
dopamine-depleting and blocking agents; contralateral thalamotomy or pallidotomy may be beneficial in severe disease
45
dystonia
sustained muscle contraction leading to repetitive twisting movments or abnormal postures
46
dystonic movements can be extignuished
by gently touchign the affected body part (geste antagoniste)
47
what exacerbates dystonia
fatigue, stress, and emotional states; worsens during voluntary movement; may be suppressed by relaxation and sleep
48
progression of dystonia
usually starts as action dystonia and progresses to dystonia at rest
49
idiopathic torsion dystonia
primary dystonia; gene for the autosomal dominant familial form is on chrom 9q (mutations in torsin A)
50
treatment for idiopathic torsion dystonia
botulinum toxin
51
manifestations of idiopathic torsion
torticollis, writer's cramp, blepharospasm, or spasmodic dysphonia
52
myoclonus
sudden lightening-like movement produced by abrupt and brief mucle contraction or inhibition
53
essential myoclonus
occurs in isolation without other neuro signs; some patients have improvements with small amt of alcohol
54
treatment for myoclonus
clonazepam and valproate
55
tourette syndrome
motor and vocal tics; more common in boys; periodic remission and exacerbation
56
PANDAS
pediatric autoimmune neuro disorders assoc with strep infection; tics, OCD or both following GABHS infection
57
treatment for tics
dopamine antagonists (haloperidol or the atypical antipsychotics) work best but due to side effecs try clonazepam and clonidine first
58
wilson disease
autosomal recessive; defect in copper metabolism; presents with liver dysfunciton and neuropsych symptoms
59
wilson disease copper accumulates where
liver and spillover into systemic circ, with deposition in the kidney, cornea, and CNS
60
kayser-fleischer ring
copper deposition in Descemet's membrane
61
diagnosis of wilson's disease
increased serum copper and decreased serum cerulosplasmin; increased 24 hour urinary copper excretion
62
treatment of wilsons disaese
copper chelating with D-penicillamine or the less toxic chelator, trientine, in conjunction with zinc
63
paroxysmal dyskinesias
tx with carbamazepine; recurrent attacks fo hyperkinesis with preserved consciousness; examples are paroxysmal kinesigenic choreoathetosis and paroxysmal dystonic choreoathetosis
64
paroxysmal kineigenic choreoathetosis
episodes of chorea, athetosis, or dystonia are triggered by sudden movements and last for seconds to mins
65
paroxysmal dystonic choreoathetosis
last minutes to hours; triggered by alcohol, fatigue, and stress;
66
treatment for acute dystonic reaction caused by a drug
give an anticholinergic like benadryl

Neuro (43 decks)

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