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neuro16 Flashcards

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
Q

when patient gets L-dopa induced hallucinations

A

decrease dose of L-dopa; can consider giving low dose atypical antipsychotic

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26
Q

what to do when patient has dyskinesia and PD

A

reduce dose of L-dopa, add or increase dose of dopamine agonist

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27
Q

stiff-man sydnrome

A

fluctuating and progressive muscle rigidity with spasms; may be autoimmune or paraneoplastic process; lumbar hyperlordosis; painful spasms

28
Q

diagnosis of stiff man syndrome

A

characteristic clinical pic and demonstration of continuou motor unit activity without evidence of neuromyotonia, pyramidal or extrapyramidal dysfunction, or structural SC diseaes

29
Q

may see what kind of antibodies in stiff man syndrome/

A

antibodies directed against glutamic acid decarboxylase (GAD) or amphiphysin

30
Q

antispacity agents used in stiff man syndrome

A

benzos and baclofen

31
Q

baclofen

A

GABA agonist

32
Q

what is intention tremor?

A

action tremor that increases as the target is approached

33
Q

essential tremor

A

postural tremor but no resting or action tremor

34
Q

essential tremor affects which body parts

A

hands most commonly; but also the head, face, voice, trunk, or legs; bilateral;

35
Q

people with essential tremor get improvement with what

A

small amounts of alcohol

36
Q

treatment of ET

A

primidone and propranolol

37
Q

action or intenton tremor localizes to where?

A

cerebellum or its connection

38
Q

typical cause of resting tremor

A

parkinsons

39
Q

typicla cause of postural tremor

A

ET

40
Q

typical cause of action tremor

A

crbellum and cerbellar outflow tract dysfunction

41
Q

causes o fchorea

A

huntingtons, neuroacanthocytosis, Wilson disease, neuroleptics, antiparkinsonian meds, alcohol, anoxia, carbon monoxide, metabolic causes, SLE, post-strep

42
Q

ballism

A

large amplitude and poorly paterned flinging or flailing movements or a limb; frequently unilateral (hemiballismus)

43
Q

cause of ballism

A

contralateral lesion in the caudate putamen or subthal nuc; stroke is most common; hyperglycemia may also cause

44
Q

treatment for ballism

A

dopamine-depleting and blocking agents; contralateral thalamotomy or pallidotomy may be beneficial in severe disease

45
Q

dystonia

A

sustained muscle contraction leading to repetitive twisting movments or abnormal postures

46
Q

dystonic movements can be extignuished

A

by gently touchign the affected body part (geste antagoniste)

47
Q

what exacerbates dystonia

A

fatigue, stress, and emotional states; worsens during voluntary movement; may be suppressed by relaxation and sleep

48
Q

progression of dystonia

A

usually starts as action dystonia and progresses to dystonia at rest

49
Q

idiopathic torsion dystonia

A

primary dystonia; gene for the autosomal dominant familial form is on chrom 9q (mutations in torsin A)

50
Q

treatment for idiopathic torsion dystonia

A

botulinum toxin

51
Q

manifestations of idiopathic torsion

A

torticollis, writer’s cramp, blepharospasm, or spasmodic dysphonia

52
Q

myoclonus

A

sudden lightening-like movement produced by abrupt and brief mucle contraction or inhibition

53
Q

essential myoclonus

A

occurs in isolation without other neuro signs; some patients have improvements with small amt of alcohol

54
Q

treatment for myoclonus

A

clonazepam and valproate

55
Q

tourette syndrome

A

motor and vocal tics; more common in boys; periodic remission and exacerbation

56
Q

PANDAS

A

pediatric autoimmune neuro disorders assoc with strep infection; tics, OCD or both following GABHS infection

57
Q

treatment for tics

A

dopamine antagonists (haloperidol or the atypical antipsychotics) work best but due to side effecs try clonazepam and clonidine first

58
Q

wilson disease

A

autosomal recessive; defect in copper metabolism; presents with liver dysfunciton and neuropsych symptoms

59
Q

wilson disease copper accumulates where

A

liver and spillover into systemic circ, with deposition in the kidney, cornea, and CNS

60
Q

kayser-fleischer ring

A

copper deposition in Descemet’s membrane

61
Q

diagnosis of wilson’s disease

A

increased serum copper and decreased serum cerulosplasmin; increased 24 hour urinary copper excretion

62
Q

treatment of wilsons disaese

A

copper chelating with D-penicillamine or the less toxic chelator, trientine, in conjunction with zinc

63
Q

paroxysmal dyskinesias

A

tx with carbamazepine; recurrent attacks fo hyperkinesis with preserved consciousness; examples are paroxysmal kinesigenic choreoathetosis and paroxysmal dystonic choreoathetosis

64
Q

paroxysmal kineigenic choreoathetosis

A

episodes of chorea, athetosis, or dystonia are triggered by sudden movements and last for seconds to mins

65
Q

paroxysmal dystonic choreoathetosis

A

last minutes to hours; triggered by alcohol, fatigue, and stress;

66
Q

treatment for acute dystonic reaction caused by a drug

A

give an anticholinergic like benadryl

Neuro (43 decks)

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