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export_movement disorders Flashcards

1
Q

Basal Ganglia structures

A

-Involved in motor control and planning as well as emotions, cognition, and eye movements -Caudate & Putamen ( striatum) Putamen & globus pallidus (lenticular nuc) -putamen, GP, Caudate nucleus, subthalmic nucleus, and substantia nigra -Reciprocal connections to cortex via thalamus -Main site of dopamine for 3/4 pathways: nigrostriatal, mesolimbic, mesocortical

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2
Q

Chorea/athetosis

A

-‘dance-like; irregular, asymmetric, random and continuous. not rhythmic but flow from one group to another. -Athetosis: snake-like: slow twisting/writhing- more distal muscles of fingers, arms, legs, neck

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3
Q

Ballism

A

Violent, involuntary flailing of extremities

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4
Q

Tremor

A

-most common - rhythmic movement due to alternating contractions between agonist and antagonist

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5
Q

Tics

A

-Random, repetitive, rapid, purposeless that occur at random intervals -Motor or vocal - Semi-voluntary: can be suppressed with hard concentration

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6
Q

Myoclonus

A

rapid, brief, uncontrollablejerks

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7
Q

Dystonia

A

sustained, abnormal posture caused by simultaneous activation of both agonist and antagonist muscles

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8
Q

Akathisia

A

subjective sense of inner restlessness where pts feel compelled to move continuously

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9
Q

Parkinson’s disease-cardinal

A

-Resting tremor, disappears with movement/sleep -Rigidity- cogwheel -Akinesia/Bradykinesia: slowness of movement -Postural instability- shuffling, festinating gait, freezing

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10
Q

PD:other symptoms

A

depression, apathy, make facies, anosmia, dysphagia, dysarthria, micrographia, autonomic dysfunction, Rem sleep disturbances

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11
Q

PD pathopysiology

A

-only shows up when 80% neurons at Sn gone -Lewy Bodies -Clinical dx but DaTscan can be used. to check for DA transporters

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12
Q

Progressive Supranuclear Palsy

A

-Taupathy, no tx -Reptilian stare, vertical gaze parlies ( esp down), -Hummingbird, MMouse sign in MRI (smaller MRI) -Mood changes, progressive mild dementia -Spectacular falls early on

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13
Q

Essential Tremor

A

-Action tremor - OFten b/l -Often familial -Improves with alcohol - Lack of bradykinesia, rigidity, gait abnormalities -Much more common than PD

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14
Q

Essential Tremor-TX

A

-Propanolol/Inderal (non-selective) -Primidone -DON’T want b1 blockers

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15
Q

Med induced Parkinsonianism

A

High potency anti-psychotics and anti-emetics

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16
Q

MSA

A

-Lack of med response -Wide based gate -Eye movement problems -Symmetric onset -Rapid onset –within 5 yrs - Early onset falling -Prominent autonomic dysfunction

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17
Q

Sinemet

A

-Carbidopa/levadopa -Carbadopa prevents conversion of levadopa to DA, also doesn’t cross BBB - Levadopa then can be converted in CNS -Can cause dyskinesias - Psych side effects

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18
Q

Requip/Ropinirole OR Mirapex/Pramipexole

A

-CI in elderly due to psych SE - DA Agonist; first line -Less powerful but good for early stage - Can cause disinhibition

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19
Q

COMT inhibitors

A

-entacapone, tolcapone -Usually with Sinemet and prolongs it ( delays wearing off) -Blocks enzyme that breaks down levadopa

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20
Q

Other PD drugs

A

amantadine, MAO-inhibitors (selegiline), -Anti-ACH such as trihexyphenidyl (Artane)

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21
Q

MSA

A

-hot crossed buns sign

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22
Q

Huntington’s Disease

A
  • Atrophy of head of caudate - Boxcar ventricle -Late onset ( >40 y.o) - AD, CAG repeats with anticipation; Chr 4 - > 40 repeats is associated with disease -familial dementia; Chorea, psychosis -tx : haloperidol; tetrabenazine ( DA depleting) -relentless deterioration and death after 15 yrs
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23
Q

Chorea Gravidarum

A

-Occurs in pregnant ladies

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24
Q

Syndenham’s Chorea

A

-from childhood infection with Group b hemolytic strep and occurs in its with rheumatic fever

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25
Q

Wilson’s Disease

A

-Abnormal Cu accumulation -Hepatolenticular degeneration -KF rings in descemet’s membrane, giant panda sign in midbrain - Low serum ceruloplasmin ( 50 mcg/day elevated LFTS: AST, ASLT, PT&PTT; low albumin -AR disease

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26
Q

Wilson’s disease –presentation

A

-Wing flap tremor -Mood/Psych symptoms

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27
Q

Wilson’s Disease TX

A

-Low Cu diet (no chocolate, nuts, shellfish) -Zn salts block absorption -Trientine> Penicillamine (chelators) -Severe Liver tx

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28
Q

CO poisoning

A

-Parkinsonism -B/l signal abnromality in Glob pallidus

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29
Q

Botox

A

-Treatment of choice for torticolis/ Blepherospasm (involuntary, forceful closure of the eyelids)

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30
Q

Restless Leg Syndrome

A

-Uncomfortable sensation: starts during rest, relief w/ movement, worsening of symptoms int he evening. -Often precedes more serious PD; can be SE of SSRI’s -Check for Fe defeciency -1st line: DA agonist, i.e. roperinole

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31
Q

Akathisia Tx

A

treat with propranolol/inderal

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32
Q

Tx for fever/rigidity with haloperidol

A

Dopamine agonists ie. bromocriptine CCB (ie. Dantrolene is also used)

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33
Q

Copropraxia

A

obscene gestures in Tourette’s

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34
Q

Coprolalia

A

-Obscene vocalizations

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35
Q

Hepatic encephalopathy

A
  • AMS, spider angiomas,icteric sclera, increased muscle tone, hyperreflexia, and + Babanski -+ asterixis -tx: lactulose
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36
Q

Tourette’s dx

A

Both multiple motor and at least one vocal tic Tic that occurs many times a day ( usually in bouts) nearly everyday or intermittently through a period of more than 1 yr. during this period there is never a tic free interval greater thAn 3 months. Before 18 yo

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37
Q

Tourette’s tx

A

Guanfacine/tenex or clonidine Antipsychotics like haldol and risperdal are also. In refractory, terra benzine is an option Tx when child is bothered not parent

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38
Q

PANDAS

A

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections is neuro-psych disorder that also manifests with tics and OCD symptoms

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39
Q

If a patient presents with a head tremor, the most likely cause is?

A

Essential tremor

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40
Q

What type of tremor begins at rest and intensifies with movement?

A

Rubral tumor 2/2 midbrain injury that interferes a/ cerebellar outflow tracts Low hz freq (3-5) compared to pd (5-7) or physiologic (10-12)

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41
Q

A physiologic tumor can be amplified by

A

Sympathetic activity–beta adregenergic drugs Is the most common cause of postural and action tremors

42
Q

What is the most common cause of postural and action tremors?

A

Physiologic tremor that is amplified by sympathetic activity

43
Q

Intention tremor is distinguished from an action tremor by

A

Intention worsens as the hand moves closer to its target whereas action postural tremors will stay the same throughout movement

44
Q

50% of pts w/ ET have a family hx. ET has this mode of inheritance

A

AD

45
Q

a glass of wine or beer is known to decrease the intensity of this tremor…

A

ET

46
Q

what is the westphal variant

A

variant of hungintons disease that develops in young children and is charaterized by a more parkinsonian picture that includes -bradykinesia -rigidity -dystonia -myoclonus -seizures

47
Q

how is huntingtons disease best diagnosed?

A

genetic testing for CAG repeats > 40 consistent with disease -look at huntingtin gene on chromosome 4

48
Q

what is chorea? what disease is it commonly associated with?

A

sudden jerky irregular movements w/ muscle contractions that appear to flow from one to the next “dance like” huntingtons disease

49
Q

what is athetosis? what disease is it commonly associated with?

A

HD twisting and writhing movements–often associated with chorea

50
Q

what is tardive dyskinesia?

A

neurologic disorder 2/2 to long term use of high dose dopamine antagonists -impairment of voluntary movement that continues even after drugs are d/c

51
Q

in HD we would expect the patients reflexes to be—

A

hyperreflexic

52
Q

-Involved in motor control and planning as well as emotions, cognition, and eye movements -Caudate & Putamen ( striatum) Putamen & globus pallidus (lenticular nuc) -putamen, GP, Caudate nucleus, subthalmic nucleus, and substantia nigra -Reciprocal connections to cortex via thalamus -Main site of dopamine for 3/4 pathways: nigrostriatal, mesolimbic, mesocortical

A

Basal Ganglia structures

53
Q

-‘dance-like; irregular, asymmetric, random and continuous. not rhythmic but flow from one group to another. -Athetosis: snake-like: slow twisting/writhing- more distal muscles of fingers, arms, legs, neck

A

Chorea/athetosis

54
Q

Violent, involuntary flailing of extremities

A

Ballism

55
Q

-most common - rhythmic movement due to alternating contractions between agonist and antagonist

A

Tremor

56
Q

-Random, repetitive, rapid, purposeless that occur at random intervals -Motor or vocal - Semi-voluntary: can be suppressed with hard concentration

A

Tics

57
Q

rapid, brief, uncontrollablejerks

A

Myoclonus

58
Q

sustained, abnormal posture caused by simultaneous activation of both agonist and antagonist muscles

A

Dystonia

59
Q

subjective sense of inner restlessness where pts feel compelled to move continuously

A

Akathisia

60
Q

-Resting tremor, disappears with movement/sleep -Rigidity- cogwheel -Akinesia/Bradykinesia: slowness of movement -Postural instability- shuffling, festinating gait, freezing

A

Parkinson’s disease-cardinal

61
Q

depression, apathy, make facies, anosmia, dysphagia, dysarthria, micrographia, autonomic dysfunction, Rem sleep disturbances

A

PD:other symptoms

62
Q

-only shows up when 80% neurons at Sn gone -Lewy Bodies -Clinical dx but DaTscan can be used. to check for DA transporters

A

PD pathopysiology

63
Q

-Taupathy, no tx -Reptilian stare, vertical gaze parlies ( esp down), -Hummingbird, MMouse sign in MRI (smaller MRI) -Mood changes, progressive mild dementia -Spectacular falls early on

A

Progressive Supranuclear Palsy

64
Q

-Action tremor - OFten b/l -Often familial -Improves with alcohol - Lack of bradykinesia, rigidity, gait abnormalities -Much more common than PD

A

Essential Tremor

65
Q

-Propanolol/Inderal (non-selective) -Primidone -DON’T want b1 blockers

A

Essential Tremor-TX

66
Q

High potency anti-psychotics and anti-emetics

A

Med induced Parkinsonianism

67
Q

-Lack of med response -Wide based gate -Eye movement problems -Symmetric onset -Rapid onset –within 5 yrs - Early onset falling -Prominent autonomic dysfunction

A

MSA

68
Q

-Carbidopa/levadopa -Carbadopa prevents conversion of levadopa to DA, also doesn’t cross BBB - Levadopa then can be converted in CNS -Can cause dyskinesias - Psych side effects

A

Sinemet

69
Q

-CI in elderly due to psych SE - DA Agonist; first line -Less powerful but good for early stage - Can cause disinhibition

A

Requip/Ropinirole OR Mirapex/Pramipexole

70
Q

-entacapone, tolcapone -Usually with Sinemet and prolongs it ( delays wearing off) -Blocks enzyme that breaks down levadopa

A

COMT inhibitors

71
Q

amantadine, MAO-inhibitors (selegiline), -Anti-ACH such as trihexyphenidyl (Artane)

A

Other PD drugs

72
Q

-hot crossed buns sign

A

MSA

73
Q
  • Atrophy of head of caudate - Boxcar ventricle -Late onset ( >40 y.o) - AD, CAG repeats with anticipation; Chr 4 - > 40 repeats is associated with disease -familial dementia; Chorea, psychosis -tx : haloperidol; tetrabenazine ( DA depleting) -relentless deterioration and death after 15 yrs
A

Huntington’s Disease

74
Q

-Occurs in pregnant ladies

A

Chorea Gravidarum

75
Q

-from childhood infection with Group b hemolytic strep and occurs in its with rheumatic fever

A

Syndenham’s Chorea

76
Q

-Abnormal Cu accumulation -Hepatolenticular degeneration -KF rings in descemet’s membrane, giant panda sign in midbrain - Low serum ceruloplasmin ( 50 mcg/day elevated LFTS: AST, ASLT, PT&PTT; low albumin -AR disease

A

Wilson’s Disease

77
Q

-Wing flap tremor -Mood/Psych symptoms

A

Wilson’s disease –presentation

78
Q

-Low Cu diet (no chocolate, nuts, shellfish) -Zn salts block absorption -Trientine> Penicillamine (chelators) -Severe Liver tx

A

Wilson’s Disease TX

79
Q

-Parkinsonism -B/l signal abnromality in Glob pallidus

A

CO poisoning

80
Q

-Treatment of choice for torticolis/ Blepherospasm (involuntary, forceful closure of the eyelids)

A

Botox

81
Q

-Uncomfortable sensation: starts during rest, relief w/ movement, worsening of symptoms int he evening. -Often precedes more serious PD; can be SE of SSRI’s -Check for Fe defeciency -1st line: DA agonist, i.e. roperinole

A

Restless Leg Syndrome

82
Q

treat with propranolol/inderal

A

Akathisia Tx

83
Q

Dopamine agonists ie. bromocriptine CCB (ie. Dantrolene is also used)

A

Tx for fever/rigidity with haloperidol

84
Q

obscene gestures in Tourette’s

A

Copropraxia

85
Q

-Obscene vocalizations

A

Coprolalia

86
Q
  • AMS, spider angiomas,icteric sclera, increased muscle tone, hyperreflexia, and + Babanski -+ asterixis -tx: lactulose
A

Hepatic encephalopathy

87
Q

Both multiple motor and at least one vocal tic Tic that occurs many times a day ( usually in bouts) nearly everyday or intermittently through a period of more than 1 yr. during this period there is never a tic free interval greater thAn 3 months. Before 18 yo

A

Tourette’s dx

88
Q

Guanfacine/tenex or clonidine Antipsychotics like haldol and risperdal are also. In refractory, terra benzine is an option Tx when child is bothered not parent

A

Tourette’s tx

89
Q

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections is neuro-psych disorder that also manifests with tics and OCD symptoms

A

PANDAS

90
Q

Essential tremor

A

If a patient presents with a head tremor, the most likely cause is?

91
Q

Rubral tumor 2/2 midbrain injury that interferes a/ cerebellar outflow tracts Low hz freq (3-5) compared to pd (5-7) or physiologic (10-12)

A

What type of tremor begins at rest and intensifies with movement?

92
Q

Sympathetic activity–beta adregenergic drugs Is the most common cause of postural and action tremors

A

A physiologic tumor can be amplified by

93
Q

Physiologic tremor that is amplified by sympathetic activity

A

What is the most common cause of postural and action tremors?

94
Q

Intention worsens as the hand moves closer to its target whereas action postural tremors will stay the same throughout movement

A

Intention tremor is distinguished from an action tremor by

95
Q

AD

A

50% of pts w/ ET have a family hx. ET has this mode of inheritance

96
Q

ET

A

a glass of wine or beer is known to decrease the intensity of this tremor…

97
Q

variant of hungintons disease that develops in young children and is charaterized by a more parkinsonian picture that includes -bradykinesia -rigidity -dystonia -myoclonus -seizures

A

what is the westphal variant

98
Q

genetic testing for CAG repeats > 40 consistent with disease -look at huntingtin gene on chromosome 4

A

how is huntingtons disease best diagnosed?

99
Q

sudden jerky irregular movements w/ muscle contractions that appear to flow from one to the next “dance like” huntingtons disease

A

what is chorea? what disease is it commonly associated with?

100
Q

HD twisting and writhing movements–often associated with chorea

A

what is athetosis? what disease is it commonly associated with?

101
Q

neurologic disorder 2/2 to long term use of high dose dopamine antagonists -impairment of voluntary movement that continues even after drugs are d/c

A

what is tardive dyskinesia?

102
Q

hyperreflexic

A

in HD we would expect the patients reflexes to be—

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