export_movement disorders Flashcards by Ahana Sen

Basal Ganglia structures

-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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Chorea/athetosis

-‘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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Ballism

Violent, involuntary flailing of extremities

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Tremor

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

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Tics

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

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Myoclonus

rapid, brief, uncontrollablejerks

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Dystonia

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

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Akathisia

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

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Parkinson’s disease-cardinal

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

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PD:other symptoms

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

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PD pathopysiology

-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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Progressive Supranuclear Palsy

-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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Essential Tremor

-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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Essential Tremor-TX

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

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Med induced Parkinsonianism

High potency anti-psychotics and anti-emetics

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MSA

-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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Sinemet

-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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Requip/Ropinirole OR Mirapex/Pramipexole

-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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COMT inhibitors

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

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Other PD drugs

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

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MSA

-hot crossed buns sign

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Huntington’s Disease

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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Chorea Gravidarum

-Occurs in pregnant ladies

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Syndenham’s Chorea

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

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Wilson's Disease

-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

Wilson's disease --presentation

-Wing flap tremor -Mood/Psych symptoms

Wilson's Disease TX

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

CO poisoning

-Parkinsonism -B/l signal abnromality in Glob pallidus

Botox

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

Restless Leg Syndrome

-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

Akathisia Tx

treat with propranolol/inderal

Tx for fever/rigidity with haloperidol

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

Copropraxia

obscene gestures in Tourette's

Coprolalia

-Obscene vocalizations

Hepatic encephalopathy

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

Tourette's dx

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

Tourette's tx

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

PANDAS

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

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

Essential tremor

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

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 physiologic tumor can be amplified by

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

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

Physiologic tremor that is amplified by sympathetic activity

Intention tremor is distinguished from an action tremor by

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

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

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

what is the westphal variant

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

how is huntingtons disease best diagnosed?

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

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

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

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

HD twisting and writhing movements--often associated with chorea

what is tardive dyskinesia?

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

in HD we would expect the patients reflexes to be---

hyperreflexic

Basal Ganglia structures

-'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

Chorea/athetosis

Violent, involuntary flailing of extremities

Ballism

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

Tremor

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

Tics

rapid, brief, uncontrollablejerks

Myoclonus

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

Dystonia

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

Akathisia

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

Parkinson's disease-cardinal

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

PD:other symptoms

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

PD pathopysiology

-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

Progressive Supranuclear Palsy

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

Essential Tremor

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

Essential Tremor-TX

High potency anti-psychotics and anti-emetics

Med induced Parkinsonianism

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

MSA

-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

Sinemet

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

Requip/Ropinirole OR Mirapex/Pramipexole

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

COMT inhibitors

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

Other PD drugs

-hot crossed buns sign

MSA

- 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

Huntington's Disease

-Occurs in pregnant ladies

Chorea Gravidarum

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

Syndenham's Chorea

Wilson's Disease

-Wing flap tremor -Mood/Psych symptoms

Wilson's disease --presentation

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

Wilson's Disease TX

-Parkinsonism -B/l signal abnromality in Glob pallidus

CO poisoning

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

Botox

Restless Leg Syndrome

treat with propranolol/inderal

Akathisia Tx

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

Tx for fever/rigidity with haloperidol

obscene gestures in Tourette's

Copropraxia

-Obscene vocalizations

Coprolalia

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

Hepatic encephalopathy

Tourette's dx

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

Tourette's tx

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

PANDAS

Essential tremor

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

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)

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

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

A physiologic tumor can be amplified by

Physiologic tremor that is amplified by sympathetic activity

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

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

Intention tremor is distinguished from an action tremor by

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

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

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

what is the westphal variant

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

how is huntingtons disease best diagnosed?

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

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

HD twisting and writhing movements--often associated with chorea

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

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

what is tardive dyskinesia?

hyperreflexic

in HD we would expect the patients reflexes to be---