Movement Disorders Flashcards
Hypokinetic Disorders
Parkinsons Disease
Parkinsonian like disease
Hyperkinetic disorders or symptoms
termor
dystonia
chorea
Parkinson Disease (PD)- pathology
Lose DA projections from substantia nigra to the caudate and putamen- the nigrostriatal tracts
PD and Lewy bodies
Lewy bodies are alpha synuclein deposits ni the cytoplasm of the nigrostriatal pathway neurons
SSx and PD
STRAPD shuffling gait tremor- resting, pill rolling Rigidity- cogwheel rigidity Akinesia/bradykinesia- slow absence of movement Autonomic dysfunction- orthostasis, neurognenic bladder, constipation, ED Postural Instability Dementia, depression
Dx
Clinical
can only see substantia nigra is on autopsy
Treatment-
Cinemet- Carbidopa/ Levadopa
anticolonergics- benztropine
dopamine receptor agonists
COMT and MAOD inhibitors
Best drug for depression in PD
Amitryptilline
Best drugs for psychosis in PD
Clonazapine, Olanzapine, Quitiapine
Best drugs for dementia in PD
donepezil and rivastigmine
Surgery and PD tx
use DBS of STB, GPI and VIM
Essential tremor
Rhythmic oscillating movements of agonits and antagonist resulting in an intention tremor.
most common hyperkinetic disorder
What parts of the body does ET affect
posture, heads, neck, and hands
worsens with movement
Essential tremor- etiology
AD inheritance
ET- age of onset
bimodal distribution in 20s and 60s
ET- treatment
Pts often self medicate with alcohol
beta blockers ie propranolol work
Dystonia- definition
involuntary sustained muscle contraction leading to twisting or repetitive movements.
Focal dystonia- types
Torticollis
Bleophasm
Global dystonia- etiology
hereditary
or medication induced- antipsychotics, metaloclopramide
Treatment of Dystonia
Anticholinergics Muscle relaxants Botox Sx DBS
How does botox work
Botox blocks presynaptic ACH receptors by inhibiting one of the 3 types of proteins that make up the SNARE complex.
Each type of botox (A,B,C) inhibit a dif protein in the SNARE complex.
Torticollis
dystonia of the SCM and Trap so the head points towards the effected SCM and the chin points away from it
Huntington disease- etiology
AD mutation on chromosome 4- CAG repeats
HD- changes in the brain
atrophy of caudate caused by frank neuronal loss
What is anticipation, how does it relate to HD
HD exhibits anticipation
diz presents earlier and causes death earlier the larger the repeat gets.
the repeat ges larger every generation
HD- SSx
chorea dementia lack of coordination personality changes depression and anxiety
HD- Dx
chromosomal analysis
HD- tx
none
symptomatic relief with neuroleptics and benzos
Wilsons dz
hepatorenticuar degeneration
WD- damages what organs
liver and brain
WD liver damage
defective hepatic transport of Cu into bile for excretion –> cirrhosis, inc Cu in liver and spleen
WD- lenticular damage
Cu builds up in putamen and subhalamic nucleus –> PD like movement disorders or hemiballismus
WD- lab findings
Cu deposits in tissues
total serum Cu is low but free urine and reum Cu are higher in later stages
Serum Ceruloplasmin is low
WD- clinical findings
Kayser Fleischer ring
movement disorders- hemibalismus, dementia
hepatosplenomegaly
hemolytic anemia
renal dz- fanconi syndrome b.c of damage to proximal tubules –> inc excretion of glucose AA, and Phosphate
WD- tx
penicilalmine
Zn
liver transplant