Movement Disorders Flashcards
Hyperkinesias
Moving too much
Tremor Chorea Tics Dystonia Myoclonus
Hypokinesias
Not moving enough
Parkinson’s
Summary of basil ganglia function
Responsible for unconscious motor control: smoothness of the movements, coordination between different muscle groups muscle tone etc
What is the key neurotransmitter in the extrapyramidal system, responsible for motor control?
Dopamine
What is the most common movement disorder in the general population?
Restless leg syndrome (12% in general population)
What is the most common movement disorder seen in clinic?
Parkinson’s disease
Tremor
Definition: Rhythmic oscillatory movement produced by alternating or synchronous contraction of antagonist muscles
Happens most when resting, action (intentional), postural (with sustained posture)
Frequency- Slow or fast ( ex. PD tremor 6 HZ)
Regular or jerky
Essential tremor
Clinical features:
Tremor with posture and action Upper extremities > lower, head Insidious onset Worsens with age 75% respond to alcohol
Usually familial
Tics
Definition:
Brief, intermittent movements or sounds Sudden, abrupt, transient Repetitive and coordinated Vary in intensity, repeated at irregular intervals May resemble gestures, normal behavior
Tourettes
Age of onset 1 year
Clinical features:
Onset in youth, more common males
Associated with:
ADHD,
OCD,
poor impulse control
Tics persist into adulthood 25%
Chin tremor
Parkinson’s
Head and voice tremor
Essential tremor
Tremor in young pts is suspicious for what?
Wilson’s disease
Especially if floppy tremor
Most common movement disorder in children
tics
Treatment of Tourettes
Educate family, patient, school Support groups Treat tics only if interfering with life Treat OCD, ADHD Cognitive-Behavioral Therapy (CBT) (BEST BEHAVIORAL INTERVENTION THAT HAS BEEN STUDIED) Biofeedback
Meds for Tourettes
Clonidine - antihypertensive
SSRIs: OCD
Neuroleptics:
Fluphenazine, aripiprazole (Side effects: weight gain, sedation)
Tetrabenazine –Dopamine depleting drug,
Clonazepam
Chorea
Irregular, brief, dancing like, jerky
Move from one body part to another
What diseases have Chorea?
Huntington disease
Sydenham chorea
Chorea gravidarum
Lupus
Dystonia
Co-contraction of muscles agonists and antagonists
Sustained muscle contractions causing twisting, abnormal postures
Can be associated with tremor
Position dependent Presence of “Null point” Alleviating maneuver (sensory tricks, geste antagoniste)
What is the etiology of primary distonia
DYT 1 – Protein Torsin
Causes of secondary distonia
Cerebral palsy
Neurodegenerative disorders (Wilson’s Disease, Panthokinase-assocoated neurodegeneration (PKND), Other ferritinopathies (NBIA), Neuroacantocytosis)
Hypoxic- ischemic brain injury
Poststroke
Posttraumatic
Medications, toxins
3 cardinal symptoms of Parkinson’s
Resting tremor
Bradykinesia or akinesia
Rigidity
Red flags that a patient might have an atypical form of Parkinson’s
Rapid progression of the disease
Early onset of dementia
Early onset of autonomic instability
Early onset or predominance of postural instability
Confusion or hallucinations, unrelated to medications
Ataxia
Downgaze impairment
Atypical parkinsonian syndromes
Prognosis: average 7-10 years
Worse than Parkinson disease
Treatment:
Only 10-15% respond to dopaminergic therapy
Rare
LBD – Lewy Bodies Dementia
FTDP – Frontotemporal dementia-parkinsonism
PSP – Progressive Supranuclear Palsy
CBD – Corticobasal degeneration
MSA – Multiple Systems Atrophy
FRAXTAS – Fragile X associated Ataxia Tremor syndrome
Progressive supranuclear palsy (PSP)
Progressive, onset > 50, Impaired eye movements Downgaze ! “Reptilian” or scared look Early onset of postural instability Falls within the first year ! Retrocollis
Multiple systems atrophy (MSA)
Average life expectance 7-9 years
Poor response to dopamine replacement treatments ( 25% can be responsive)
What is the typical presentation of a movement disorder?
Usually start insidiously and progress slowly
Sudden onset is suspecious for psychogenic movement disorder
Movement disorders and sleep
Usually movement disorders disappear while at sleep
Movement disorders that persist or start during sleep:
REM sleep behavioral disorder
PLMS
Whipple’s disease
RLS is a disorder of restful wakefulness
Hypnogogic myoclonus happen while drowsy
