Movement Disorders Flashcards
Basal ganglia
Responsible for unconscious motor control, smoothness of movements, coordination between different muscle groups; muscle tone
Direct and Indirect Pathways
D1: Direct pathway–> facilitates movement
D2: indirect pathway–>inhibits movement
–Normally dopamine: turns up direct, turns down indirect
**So: dopamine facilitates movement (DA is the key NT in extrapyramidal system, responsible for motor control)
Hyperkinesias
moving too much: tremor chorea tics dystonia myoclonus restless legs
Hypokinesias
not moving enough
Parkinsonism
Tremor
Rhythmic oscillatory movement produced by alternating or synchronous contraction of antagonist muscles Happens most: resting action (intentional)--> w/ ET postural
*Chin tremor seen with Parkinson’s
Essential Tremor
*tremor with posture and action (not chin) -upper ext>lower,head -insidious onset -worsens with age (peaks 20-30, then 60-70) -75% respond to alcohol
Tics
- Brief intermittent movements or sounds
- Sudden, abrupt, transient
- Repetitive and coordinated
- Vary in intensity, repeated at irregular intervals
- May resemble gestures, normal behavior
Can cause emergency if neck involvement and spinal cord damage over time.
Tourette Syndrome
Age of onset1 yr
- More common in males
- Assoc w/: ADHD, OCD, poor impulse control
- 25% of tics persist into adulthood
Pathogenesis:
SLITRK1 gene, chrom 13 (one pt only)
others: AD inheritance
Otherwise idiopathic
Treat tics if interfering with life
Treat OCD/ADHD
CBT
Biofeedback
Meds: Clonidine (antiHTN) SSRIs (OCD) Neuroleptics (fluphenazine, aripiprazole: SE weight gain, sedation) Tetrabenazine Clonazepam
Chorea
irregular, brief, dancing like, jerky movement
Diseases:
-Huntington (CAG repeat, chrom 4, anticipation; near 100% penetrant)
-Sydenham chorea (usally bilateral and transient)
-chorea gravidarum
-lupus (three neuro complications: abnormal movement, behavioral and mood problems, epilepsy)
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”: can be completely abolished in position of the affected part of the body
- Alleviating maneuver: actions that can improve or abolish dystonic posturing or tremor
- Note timing/duration
Etiology of Dystonia
Primary: DYT 1-12 (DYT1–protein torsin)
Secondary: cerebral palsy Neurodegen disorders (wilson's, PKND,ferritinopathies (NBIA), neruoacantocytosis) Hypoxic-ischemic brain injury post-stroke post-traumatic -meds/toxins
Classification of dystonia
Focal: blepharospasm hemifacial spasm oromandibular dystonia laryngeal dystonia spasmodic torticolis (cervical dystonia-->botox can help with this and Meige syndrome: belpharospasm and facial dystonia)
Segmental
Multifocal
Generalized
DBI for dystonia can also help
Athetosis
slow distal twisting movements
Myoclonus
sudden, brief, shock-like movements
Ataxia
poorly coordinated, clumsy movement
Parkinson disease (motor, non-motor sx)
resting tremor
bradykinesia or akinesia
rigidity
(others)
nonmotor: anosmia, dep, anxiety, constipation, cognitive impairment, drooling, visual disturbances, sensory changes, autonomic instability, sleep disturbance
ASYMMETRIC
DDx Parkinsonism
vascular, drug-induced, post-traumatic, post-infectious Lewy Body dementia FTDP PSP corticobasal degen Multiple Systems atrophy FRAXTAS
Atypical features for DDx Parkinsonism
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 Prominent retrocollis or anterocollis Eye movements abnormalities (downgaze!) Poor response to dopaminergic treatment
[Atypical parkinsonian syndromes: prognosis 7-10 y; only 10-15% respond to DA therapy; RARE]
PSP
Progressive, onset >50 impaired eye movements (downgaze!) Reptilian or scared look early onset of postural instability (falls w/in first yr) retrocollis
Multiple Systems Atrophy (MSA)
Avg life expectance 7-9 yr
Poor response to DA (25% are responsive)
Subtypes:
Parkinsonism (MSA-P)–striatonigral degeneration
Cerebellar sx, ataxia (MSA-C)–olivopontocerebellar degen
Autonomic sx (MSA-A)–Primary autonomic insufficiency; Shy-Dragger syndrome
Most common movement disorders in adults/kids
Adults
- restless leg syndrome
- essential tremor
- parkinson disease
(Children: tics)
Description of most movement disorder progression
usually start insidiously and progress slowly
–sudden onset and waxing and waning course are suspicious for Psychogenic movement disorder
Exceptions:
-waxing and waning course typical for tics
-Sudden onset:
poststroke, postischmic
toxin induced
postinfections
metabolic (mitochondrial)
Sensorimotor disorders
RLS
Tics
Akasthesia
Painful limbs/toes
Movement disorders of sleep
usually movement disorders disappear while asleep
Persisting in sleep include: REM sleep behavioral disorder; PLMS; Whipple’s disease
RLS is a disorder of restless wakefulness
Hypnogogic myoclonus happens while drowsy
**Most common movement disorder in children
TICS
**Indication of Wilson’s disease
Tremor in young person (especially big floppy tremor)–Wilson’s until proven otherwise
Don’t miss because chelation therapy available and can stop progression
