Movement Disorders

Question 1

Basal ganglia

Answer 1

Responsible for unconscious motor control, smoothness of movements, coordination between different muscle groups; muscle tone

Question 2

Direct and Indirect Pathways

Answer 2

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)

Question 3

Hyperkinesias

Answer 3

moving too much:
tremor
chorea
tics
dystonia
myoclonus
restless legs

Question 4

Hypokinesias

Answer 4

not moving enough

Parkinsonism

Question 5

Tremor

Answer 5

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

Question 6

Essential Tremor

Answer 6

*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

Question 7

Tics

Answer 7

• 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.

Question 8

Tourette Syndrome

Answer 8

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

Question 9

Chorea

Answer 9

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)

Question 10

Dystonia

Answer 10

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

Question 11

Etiology of Dystonia

Answer 11

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

Question 12

Classification of dystonia

Answer 12

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

Question 13

Athetosis

Answer 13

slow distal twisting movements

Question 14

Myoclonus

Answer 14

sudden, brief, shock-like movements

Question 15

Ataxia

Answer 15

poorly coordinated, clumsy movement

Question 16

Parkinson disease (motor, non-motor sx)

Answer 16

resting tremor
bradykinesia or akinesia
rigidity
(others)

nonmotor: anosmia, dep, anxiety, constipation, cognitive impairment, drooling, visual disturbances, sensory changes, autonomic instability, sleep disturbance

ASYMMETRIC

Question 17

DDx Parkinsonism

Answer 17

vascular, drug-induced, post-traumatic, post-infectious
Lewy Body dementia
FTDP
PSP
corticobasal degen
Multiple Systems atrophy
FRAXTAS

Question 18

Atypical features for DDx Parkinsonism

Answer 18

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]

Question 19

PSP

Answer 19

Progressive, onset >50
impaired eye movements (downgaze!)
Reptilian or scared look
early onset of postural instability (falls w/in first yr)
retrocollis

Question 20

Multiple Systems Atrophy (MSA)

Answer 20

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

Question 21

Most common movement disorders in adults/kids

Answer 21

Adults

1. restless leg syndrome
2. essential tremor
3. parkinson disease

(Children: tics)

Question 22

Description of most movement disorder progression

Answer 22

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)

Question 23

Sensorimotor disorders

Answer 23

RLS
Tics
Akasthesia
Painful limbs/toes

Question 24

Movement disorders of sleep

Answer 24

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

Question 25

**Most common movement disorder in children

Answer 25

TICS

Question 26

**Indication of Wilson’s disease

Answer 26

Tremor in young person (especially big floppy tremor)–Wilson’s until proven otherwise

Don’t miss because chelation therapy available and can stop progression