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Movement Disorders Flashcards

1
Q

Movement Disorders

A
  • result of dysfunction or damage to portions of the brain (basal ganglia)
  • to little movement (hypokinetic)
  • to much unwanted movement (hyperkinetic)
  • mixed movement disorders
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2
Q

Function of the Basal Ganglia

A

-Modulate Movement
Facilitate intended movements
Suppress unwanted movements

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3
Q

Hypokinetic Movement Disorders

A
  • Parkinson’s disease
  • Progressive supranuclear palsy
  • Multiple system atrophy
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4
Q

Parkinson’s Disease: Demographics

A

-common neurological disease
100 per 100,000 people (2000 in above age 60)
-unknown etiology, genetic factors important if onset before age 50, others important if later (env.)
-typical age of onset: 55-65
-life expectancy (with treatment) nears normal
-10% develop symptoms before 40

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5
Q

Parkinson’s Disease: Neurologic Features

A
  • asymmetric onset - eventually bilateral
  • primary extrapyramidal features:
    1. rest tremor
    2. rigidity - inc. muscle tone in passive movement
    3. bradykinesia - hard to start/stop movement, slow to cary out, hard to repeat motion
    4. postural instability (late), balance impairment
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6
Q

Parkinson’s Disease: Motor Features

A
  • secondary extrapyramidal features
  • masked face - dec. facial expression
  • diminished blink frequency
  • stooped posture
  • small-stepped, shuffling gait
  • reduced armswing
  • hypokinetic dysarthria - softer, slurred speech
  • micrographia - smaller handwriting
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7
Q

Parkinson’s Disease: Non-Motor Features

A
  • depression 40-50%, may precede motor
  • anxiety 40%
  • cognitive impairment (frontal lobe (executive)) with progression: if early not it
    • executive: difficulty making decisions & carrying out plans
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8
Q

Parkinson’s Disease: Autonomic Dysfunction

A
  • Gastrointestinal: constipation
  • Urinary: over/under activing
  • Sexual: ED in men, dec. libido
  • Cardiovascular: orthostatic HTN
  • Thermoregulatory: sweating
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9
Q

Parkinson’s Disease: Others

A
  • olfactory dysfunction
  • visual dysfunction
  • sensory symptoms (pain)
  • Sleep disturbances (sleep fragmentation, REM sleep behavior disorder)
  • Fatigue
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10
Q

Parkinson’s Disease: Pathologic Features

A
  • degeneration of pigmented neurons
    • substantia nigra pars compacta
    • locus ceruleus
    • dorsal vagal nucleus
  • Lewy body formation (cytoplasmic inclusion bodies) “fried egg” appearance
    • central/enteric nervous system
    • protein: alpha-synuclein (synucleinopathies)
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11
Q

Parkinson’s Disease: Neurochemical Features

A
  • Dopamine deficiency (nigrostriatal, mesolimbic, hypothalamic, retinal)
  • Norepinephrine
  • Serotonin
  • Glutamate
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12
Q

Progressive Supranuclear Palsy

A
  • characterized by features of Parkinsonism but some additional ones
  • Parkinsonism-plus syndrome
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13
Q

Progressive Supranuclear Palsy: Demographics

A
  • sporadic
  • prevalence is 1-6.5 per 100,000
  • onset 50-60
  • life expectancy is ~10 yrs
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14
Q

Progressive Supranuclear Palsy: Neurologic Features

A

Extrapyramidal Features

  • Rigidity: especially axial rigidity, may produce neck hyperextension, symmetric
  • Bradykinesia
  • “Astonished” facial expression
  • Dysarthria
  • gait disturbance (early)
  • postural instability (falling) (early)
  • tremor is unusual
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15
Q

Progressive Supranuclear Palsy: Behavioral Features

A
  • emotional lability

- dementia

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16
Q

Progressive Supranuclear Palsy: Ophthalmologic Features

A
  • supranuclear gaze palsy
    • vertical (downwardgaze) first (messy eater, “dirty tie” sign, difficulty descending stairs
    • horizontal later
    • oculocephalic reflex intact
  • apraxia of eyelid opening (hard to open when asked, can do it spontaneously)
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17
Q

Progressive Supranuclear Palsy: Other Neurologic Features

A
  • pyramidal tract signs (Babinski sign)

- sleep disturbances

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18
Q

Progressive Supranuclear Palsy: Pathologic Features

A
  • Midbrain & cerebral cortical atrophy
  • Neuronal loss & gliosis (multiple areas)
  • Neurofibrillary tangles (globose type), composed of unpaired straight filaments, contain abnormally phosphorylated tau protein (tauopathy)
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19
Q

Progressive Supranuclear Palsy: Neurochemical Features

A
  • striatal domamine deficiency

- dec. ACh, GABA, Norepinephrine

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20
Q

Multiple System Atrophy

A

-Parkinsonism-plus syndrome

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21
Q

Multiple System Atrophy: Demographics

A
  • Sporadic
  • Prevalence is 2.3-5.7 per 100,000
  • symptom onset typically age 50-55
  • life expectancy is ~5-10 yrs
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22
Q

Multiple System Atrophy: Neurologic Featuers

A
  • present: parkinsonism, autonomic failure, or cerebellar syndrome
  • Extrapyramidal (89%)
  • Rigidity
  • Bradykinesia
  • Postural instability (early)
  • Tremor is unusual
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23
Q

Multiple System Atrophy: Neurologic: Autonomic

A

78%

  • urinary dysfunction
  • orthostatic HTN
  • impotence (males)
  • gastrointestinal dysfunction
  • thermoregulatory dysfunction
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24
Q

Multiple System Atrophy Neurologic - Cerebellar

A

55%

  • ataxia
  • dysarthria
  • oculomotor abnormalities
  • exaggerated rebound
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25
Multiple System Atrophy: Neurologic - Pyramidal
61% - hyperreflexia - Babinski responses - Spasticity - Psuedobulbar palsy
26
Multiple System Atrophy: Neurologic - Behavioral
22% - personality changes - depression - cognitive dysfunction (frontal lobe executive dysfunction) - dementia typically does not develop
27
Multiple System Atrophy: Neurologic - Other
- respiratory stridor, vocal cord abductor weakness, 33%, inc. sudden nocturnal death - involuntary sighing - Raynaud's phenomenon - postural myoclonus of the hands
28
Multiple System Atrophy: Pathologic Features
- Cell loss & Gliosis: basal ganglia, brainstem, cerebellum, spinal cord - Glial cytoplasmic inclusion bodies - alpha-synuclein
29
Multiple System Atrophy: Neurochemical Features
-not well characterized
30
Hyperkinetic Movement Disorders
- Huntington's disease - Tourette's Syndrome - Primary (idiopahtic) Dystonia
31
Huntington's Disease: Demographics
- autosomal dominant inheritance (mutation on short arm of chromosome 4, expanded trinucleotide repeat, codes for "huntingtin" protein - onset 35-45 - younger age of onset associated with more rapid course - after symptoms: life 15-20 yrs
32
Huntington's Disease: Neurologic - Extrapyramidal
- chorea is common early - dystonia emerges as disease progresses - hyperkinetic dysarthria - Parkinsonism (juvenile form is onset) - Parkinsonism (advanced disease in adults)
33
Chorea
-rapid, random jerky movements that seem to flow from one movement into another & impart a restless "wiggly" or dancing appearance to patient
34
Dysarthria
-result of choreiform movements of the tongue & lips interfering with speech
35
Huntington's Disease: Neurologic - Behavioral
- personality changes (impulsiveness, irritability, obsessive behavior, aggression) - depression - dementia (executive dysfunction, impairment of planning, organizing, reasoning, abstraction, judgement)
36
Huntington's Disease: Other Features
- Oculomotor Disturbances (difficulty initiating saccades, slowed saccades, glazed impersistence) - Cachexia
37
Huntington's Disease: Pathologic Features
- striatum (especially caudate), atrophy, neuronal loss, gliosis - cortex - atrophy, neuronal loss
38
Tourette's Syndrome: Demographics
100-1000 per 100,000 - genetic mutation identified in a small fraction of patients; suspected in many more - 3:1 male predominance - symptom onset b/w age 2-15 - symptoms often diminish in adulthood - normal life expactancy
39
Tourette's Syndrome: Clinical Features
- Motor tics (stereotypic, sudden movements), may be simple or complex, preceded by a premoition or urge, may be temporarily suppressed - Vocal (phonic) tics - similar to motor tics but involve sound rather than movement - Sensory tics - may occur - Tics vary in location, frequency, character, & severity over time
40
Tourette's Syndrome: Formal Diagnostic Criteria
- Multiple motor tics & at least one vocal tic must be present at some point during the course, though not necessarily concurrently - Tics must occur many times per day, almost every day or intermittently over the course of more than a year, wit no tic-free period of greater than 3 consecutive months - Onset must be before age 18 - The disorder must not be explained by any other condition
41
Motor Tics
Simple: muscle jerks, head shaking, shoulder shrugging, eye blinking, lip pouting complex: jumping, throwing, clapping, touching, echopraxia (mimic others movement), copropraxia (obscene or forbidden gestures)
42
Vocal Tics
Simple: sniffing, grunting, barking, hissing, clearing throat Complex: words, phrases, sentences, echolalia (mimic vocal), palilalia (rapid repetition or echoing of one's own words), coprolalia (swearing or obscene derogatory words)
43
Tourette's Syndrome: Behavioral Features
- Attention deficit hyperactivity disorder: 35-90% of children with TS - Obsessive-Compulsive disorder: 30-50% of persons with TS
44
Tourette's Syndrome: Pathologic Features
-no abnormality has been identified y
45
Tourette's Syndrome: Neurochemical Features
- no definitive abnormality - some dopaminergic disturbance suspected - others: monoaminergic (norepinephrine, serotonin,), opioid, adenosine
46
Dystonia
-sustained muscle contraction that produces sustained, & sometimes repetitive, twisting movements that result in abnormal posture
47
Dystonia can be Classified by?
- Etiology: primary (unknown) & secondary (disease) | - Topographic distribution: generalized (muscles throughout the body) & focal (only one or a group)
48
Dystonia: Demographics
- estimated prevalence is 3 per 100,000 - onset during childhood - Ashkenazi Jews - autosomal dominant inheritance (DYT1 gene mutation on chromosome 9, glutamate deletion in the protein, Torsin A, penetrance rate is 30-40%) - other mutations have also been identified
49
Dystonia: Neurologic Features
- focal onset - initially is action dystonia - lower extremity usually involved first (ankle inversion, plantar flexion of the toes) - axial muscles subsequently involved - gait & posture abnormalities develop "dromedary" appearance to gain, exaggerated hip abduction, knee hyperextension" - eventually these postures become fixed - dystonia remains sole feature (except for tremor in some)
50
Dystonia: Pathologic Features
-no consistent abnormality | basal ganglia or cerebellum is origin
51
Dystonia: Neurochemical Features
- no consistent abnormality | - some studies show: dopamine, norepinephrine, GABA
52
Primary Focal Dystonia: Demographics
- adult onset - traditionally considered to be sporadic - genetic basis recently has been identified in some - 30 per 100,000
53
Primary Focal Dystonia: Neurologic Features
- various muscle groups affected - muscles are usually above the waist - typically progress over several years, then static - may be task-specific - may be relieved by sensory tricks - Cranial dystonia (Blepharospasm, Oromandibular, Laryngeal (spasmodic dysphonia)) - Cervical Dystonia (spasmodic torticollis) - Limb Dystonia (Writer's cramp, Musician's dystonia)
54
Primary Focal Dystonia: Pathologic Features
-no consistent abnormality
55
Primary Focal Dystonia: Neurochemical Features
-no consistent abnormality
56
Mixed Hypokinetic-Hyperkinetic Movement Disorders
-Wilson's Disease
57
Wilson's Disease: Demographics
- 3 per 100,000 - 1 in 90 is a carrier - autosomal recessive inheritance (mutation long arm of chromosome 13, gene product is ATP7B, copper-transporting APTase, 380 mutations identified)
58
Wilson's Disease: Presentation
-onset 10-20, early as 3, late as 70s -clinical presentation: hepatic, neurologic, psychiatric
59
Wilson's Disease: Hepatic Features
- acute transient hepatitis - acute fulminant hepatitis - chronic active hepatitis - progressive cirrhosis
60
Wilson's Disease: Neurologic Features
- parkinsonism - chorea - dystonia - kinetic (intention) tremor - dysarthria - incoordination
61
Wilson's Disease: Psychiatric Features
- personality change - depression - mania - psychosis - dementia
62
Wilson's Disease: Ophthalmologic Features
- Kayser-Fleischer rings: brown pigment around the outer rim of the cornea (Cu deposits in Descemet's membrane) - Sunflower Cataracts: only seen on slit-lamp exam, due to Cu deposits in the lens
63
Wilson's Disease: Pathologic Features
- Cu deposition in various tissues - Hepatic fibrosis & cirrhosis - Neuronal loss & gliosis (putamen, thalamus, cerebral cortex) - Opalski cell formation (may originate from degenerating astrocytes)
64
Opalski Cell
Seen in Wilson's Disease
65
Wilson's Disease: Chemical Features
- dec. serum ceruloplasmin - inc. serum free copper (dec. serum total copper, because of dec. ceruloplasmin) - inc. urinary copper - inc. hepatic Cu

Neurology (18 decks)

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