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movement disorders Flashcards

1
Q

what is the basic function of the basal ganglia?

A

movement and modulation

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2
Q

5 nuclei of basal ganglia?

A

caudate

putamen

globus pallidus

subthalamic nucleus

substania nigra

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3
Q

2 functions of basal ganglia?

A

modulate movement:

  1. facilitate intended movements
  2. suppress unwanted movements
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4
Q

three types of movement disorders?

A
  1. hypokinetic - too little movement
  2. too much movement - hyperkinetic
  3. mixed movemet
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5
Q

3 hypokinetic disorders?

A

Parkinson’s disaease

progressive supranuclear palsy

multiple system atrophy

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6
Q

Parkinson’s disease:

  1. how common?
  2. when are genetics importat ?
  3. typical age of onset?
  4. life expectance
A
  1. prevalence: 100 per 100,000 gen pop; 2000 per 100,000 >60yrs
  2. genetics important if onset before age 50
  3. onset: 55-65
  4. near normal life expectancy w/treatment
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7
Q

neurologic features of parkinsons:

onset?

what are the 4 cardinal features of Parkinsons?

A

asymmetric ONSET

primary extrapyramidal features:

  1. rest tremor
  2. rigidity
  3. bradykinesia
  4. postural instability - (later in course of disease - if early, think of differential )
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8
Q

what are 7 secondary extrapyramidal features of Parkinson’s

face: 3
back: 1
extremeties: 3

A

face: masked face, decreased blink frequency, hypokinetic dysarthria
body: stooped posture
extremities: decreased arm swing, micrographia, shuffling gait,

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9
Q

non-motor features of Parkinson’s

behavioral features: 3

A

Behavioral

  • depression (may precede motor features)
  • anxiety
  • cognitive impairment: frontal lobe(executive dysfunction -can’t make decisions or plans, pure memory intact)

NOTE: if cognitive impairment early disease, consider Lewy Body demetnia

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10
Q

autonomic dyfunction in Parkinsons (5)

A

GI- constipation

urinary - usually increased frequency

sexual - decreased libido, erectile dysfunction

Cardiovascular - orthostatic hypotension (often due to meds, but can be part of disease itself)

**Cardiac sympathetic denervation **

Thermoreg - episodic profuse sweating

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11
Q

what are 5 neurologic features of Parkinson’s?

5 senses here.

A
  1. olfactory dysfunction
  2. **visual dysfunction **-blurred vision
  3. sensory symptoms: pain esp shoulder pain that can be presenting symptom(often misdiagnosed as bursitis)
  4. sleep disturbances: sleep fragmentation, REM sleep behavior disorder (act out their dreams)
  5. **fatigue **
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12
Q

Which three areas of the brain do you see degeneration of dopaminergic pigmented neurons in Parkinson’s

A

_1. Substantia Nigra pars compacta _

  1. locus ceruleus
  2. dorsal vagal nuclei
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13
Q

Where are Lewy bodies found in Parkinson’s?

what are Lewy bodie made out of?

A

location: CNS and enteric nervous system

Lewy bodies = alpha-synuclien protein aggregates in surviving pigmented neurons

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14
Q

fried egg appearing inclusion in pigmented neuron in Parkinson’s =

A

Lewy body

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15
Q

neuro chem hallmark of Parkinson’s

A

dopamine deficiency

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16
Q

where do you see dopamine neuron loss in Parkinson’s?

A
  1. substantia nigra
  2. striatum within the cerebrum
    also: mesolimbic, hypothalamic, retinal
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17
Q

apart from dopamine which other neurotransmitters are affected in Parkinson’s? (3)

A

norepi

serotonin

glutamate

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18
Q

What is the etiology of Progressive Supranuclear Palsy?

prevalence?

age of onset?

life expectancy?

A

sporadic

  1. 0-6.5 per 100,000
    onset: 50-60yrs old

life expectancy: 10 years

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19
Q

what are 4 extrapyramidal features of Progressive supranuclear atrophy disease (2 similar to Parkinson’s)

A
  1. ridigidty: esp axial rigidity; may produce neck hyperextenstion
  2. bradykinesia
  3. astonished facial expression
  4. dysarthria -

NOTE: features are SYMMETRIC (compare to Parkinson’s disease = non-symettric)

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20
Q

2 early features of Progressive supranuclear palsy?

which feature is unusual in this disease?

A

2 features: gait disturbance and postural instability (unexplained falling)

TREMOR UNUSUAL

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21
Q

2 behavioral features of Progressive supranuclear palsy?

A

Emotional lability

dementia

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22
Q

which clinical feature of progressive supranuclear palsy separates it from Parkinson’s?

A

supranuclear gaze palsy: vertical downgaze first(messy eater, dirty tie, difficulty desceding stairs) –> horizontal later. **oculocephalic reflex intact (eyes are actually still capable of moving) **

apraxia of eyelid opening- difficulty opening closed eye.

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23
Q

pyramidal tract signs and sleep disturbances are characteristic of which disorder?

A

Progressive supranuclear palsy

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24
Q

what are three pathologic features of Progressive Supranuclear palsy?

what are the neurofibrillary tangles composed of?

A
  1. midbrain and cerebral cortical atrophy (
  2. neuronal loss and gliosis in multiple areas(substantia nigra, rostral midbrain, pedunculopontine nucleus, globus pallidus)
  3. neurofibrillary tangles(globose type)-

co_mposed of tau protein(abnormally phosphorylated) _

composed of unpaired straight filaments

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25
Q

most prominent neurochem feature of PSP?

which other neurotransmitters are reduced? (NAGB)

A

dopamine deficiency in th_e striatum _

other neurotransmitters reduced:

  • Acetylcholine
  • GABA
  • norepinephrine
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26
Q

what is multiple system atrophy?

A

Parkinson -plus syndrome characterized by parkinsonian features + addl distinctive clnical and pathological abnormalities

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27
Q

3 diseases that make up multiple system atrophy?

A
  1. shy-drager syndrome
  2. olivopontocerebellar atrophy
  3. striatonigral degeneration
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28
Q

multiple system atrophy:

demographics

prevalence?

onset?

life expec?

A

sporadic

2.3-5.7 per 100,000

symptom onset: 50-55

life expec: 5-10 years

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29
Q

multiple system atrophy triad:

A

parkinsonianism

autonomic failure

cerebellar syndrome

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30
Q

which 4 extrapyramidal parkinson like features do pts with multiple system atrophy develop?

A

TRAP w/out the tremor

  1. rigidity
  2. bradykinesia

3. postural instability(early)

  1. TREMOR UNUSUAL, if develops use levodopa
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31
Q

what is the hallmark feature in multiple system atrophy?

A

AUTONOMIC function def: **MC cardiovascular dysfunction with orthostatic hypotension **

-urogenital dysfunction -

compared with PD, autonomic dysfunction in MSA begins earlier and is more severe

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32
Q

what are the cerbellar features of multiple system atrophy? (An ODE)

A

ataxia

dysarthria

oculomotor abnormalities

exagerrated rebound

33
Q

pryamidal features (61%) of MSA?(4)

A

hyperreflexia

babinski responses

spasticity

pseudobulbar palsy

34
Q

Behavioral features of MSA (22%) ? 4 types

A

personality changes

depression congitive dysfuction(frontal lobe executive dysfunction kind of like in Parkinson’s)

_dementia typically does not develop _

35
Q

respiratory stridor

involuntary sighing

raynaud phenomenon

postural myoclonus of the hands = features of which disease?

A

multiple system atrophy

36
Q

where does cell loss and gliosis occur in MSA? (4)

what type of inlcusion bodies found?

A

cell loss and gliosis in: basal ganglia(posterior putamen and substantia nigra), brainstem, cerebellum , spinal cord

-cortex usually spared

GLiAL cytoplasmic inlcusion bodies that stain for ALPHA SYNUCLEIN

37
Q

neurochem features for multipe system atrophy?

A

not well characterized.

38
Q

name 3 hyperkinetic movement disorders;

A

Huntington

Tourette’s

Primary idiopathic dystonia

39
Q

type of inherintannce in HUntington?

which chormosome mtuation?

name of mutated protien?

classic feature of disease

A

AD

mutation on short arm of chromosome 4

expanded trinucleotide (CAG) repeat

codes for _huntingtin protein _

40
Q

huntington demographics:

  1. onset?
  2. younger age of onset asociated with?
  3. life expectance after symptoms?
A
  1. 35-45
  2. younger onset = more rapid course
  3. life exectancy after symptom onset = 15-20years
41
Q

nuerological hallmark for HD?

A

chorea

42
Q

What are the extrapyramidal features of HD?

what does early onset in teenage years(junvenille form) look like?

A
  1. Chorea(early)–>dystonia(as disease progresses)–> Parkinsonianism(advanced disease) and hyperkinetic dysarthria
  2. in young with long CAG repeats, skips chorea and **goes straight to Parkinsonism **
43
Q

3 beharvioral features of HD? (PDD)

A
  1. personality changes: impulsiveness, irritablity, obsessive behavior, agression
  2. depression
  3. dementia - subcortical - impaired executive function
44
Q

oculomotor disturbances

-difficulty initating saccades

slowed saccades

-gaze impersistence

and cachexia describe which disease?

A

Huntington

45
Q

what are 3 pathologic features of HD in the striatum (esp caudate)?cortex?

A

atrophy

neuronal loss

gliosis

cortex: atrophy and neuronal loss

46
Q

Tourette’s syndrome:

  1. prevalence?
  2. which mutation?
  3. gender predom?
  4. onset?
  5. when do symptoms go away?
  6. life expec?
A

prevalence: 100-1000/100,000
- genetic mutation identified in a small fraction of pts; suspected in many more
- 3:1 male predominance
- onset: 2-15 years old
- symptoms often diminish in adulthood
- life expec normal

47
Q

what is a sudden sterotyped, non-rhythmic movement or vocalizations

A

tic

48
Q

3 tics in tourettes?

A

motor tics: stereotypic, sudden movements, may be simple or complex, are preceded by a premonition or urge, may be temporarily suppressed

vocal tics: similar to motor tics but involve sound rather than movement

sensory tics may also occur

tics vary in location, frequency, character and severity over time

49
Q

3 aspects of formal diagnostic criteria for Tourette’s?

A
  1. multiple motor tics and at least one vocal phonic tic (must be present at some pt during the course)
  2. tics must occur many times/day, almost every day, or intermittently over course of more than a year with no tic free period of greater than 3 consecutive months
  3. onset before age 18
  4. disorder must not be explained by any other condition
50
Q

muslcle jerks

head shaking

shoulder shrugging

eye blinking and lip pouting are examples of _______

A

simple motor tics

51
Q

jumping

throwing

clapping

touching

echopraxia = involuntary repeat of another person’s movement

copropraxia = involuntary performance of forbidden gestures

all examples of ?

A

complex motor tics

52
Q

sniffing

grunting

barking

hissing and clearing throat = examples of what type of tics?

A

simple vocal tics

53
Q

words, phrases, sentences

echolalia- repead vocalization made by another person

palilaia = rapid repetition or echoing of one’s words

coprolalia = involuntary swearing or utterance of obsene, socially inappropriate derogatory words

all examples of

A

complex vocal tics

54
Q

what are the behavioral features of Tourrettes? (2)

A

ADHD (35-90% of ppl with TS) and OCD (usually obsessive about thoughts about symmetry, counting, sex, and violence; compulsive about: countng, touching, checking, making sure things are just right)

55
Q

pathologic feature of tourettes?

A

no one really knows.

1st thought to be basal ganglia. now thought to be PFC

56
Q

neurochem features of tourettes?

A

no definitive abnormality seen:

some dopamine involvement

other systems potentially involved:

  1. monaminergic
  2. opiod
  3. adenosine
57
Q

what is characterized by sustained muscle contraction that produces sustained and sometimes repetitive, twisting, movments that result in abnormal postures?

A

dystonia

58
Q

Primary generalized dystonia:

prevalence:
onset:

MC in ______ppl

inhertiance?

A

prevalence: 3 per 100,000

onset during childhood(7-10yrs old)

MC in Ashkenazi Jews

Autosomal dominant nheritance: **DYT1 gene mutation in chromosome 9 **

Glutamate deletion TORSIN A (GAG deletion)

penetrance = 30-40%

59
Q

disease characterized by:

focal onset initially as action dystonia with lower extremity usually involved; ankle inversion; plantar flexiion ofthe toes and axial muscles subsequently involved

A

Dystonia

60
Q

what are neurologic features of primary generalized dystonia?

(dromedry)

A

dromedry = exaggerated hid abduction and KNEE hyperextension

gait and posture abnormalities develop –> fixed postures

-dystonia remain sole feature (except for tremor in some)

61
Q

pathogenesis of dystonia

A

no one knows;

proposed that dysfunction starts at CNS or basal ganglia and cerebellum

62
Q

neurochem abnormality in dystonia

A

dopamine

NE

GABA

63
Q

primary focal dystonia

onset:

prevalence

A

adult onset and sporadic (genetic basis in some)

prevalence: 30 per 100,00(more common than primary dystonia)

64
Q

which disease characterized by?

various muscle groups affected

_muscles are usually above the waist _

progress over serveral years –>static

task-specific (writing, playing instrument)

may be relieved by sensory tics

A

Primary focal dystonia

65
Q

ID 3 types of primary focal dystonia:

A

cranial

cervical

limb

66
Q

features of cranial dystonia:

A

blepharospasm - involuntary eye closure

oromandibular - muscles aroud jaw and mouth

laryngeal - (spasmodic dysphonia) - vocal cords closed (strained, strangled quality) and vocal cords open (breathy, whispering quality)

67
Q

features of cervical dystonia?

A

spasmodic torticollis - dystonia involving neck muscles

68
Q

features of limb dystonia(2)

A

writer’s cramp

musician’s dystonia

69
Q

path/neurochem of dystonia?

A

no consistent abnormality

70
Q

example of mixed hypokinetic-hyperkinetic movement disorder:

A

Wilson’s disease

71
Q

Wilson’s disease:

  1. prevalence
  2. carrier frequency
  3. inheritance?
  4. cause of mutation
A
  1. 3 per 100,000
  2. carrier freqeuncy is 1 per 90
  3. AR inheritance

mutations is on long arm of chromosome 13

gene product is ATP7B

copper-transporting ATPase

approx 380 diff mutations IDentified

72
Q

Wilson’s disease

age of onset?

3 types of clinical presentation?

A

age of onset:

typical: 10-20; as late as 70 as early as 3

clinical presentation: _hepatic, neurologic(later onset) , psychiatric _

73
Q

following hepatic features of which disease:

acute transient hepatitis

acute fulminant hepatitis (need liver transplant)

chronic active hepatitis

progressive cirrhosis

A

Wilson’s disease

74
Q

neurologic features of this disease inlcude:

Parkinsonism - tremor MC feature

chorea

dystonia

Kinetic (intention motor)

dysarthria(hyperkinetic or cerbellar)

incoordnation

A

Wilson’s disease

75
Q

psychiatric features ilcude:

personality change

depression

mania

psychosis

and RARE demetia

A

Wilson’s

76
Q

ophthalmologic features of Wilson’s?

A

Kayser-Fleirscher rings(copper deposition in Descement’s membrane within cornea)

and sunflower cataracts (copper deposition in the lens)

77
Q

microscopic hallmark of Wilson’s disease

A

Opalski cell = altered glial cell that can be found in basal ganglia of individuals with WD.

78
Q

in Wilson’s where is neuronal loss and gliosis most common?

A

putamen

thalamus

cerebral cortex

79
Q

4 chemical features of Wilson’s disease? all dealing with copper

A

Decrease serum ceruloplasmin

increased serum free copper (decreased serum total copper)

increased urinary copper

increased hepatic copper (most accurate, but must get liver biopsy)

Neuro week1 (9 decks)

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