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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
most prominent neurochem feature of PSP? which other neurotransmitters are reduced? (NAGB)
dopamine deficiency in th**_e striatum _** other neurotransmitters reduced: - Acetylcholine - GABA - norepinephrine
26
what is multiple system atrophy?
Parkinson -plus syndrome characterized by parkinsonian features + addl distinctive clnical and pathological abnormalities
27
3 diseases that make up multiple system atrophy?
1. shy-drager syndrome 2. olivopontocerebellar atrophy 3. striatonigral degeneration
28
multiple system atrophy: demographics prevalence? onset? life expec?
sporadic 2.3-5.7 per 100,000 symptom onset: 50-55 life expec: 5-10 years
29
multiple system atrophy triad:
parkinsonianism autonomic failure cerebellar syndrome
30
which 4 extrapyramidal parkinson like features do pts with multiple system atrophy develop?
TRAP w/out the tremor 1. rigidity 2. bradykinesia **3. postural instability(early)** 4. TREMOR UNUSUAL, if develops use levodopa
31
what is the hallmark feature in multiple system atrophy?
AUTONOMIC function def: **MC cardiovascular dysfunction with orthostatic hypotension ** -urogenital dysfunction - compared with PD, autonomic dysfunction in MSA begins earlier and is more severe
32
what are the cerbellar features of multiple system atrophy? (An ODE)
ataxia dysarthria oculomotor abnormalities exagerrated rebound
33
pryamidal features (61%) of MSA?(4)
hyperreflexia babinski responses spasticity pseudobulbar palsy
34
Behavioral features of MSA (22%) ? 4 types
personality changes depression congitive dysfuction(frontal lobe executive dysfunction kind of like in Parkinson's) _**dementia typically does not develop** _
35
respiratory stridor involuntary sighing raynaud phenomenon postural myoclonus of the hands = features of which disease?
multiple system atrophy
36
where does cell loss and gliosis occur in MSA? (4) what type of inlcusion bodies found?
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
neurochem features for multipe system atrophy?
not well characterized.
38
name 3 hyperkinetic movement disorders;
Huntington Tourette's Primary idiopathic dystonia
39
type of inherintannce in HUntington? which chormosome mtuation? name of mutated protien? classic feature of disease
AD mutation on short arm of chromosome 4 expanded trinucleotide (CAG) repeat codes for **_huntingtin protein _**
40
huntington demographics: 1. onset? 2. younger age of onset asociated with? 3. life expectance after symptoms?
1. 35-45 3. younger onset = more rapid course 3. life exectancy after symptom onset = 15-20years
41
nuerological hallmark for HD?
chorea
42
What are the extrapyramidal features of HD? what does early onset in teenage years(junvenille form) look like?
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
3 beharvioral features of HD? (PDD)
1. personality changes: impulsiveness, irritablity, obsessive behavior, agression 2. depression 3. dementia - subcortical - impaired executive function
44
oculomotor disturbances -difficulty initating saccades slowed saccades -gaze impersistence and cachexia describe which disease?
Huntington
45
what are 3 pathologic features of HD in the striatum (esp caudate)?cortex?
atrophy neuronal loss gliosis cortex: atrophy and neuronal loss
46
Tourette's syndrome: 1. prevalence? 2. which mutation? 3. gender predom? 4. onset? 5. when do symptoms go away? 6. life expec?
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
what is a sudden sterotyped, non-rhythmic movement or vocalizations
tic
48
3 tics in tourettes?
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
3 aspects of formal diagnostic criteria for Tourette's?
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
muslcle jerks head shaking shoulder shrugging eye blinking and lip pouting are examples of \_\_\_\_\_\_\_
simple motor tics
51
jumping throwing clapping touching echopraxia = involuntary repeat of another person's movement copropraxia = involuntary performance of forbidden gestures all examples of ?
complex motor tics
52
sniffing grunting barking hissing and clearing throat = examples of what type of tics?
simple vocal tics
53
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
complex vocal tics
54
what are the behavioral features of Tourrettes? (2)
**_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
pathologic feature of tourettes?
no one really knows. 1st thought to be basal ganglia. now thought to be PFC
56
neurochem features of tourettes?
no definitive abnormality seen: some dopamine involvement other systems potentially involved: 1. monaminergic 2. opiod 3. adenosine
57
what is characterized by **_sustained muscle contraction_** that produces sustained and sometimes repetitive, twisting, movments that result in abnormal postures?
dystonia
58
Primary generalized dystonia: prevalence: onset: MC in \_\_\_\_\_\_ppl inhertiance?
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
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
Dystonia
60
what are neurologic features of primary generalized dystonia? (dromedry)
dromedry = exaggerated hid abduction and KNEE hyperextension gait and posture abnormalities develop --\> fixed postures -dystonia remain sole feature (except for tremor in some)
61
pathogenesis of dystonia
no one knows; proposed that dysfunction starts at CNS or basal ganglia and cerebellum
62
neurochem abnormality in dystonia
dopamine NE GABA
63
primary focal dystonia onset: prevalence
adult onset and sporadic (genetic basis in some) prevalence: 30 per 100,00(more common than primary dystonia)
64
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
Primary focal dystonia
65
ID 3 types of primary focal dystonia:
cranial cervical limb
66
features of cranial dystonia:
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
features of cervical dystonia?
spasmodic torticollis - dystonia involving neck muscles
68
features of limb dystonia(2)
writer's cramp musician's dystonia
69
path/neurochem of dystonia?
no consistent abnormality
70
example of mixed hypokinetic-hyperkinetic movement disorder:
Wilson's disease
71
Wilson's disease: 1. prevalence 2. carrier frequency 3. inheritance? 4. cause of mutation
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
Wilson's disease age of onset? 3 types of clinical presentation?
age of onset: typical: 10-20; as late as 70 as early as 3 clinical presentation: _**hepatic, neurologic(later onset) , psychiatric** _
73
following hepatic features of which disease: acute transient hepatitis **acute fulminant hepatitis (need liver transplant)** chronic active hepatitis progressive cirrhosis
Wilson's disease
74
neurologic features of this disease inlcude: Parkinsonism - tremor MC feature chorea dystonia Kinetic (intention motor) **dysarthria(hyperkinetic or cerbellar)** incoordnation
Wilson's disease
75
psychiatric features ilcude: personality change depression mania psychosis and **_RARE demetia_**
Wilson's
76
ophthalmologic features of Wilson's?
Kayser-Fleirscher rings(copper deposition in Descement's membrane within cornea) and sunflower cataracts (copper deposition in the lens)
77
microscopic hallmark of Wilson's disease
Opalski cell = altered glial cell that can be found in basal ganglia of individuals with WD.
78
in Wilson's where is neuronal loss and gliosis most common?
putamen thalamus cerebral cortex
79
4 chemical features of Wilson's disease? all dealing with copper
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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