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Flashcards in Week 6 Movement Disorders Deck (19)
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1

Define hypokinesias: akinesia, bradykinesia, rigidity.

hypokinesias-too little movement
akinesia-loss or absence of movement
bradykinesia-slowness of movement
rigidity-stiffness of muscle tone with passive movement of the joint

2

Define tremor.

Oscillatory, rhythmical, and regular movement affecting one or more body parts.
-at rest-limb supported against gravity
Action Tremor
-kinetic tremor-while performing task
-postural tremor-during a sustained posture, usually limb outstretched
-intention tremor-present during pursuit of a goal, usually increasing as target is approached (from cerebellar lesion)
causes: damage to substantia nigra, cerebellum

3

Define chorea.

Involuntary, irregular, nonrhythmic, rapid jerky movements that flow from one body part to another
-looks like fidgeting or clumsiness
-gait can be lurching or "dancing"
-associated w/ cell loss in caudate and putamen

4

Define ballism.

large amplitude, proximal movements resulting in flinging limb movements
-can be considered variant of chorea
-associated with lesion in STN

5

Define dystonia.

Sustained involuntary abnormal posturing. Continuous muscle contractions cause sustained and painful postures.
-focal: single muscle group
-segmental: 2 contiguous areas of body
-generalized: legs and 2 contiguous parts of the body
-unclear pathophysiology

6

Define myoclonus.

Sudden, brief, twitch like involuntary movements usually caused by muscular contractions (like the twitch/jerk while falling asleep)
-can be focal, segmental, generalized
-can be assoc. w/ lesions of cortex, brainstem, spinal cord

7

Define tics.

Semi-voluntary, repetitive, intermittent, stereotypic movements or sounds. Preceded by an urge to perform the movement, followed by temporary relief of urge after the tic
-tics can be suppressed temporarily, but often have rebound in tics afterward

8

List the movement time and the anatomic region involved for the following syndromes: Parkinson's, Hemiballism from stroke, Hungtinton's disease, idiopathic torsion dystonia, Tourette's syndrome, essential palatal myoclonus

1. Parkinson's disease: rest tremor & bradykinesia, substantia nigra
2. Hemiballism: ballism, STN
3. Huntington's disease, chorea, caudate
4. Idiopathic torsion dystonia: dystonia, putamen
5. Tourette's syndrome: tics, Basal ganglia/limbic
6. essential palatal myoclonus: myoclonus (palate), Mollaret's triangle

9

What are the cardinal signs of parkinson's disease?

TRAP: tremor, rigidity, akinesia, postural disturbances
-diminished facial expression
-diminished movements, e.g. swinging arms while walking, blinking
-reduction in speed and amplitude of voluntary movements
-freezing: intermittent, severe inability to initiate walking

10

What are some late complications of PD treatment?

-motor complications and psychosis
-wearing off:when patient loses sustained effect of medication and needs the next dose earlier
-dose related dyskinesias: abnormal involuntary movements in any body location which occur following each medication dose at peak bioavailability
-on-off fluctuations: knife edge phenomenon where with abrupt on and off periods of improvements and periods of parkinsonian state

11

Describe Essential Tremor (ET)

features: causes action tremor with prominent postural and kinetic components
-usually upper extremities
-any age, more common in elderly
-etiology: family hx common
-treatment: beta blocker, primidone (barbiturate anticonvulsant), deep brain stimulation, thalamotomy

12

What are diseases that have chorea as a component?

Sydenham's chorea
Huntington's Disease
Wilson's disease
hyperthyroidism
Drug related, Tardive's syndrome

13

Describe the clinical features of Huntington's Disease.

Progressive chorea and dementia
-inherited: autosomal dominant
-onset: age 30-50
Major features:
1. psychiatric disturbance: personality changes, depression, schizophrenia
2. progressive dementia
3. Progressive chorea
-milk maid grip: inability to squeeze examiner's fingers
-motor impersistence: inability to maintain a sustained posture
Pathology: neuronal loss in caudate and putamen

14

Describe Sydenham's Chorea.

-late feature of acute rheumatic fever
-7-12 year olds, commonly female

15

Describe Idiopathic torsion dystonia

syndrome of progressive generalized dystonia with no identifiable cause
-DYT1: autosomal dominant disorder
-limbs, neck, head, trunk twist into particular postures
-mentation spared
-treatment with anticholinergics, benzodiazepines, anti spasticity agents

16

Describe focal dystonias.

-Limited to one or two body areas
-begins in adulthood
-e.g. writer's cramp
-treat with botulinum toxin

17

Describe Gilles de la Tourette syndrome

1. symptoms start before 21
2. motor and vocal tics
3. last more than 6 months
-frequently associated with OCD and ADHD
-treat with dopamine antagonists

18

Describe tardive syndromes

-associated with use of neuroleptics, which block dopamine receptors
-Tardive Dyskinesia*: months to years after neuroleptic started, oral facial, choreaathetosis
-acute dystonia: spasms of neck, tongue, face
-akathesia: motor restlessness
-Parkinsonism: bradykinesia, rigidity, tremor
-neurolepic malignant syndrome: catatonia, fever, unstable BP

19

Describe wilson's disease.

present with any combination of abnormal movement types
-defect in copper metabolism
-copper deposition and damage to putamen and globus pallidus results in movement disorders
-Kayser fleisher ring
-treatment: copper chelator

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