Week 6 Movement Disorders

Question 1

Define hypokinesias: akinesia, bradykinesia, rigidity.

Answer 1

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

Question 2

Define tremor.

Answer 2

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

Question 3

Define chorea.

Answer 3

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

Question 4

Define ballism.

Answer 4

large amplitude, proximal movements resulting in flinging limb movements

• can be considered variant of chorea
• associated with lesion in STN

Question 5

Define dystonia.

Answer 5

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

Question 6

Define myoclonus.

Answer 6

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

Question 7

Define tics.

Answer 7

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

Question 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

Answer 8

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

Question 9

What are the cardinal signs of parkinson’s disease?

Answer 9

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

Question 10

What are some late complications of PD treatment?

Answer 10

• 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

Question 11

Describe Essential Tremor (ET)

Answer 11

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

Question 12

What are diseases that have chorea as a component?

Answer 12

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

Question 13

Describe the clinical features of Huntington’s Disease.

Answer 13

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

Question 14

Describe Sydenham’s Chorea.

Answer 14

• late feature of acute rheumatic fever

- 7-12 year olds, commonly female

Question 15

Describe Idiopathic torsion dystonia

Answer 15

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

Question 16

Describe focal dystonias.

Answer 16

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

Question 17

Describe Gilles de la Tourette syndrome

Answer 17

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

Question 18

Describe tardive syndromes

Answer 18

• 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

Question 19

Describe wilson’s disease.

Answer 19

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