Hyperkinetic Movement Disorders

Question 1

Ataxia…what is it, what is it dysfunction of, and when is it seen?

Answer 1

Breakdown of normally coordinated voluntary movements

Dysfunction of cerebellum pathways

Only seen during voluntary movements, NOT at rest

Question 2

Dysmetria 
Dusdiadochokinesia Loss of check
Rbound
Ataxic gait 
Truncal ataxia

Answer 2

Incoordinated movement toward a target
Breakup with rapid alternating mvmt
Unable to stop fast voluntary movement at the target
SUdden displacement results in over correction
Wide base, body sway, and inability to tandem
(vermian)

Question 3

Other cerebellar signs besides ataxia

Answer 3

Nystagmus (with slowed or jerky saccades)

Dysarthria and scannign speech

Question 4

HD genetic numbers

Answer 4

Normal is 6-26, pre is 35-38, more than 38 is HD

Can expand if passed from paternal side

Question 5

Chorea

Answer 5

Involuntary, irregular, purposeless, nonrhytmic, abrupt, rapid, unssustained movements

Flow from one body part to another

Unpredictable

Can be camouflaged as semipurposeful moveemnts

Thin HD

Question 6

HD motor impersistence, type of kinesia, visual, reflexes, cog, psych

Answer 6

Milkmaids grip and inability t keep tongue protruded
Parakinesia camouflages movements
Slowed saccades 
Pendualr reflexes 
Cog impaired and psych often first

Also dysarthria/dysphagia and unsteady gait

Question 7

MRI of HD

Answer 7

Atrophy of caudate and putamen

Uf hyperintense on CT, NOT calcium

Hypointense on T2 at basal ganglia and GP

Question 8

Dystonia

Answer 8

Twisting, repetitive, often sustained and tremulous

Co contraction of agonist and antagonist

Can lead to fixed postures

Worse by voluntary action

May have sensory tick

Question 9

Age of onset
Distribution
And etiology of dystonia

Answer 9

Early<26…late>26
Focal (50-60), segmental (30-35), generalized (8-15)
Primary, secondary, heredodegenerative, dystonia-plus

Question 10

Pathophys of dystonia

Answer 10

Loss of surround inhibition
Reduced firing from the GPi and GPe therefore reduced inhibition of the thalamus

Increased plasticity of neural connections in sensorimotor circuits at BS and SC

Question 11

Tx of dystonia and what to do with child

Answer 11

Botulinum toxin can be used

Try levodopa and make sure it is not Wilson’s dz

Question 12

Tremor and types

Answer 12

Oscillatory, rhythmic and regular

Rest, postural, action (kinetic and intention)

Question 13

Physiologic tremor
Essential tremor
Rest tremor

Answer 13

Postural, slowed by mass loading (8)
Amplitude increases with age while frequency decreases…(4-10)
Parkinsons dz, prkinsonism (4-6)

Question 14

Dystonic tremor

Orthostatic tremor

Answer 14

Irregular and position sensitive (null point)

Mainly legs and trunk on standing (14-16)