Non-Parkinsonian Movement Disorders

Question 1

Dystonia

Pathophysiology
Clinical Features

Answer 1

Result of loss of movement inhibition and increased plasticity of neural connections.

Involuntary co-contraction of antagonistic muscles, resulting in abnormal twisting–can involve any part of the body! Can be associated with “sensory trick” (geste antagoniste), where touching certain parts of the body lessens movement.

Question 2

Treatment for focal vs. generalized dystonia

Answer 2

Focal dystonia - treat with botulinum toxin

Generalized dystonia - treatments not necessarily as effective as that for focal condition; can be pharmacologic but can also use DBS

Question 3

Huntington disease - what are the triad of symptoms? What would you see grossly?

Treatment?

Answer 3

Chorea/movement disorder, psychiatric issues, cognitive decline (later in disease)

Would see atrophy of caudate (and maybe expanded lateral ventricles from hydrocephalus)

Dopamine antagonists (Risperidone), Tetrabenazine (VMAT 2 inhibitor)

Question 4

Features of ataxia?

Answer 4

Localizes primarily to cerebellum (can see atrophy grossly) with various etiologies.

Loss of limb/speech coordination, nystagmus, wide unstable gait

Question 5

Features of myoclonus?

Answer 5

Quick, “shock-like,” simple muscle jerks–can be affecting cortex, brainstem, or spinal cord

Question 6

Tics vs. Tourette Syndrome

Answer 6

Tics = simple/complex brief repetitive movements or sounds that abruptly interrupt normal activity with return to normal in between. Urge/discomfort that is eased by performing tic. Suppressable if asked to do so momentarily!

Tourette Syndrome = two or more MOTOR tics and at LEAST one vocal tic