Hyperkinetic Movement Disorders

Hyperkinetic Movement Disorders. 1

Dystonia (Athetosis) 1

Definitions. 1

Epidemiology. 1

Pathogenesis. 1

Clinical manifestations. 1

Pseudo-athetosis. 2

Chorea. 2

Hemiballismus. 2

Pseudoathetosis. 2

Dystonia (Athetosis)

Definitions

• A syndrome of sustained muscle contractions
• Frequently causing twisting and repetitive movements or abnormal postures
• Often proximal >distal

Epidemiology

Pathogenesis

• Primary
• Secondary
• Metabolic
• Drugs
• Hereditary
• Degenerative

Clinical manifestations

• Types
• Generalized
• Segmental
• Focal
• Hemi-dystonia
• Primary Torsion dystonia
• Onset in early life (<20yrs)
• No history of developmental disorders etc.
• Many different patterns
• Disappears with sleep
• Genetic link
• Dopa responsive dystonia
• DYT-5 gene
• Parkinsons features may also be present
• Responds to dopa

Pseudo-athetosis

• Athetoid movements of fingers in patients with proprioceptive loss.

Chorea

• Rapid irregular muscle jerks that occur involuntarily
• Mild forms may look like restlessness
• Excess of dopamine over cholinergic system

Causes

• Very long list including,
• Huntington’s
• SLE
• Sydenham’s chorea (rheumatic fever)
• Drugs
• Pregnancy

Rx

· Dopamine blocking agents may be helpful

Hemiballismus

• Unilateral
• Violent, jerking, flinging movement
• Affects proximal muscles
• Due to lesion of subthalamic nucleus

Pseudoathetosis