Fast Five Quiz: Movement Disorders

Secondary dystonia may result from a wide variety of neurologic diseases or inherited metabolic defects, including:

Huntington disease
Hallervorden-Spatz disease
Wilson disease (hepatolenticular degeneration)
Leigh disease
Lipid storage disease
Parkinsonism
Central nervous system infections
Cerebral or cerebellar tumors
Drug intoxication: dopamine antagonists, neuroleptics, metoclopramide, and haloperidol, among others
Structural or hypoxic injury to the basal ganglia brainstem structures

Cervical dystonia, or torticollis, is the most common focal dystonia. It has an insidious onset in people aged 30-50 years, although it can begin earlier. Cervical dystonia commonly affects women. Intermittent spasms of the neck muscles or abnormal head movements occur because of contractions of the sternocleidomastoid, trapezius, and posterior cervical muscles. This effect results in a patterned, repetitive, and spasmodic movement that causes the head to twist (rotational torticollis), extend (retrocollis), flex (anterocollis), or tilt toward the shoulder (laterocollis). The patient may display more than one of these head movements simultaneously.

Genetic screening for DYT gene abnormalities and genetic counseling are important for patients who have had an onset of primary dystonia before age 30 years or for persons who have an affected relative.

Baclofen, given intrathecally by an implanted pump, can be very effective in certain types of dystonia, especially if spasticity coexists. Due to the low prevalence of side effects when the medicine is delivered into the cerebrospinal fluid, the ability to deliver the medicine continuously, and the ability to test the therapeutic effect prior to proceeding with surgery, this option may provide effective treatment for many patients.