torsion dystonia

dys·to·ni·a mus·cu·lo·rum de·for·mans

a genetic, environmental, or idiopathic disorder, usually beginning in childhood or adolescence, marked by muscular contractions that distort the spine, limbs, hips, and sometimes the cranial innervated muscles. The abnormal movements are increased by excitement and, at least initially, abolished by sleep. The musculature is hypertonic when in action, hypotonic when at rest. Hereditary forms usually begin with involuntary posturing of the foot or hand (autosomal recessive form [MIM*224500]) or of the neck or trunk (autosomal dominant form [MIM*128100]); both forms may progress to produce contortions of the entire body.

Synonym(s): torsion disease of childhood, torsion dystonia, Ziehen-Oppenheim disease

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torsion dystonia

Neurology An AD, possibly also AR condition most common in Jews, onset age 5 to 16 Clinical Gait defects, involuntary contractions and distortion of spine, hands, feet, hips, and eventually neck; lesions typically start in one body region, usually in an arm or leg, and spread to the rest of the body in ±5 yrs; most Pts are wheelchair bound by adulthood; muscles are hypertonic, under voluntary control, hypotonic at rest; IQ unaffected

McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.

Flatau,

Edward, Polish neurologist, 1869-1932.

Flatau law - a law concerning the eccentric position of the long spinal tracts.

Flatau syndrome - recessive trait resulting in bizarre movements brought on by physical activity. Synonym(s): torsion dystonia

Flatau-Schilder disease - Synonym(s): Schilder disease

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