Myoclonic dystonia.

Abstract

Myoclonic dystonia is a rare disorder that occurs in an hereditary and a sporadic form. The autosomal-dominantly inherited form is responsive to alcohol but not to other drugs. The sporadic form has been relatively resistant to drug treatment. We report a young man with myoclonic dystonia who displayed only little response to alcohol but improved significantly with a combination of sodium valproate for myoclonus and trihexiphenidyl hydrochloride for dystonia. His rehabilitation, however, was confounded by public authorities who thought the patient's appearance was indicative of drug use.

• Authors: S M Pueschel, J H Friedman, T Shetty
• Journal: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery (Childs Nerv Syst) Vol. 8 Issue 2 Pg. 61-6 (Mar 1992) ISSN: 0256-7040 [Print] Germany
• PMID: 1591746 (Publication Type: Case Reports, Journal Article)

Chemical References

• Valproic Acid
• Trihexyphenidyl

Topics

• Adult
• Biopsy
• Cerebellum (physiopathology)
• Diseases in Twins (genetics)
• Drug Therapy, Combination
• Dystonia (diagnosis, genetics, physiopathology, rehabilitation)
• Epilepsies, Myoclonic (diagnosis, genetics, physiopathology, rehabilitation)
• Gait (physiology)
• Humans
• Magnetic Resonance Imaging
• Male
• Muscles (pathology)
• Myoclonus (diagnosis, genetics, physiopathology, rehabilitation)
• Neurologic Examination (drug effects)
• Posture (physiology)
• Rehabilitation, Vocational
• Tomography, X-Ray Computed
• Trihexyphenidyl (administration & dosage)
• Valproic Acid (administration & dosage)