title: Clinical, biochemical, and physiological features distinguishing myoclonus responsive to 5-hydroxytryptophan, tryptophan with a monoamine oxidase inhibitor, and clonazepam.
abstract: Fifteen patients with a variety of myoclonic syndromes were studied clinically, pharmacologically, and physiologically. CSF tryptophan, 5HIAA, and HVA were also measured. Of these patients, 8 were improved to varying degrees by therapy with 5HTP, tryptophan in combination with MAOI (but not tryptophan alone), and clonazepam. This group included 6 cases of post-anoxic myoclonus, one case of post-traumatic myoclonus and one undiagnosed case of non-progressive focal myoclonus and epilepsy. In this group low levels of CSF 5HIAA were found compared to non-responsive cases and controls. Two cases of dysynergia cerebellaris myoclonica, 2 cases of undiagnosed aetiology, 2 cases of essential myoclonus, and one case of palatal myoclonus failed to respond to drug therapy. However, even amongst the responsive group the improvement varied. The most dramatic responses were seen in those patients in whom physiological study suggested that myoclonus was mediated by brain-stem structures. Less dramatic responses were seen in patients in whom the myoclonus appeared to originate from cortical structures. The neurochemical basis of myoclonus responding to 5HT precursors and clonazepam is discussed. It is suggested that such myoclonus arises from a relative hypoactivity of the 5HT neuronal system which results in a release of abnormal responses to sensory stimuli which characterize this type of myoclonus.
mesh_terms: D006916:5-Hydroxytryptophan / Q000627:therapeutic use*; D000293:Adolescent; D000328:Adult; D000368:Aged; D001570:Benzodiazepinones / Q000627:therapeutic use*; D002230:Carbidopa / Q000627:therapeutic use*; D002675:Child, Preschool; D002998:Clonazepam / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D004569:Electroencephalography; D004576:Electromyography; D004831:Epilepsies, Myoclonic / Q000188:drug therapy; D004834:Epilepsy, Post-Traumatic / Q000188:drug therapy; D005260:Female; D006719:Homovanillic Acid / Q000134:cerebrospinal fluid; D006801:Humans; D006834:Hydrazines / Q000627:therapeutic use*; D006897:Hydroxyindoleacetic Acid / Q000134:cerebrospinal fluid; D002534:Hypoxia, Brain / Q000150:complications; D007520:Isocarboxazid / Q000627:therapeutic use; D008297:Male; D008784:Methysergide / Q000627:therapeutic use; D008875:Middle Aged; D009207:Myoclonus / Q000134:cerebrospinal fluid / Q000188:drug therapy*; D011292:Premedication; D014364:Tryptophan / Q000627:therapeutic use*
chemical_list: D001570:Benzodiazepinones; D006834:Hydrazines; D007520:Isocarboxazid; D006897:Hydroxyindoleacetic Acid; D002998:Clonazepam; D014364:Tryptophan; D006916:5-Hydroxytryptophan; D002230:Carbidopa; D006719:Homovanillic Acid; D008784:Methysergide