Movement Disorders Vol. 6 , No. 2, 1991, pp. 183-188. 0 1991 Movement Disorder Society
Brief Communications References
Action Myoclonus (Lance-Adam Syndrome) Secondary to Strangulation with Dramatic Response
1 . Lance JW, Adam RD. The syndrome of intention or action myoclonus as a sequel to hypoxic encephalopathy. Bruin 1963;876:1 1 1-36. 2. Plum F, Posner JB, Hain RF. Delayed neurological deterioration after anoxia. Arch Intern Med 1962;110:56-7. 3. Fahn S. Posthypoxic action myoclonus. Review of the literature and report of two new cases with response to valproate and estrogen. Adv Neurol 1979;26:4!%84. 4. Van Woert HM, Sethy VH. Therapy of intention myoclonus with L-5-hydroxytryptophanand a peripheral decarboxylase inhibitor MK 486. Neurology 1975;25:135-40.
to Alcohol To the Editor: A 32-year-old man was accidentally strangled while operating a machine on January 29, 1989. He was brought to the hospital unconscious. After a week, he recovered but developed severe action myoclonus and an ataxic gait. Clonazepam, combined subsequently with sodium valproate, produced minimal improvement. Interestingly, after consumption of alcohol (30 ml of whiskey), the patient showed dramatic improvement (videotape segment). Initially, 30 ml of whiskey (42% alcohol) three times a day was given to the patient. Surprisingly, 30 min after each dose of alcohol, he could manage daily routine activities. The effect of each dose lasted 4-6 h. Later, piracetam was added and produced improvement. Nevertheless, for a sustained, smooth, and prolonged benefit, additional alcohol was required. Postanoxic action myoclonus was described by Lance and Adam in 1963 (1). Many cases have since been described secondary to anoxia, drug overdose, and mercury poisoning, etc. Action myoclonus has not previously been reported secondary to strangulation, although a late encephalopathy (2) has been described. Diazepam, phenobarbitone, clonazepam (3), sodium valproate, and 5hydroxytryptophan (4) have been used in the management of this condition. The beneficial effect of alcohol has not previously been described.
Antineuronal Antibodies in Cranial Dystonia To the Editor: The presence of autoimmune disorders in a number of patients with cranial dystonia (CD) (1,2) makes it more likely that antibodies capable of damaging discrete neuronal populations might be involved in the pathophysiology of CD. However, it was recently demonstrated that sera from patients with CD did not stain postmortem sections from different regions of normal human brain (3). Therefore, we approached the problem of antineuronal antibodies in CD differently. It is well known that complement-mediated cytotoxicity is one of the most important mechanisms of antibodydependent cell damage. Thus, we evaluated whether serum from patients with idiopathic CD contained factor(s) exerting complement-mediated injury against neuronal systems which might be implicated in CD. We focused on mesencephalic dopamine (DA) and GABA neurons and striatal GABA neurons. In fact, rostra1 brainstem or basal ganglia lesions may sometimes underlie CD, and isolated or combined disruption of dopaminergic (hypofunction or preponderance), GABAergic (hypofunction), and cholinergic (preponderance) systems have been proposed (1,4,5). Thus, serumless dissociated mesencephalicstriatal rat primary culture (6) expressing neurotransmitter-related properties, i.e., high affinity 3H DA and 14C GABA uptake, was used as the bioassay system. Serum samples were obtained from 3 female and 1 male patient (mean age 61 years, range 48-76 years) suffering for 2 to 6 years (mean = 3.6) from blepharospasm (BS) (n = 2) or BS oromandibular dystonia (n = 2). No patient had a history of diabetes or hypertension, and computed tomographic scan was unrevealing. Eight age- and sexmatched healthy persons were used as controls. Heat-inactivated (56°C for 30 min) serum samples were added to cultures on day 4 in vitro at dilutions ranging from 1 to 10%. Twenty-four h later, serum-free and serum-containing cultures were exposed for 60 min to active or heat-inactivated (56°Cfor 30 min) rabbit comple-
Acknowledgment: We are thankful to Dr. R. J. Chaube, Director, Medical and Health Services, JLN Hospital and Research Centre, Bhilai for permitting us to publish the case and to Mrs. S. Kaur for secretarial assistance.
Legend to the Videotape The patient is unable to get out of a car. Marked ataxia and myoclonus are visible; finger-nose testing is impaired; he is not able to drink; climbing is difficult. He imbibes whiskey, 60 ml of 42% alcohol. After half an hour, he is able to walk; after 1 h he is able to write; finger-nose and alternating movements are normal; he is able to dress himself, walk, and enter his car. The effect of alcohol lasted for 4-6 h. Myoclonus and ataxia reappeared after the effect of alcohol wore off.
+
Shailendra Jain Meena Jain JLN Hospital and Research Centre Bhilai, India
183
Brief Communications References
Action Myoclonus (Lance-Adam Syndrome) Secondary to Strangulation with Dramatic Response
1 . Lance JW, Adam RD. The syndrome of intention or action myoclonus as a sequel to hypoxic encephalopathy. Bruin 1963;876:1 1 1-36. 2. Plum F, Posner JB, Hain RF. Delayed neurological deterioration after anoxia. Arch Intern Med 1962;110:56-7. 3. Fahn S. Posthypoxic action myoclonus. Review of the literature and report of two new cases with response to valproate and estrogen. Adv Neurol 1979;26:4!%84. 4. Van Woert HM, Sethy VH. Therapy of intention myoclonus with L-5-hydroxytryptophanand a peripheral decarboxylase inhibitor MK 486. Neurology 1975;25:135-40.
to Alcohol To the Editor: A 32-year-old man was accidentally strangled while operating a machine on January 29, 1989. He was brought to the hospital unconscious. After a week, he recovered but developed severe action myoclonus and an ataxic gait. Clonazepam, combined subsequently with sodium valproate, produced minimal improvement. Interestingly, after consumption of alcohol (30 ml of whiskey), the patient showed dramatic improvement (videotape segment). Initially, 30 ml of whiskey (42% alcohol) three times a day was given to the patient. Surprisingly, 30 min after each dose of alcohol, he could manage daily routine activities. The effect of each dose lasted 4-6 h. Later, piracetam was added and produced improvement. Nevertheless, for a sustained, smooth, and prolonged benefit, additional alcohol was required. Postanoxic action myoclonus was described by Lance and Adam in 1963 (1). Many cases have since been described secondary to anoxia, drug overdose, and mercury poisoning, etc. Action myoclonus has not previously been reported secondary to strangulation, although a late encephalopathy (2) has been described. Diazepam, phenobarbitone, clonazepam (3), sodium valproate, and 5hydroxytryptophan (4) have been used in the management of this condition. The beneficial effect of alcohol has not previously been described.
Antineuronal Antibodies in Cranial Dystonia To the Editor: The presence of autoimmune disorders in a number of patients with cranial dystonia (CD) (1,2) makes it more likely that antibodies capable of damaging discrete neuronal populations might be involved in the pathophysiology of CD. However, it was recently demonstrated that sera from patients with CD did not stain postmortem sections from different regions of normal human brain (3). Therefore, we approached the problem of antineuronal antibodies in CD differently. It is well known that complement-mediated cytotoxicity is one of the most important mechanisms of antibodydependent cell damage. Thus, we evaluated whether serum from patients with idiopathic CD contained factor(s) exerting complement-mediated injury against neuronal systems which might be implicated in CD. We focused on mesencephalic dopamine (DA) and GABA neurons and striatal GABA neurons. In fact, rostra1 brainstem or basal ganglia lesions may sometimes underlie CD, and isolated or combined disruption of dopaminergic (hypofunction or preponderance), GABAergic (hypofunction), and cholinergic (preponderance) systems have been proposed (1,4,5). Thus, serumless dissociated mesencephalicstriatal rat primary culture (6) expressing neurotransmitter-related properties, i.e., high affinity 3H DA and 14C GABA uptake, was used as the bioassay system. Serum samples were obtained from 3 female and 1 male patient (mean age 61 years, range 48-76 years) suffering for 2 to 6 years (mean = 3.6) from blepharospasm (BS) (n = 2) or BS oromandibular dystonia (n = 2). No patient had a history of diabetes or hypertension, and computed tomographic scan was unrevealing. Eight age- and sexmatched healthy persons were used as controls. Heat-inactivated (56°C for 30 min) serum samples were added to cultures on day 4 in vitro at dilutions ranging from 1 to 10%. Twenty-four h later, serum-free and serum-containing cultures were exposed for 60 min to active or heat-inactivated (56°Cfor 30 min) rabbit comple-
Acknowledgment: We are thankful to Dr. R. J. Chaube, Director, Medical and Health Services, JLN Hospital and Research Centre, Bhilai for permitting us to publish the case and to Mrs. S. Kaur for secretarial assistance.
Legend to the Videotape The patient is unable to get out of a car. Marked ataxia and myoclonus are visible; finger-nose testing is impaired; he is not able to drink; climbing is difficult. He imbibes whiskey, 60 ml of 42% alcohol. After half an hour, he is able to walk; after 1 h he is able to write; finger-nose and alternating movements are normal; he is able to dress himself, walk, and enter his car. The effect of alcohol lasted for 4-6 h. Myoclonus and ataxia reappeared after the effect of alcohol wore off.
+
Shailendra Jain Meena Jain JLN Hospital and Research Centre Bhilai, India
183
