|Year : 1996 | Volume
| Issue : 2 | Page : 101-102
Opsoclonus - myoclonus syndrome in an adult: A case report and response to clonazepam
RK Garg, AM Kar, V Dixit
King George's Medical College, Lucknow, India
R K Garg
Department of Neurology, King George's Medical Colllege, Lucknow 226003
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Garg R K, Kar A M, Dixit V. Opsoclonus - myoclonus syndrome in an adult: A case report and response to clonazepam. Indian J Ophthalmol 1996;44:101-2
|How to cite this URL:|
Garg R K, Kar A M, Dixit V. Opsoclonus - myoclonus syndrome in an adult: A case report and response to clonazepam. Indian J Ophthalmol [serial online] 1996 [cited 2015 Jul 5];44:101-2. Available from: http://www.ijo.in/text.asp?1996/44/2/101/24585
Opsoclonus is a striking disorder of eye movement reflecting the presence of unwanted saccades. It consists of involuntary, arrhythmic, multidirectional, high amplitude, conjugate saccades. Opsoclonus is exacerbated by attempts at visual pursuit or refixation, persists when eyes are closed and during sleep. The eye movements often are continuous, but may be interrupted by brief pauses. In most patients it is often accompanied by truncal ataxia, myoclonus of limbs (dancing eye and dancing feet syndrome), and encephalopathy.,
Approximately 50% of children with opsoclonus and myoclonus have neuroblastoma, while only 20% of adults have an underlying cancer. In adults opsoclonus-myoclonus syndrome is most frequently because of viral infections., We are reporting such a case who showed remarkable improvement with clonazepam.
| Case report|| |
A 36 year old man complained of high grade fever of 7 days duration which persisted till 3 days prior to admission, when he developed complaint of blurring and shaking of vision. Subsequently, he developed the severe difficulty in walking. Patient was unable to sit, even with support.
At the time of admission results of general physical examination were normal. Patient was afebrile. He was alert and oriented. His other mental functions were also normal. Cranial nerve examination showed conjugate, arrhythmic, chaotic, large amplitude eye movements, predominantly in horizontal direction. Patient could not cooperate for optokinetic nystagmus testing. However, voluntary ocular movements were full but tended to exacerbate the opsoclonus. Patient had severe truncal and appendicular ataxia, along with truncal and limb myoclonus. His myoclonic movements markedly increased on attempted movement. Remaining neurological and systemic examinations were normal, except there was mild splenomegaly. He had received a course of chloroquine (1500 mg of base given over 48 hours) prior to admission.
All routine blood and urine parameters were normal. Malarial parasites in blood smear were absent. Cerebrospinal fluid examination revealed a protein concentration of 30 mg/dl, a glucose level 63 mg/dl, and cells <5/Cu mm, all mononuclears. Electroencephalogram showed intermittent generalised theta activity. Chest radiogram, abdominal ultrasound, electrocardiogram and cranial computerised tomographic scan were normal.
Soon after admission patient was treated with clonazepam 0.5 mg three times a day which gradually increased to 1 mg three times a day. Patient improved considerably in 2 weeks duration. His opsoclonus and myoclonus were reduced, and he was able to sit and stand with support. The treatment with clonazepam was continued for 4 weeks then it was gradually withdrawn in the next 2 weeks without any evidence of reappearance of symptoms.
| Discussion|| |
Opsoclonus-myoclonus syndrome is more frequent in children. In children there are three major settings in which this syndrome occurs: (1) Paraneoplastic (Neuroblastoma related), (2) Parainfectious and encephalitic, and (3) Neonatal or congenital., In more than 50% of children with opsoclonus occult neuroblastoma may be detected. In adults this syndrome is most frequently reported as a sequelae to viral infection. However, only in a few cases specific infection had been identified. These included Psittacosis, Salmonella, St. Louis encephalitis, Rickettsia and Coxsackie virus infections.,, In about 20% of adult patients this syndrome constitute paraneoplastic manifestation of an occult malignancy.,, An antinueral antibody (anti Ri) has been reported in opsoclonus patients with breast cancer. In India few cases in relation to malaria have also been described. High grade pyrexia due to malaria may some way activate a dormant neurotropic virus resulting in this syndrome. In our case also as we could not find anything that was suggestive of some occult malignancy, a viral or immunological affliction could be the probable aetiology. The pathology underlying opsoclonus-myoclonus syndrome seems to be immune mediated damage to Purkinje cells in the cerebellum.,
Treatment for opsoclonus-myoclonus syndrome from whatever the cause has not been uniformly successful. In children this syndrome usually resolves with adrenocorticotropic hormone (ACTH), or corticosteroid, but in most of the patients symptoms recur after withdrawal. Most authors believe that in adults this syndrome need no treatment as symptoms are self limiting and resolve within 6-8 weeks. There are other reports indicating that clonazepam,,, baclofen,valproate, 5-hydroxytryptophan had provided much needed symptomatic relief to these patients. In our patient we also observed significant response with clonazepam. The improvement was evident even after few doses of the drug. Our case suggests that the use of clonazepam may improve symptomatology in some adults with opsoclonus and myoclonus. Moreover, clonazepam has also been found effective in other types of myoclonus e.g. post-anoxic action myoclonus. The main action of clonazepam is its ability to interact with benzodiazepine receptors in the brain that facilitate inhibitory GABAergic transmission. However, the reason why clonazepam is more effective than other benzodiazepines in the treatment of myoclonus is not clear.
| References|| |
Anderson NE, Budde-Steffen C, Rosenblum MK et al. Opsoclonus, myoclonus, ataxia and encephalopathy in adults with cancer. A distinct paraneoplastic syndrome. Medicine 67:100-109, 1988.
Digre KB. Opsoclonus in adults. Arch Neurol 43:1165-1175,1986.
Altman AJ, Baehner RL. Favourable prognosis for survival in children with coincident opsomyoclonus and neuroblastoma. Cancer 37:846-852,1976.
Kuban KC, Ephros MA, Freeman RL, et al. Syndrome of opsoclonus-myoclonus caused by Coxsackie B3 infection. Ann Neurol 13:69-71,1983.
Koh PS, Raffensperger JG, Berry S et al. Long-term outcome in children with opsoclonus-myoclonus and ataxia and coincidental neuroblastoma. J Pediatr 125:712-716,1994.
Lugue FA, Furneaux HM, Ferziger R et al. Anti-Ri antibody associated with paraneoplastic opsoclonus and breast cancer. Ann Neurol 29:241-251, 1991.
Sheinman BD, Gawler J. Opsoclonus and polymyoclonia complicating oat cell carcinoma of bronchus. Postgraduate Med J 58:704-705,1982.
Motiani R, Agarwal S, Saifee AA, et al. Opsoclonus-Ataxia, as an unusual presentation of Malaria. Neurology India 39:39-40, 1991.
Dropco EJ, Kline LB, Riser J. Antineuronal (Anti Ri) antibodies in a patient with steroid responsive opsoclonus-myoclonus. Neurology 43:207-211,1993.
Spalton DJ, Sanders MD, Gresty M et al. Ocular myoclonyus and oscillopsia. Trans Ophthalmol Soc UK 100:507-508,1980.
Jenner P, Pratt JA, Marsden CD. Mechanism of action of clonazepam in myoclonus in relation to effects on GABA and 5-HT. In: Advances in Neurology Vol 43: Myoclonus. S Fahn et al. (Eds.). Raven Press, New York 629-643, 1986.