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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 24  |  Issue : 1  |  Page : 59-62

Opsoclonus-myoclonus-ataxia syndrome presenting in Enugu, Southeast Nigeria: A case report


Department of Medicine, University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria

Date of Web Publication1-Aug-2019

Correspondence Address:
Dr. Chiamaka E Okereke
Department of Neurology, University of Nigeria Teaching Hospital, PMB 01129, Enugu
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmh.IJMH_4_19

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  Abstract 

Opsoclonus – myoclonus- ataxia syndrome is an extremely rare neurological condition of probable autoimmune aetiology. It has not been previously described in medical literature from Nigeria and West Africa. This is a case report of a 43- year old Nigerian woman who presented to the University of Nigeria Teaching Hospital Enugu Emergency Department in July 2018 with a 5 weeks history with vertigo, diplopia, dancing eyes, muscular twitches and incoordination two weeks after a caesarean section complicated by intra-operative bleeding. No other significant medical history. She had been managed as a case of cerebellar haemorrhage before referral to the Neurology Unit. On examination she was lucid, had normal vital signs, opsoclonus, motor ataxia and myoclonus affecting the left face as well as left upper extremity. Investigations done were normal except for reduced cerebrospinal fluid ( CSF) protein. Her symptoms responded substantially to prednisolone and clonazepam. The relevant literature was reviewed. Opsoclonus myoclonus ataxia syndrome though rarely described in Africans may occur and may be misdiagnosed by inexperienced physicians leading to undesirable consequences.

Keywords: Ataxia, myoclonus, Nigeria, opsoclonus


How to cite this article:
Ikemeh A, Onwuekwe I, Okereke CE, Ekenze O. Opsoclonus-myoclonus-ataxia syndrome presenting in Enugu, Southeast Nigeria: A case report. Int J Med Health Dev 2019;24:59-62

How to cite this URL:
Ikemeh A, Onwuekwe I, Okereke CE, Ekenze O. Opsoclonus-myoclonus-ataxia syndrome presenting in Enugu, Southeast Nigeria: A case report. Int J Med Health Dev [serial online] 2019 [cited 2019 Aug 21];24:59-62. Available from: http://www.ijmhdev.com/text.asp?2019/24/1/59/263546




  Introduction Top


Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare clinical entity characterized by chaotic eye movements and myoclonus.[1] It can occur in association with a neoplasm or following viral or bacterial infections.[2],[3],[4] Early commencement of treatment, which includes immunomodulators and cerebellar sedatives, is important in ensuring a better outcome for the patient. This case report is necessary because to the best of our knowledge, it is the first documented case seen in Southeast Nigeria, which had been misdiagnosed before appropriate referral to the neurologist.


  Case Summary Top


A 43-year-old, married Nigerian woman of the Igbo tribe was admitted into the Female Medical Ward of the University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria, via the Emergency Department in July 2018 as a possible case of cerebellar hemorrhage. She was then referred to the Neurology Unit after 2 days of no improvement. She presented with sudden-onset vertigo and double vision of 5 weeks duration. The symptoms were moderate in intensity initially but later became severe and disabling. Two days later, she developed tremors and repetitive muscle twitching involving the left upper limb, which was aggravated by use. These soon affected the left side of her face. She also was unable to stand without support.

There was no fever, alteration in consciousness, headache, vomiting, numbness, limb weakness, or other relevant associated symptoms. She was not known to have either hypertension, diabetes mellitus, or dyslipidemia.

She had an elective cesarean section 2 weeks before the onset of her symptoms. Surgery was said to have lasted for about 6h due to intraoperative hemorrhage but she was not transfused. Baby was normal. Postoperatively, she developed severe, generalized, nonpulsatile headache, which lasted for 1 week. She did not have a record of fever, neck stiffness, photophobia, or altered behavior associated with the headache.

She was lactating adequately before the onset of illness. She had no significant family history and had negligible alcohol intake.

Notably on neurological examination, she was conscious, well oriented, and her neck was supple. Rapid myoclonic activity prominent on the left side of the face and the left upper limb was observed. She also had rapid, continuous, involuntary horizontal, and vertical movements of both eyes akin to dancing eyes, which were present in all ranges of eye movement (opsoclonus). There was severe truncal ataxia. The rest of the systemic examination was essentially unremarkable. Her blood pressure was 122/80mm Hg, whereas her pulse was 78 beats/min regular and full volume. The rest of the systemic examination was normal.

A diagnosis of OMAS of yet unexplained etiology was made by the consultant neurologist based on the aforementioned clinical findings. Relevant investigations were requested for and the results were obtained as shown in [Table 1].
Table 1: Results of laboratory investigations

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Brain magnetic resonance imaging (MRI) showed no abnormalities in T1, T2, and Fluid arrenuated inversion recovery sequences.

The patient was treated with tab clonazepam (1mg bid), tab prednisolone (30mg od), tab prochlorperazine (5mg tid), and tab cinnarizine (25mg bid). She made an impressive recovery. The myoclonus, ataxia, and vertigo subsided remarkably within a week of commencement of medications. The opsoclonus substantially reduced but did not stop completely.

She was discharged home after 2 weeks of admission and was followed up once at the outpatient clinic with continued stable state.




  Discussion Top


OMAS is an inflammatory neurological disorder, which has equally been recognized as a part of paraneoplastic syndrome, characterized by ocular, motor, behavioral, sleep, and language disturbances usually with an abrupt, severe onset and can also become chronic.[3],[4]

It is an extremely rare condition affecting one in one million people per year. Its prevalence is not known because of its rarity. It affects 2%–3% of children with neuroblastoma.[1],[2] In adults, most cases are associated with breast carcinoma or small cell lung carcinoma.[5]

There has also been a hypothesis that viral infections (putatively St. Louis encephalitis, Epstein–Barr, and Coxsackie B) contribute to the cause of a number of cases of OMAS although a direct connection has not been proven.[6] In a few cases, it has followed Lyme disease.[7] In other cases, as in our index case, the cause remains unknown.[4],[8] Manifestations include opsoclonus, which are rapid, involuntary, multidirectional, and unpredictable fast eye movements; myoclonus, described as brief, involuntary twitching of a muscle or group of muscles; and cerebellar ataxia, presenting as either of or both truncal and appendicular. Additional features noted to occur include aphasia, strabismus, vomiting, and sleep disorders.[4],[8]

The diagnosis of OMAS is basically clinical. No diagnostic tests are available yet as the antigen remains unidentified. Clinical diagnosis is based on the presence of any three of the four following criteria: neuroblastoma, opsoclonus, myoclonus and/or ataxia, and behavioral or sleep disturbance. Our patient satisfied three of the four criteria for OMAS diagnosis. No clinical basis was available to suspect a neoplastic or encephalitic process in the background. The role of the initial headache was unclear as was the reduced cerebrospinal fluid protein level.

No heredity is involved in OMAS. The disorder is sporadic and occurs in people with no family history of the condition.[9]

Laboratory tests can be carried out in some cases to detect certain antibodies and/or abnormal white blood cells such as the Hu anti-neuronal nuclear antibodies (anti-Hu) present in the blood of adults but not in children. MRI in most instances shows neuroblastoma when present.[8],[9]

Pharmacological treatment uses steroids or adrenocorticotropic hormone. Tumor when present should be aggressively approached with appropriate chemotherapy, surgery, and/or radiation. Treating the underlying cause may result in symptomatic improvement.[3],[4],[8] There may be a role for plasmapheresis, human intravenous immunoglobulin, and immunosuppressive agents such as rituximab.[10] In many cases, a combination of medications produces most remarkable results as witnessed in our patient, who responded eloquently to a combination of prednisolone, clonazepam, and cerebellar sedatives (prochlorperazine and cinnarizine).[4],[8],[10]

It has been recognized that there is a risk of persistent neurological deficit following apparent recovery from OMAS. Those with milder symptoms recover better, whereas those with severe symptoms have the highest risk for permanent neurological problems.[8]


  Conclusion Top


OMAS remains a rare disorder with potentially alarming consequences. Prompt diagnosis and immediate commencement of treatment options remain the bedrock for ensuring good quality life in affected patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Baets J, Pals, P, Bergmans FE, Smets K, Hauman H, Vanderwegen L et al. Opsoclonus-myoclonus syndrome: A clinicopathological confrontation. Acta Neurologica Belgica 2006;106:142-6.  Back to cited text no. 1
    
2.
Deconinck N, Scaillon M, Segers V, Groswasser JJ, Dan B Opsoclonus-myoclonus associated with celiac disease. Paediatric Neurology 2006;34:312-4.  Back to cited text no. 2
    
3.
Pranzatelli MR Opsoclonus-Myoclonus Syndrome. National Organization For Rare Disorders (NORD) [ Internet]2009; [cited 2018 Oct 14]. Available from: https://rarediseases.org/rare-diseases/opsoclonus-myoclonus-syndrome/.  Back to cited text no. 3
    
4.
https://www.ninds.nih.gov/Disorders/All-Disorders/Opsoclonus-Myoclonus-Information-Page.  Back to cited text no. 4
    
5.
Dropcho EJ Paraneoplastic Opsoclonus in Adults. Clinical Summary. MedLink Corporation. [Updated 2018 Jan;cited 2018 Oct 15]. Available from: http://www.medlink.com/article/paraneoplastic_opsoclonus_in_adults.  Back to cited text no. 5
    
6.
Shukla R, Ahuja RC, Kumar R, Singh D, Sinha M Opsoclonus-Myoclonus syndrome caused by varicella-zoster virus. Annals of Indian Academy of Neurology 2010;13:211-2.  Back to cited text no. 6
    
7.
Peter L, Jung J, Tilikete C, Ryvlin P, Maugulere F Opsoclonus-myoclonus as a manifestation of Lyme disease. Journal of Neurology, Neurosurgery & Psychiatry 2006;77:1090-1.  Back to cited text no. 7
    
8.
What is the Opsoclonus-Myoclonus Syndrome? National Pediatric Neuroinflammation Organization, Inc. [cited 2018 Oct 14]. Available from:http://www.omsusa.org/pranzatelli-Brochure1.htm.  Back to cited text no. 8
    
9.
Opsoclonus-myoclonus syndrome. Orphanet. [updated 2014 November; cited 2018 Oct 13]. Available from:http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=1183.  Back to cited text no. 9
    
10.
Dalmau J, Rosenfeld. Opsoclonus myoclonus ataxia. UpToDate [internet].2016 May [cited 2018 Oct 13]. Available from: http://www.uptodate.com/contents/opsoclonus-myoclonus-ataxia.  Back to cited text no. 10
    



 
 
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