Neurology India
Open access journal indexed with Index Medicus
  Users online: 184  
 Home | Login 
About Editorial board Articles NSI Publications Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Resource Links
  »  Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
 »Related articles
  »  Article in PDF (1,014 KB)
  »  Citation Manager
  »  Access Statistics
  »  Reader Comments
  »  Email Alert *
  »  Add to My List *
* Registration required (free)  

  In this Article
 »  Abstract
 » Patients and Methods
 » Results
 » Discussion
 »  References
 »  Article Figures
 »  Article Tables

 Article Access Statistics
    PDF Downloaded42    
    Comments [Add]    

Recommend this journal


Table of Contents    
Year : 2018  |  Volume : 66  |  Issue : 5  |  Page : 1332-1337

Pediatric opsoclonus-myoclonus-ataxia syndrome: Experience from a tertiary care university hospital

1 Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India
2 Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India

Date of Web Publication17-Sep-2018

Correspondence Address:
Dr. Parayil S Bindu
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru - - 560 029, Karnataka
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.241404

Rights and Permissions

 » Abstract 

Background: Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare disorder; there is limited experience regarding its clinical course and therapeutic response.
Aims and Objectives: To describe the clinical profile, investigations, and therapeutic outcome in pediatric OMAS.
Patients and Methods: Fourteen children (age: 27.1 ± 7 months; male: female = 1:2.3) suffering from OMAS seen over a period of 10 years (2006–2015) were included in the study. Their clinicodemographic profile, investigations, therapeutic outcome at follow-up, and relapses were reviewed.
Results: Ten children reported antecedent events (respiratory infection: 7; gastrointestinal infection: 1; vaccination: 2). The most common referral diagnosis was acute cerebellitis (n = 8). Hypotonia (n = 9), abnormal behavior (n = 10), and neuroregression (n = 6) were also the frequent manifestations. Brain magnetic resonance imaging, cerebrospinal fluid, and urinary vanillylmandelic acid were normal in all the patients. Seven patients had an underlying tumor (abdomen: 4; thorax: 2; neck: 1) detected by ultrasound (n = 2/14), computed tomography (CT) (n = 6/12), and fluorodeoxyglucose - positron emission tomography (n = 2/2). CT scan identified the tumor in 2 patients where metaiodobenzylguanidine scintigraphy was negative. All patients received steroids for 22.3 ± 20 months (3 months to 5 years). Eight required prolonged immunomodulation (>12 months). Complete remission after follow-up of 31.3 ± 19 months (7 months to 5 years) was noted in 5 patients, whereas the rest had persisting behavioral and cognitive abnormalities. Relapses were noted in 6 patients related to intercurrent infections (n = 5) and discontinuation of steroids (n = 1). The patients presented with isolated symptoms of the full-blown syndrome during their relapses.
Conclusion: OMAS in children runs an indolent course requiring careful monitoring and long-term immunomodulation. An abnormal behavior is common and the outcome is variable.

Keywords: Methyl prednisolone, neuroblastoma, paraganglioma, paraneoplastic, pediatric opsoclonus-myoclonus-ataxia, steroids
Key Message: Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare syndrome of early childhood characterized by truncal and appendicular ataxia, rapid conjugate multidirectional eye movements (opsoclonus), and jerky movements of the body with tremulousness, behavioral, and sleep disturbances. It is associated with a tumor, such as a neuroblastoma or a ganglioneuroma, in 40-50% of the cases and has a good therapeutic response to immunomodulation. Relapses are related to intercurrent infections and discontinuation of steroids. Patients often present with isolated symptoms of the syndrome during their relapses and may also have neuropsychiatric sequele, especially hyperactivity and poor scholastic performance. The study highlights the therapeutic response to pulsed or oral steroids and the requirement for a prolonged maintenance therapy.

How to cite this article:
Huddar A, Bindu PS, Nagappa M, Bharath RD, Sinha S, Mathuranath PS, Taly AB. Pediatric opsoclonus-myoclonus-ataxia syndrome: Experience from a tertiary care university hospital. Neurol India 2018;66:1332-7

How to cite this URL:
Huddar A, Bindu PS, Nagappa M, Bharath RD, Sinha S, Mathuranath PS, Taly AB. Pediatric opsoclonus-myoclonus-ataxia syndrome: Experience from a tertiary care university hospital. Neurol India [serial online] 2018 [cited 2019 Feb 16];66:1332-7. Available from:

Opsoclonus-myoclonus-ataxia syndrome (OMAS) was first described by Kinsborne in 1962.[1] It is a rare and distinct syndrome of early childhood characterized by truncal and appendicular ataxia, rapid conjugate multidirectional eye movements (opsoclonus), and jerky movements of the body with tremulousness, behavioral, and sleep disturbances.[2] OMAS is often misdiagnosed as acute cerebellar ataxia in children.[3] It is associated with a tumor, viz. neuroblastoma and ganglioneuroma among others, in 40–50% of the cases.[2] OMAS has a good therapeutic response to immunomodulation. Early diagnosis and prompt treatment prevent neuropsychiatric sequelae and morbidity in children. There are relatively few studies which describe the therapeutic response and outcome in children with OMAS and only one case series from India.[4],[5],[6],[7],[8],[9],[10] In this study, we retrospectively analyzed the clinical profile, laboratory investigations, and therapeutic outcome in pediatric OMAS.

 » Patients and Methods Top

This study was carried out at the National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, India, which is a tertiary care referral centre for neuropsychiatric cases. Patients were diagnosed with OMAS based on the previously proposed criteria, i.e., the presence of three or more of the following: (1) Opsoclonus; (2) myoclonus and/or ataxia; (3) behavioral abnormalities and/or sleep disturbances; and (4) the presence of a neuroblastoma.[5] Patients aged 5 years or less, diagnosed with OMAS between 2006 and 2015, who were evaluated and followed up in a single neurology unit were included in the study. Their case records were retrieved and reviewed.

Clinicodemographic data were extracted from case files. The data included the patients' age, gender, birth and development history, and preceding events, if any. Clinical profile details including the age of onset, duration, symptomatology, investigations, follow-up duration, relapses, and response to treatment were noted. All patients underwent brain magnetic resonance imaging (MRI), urinary vanillylmandelicacid (VMA), and lumbar cerebrospinal fluid (CSF) analysis. Other laboratory tests such as the complete blood count, hepatic and renal function tests, lactate, and vitamin B12, among others, were performed based on the clinical discretion. Imaging studies performed to identify the underlying tumor included computed tomography (CT) scan of the abdomen (n = 12), thorax (n = 3), and neck (n = 1); metaiodobenzylguanidine (MIBG) scan (n = 3); and fluorodeoxyglucose positron emission tomography [FDG-PET] (n = 2). The protocol used for various imaging studies was as follows. A helical CT scan from the diaphragm to the pelvic diaphragm was performed using 90 kV and 200 mAs (Philips Brilliance Royal Philips Electronics, The Netherlands). An oral (1200 ml of 2% ionic contrast) and an intravenous contrast (50 ml of nonionic contrast) 0.938 pitch was also simultaneously administered. Serial 5-mm axial reconstructions together with coronal and sagittal constructions were obtained. The MIBG scan was performed 24 and 48 hours after intravenous injection of 300 mci of MIBG. Whole body FDG-PET was performed in fasting state 60 minutes after the intravenous injection of 3.5 mci of gallium (Ga)-68 DOTANOC/10 mci of fluoro-18-L-dihydroxyphenylalanine (18F-FDOPA). CT images were retrieved from the Picture Archival and Communication System (PACS) and reviewed (AH, PSB, MN, and RDB). Doubts were resolved by discussion with other authors (ABT and SS).

All patients received intravenous methylprednisolone (20–30 mg/kg/day infusion for 5 days) as induction therapy. Subsequently, the patients received oral prednisolone (0.75–1.0 mg/kg/day) or monthly intravenous pulsed methylprednisolone. The patients were followed up and assessed clinically for symptomatic response to treatment, relapses, persisting behavioral, and neurological abnormalities. The data were entered into a predesigned proforma and incorporated into a Microsoft Excel spreadsheet for analysis.

 » Results Top

Clinical profile

Twenty-three patients were diagnosed with OMAS during the study period. Of these, 14 were aged 5 years or less (the mean age: 21.7 ± 7 months, age range: 10–36 months, male:female = 1:2.3) and were included for analysis. The mean duration of symptoms was 3.8 ± 5.0 months (range: 15 days to 16 months). All patients had uneventful birth and development milestones. [Table 1] summarizes and compares the clinical features, treatment, and follow-up details of patients with and without tumor. The most common referral diagnosis was acute cerebellar ataxia (n = 8). Antecedent events were noted in 10 patients, with the most common being upper respiratory tract infection (50%). The clinical features noted at presentation in the order of frequency were ataxia (71%), tremulousness (57%), abnormal eye movements (50%), behavioral abnormalities (28%), and regression of milestones (21%). Behavioral abnormalities included irritability (60%) followed by anger outbursts (40%) and clinging to mother (20%).
Table 1: Comparison of clinical features and outcome in pediatric OMAS with and without an underlying tumor (n=14)

Click here to view


Brain MRI, urinary vanillylmandelic acid (VMA), cerebrospinal fluid (CSF) analysis, complete blood count, hepatic and renal function tests, and lactate were normal in all patients. An underlying neuroblastoma/paraganglioma was detected in 7 patients. The sites of the neuroblastoma included the abdomen (n = 4), thorax (n = 2), and neck (n = 1) [Figure 1]. Histopathological confirmation was available in 4 patients.
Figure 1: CT scan of thorax of a 14-month old girl (a: plain; b: contrast enhanced) shows a paraganglioma in the right cervicothoracic paraspinal region (arrows). CT scan of the thorax of a 10-month old girl shows a paraganglioma in the right thoracic paraspinal region (c, arrow). CT scan of the abdomen of a 3-year old boy (d: contrast enhanced coronal section; e: plain axial section; f: contrast-enhanced axial section) shows a paraganglioma of the abdomen (arrows)

Click here to view

Treatment and follow-up

The mean duration between the onset of symptoms and the initiation of immunomodulation was 6.1 ± 7.5 months (range: 2–24 months), and the mean duration of treatment was 22.3 ± 20 months (range: 3 months to 5 years). Intravenous immunoglobulin (IVIG) was administered in 1 patient because of the presence of severe disease. Following induction with intravenous methylprednisolone, maintenance therapy was initiated (n = 13), including monthly methylprednisolone alone (n = 4), methylprednisolone followed by oral steroids (n = 4), and oral steroids alone (n = 5). The number of pulses of methylprednisolone was decided based on the clinical response and the patient's compliance to treatment. Among patients who were on oral steroids, 3 received pulsed methylprednisolone for relapse of the symptoms. Symptomatic treatment included sodium valproate (n = 5), clonazepam (n = 6), and clonidine (n = 2).

Out of 7 patients with tumor, 3 patients underwent surgical removal. The mean duration between the onset of symptoms and surgery (n = 3) was 28 ± 17 months (12–46 months). The duration of follow-up following surgery ranged from 2 weeks to 2 years. Two patients had persisting symptoms and required immunomodulation even after surgery.

The mean follow-up (n = 13) was 31.3 ± 19 months (range: 7–62 months). Three patients did not attain remission. In the remaining patients, the mean duration from initiation of treatment to remission was 12 ± 14 months (range: 2 months to 4 years). Twelve relapses were noted in 6 patients. In 5 patients, relapses were precipitated by fever and infection and in 1 patient due to stopping of steroids. Relapses were mainly in the form of increased ataxia or tremulousness, rather than the presence of a full syndrome. At the time of the last follow-up, only 5 patients had achieved complete remission, whereas the rest had persisting mild behavioral and cognitive abnormalities [Table 1]. In 4 patients, an underlying neuroblastoma or a paraganglioma was detected at the time of first evaluation itself, whereas in the remaining 3 patients, it was detected during the follow-up visit.

 » Discussion Top

OMAS is rare with an incidence of 0.27 cases/million population in Japan and 1.8/million in the United Kingdom. A previous study from India showed that this disorder contributes to nearly 7% of acute movement disorders in children.[11] There is only one case series from India describing 11 children with OMAS.[6] The most common age of onset is 1–3 years.[3],[5],[7] The common underlying etiologies include paraneoplastic (21%), and parainfectious causes (55%).[5] Less common etiologies such as vaccination and intoxication with phenytoin have been proposed.[12],[13],[14],[15],[16],[17],[18],[19] Recent case studies from India have reported infectious causes such as scrub typhus,[20],[21] dengue,[22] malaria,[23] and varicella zoster virus.[24] Due to these associations and symptomatic response to immunomodulatory therapies, the pathophysiology is thought to be autoimmune in nature.[2],[19],[25] Autoantibodies against antigens on neuronal and dendritic surface, namely N-methyl-D-aspartate (NMDA), glutamic acid deacetylase (GAD), anti-Hu and gamma amino butyric acid (GABA B), are reported.[26],[27],[28],[29],[30] Recently, glucose transporter-1 deficiency has also been suggested as an etiological cause.[31] Recent studies have reported a new hypothesis that proposes disinhibition of the fastigial nucleus in the cerebellum. This is evidenced by an increased activity in bilateral cerebellar nuclei, as detectable by functional MRI scans, in response to administration of clonazepam;[21],[32],[33] and, an atrophy of vermis and floculonodular lobes in patients with persisting and severe symptoms.[34] Damage to omnipause cells in the nucleus raphe interpositus of pons (which prevents excessive saccades by inhibiting burst neurons in the para-pontine reticular formation) and rostral interstitial nucleus of Cajal has also been reported.[35]

In this study, children constituted 60% of the entire cohort. Similar to previous studies, we observed that respiratory illness was the most common antecedent event and ataxia was the most common presenting feature. Behavioral and sleep abnormalities were noted in 70–80% of the patients, which is also similar to previous studies.[4],[5],[8] However, girls outnumbered boys in contrast to earlier studies.[4],[5],[6],[7],[8] Klein et al., also reported a female preponderance.[9] There was no significant difference in the clinical profile or sequelae between the patients with or without tumor, a finding that was similar to that obtained in the previous studies.[4],[5] A comparison between this study and previous case series is presented in [Table 2].
Table 2: Comparison of current study with other studies

Click here to view

Our study highlights that, even when urinary VMA and iodine-123 meta-iodobenzylguanidine (MIBG) are normal, the CT scan aids in detection of an underlying tumor. This has been noted in previous studies and has been attributed to the low grade of the tumor.[2] A neuroblastoma/paraganglioma was detected in 50% of the patients, whereas in the previous studies, this incidence ranged between 7–65%.[4],[5],[6],[7],[8],[9] Thus, a diligent search with a CT scan of the abdomen, thorax, and neck not only has a high yield but is also cost-effective compared to the FDG-PET.[36] This observation is particularly relevant in countries such as India where cost and availability of investigations is a major constraint. In recent studies, the presence of infection has been found to influence the treatment and outcome.[20],[21] In the current study, investigation for infectious causes was not done as most of cases were referred in the subacute or chronic stage.

A good therapeutic response to immunomodulation was noted, similar to the findings of previous studies.[4],[5],[6],[7],[8],[9],[10] Steroids were used as the mainstay of treatment in the current study, either in the form of intravenous pulse methylprednisolone and/or oral prednisolone. Clinical remission was noted in a majority of the patients (>80%), thus eliminating the need for other agents such as cyclophosphamide and rituximab, which are more toxic and expensive. Clinical remission, relapses, and sequelae noted in the present study were similar to the findings of previously reported studies, where intravenous immunoglobulin (IVIG), adrenocorticotrophic hormone (ACTH), and immunomodulation were used.[6],[7] Rituximab and cyclophosphamide were not used in the present study in view of a good therapeutic response with steroids as well as the cost constraints. However, most of the patients required a prolonged immunomodulation therapy. The usefulness of IVIG [37] and plasmapheresis [38] in cases with partial recovery to prednisolone has been reported. In this study, only 1 patient received IVIG due to partial recovery with prednisolone. Recent studies have suggested a multimodal approach and the use of rituximab based on the role of B cell and B cell activating factor (BAFF) in the pathogenesis of OMAS.[39],[40],[41] Monotherapy with clonazepam [32],[33],[35] or doxycycline in the case of an infectious etiology, particularly due to scrub typhus,[20],[21] leading to complete recovery, has also been reported. Based on the current literature, we propose an algorithm for the evaluation and management of OMAS [Figure 2].
Figure 2: An algorithm for the evaluation and management of opsoclonus myoclonus ataxia syndrome

Click here to view

There was no difference in the duration of immunomodulation and outcome in patients with or without a tumor. This observation is similar to the findings of previous studies.[4-7,9] In contrast, Singhi et al., reported that the presence of a tumor was an indicator of a worse outcome.[9] Larger randomized controlled studies are required for confirming these results. It was noted that most patients required prolonged treatment for maintenance of their improved outcome.

Approximately half of the patients had relapses that were temporally related to infection or tapering of steroids. Persisting neuropsychiatric features, including hyperactivity and a poor scholastic performance, were noted in less than half of the cohort, in contrast to the speech and motor disturbances noted in the previous studies [Table 2]. A follow-up is required in these patients not only for monitoring treatment, relapses and persisting abnormalities but also for the detection of tumor, as an underlying tumor was detected in 3 patients during the follow-up visit in the present study.

This study has limitations of being a retrospective, smaller cohort study and a lack of a uniform treatment protocol. Nevertheless, it highlights the therapeutic response to pulsed or oral steroids and the requirement for a prolonged maintenance therapy. We already know that OMAS is a rare and distinct pediatric syndrome associated with an underlying tumor in 40–50% of cases. Behavioral and sleep disturbances are commonly seen. It is responsive to immunomodulation. What the current study adds is that there is a female preponderance, especially in the subset with an underlying tumor. A diligent search with CT scan of the abdomen, thorax, and neck is the most sensitive, cost-effective, and yielding investigation to detect an underlying tumor. Monotherapy with pulse methylprednisolone, both for acute and maintenance phase, appears to be a good therapeutic option. What remains to be known is the optimal duration of immunomodulation required to balance the relapse rates versus the side effects of treatment.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 » References Top

Kinsbourne M. Myoclonic encephalopathy of infants. J Neurol Neurosurg Psychiatry 1962;25:271-6.  Back to cited text no. 1
Pike M. Opsoclonus-myoclonus syndrome. Handb Clin Neurol 2013;112:1209-11.  Back to cited text no. 2
Desai J, Mitchell WG. Acute cerebellar ataxia, acute cerebellitis, and opsoclonus-myoclonus syndrome. J Child Neurol 2012;27:1482-8.  Back to cited text no. 3
Tate ED, Allison TJ, Pranzatelli MR, Verhulst SJ. Neuroepidemiological trends in 105 US cases of pediatric opsoclonus-myoclonus syndrome. J Pediatr Oncol Nurs 2005;22:8-19.  Back to cited text no. 4
Brunklaus A, Pohl K, Zuberi SM, Sousa C De. Outcome and prognostic features in opsoclonus- myoclonus syndrome from infancy to adult life. Pediatrics 2011;128:e388-94.  Back to cited text no. 5
Pohl KR, Pritchard J, Wilson J. Neurological sequelae of dancing eye syndrome. Eur J Padiatr 1996;155:237-44.  Back to cited text no. 6
Hasegawa S, Matsushige T, Kajimoto M, Inoue H. A nationwide survey of opsoclonus – myoclonus syndrome in Japanese children. Brain Dev 2015;37:656-60.  Back to cited text no. 7
Singhi P, Sahu JK, Sarkar J, Bansal D. Clinical profile and outcome of children with opsoclonus-myoclonus syndrome. J Child Neurol 2014;29:58-61.  Back to cited text no. 8
Klein A, Schmitt B, Boltsheuser E. Long term outcome of ten children with opsoclonus-myoclonus syndrome. Eur J Pediatr 2007;166:359-63.  Back to cited text no. 9
Pang KK, Sousa C De, Lang B, Pike MG. A prospective study of the presentation and management of dancing eye syndrome/opsoclonus – myoclonus syndrome in the United Kingdom. Eur J Paediatr Neurol 2010;14:156-61.  Back to cited text no. 10
Goraya JS. Acute movement disorders in children: Experience from a developing country. J Child Neurol 2015;30:406-11.  Back to cited text no. 11
Cooper CJ, Said S, Paso E. West Nile virus encephalitis induced opsoclonus-myoclonus syndrome. Neurol Int 2014;6:5359.  Back to cited text no. 12
Vale TC, Silva RA, Cunningham MC, Maia DP, Camargos ST, Cardoso F. Opsoclonus-myoclonus-ataxia syndrome in an AIDS patient. Einstein (Sao Paulo) 2013;11:533-4.  Back to cited text no. 13
Kang B, Kim J. Opsoclonus-myoclonus syndrome associated with mumps virus infection. J Clin Neurol 2014;10:272-5.  Back to cited text no. 14
Belcastro V, Piola M, Binda S, Santoro D, Rezzonico M, Arnaboldi M. Opsoclonus-myoclonus syndrome associated with human herpes virus-6 rhomboencephalitis. J Neurol Sci 2014;341:165-6.  Back to cited text no. 15
Lapenna F, Lochi L, Mari M De, Iliceto G, Lamberti P. Post-vaccinic opsoclonus myoclonus syndrome: A case report. Parkinsonism Relat Disord 2000;6:241-2.  Back to cited text no. 16
Laroumagne S, Elharrar X, Coiffard B, Plojoux J, Dutau H, Breen D, et al. “Dancing Eye Syndrome” secondary to opsoclonus-myoclonus syndrome in small-cell lung cancer. Case Rep Med 2014;2014:545490.  Back to cited text no. 17
Krug P, Schleiermacher G, Michon J, Valteau-Couanet D, Brisse H, Peuchmaur M, et al. Opsoclonus-myoclonus in children associated or not with neuroblastoma. Eur J Paediatr Neurol 2010;14:400-9.  Back to cited text no. 18
Raffaghello L, Conte M, De Grandis E, Pistoia V. Immunological mechanisms in opsoclonus-myoclonus associated neuroblastoma. Eur J Paediatr Neurol 2009;13:219-23.  Back to cited text no. 19
Koti N, Mareddy AS, Nagri SK, Kudru CU. Dancing eyes and dancing feet in scrub typhus. Implications for practice. Australian Med J 2015;8:371-2.  Back to cited text no. 20
S D'sa, Singh S, Sowmya S. Opsoclonus in scrub typhus. J Postgrad Med 2012;58:296-8.  Back to cited text no. 21
Verma R, Sharma P, Garg RK, Atam V, Singh MK. Neurological complications of dengue fever: Experience from a tertiary center of north India. Ann Indian Acad Neurol 2011;14:272-8.  Back to cited text no. 22
[PUBMED]  [Full text]  
Garg RK, Karak B, Misra S. Neurological manifestations of malaria: An update. Neurol India 1999;47:85-91.  Back to cited text no. 23
[PUBMED]  [Full text]  
Singh D, Sinha M, Kumar R, Shukla R, Ahuja RC. Opsoclonus-myoclonus syndrome caused by varicella-zoster virus. Ann Indian Acad Neurol 2010;13:211-2.  Back to cited text no. 24
[PUBMED]  [Full text]  
Hero B, Schleiermacher G. Update on pediatric opsoclonus myoclonus syndrome. Neuropediatrics 2013;44;324-9.  Back to cited text no. 25
Player B, Harmelink M, Bordini B, Weisgerber M, Girolami M, Croix M. Pediatric opsoclonus-myoclonus-ataxia syndrome associated with anti- N -methyl-D-aspartate receptor encephalitis. Pediatr Neurol 2015;53:456-8.  Back to cited text no. 26
Panzer JA, Anand R, Dalmau J, Lynch DR. Antibodies to dendritic neuronal surface antigens in opsoclonus myoclonus ataxia syndrome. J Neuroimmunol 2015;286:86-92.  Back to cited text no. 27
DeFelipe-Mimbrera A, Masjuan J, Corral Í, Maria L, Graus F, García-barragán N. Opsoclonus-myoclonus syndrome and limbic encephalitis associated with GABA B receptor antibodies in CSF. J Neuroimmunol 2014;272:91-3.  Back to cited text no. 28
Blaes F, Dharmalingam B. Childhood opsoclonus-myoclonus syndrome: Diagnosis and treatment. Exp Rev Neurother 2016;16:641-8.  Back to cited text no. 29
Bhandari HS. Rare disease Presentation of opsoclonus myoclonus ataxia syndrome with glutamic acid decarboxylase antibodies. BMJ Case Rep 2012;8:1-3.  Back to cited text no. 30
Appavu B, Mangum T, Obeid M. Glucose transporter-1 deficiency: A treatable cause of opsoclonus and epileptic myoclonus. Pediatr Neurol 2015;53:364-6.  Back to cited text no. 31
Paliwal VK, Chandra S, Verma R, Kalita J, Mishra UK. Clonazepam responsive opsoclonus myoclonus syndrome: Additional evidence in favour of fastigial nucleus disinhibition hypothesis? J Neural Transm 2010;117:613-5.  Back to cited text no. 32
Garg RK, Kar AM, Dixit V. Opsoclonus-myoclonus syndrome in an adult: A case report and response to clonazepam. Indian J Ophthalmol 1996;44:101-3.  Back to cited text no. 33
[PUBMED]  [Full text]  
Anand G, Bridge H, Rackstraw P, Chekroud AM, Yong J, Stagg CJ, et al. Cerebellar and cortical abnormalities in paediatric opsoclonus-myoclonus syndrome. Dev Med Child Neurol 2015;57:265-72.  Back to cited text no. 34
Sahu JK, Prasad K. The opsoclonus-myoclonus syndrome. Pract Neurol 2011;11:160-6.  Back to cited text no. 35
Takama Y, Yoneda A, Nakamura T, Nakaoka T, Higashio A, Santo K, et al. Early detection and treatment of neuroblastic tumor with opsoclonus-myoclonus syndrome improve neurological outcome: A review of five cases at a single institution in Japan. Eur J Pediatr Surg 2016;26:54-9.  Back to cited text no. 36
Unal A, Sumer MM, Atasoy HT, Atasoy N. Treatment of idiopathic opsoclonus-myoclonus syndrome with intravenous immunoglobulin. Neurol India 2004;52:520-1.  Back to cited text no. 37
[PUBMED]  [Full text]  
Sheela SR, Mani PJ. Opsoclonus myoclonus syndrome: Response to plasmapheresis. Indian Pediatr 2004;41:499-502.  Back to cited text no. 38
Toyoshima D, Morisada N, Takami Y, Kidokoro H, Nishiyama M, Nakagawa T, et al. Rituximab treatment for relapsed opsoclonus-myoclonus syndrome. Brain Dev 2016;38:346-9.  Back to cited text no. 39
Sinha S, Sarin YK. Rituximab for opsoclonus myoclonus ataxia syndrome associated with neuroblastoma. India J Pediatr 2014;81:218-9.  Back to cited text no. 40
Tate ED, Pranzatelli MR, Verhulst SJ, Malkwell SJ, Franz DN, Graf WD, et al. Active Comparator-controlled rated blinded study of corticotrophin based immunotherapies for opsoclonus myoclonus syndrome. J Child Neurol 2012;27:875-84.  Back to cited text no. 41


  [Figure 1], [Figure 2]

  [Table 1], [Table 2]


Print this article  Email this article
Online since 20th March '04
Published by Wolters Kluwer - Medknow