Year : 2022 | Volume
: 5 | Issue : 3 | Page : 189--191
Opsoclonus–myoclonus–ataxia syndrome associated with dengue encephalitis: A rare presentation
Kusum Sikariya, Nishant R Agrawal, Dinesh Chouksey, Rahul Jain, Akansha Jain, Ajoy Sodani
Department of Neurology, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India
Department of Neurology, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh
Opsoclonus–myoclonus–ataxia syndrome (OMAS) is an inflammatory neurological disordercharacterized by chaotic uncontrolled movements of the eyes and involuntary jerk-like movements of the body. We report the case of a 45-year-old man who presented with fever without any seizures or focal deficits. On examination, he had opsoclonus in his eyes and cortical myoclonus in his hands and body. On evaluation, he tested positive for the dengue NS1 antigen. Furthermore, he had low platelets, normal metabolic workup, normal brain imaging, and normalcerebrospinal fluid analysis. He was managed conservatively and showed improvement in opsoclonus–myoclonus by day 7 of his illness and complete recovery in 2 weeks. Although dengue is primarily considered to be a hematotropic virus, it can involve the nervous system and manifest with OMAS. To the best of our knowledge, OMAS has been reported in only four cases of dengue infection in adults to date.
|How to cite this article:|
Sikariya K, Agrawal NR, Chouksey D, Jain R, Jain A, Sodani A. Opsoclonus–myoclonus–ataxia syndrome associated with dengue encephalitis: A rare presentation.Ann Mov Disord 2022;5:189-191
|How to cite this URL:|
Sikariya K, Agrawal NR, Chouksey D, Jain R, Jain A, Sodani A. Opsoclonus–myoclonus–ataxia syndrome associated with dengue encephalitis: A rare presentation. Ann Mov Disord [serial online] 2022 [cited 2023 Jun 1 ];5:189-191
Available from: https://www.aomd.in/text.asp?2022/5/3/189/363465
The dengue virus belongs to the Flaviviridaefamily. Neurological dengue isclassified as a form of severe dengue. There are four antigenically different virus serotypes: DEN1, DEN2, DEN3, and DEN4. The neurological manifestations are mainly associated with DEN2 and DEN3. These serotypes were found in cases of encephalitis, meningitis, and myelitis. The association of movement disorder syndromes with dengue encephalitisis rare.We report arare case of opsoclonus–myoclonus–ataxia syndrome (OMAS) along with dengue encephalitis.
A 45-year-old man presented to our hospital with high-grade fever with chills and rigors on day 7 of his illness. He had leukopenia and thrombocytopenia withabnormal liver function test and splenomegaly on sonography. Dengue was confirmed by a positive diagnosis for the dengue NS1 antigen. The patient became irritable 4 days after admission. On examination,he displayed involuntary, chaotic, uncontrolled, and rapid multidirectional conjugate saccadic eye movements without intersaccadic intervals with frequent eye blinking, as well as spontaneous non-rhythmic generalized myoclonic jerks that aggravated on stimuli. He had action myoclonus that aggravated on standing and walking. In addition, he had scanning speech, intention tremor, truncal ataxia, and limb ataxia. The remaining neurological examination wasnormal.
Magnetic resonance imaging of the brain and cerebrospinal fluid analysis were normal (protein: 25 mg/dl, glucose: 61 mg/dl, corresponding RBS: 106, total cells: 01). However, his cerebrospinal fluid was not analyzed for the dengue antigen. The patient received 1000 mg/day of methylprednisolonepulse therapy for 5 days, followed by 50 mg/day of oral prednisolone that was tapered by 10 mg after every 3 daysover 2 weeks. The patient showed improvement with the treatment. Opsoclonus–myoclonus remarkably improved, followed by gradual improvement in ataxia.
OMAS is an inflammatory neurological disorder characterized by chaotic uncontrolledmovements of the eyes with involuntary jerk-like movements of the body, caused by the contraction of muscles. The underlying mechanism of OMAS is autoimmune dysfunction of Purkinje cells in the dorsal vermis. The autoimmune dysfunction leads to subsequent disinhibition of the oculomotor fastigial region and damage to the omnipause cells in the pontine raphe nucleus. Paraneoplastic OMAS is commonly associated with small-cell lung cancer, breastcancer, and ovarian cancer. In children, a neuroblastoma is detected in approximately 50% of the cases. Infectious causes of OMAS have been reported with herpes virus, arbovirus, severe acute respiratory syndrome coronavirus 2, and several parasitic infections.
The neurological manifestations associated with dengue are as follows: 1) Direct neurotropic complications,leading toencephalitis and myositis. 2) Systemic neurological complications due to metabolic diseases resulting in encephalopathy, cerebrovascular complications due to thrombocytopenia, and hypokalemic periodic paralysis. 3) Immune-mediated complications such as acute disseminated encephalomyelitis, Guillain–Barrésyndrome, and OMAS. The literature on dengue-related immune-mediated complications is scarce.
Bhushan et al. found that among 1627 patients infected with the dengue virus, 4.86%had immune-mediated neurological complications. On the spectrum of movement disorders, Misra and Kalita found that 11% of the patients with dengue encephalitis had movement disorders. The reported manifestations of movement disorders with dengue infection are OMAS, cerebellar syndrome,ocular flutter, and truncal ataxia.
The etiology ofOMAS includes paraneoplastic, parainfectious, toxic–metabolic, and idiopathic causes.Autoimmune-mediated dysfunction has been suggested to be the underlying mechanism of OMAS. It has been hypothesized that sequential arbovirus infections may cause immunological enhancement of infection, which could be related to the clinical forms of severe dengueor may trigger neurological complications. The interaction of the dengue virus with targetcells such as dendritic cells, hepatocytes, and endothelial cells has been established. Infection of these cells results in the production of immune mediators that shape the adaptive humoral and cellular immune response. The circulation of high levels of secreted NS1 in the presence of pre-existing heterologous non-neutralizing antibodies may mediate complement activation and trigger neurological complications.
Although the dysfunction of neurons in the pontine tegmentum is implicated in the pathogenesis of OMAS, magnetic resonance imaging of the brain does not usually demonstrate a parenchymal lesion in this condition. Various treatment modalities have been described for infectionsassociated with OMAS: methylprednisolone pulse therapy (1g/day for 5 days), intravenous immunoglobulin 0.4gm/kg daily for 5 days clonazepam (1mg three times/day), divalproex (1gthree times/day), and a tapering dose of oral methylprednisolone (40 mg/day). Early initiation of immunotherapy (corticosteroids, intravenous immunoglobulins, and/or plasma exchange) with the adrenocorticotropic hormonecyclophosphamide or rituximab, along with tumor resection (paraneoplastic OMAS), has been advocated by some authors to ensure an optimal neurological outcome. The patient in our case had a remarkable response with steroids.
We report this case of dengue-associated OMAS due to its rarity. In conclusion, early diagnosis and proper management are necessary for a good outcome and prognosis.
The authors are grateful to the physicians and nurses involved in the patient’s clinical treatment.
a. Writing of the first draft: by Kusum Sikariya,
b. Review and critique: by Nishant R. Agrawal, Dinesh Chouksey, Rahul Jain, Akansha Jain, and Ajoy Sodani.
Ethical compliance statement
Written informed consent was obtained from this patient for participation in the present case study.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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