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Título

ADULT ONSET OPSOCLONUS-MYOCLONUS-ATAXIA SYNDROME WITH SEIZURE AND RELATED TO EPSTEIN BARR VIRUS

RESUMO

A 49 year-old-man previously healthy started with fever, headache, malaise, swallow pain and was diagnosed with pharyngitis and treated with azitromicin and amoxicillin clavulanate, without fever resolution. Twelve days after initial symptoms, he had a generalized tremor and presented a tonic-clonic seizure. It was described anxiety and irritability of the patient. On neurological examination, he had generalized tremor with head and axial titubation, GCS 15, dysarthria, ocular motricity with saccades intrusions and opsoclonus, cerebellar ataxia, tremor with kinetic, postural and intention characteristics and myoclonus. He was started on acyclovir. Brain MRI was normal. CSF with pleocytosis and high protein. CBC had leukocytosis with lymphocytosis. HIV, HBsAg, VDRL and HCV serologies were negative. Cytomegalovirus (CMV) IgG serology was positive and IgM negative. HSV was positive for IgM and IgG. Opsoclonus Myoclonus Ataxia Syndrome (OMS) diagnosis was given. Methylprednisolone was given, with significant clinical improvement. The case presented all four symptoms of the syndrome: opsoclonus, myoclonus of trunk and extremities, step ataxic, and behavioral change. In adults, there is a strong paraneoplasic association, in particular with small cell lung carcinomas, breast and ovarian neoplasia, leading us to perform CT screening of chest, abdomen and pelvis, all negative. Brain MRI was made, also normal. Also relevant in adults are infections, being the most related EBV, CMV, HCV, HIV, HSV, post-streptococcal. In our case, the onset was compatible with infectious mononucleosis. The presence of IgM and IgG antibodies for HSV is probably a cross reaction with EBV. Echocardiogram shown a mild pericardial effusion. A remarkable characteristic is the seizure. We didn't find any case report of OMS in literature associated with tonic-clonic seizure. The investigation of the patient rule out encephalitis. Our explanation for the seizure was a cortical disfunction without patterns of encephalitis. Thus, our case report is the unique to literature with this specific type of presentation. OMS is a rare entity with difficult diagnosis. Recurrence is common, and there are few studies on prognosis of this population. In our case, a detailed history and neurological examination was crucial to suspect OMS, using opsoclonus as an important feature to accurate diagnosing. We ruled out tumors and stabilished early treatment, resulting in good clinical improvement.

Palavras Chave

OPSOCLONUS-MYOCLONUS-ATAXIA SYNDROME, EPSTEIN BARR VIRUS

Área

Neurointensivismo