Abstract
Described in 1962, the opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare, neurologically debilitating disorder with distinct characteristics that may begin in childhood or adult life. Although many cases remain without etiological diagnosis, others are related to neoplasms and infectious diseases. We report a 41-year-old previously healthy male with an 8-day history of headache, vertigo, nausea, vomiting, and nystagmus. After a normal brain computed tomography and lymphocytic pleocytosis in cerebral spinal fluid (CSF), intravenous acyclovir therapy was initiated in the emergency room. On the third day of hospitalization, the diagnosis of OMAS was made based on the presence of chaotic and irregular eye movements, dysarthric speech, gait instability, generalized tremor, and myoclonic jerks. In the face of his neurological worsening, ampicillin followed by nonspecific immunotherapy (methylprednisolone and intravenous immunoglobulin) was prescribed, with mild clinical improvement. After a thorough laboratory workup, the definite diagnosis of neuroborreliosis was established and ceftriaxone (4 g/daily/3 wks) and doxycycline (200 mg/day/2 mo) was administered. Toward the end of the ceftriaxone regimen, the neurologic signs substantially improved. We believe this to be the first case description of OMAS as clinical presentation of Brazilian Lyme disease-like syndrome (Baggio-Yoshinari syndrome).
Highlights
Described in 1962, the opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare, neurologically debilitating disorder with distinct characteristics that may begin in childhood or adult life
A new cerebral spinal fluid (CSF) test confirmed the lymphocytic pleocytosis with 210 cells/mm[3] (99% lymphocytes, 1% neutrophils), protein was 84.7 mg/dL, glucose was 51 mg/dL, lactate and chloride values were within normal limits, adenosine deaminase was 3.05 UI/L (RV: 9 UI/L)
Methylprednisolone (1g/day/5 days) regimen was administered followed by a 5-day course of intravenous immunoglobulin (400 mg/kg/ day) with mild clinical improvement
Summary
Described in 1962, the opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare, neurologically debilitating disorder with distinct characteristics that may begin in childhood or adult life. On the third day of hospitalization, despite the maintenance of normal mental status, the patient presented involuntary, fast, arrhythmic, conjugated, multidirectional eye movements preventing him from fixing his gaze (opsoclonus) [video] He developed generalized rhythmic resting and action tremors associated with myoclonic jerks at postural fixation, and volitional limb movements, in the right side. The patient was unable to stand and walk, and became completely dependent for daily activities, such as eating, brushing teeth, shaving, and bathing Based on these neurological signs, the diagnosis of opsoclonus-myoclonus-ataxia syndrome (OMAS) was raised and a wide diagnostic workup was carried out to identify unusual infectious agents and immune dysfunction, the more common cause of this syndrome in adults. Methylprednisolone (1g/day/5 days) regimen was administered followed by a 5-day course of intravenous immunoglobulin (400 mg/kg/ day) with mild clinical improvement
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