We report the case of a 30-month-old girl who came to the emergency department with mild gait instability and intentional tremor in the last two days.
The parents reported that a mild generalized rash without fever had appeared three weeks before, which resolved spontaneously in a few days.
Her personal history included no known allergies or previous trauma and her immunization schedule was updated, including chickenpox.
His psychomotor development was normal.
It was reevaluated 48 hours later, highlighting a worsening of ataxia that prevented standing, associated with severe irritation and irregular sleep.
Physical examination at admission showed good nutritional status and no meningeal signs, external signs of trauma or other pathological findings were found.
In the neurological examination, emphasis was placed on intentional speech but intelligible and appropriate to the context, ataxia with cerebellar characteristics, and intense and generalized tremor.
The cranial nerves, strength, muscle tone and osteotendinous reflexes were normal and symmetrical.
Upon admission, brain electroencephalogram (EEG) and computed tomography (CT) with contrast enhanced echocardiography were performed and revealed no electroencephalographic or structural abnormalities.
Urine toxics were negative.
Lumbar puncture showed a mild lymphorrhachia with biochemistry and negative cultures, including Herpes group, neurotropic virus and Borrelia.
Serology for Herpes simplex virus, Epstein-acquired, Varicella-zoster virus, enterovirus, Salmonella sp., Streptococcus pyogenes and Borrelia burgdoferi did not provide pathological findings
On the fifth day after the onset of symptoms, the patient presented intentional tremor and ataxia with a predominance of severe troncal ataxia that made sitting difficult.
The tremor was aggravated by probable intentional reinforcement myoclonus and the irritability was extreme.
There were rapid, saccade, multidirectional, chaotic, but conjugated eye movements, which were also appreciated with closed eyes, and which diminished when the eye was fixed.
The clinical diagnosis of opsoclonus (SOMA) began searching for a possible tumor.
An abdominal ultrasound was requested, with normal results, and a simple chest X-ray showed a left thoracic mass.
A CT scan showed a left intrathoracic mass consistent with TCN, confirmed after surgery as a localized ganglioneuroblastoma.
A 24-hour urine specific neuronal enolase and catechiae were also requested, which were normal.
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Opsoclonus decreased after the start of high-dose oral prednisone (2 mg/kg/day), and 15 days after surgery was no longer observed.
Ataxia and irritation improved markedly, but one year later treatment with oral prednisone at minimum daily doses for relapses of mild gait instability and irritation is still needed, with no evidence of tumor recurrence to date.
Psychomotor development continues to progress within normal limits.
