A Case of Acute Encephalopathy & Ataxia

From Dr. J Maestre

Admission

Discharge

2 weeks ago I've attended in ward a 7 yo boy who 5 days before admission had a non-specific upper tract respiratory infection (non-productive cough and fever). This "cold" lasted no more than 2 days. Two days before admission he began with inexplicable falls, and his gait seemed progressively clumsy; his parents brought him to Ped. ER because the child had become sleepy and apathetic. Peds. did consult us.

On physical examen, he looked like a healthy boy, although was drowsy and irritable, non cooperative then. The only positive findings on somatic examen were a brisk DTRs, equivocal plantar responses, mild finger-nose and heel-knee dysmetria, and truncal and gait ataxia. He was born from a induced labour, due to his mother' pre-eclamptic status, with the aid of forceps. He has been slow to walk alone, is a bit clumsy with his hands, is not a brilliant pupil at school, but otherwise is happy and healhty (chickenpox at age of 3). There is no family antecedents of neurological disease.

Tests:

-blood analysis: normal (including lactate and pyruvate)

-Chest Rx: normal

-CT scan: to be showed

-MRI: to be showed

-CSF: cell count, proteins and glucose, normal

-EEG: generalized high voltage delta activity (1-2 Hz)

Treatment: high dose IV steroids for 3 days, followed by slowly tapered oral prednisone.  On third day in hospital, the child was fully alert and cooperative. He was still ataxic. Two days later, he was almost asymptomatic. One week after admission his DTRs were still brisk, his plantar responses were flexor, EEG was normal, and he was discharged home jumping for joy. A second MRI -will be also shown- was performed just before he left running (literally) to home.

Could anyone guess what findings are there, and put forward any diagnosis?

Jose (Pepe) Maestre