Objective: To describe a young man with post encephalitc dystonia-ataxia with transient worsening following a febrile episode

Background: We had a 19 year old young man, who had a febrile encephalitic illness at 8 years of age. He had a prolonged course of hospitalization due to unresponsive state, not moving his limbs, was on nasogastric tube feeding and was incontinent. He recovered slowly over the next 2 months.

Method: After this recovery, the mother noted neck twisting, shaking and posturing associated with difficulty in picking up objects or using his upper limbs for other activities of daily living due to the tremors and dystonia. The patient’s symptoms were static till 10 years later, when the patient had a short acute febrile illness when his symptoms worsened, and then over the next week returned back to baseline.

Results: The MR imaging of the brain revealed symmetric central thalamic hyperintensities on T2/FLAIR images with no diffusion restriction or mineralization. The imaging was consistent with a post Japanese encephalitis sequelae. The history review revealed that there was an epidemic in their village with other children being affected as well during the same time as our patient’s childhood encephalitic illness.

Conclusion: Japanese encephalitis is commonly associated with thalamic lesions on imaging and can lead to post encephalitic parkinsonism. Thalamic lesions can also cause hyperkinetic movement disorders, but segmental dystonia-ataxia involving the bi-brachial-cervical distribution is rare.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/post-encephalitic-dystonia-ataxia-syndrome/