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Hashimoto encephalopathy presenting as progressive myoclonus epilepsy syndrome
Ravindra Arya1, Vidhu Anand, Maya Chansoria
1Comprehensive Epilepsy Center, Division of Neurology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Summary
Hashimoto encephalopathy, an autoimmune thyroiditis, can cause epilepsy and myoclonus in children. Early diagnosis and treatment with steroids and anti-seizure medications lead to significant improvement.
Area of Science:
- Neurology
- Endocrinology
- Immunology
Background:
- Hashimoto encephalopathy (HE), also known as Steroid Responsive Encephalopathy with Autoimmune Thyroiditis (SREAT), is a rare autoimmune disorder.
- It is characterized by neurological dysfunction associated with autoimmune thyroid disease.
Observation:
- An 11-year-old girl presented with multi-focal myoclonus, generalized seizures, and behavioral changes.
- Laboratory investigations revealed sub-clinical hypothyroidism and elevated anti-thyroid peroxidase antibodies.
- Brain MRI showed mild cerebral cortical grey matter atrophy, and EEG displayed intermittent slowing with generalized poly-spike-and-wave discharges.
Findings:
- The patient was diagnosed with Hashimoto encephalopathy based on clinical and laboratory findings.
- She demonstrated significant and sustained improvement with valproate, clonazepam, and long-term prednisolone treatment.
- Extensive investigations ruled out other causes of progressive myoclonus epilepsy (PME) syndrome, and no other autoimmune abnormalities were detected.
Implications:
- This case highlights the importance of considering Hashimoto encephalopathy in the differential diagnosis of progressive myoclonus epilepsy (PME) syndrome in adolescents.
- Prompt diagnosis and management of HE, particularly with immunosuppressive therapy, can lead to favorable neurological outcomes.
- Raising awareness among clinicians about this treatable neurological condition is crucial for timely intervention and improved patient prognosis.
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