Hashimoto's encephalopathy in association with retinitis pigmentosa - First reported case

Hashimoto's Encephalopathy (HE), also known as steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is a proteiform disorder known for its wide spectrum of presentations from subtle neuropsychiatric manifestations, movement disorders, seizures, stroke-like episodes to coma. Here, we report a case of HE which initially masqueraded as bipolar affective disorder (BPAD) and ultimately progressed to generalized tonic clonic seizures and coma. Although SREAT is characterized by exquisite responsive to steroid, in our case it was unresponsive to pulse methylprednisolone therapy. Rapid recovery was noted with intravenous immunoglobulin (IVIG) therapy. This case was also peculiar for its association with non-sydnromic retinitis pigmetosa (RP). To the best of our knowledge, this was the first reported case of HE which was associated with RP.

Keywords: Hashimoto's Encephalopathy; intravenous immunoglobulin; retinitis pigmentosa; thyroid.