Corticobasal syndrome like presentation of Hashimoto encephalopathy with cortical ribboning

Abstract

Abstract Hashimoto encephalopathy (HE) and corticobasal syndrome (CBS) are heterogeneous disorders characterized by variable presentation; seizure, stroke like symptoms, myoclonus, alien limb phenomena, dystonia, limb apraxia, although extrapyramidal features and alien limb phenomena are rare in HE. Here we are reporting a 70 years female who presented with 3 months history of asymmetrical onset rapidly progressive disease associated with alien limb behaviour, myoclonic jerks, dystonia, seizure, speech impairment, asymmetrical extrapyramidal features (left > right), gait ataxia and cortical ribboning on brain imaging without visual disturbances, cognitive decline or neuropsychiatric abnormalities. EEG did not show any periodic discharges or slowing. Neuronal antibody VGKC was negative but she had euthyroid state with high titre of anti TPO antibody 590 IU/ml (normal upto 95 IU/ml). Therefore, diagnosis of Hashimoto encephalopathy in a probable sporadic case of corticobasal syndrome was made. Patient was treated with antiepileptic and IV steroid for 5 days. At the time of discharge she was conscious and followed verbal commands. In every case of rapidly progressive neurodegenerative disease like CBS, investigations for HE should be planned.