In its classic presentation, Hashimoto's encephalopathy is an acute-subacute complex neuropsychiatric syndrome with cognitive impairment, hallucinations, myoclonus, tremor or ataxia, associated with elevated anti-thyroid antibodies. Corticoids and immunotherapy are dramatically effective. However, in some cases, not all the associated features are presented and this delays diagnosis and appropriate treatment. We describe a man with abrupt onset of recurrent auditory seizures resulting in refractory non-convulsive status epilepticus. The patient was diagnosed with an autoimmune encephalopathy with elevated serum and CSF anti-thyroid antibodies. None of the antiepileptic drugs were successful, however, following immune-modulating therapy, the refractory non-convulsive status epilepticus dramatically improved, as did the patient overall. We suggest that Hashimoto's encephalopathy should be suspected in otherwise healthy patients with unexplained new-onset focal recurrent auditory seizures which do not respond to antiepileptic drugs. The presence of anti-thyroid antibodies in the CSF supports this diagnosis.

Auditory seizures in autoimmune epilepsy: a case with anti-thyroid antibodies

VECCHIO, DOMIZIA;MAGISTRELLI, Luca;CANTELLO, Roberto
2017-01-01

Abstract

In its classic presentation, Hashimoto's encephalopathy is an acute-subacute complex neuropsychiatric syndrome with cognitive impairment, hallucinations, myoclonus, tremor or ataxia, associated with elevated anti-thyroid antibodies. Corticoids and immunotherapy are dramatically effective. However, in some cases, not all the associated features are presented and this delays diagnosis and appropriate treatment. We describe a man with abrupt onset of recurrent auditory seizures resulting in refractory non-convulsive status epilepticus. The patient was diagnosed with an autoimmune encephalopathy with elevated serum and CSF anti-thyroid antibodies. None of the antiepileptic drugs were successful, however, following immune-modulating therapy, the refractory non-convulsive status epilepticus dramatically improved, as did the patient overall. We suggest that Hashimoto's encephalopathy should be suspected in otherwise healthy patients with unexplained new-onset focal recurrent auditory seizures which do not respond to antiepileptic drugs. The presence of anti-thyroid antibodies in the CSF supports this diagnosis.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11579/82562
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