Autoimmune limbic encephalitis presenting with fasciobrachial dystonic seizures: a case report

Address for correspondence: Alana Bacelar Limeira Sales (email: [e-mail]).

Abstract

Case Presentation: We present the case of a 64-year-old woman, with essential hypertension, referred to our hospital for investigation of refractory epilepsy. She had a history of prolonged inpatient treatment in another hospital due to several complications of her state: bronchoaspiration, pneumonia, and hyponatremia of hard control. The patient had already received a loading dose of phenobarbital followed by phenytoin due to long-duration seizures. Upon admission, the patient exhibited decreased mental status, diurnal excessive somnolence, episodes of hyponatremia, and seizures characterized by fasciobrachial dystonias.

Discussion: While the seizures were initially controlled, the patient progressed to a non-convulsive status epilepticus, necessitating a loading dose of levetiracetam for management. Suspecting autoimmune encephalitis, we conducted thorough laboratory investigations and a lumbar puncture, initially yielding no remarkable results. Consequently, the patient received pulse therapy with methylprednisolone (1 g daily for 5 days), followed by maintenance treatment with prednisone (50 mg daily). Subsequent improvement in the patient’s condition was observed, with resolution of seizures and hyponatremia, and gradual improvement in mental status. Analysis of the autoimmune panel of the cerebrospinal fluid revealed positivity for anti-LGI1 antibodies, confirming the diagnosis of autoimmune limbic encephalitis. Autoimmune encephalitis, particularly the anti-LGI1 subtype, is often underdiagnosed despite its significant clinical manifestations, including seizures, hyponatremia, and fasciobrachial dystonic seizures. The treatment primarily involves pulsed corticosteroid therapy followed by maintenance with prednisone. Our patient exhibited an excellent response to corticosteroids, allowing for gradual tapering of prednisone dosage.

Final Comments: This case underscores the importance of considering autoimmune encephalitis in patients presenting with refractory seizures and associated clinical features. Prompt diagnosis and initiation of appropriate treatment are essential for favorable outcomes in these patients.

 

MissingLinkText

Die Autoren geben an, dass kein Interessenkonflikt besteht.

Publikationsverlauf

Artikel online veröffentlicht:
02. Oktober 2024