Rare Association of Autoimmune Limbic Encephalitis and Stiff Person Syndrome

Abdelmoniem Moustafa; Mohd Amer Alsamman; Denise Fernandes

Rare Association of Autoimmune Limbic Encephalitis and Stiff Person Syndrome

R I Med J (2013). 2023 Jul 5;106(6):7-9.

Authors

Abdelmoniem Moustafa¹, Mohd Amer Alsamman², Denise Fernandes³

Affiliations

¹ Division of Cardiovascular Medicine, University of Toledo, Toledo, OH.
² Division of Gastroenterology, Georgetown University, Washington, D.C.
³ Division of Hospital Medicine, The Miriam Hospital, The Warren Alpert Medical School of Brown University, Providence, RI.

PMID: 37368825

Abstract

Antibodies to Glutamic Acid Decarboxylase (GAD) have been implicated in the pathogenesis of both autoimmune Limbic Encephalitis (LE) and Stiff Person Syndrome (SPS). However, their association is quite rare. We present a case of a 48-year-old Caucasian female who presented with symptoms of recurrent severe headaches, behavioral and cognitive dysfunction, and an episode of seizure. She was found to have high titers of anti-GAD65 antibodies in both cerebrospinal fluid and serum. She was diagnosed with LE and SPS, and was started on immunosuppressive therapy with steroids and intravenous immunoglobulins (IVIG). The patient responded well to treatment with improvement in her symptoms.

Keywords: Limbic encephalitis; GAD 65; LE; SPS; Stiff person syndrome.

Publication types

Case Reports

MeSH terms

Autoantibodies / cerebrospinal fluid
Autoimmune Diseases* / complications
Autoimmune Diseases* / diagnosis
Autoimmune Diseases* / drug therapy
Female
Headache
Humans
Limbic Encephalitis* / complications
Limbic Encephalitis* / diagnosis
Limbic Encephalitis* / therapy
Middle Aged
Stiff-Person Syndrome* / complications
Stiff-Person Syndrome* / diagnosis
Stiff-Person Syndrome* / drug therapy

Substances

Autoantibodies

Supplementary concepts

Autoimmune limbic encephalitis