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Case Report

Multiple autoimmune antibody limbic

encephalitis: a case in a pregnant woman

1
2
1
Meha Goyal , Kasey L. Gildersleeve , Stuart L. Tomko , Joseph S. Kass 2,3
1 Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA.
2 Department of Neurology, The University of Texas Health Science Center, Houston, TX 77030, USA.
3 Department of Neurology, Ben Taub General Hospital, Houston, TX 77030, USA.
ABSTRA CT

Autoimmune limbic encephalitis is most commonly associated with antibodies against the N‑methyl‑D‑aspartate receptor (NMDAR),
among other neuronal cell surface receptors. Here, a case of a pregnant female with limbic encephalitis in the presence of multiple
additional autoimmune antibodies is described. The patient was a 36‑year‑old female who presented with 4 days of confusion,
hallucinations, hypersexuality, disinhibition, and pressured speech. The patient’s work‑up detected the presence of anti‑NMDAR
antibodies, anti‑glutamic acid decarboxylase antibodies, and a yet uncharacterized neuronal autoantibody. The patient was also
found to be pregnant. No evidence of ovarian or other pelvic malignancy was discovered. Symptomatic control was achieved with
plasma exchange.
Key words: Autoimmune, encephalitis, glutamic acid decarboxylase, limbic, N‑methyl‑D‑aspartate

INTRODUCTION been described in association with late-onset cerebellar
ataxia, epilepsy, palatal tremor, and limbic encephalitis. [4-6]
Autoimmune limbic encephalitis is most However, the clinical significance of these antibodies
commonly associated with antibodies against the in the context of encephalitis has been questioned
N-methyl-D-aspartate receptor (NMDAR). Several given the small number of cases reported as well as the
[1]
other autoimmune antibodies have been implicated frequent co-presence of antibodies against cell surface
in autoimmune limbic encephalitis, but the existence antigens (e.g. GABA-BR, AMPAR, and NMDAR). [4-6]
of several of these antibodies in one patient has rarely Cases of anti-GAD receptor encephalitis described in
been described. Autoimmune limbic encephalitis the literature generally do not follow the clinical pattern
manifests as sub-acute onset of irritability, short-term of typical limbic encephalitis. Patients with anti-GAD
memory loss, depression, sleep disturbances, receptor encephalitis present with prominent memory loss,
hallucinations, seizures, and confusion. [1,2] Anti-NMDAR medically refractory seizures, and language difficulty.
[2]
encephalitis is the most common form, whereas other In addition, patients with autoimmune encephalitis can
antibodies described with this syndrome are directed also demonstrate seropositivity for other antibodies such
at amino-3-hydroxy-5-methyl-4-isoxazolepropionic as anti-nuclear antibody and anti-thyroperoxidase (TPO).
acid receptor (AMPAR), gamma-aminobutyric Immunomodulatory treatment remains the gold standard
acid beta receptor (GABA-BR), leucine-rich glioma of therapy, with first-line treatments consisting of
inactivated (LGI1) gene, and contactin-associated corticosteroids, intravenous immunoglobulin (IVIG), or
[3]
protein 2. Anti-glutamic acid decarboxylase (GAD)
antibodies, although most commonly associated with plasma exchange (PLEX); second-line treatment includes
[1]
autoimmune-mediated Stiff-Person syndrome, have also cyclophosphamide and rituximab. Here, we describe
a case of a pregnant female found to have anti-NMDAR
encephalitis with the presence of multiple autoimmune
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Website: CASE REPORT
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DOI: A 36-year-old female presented with 4 days of confusion,
10.4103/2347-8659.149423 hallucinations, hypersexuality, disinhibition, and
pressured speech. Initial evaluation showed a thyroid

Corresponding Author: Dr. Meha Goyal, Baylor College of Medicine, 7575 Kirby Drive 2303, Houston, TX 77030, USA.
E‑mail: mgoyal@bcm.edu

46 Neuroimmunol Neuroinflammation | Volume 2 | Issue 1 | January 15, 2015