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J Neurol Sci. 2014 Aug 15;343(1-2):192-4. doi: 10.1016/j.jns.2014.05.032. Epub 2014 May 27.

Immunotherapy-responsive limbic encephalitis with antibodies to glutamic acid decarboxylase.

Author information

1
Department of Neurology, "St. Panteleimon" General State Hospital, 3 Mantouvalou St., 18454 Nikaia, Greece. Electronic address: jmarkak2@otenet.gr.
2
Neuroimmunology Unit, Department of Pathophysiology, Medical School, University of Athens, 75 Mikras Asias St., 11527 Athens Greece. Electronic address: halexo@gmail.com.
3
Laboratory of Experimental Neurophysiology, Department of Neurology, Medical School, University of Athens, 72-74 Vas. Sophias Ave., 115 28 Athens, Greece. Electronic address: cpoulop@med.uoa.gr.
4
Neuroimmunology Unit, Department of Pathophysiology, Medical School, University of Athens, 75 Mikras Asias St., 11527 Athens Greece. Electronic address: akrivousof@gmail.com.
5
Department of Neurology, Essex Centre for Neurological Sciences, Queen's Hospital, Romford, Essex, UK. Electronic address: tpapathanasiou@gmail.com.
6
Department of Radiology, "St. Panteleimon" General State Hospital, 3 Mantouvalou St., 18454 Nikaia, Greece. Electronic address: vaso@otenet.gr.
7
Department of Neurology, "St. Panteleimon" General State Hospital, 3 Mantouvalou St., 18454 Nikaia, Greece. Electronic address: geolyr@hotmail.com.
8
Department of Neurology, "St. Panteleimon" General State Hospital, 3 Mantouvalou St., 18454 Nikaia, Greece. Electronic address: g_gkekas@hotmail.com.
9
Neuroimmunology Unit, Department of Pathophysiology, Medical School, University of Athens, 75 Mikras Asias St., 11527 Athens Greece. Electronic address: mdalakas@med.uoa.gr.

Abstract

Glutamic acid decarboxylase (GAD) has been recently identified as a target of humoral autoimmunity in a small subgroup of patients with non-paraneoplastic limbic encephalitis (NPLE). We present a patient with NPLE and positive anti-GAD antibodies who showed significant improvement after long-term immunotherapy. A 48-year old female was admitted with a two-year history of anterograde amnesia and seizures. Brain MRI revealed bilateral lesions of medial temporal lobes. Screening for anti-neuronal antibodies showed high anti-GAD titers in both serum and cerebrospinal fluid (CSF) with strong evidence of intrathecal production. The patient received treatment with prednisolone and long-term plasma exchange. During a 12-month follow-up, she exhibited complete seizure remission and an improvement in memory and visuo-spatial skills. Anti-GAD antibodies may serve as a useful marker to identify a subset of NPLE patients that respond to immunoregulatory treatment.

KEYWORDS:

Autoimmune; Glutamic acid decarboxylase; Limbic encephalopathy; Plasma exchange; Steroids; Treatment

PMID:
24950904
DOI:
10.1016/j.jns.2014.05.032
[Indexed for MEDLINE]

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