A Survival Case of Super-refractory Status Epilepticus due to Glutamic Acid Decarboxylase Antibodies-associated Limbic Encephalitis

Abstract

Limbic encephalitis (LE) is a neurological syndrome that mainly affects mesial temporal lobes. It may present in association with cancer or infection. Limbic encephalitis associated with glutamic acid decarboxylase antibodies (anti-GAD) is rare. Here, we report a case of anti-GAD limbic encephalitis to heighten the awareness of this rare cause of autoimmune encephalitis. Anti-GAD-associated epilepsy is often poorly responsive to seizure medications. Treatment is challenging. Early initiation of immunotherapy is important.

Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect

“…The latter two are mainly related to amphiphysin and glycine receptor antibodies, respectively [ 138 ]. Seizures occurred in almost all patients with GAD-related LE, may present as status epilepticus, and were difficult to control even with immunotherapy [ 139 , 140 , 141 ].…”

New Onset Refractory Status Epilepticus in an Adult Patient with Autoimmune Encephalitis Responding to Vitamin B6 Pyridoxine

“…Autoimmune encephalitis, either nonparaneoplastic or paraneoplastic, was the most common reported cause of NORSE [4,[19][20][21][22] and anti-NMDAR encephalitis cases presenting as NORSE are reported [23,24] knowing that most published cases of NORSE predate the discovery of anti-NMDAR antibodies [11,25].Here, we present a case of NORSE with underlying nonparaneoplastic autoimmune encephalitis etiology (diagnosed by elevated protein in the CSF with pleocytosis, positive CSF anti-NMDAR antibodies and by brain MRI findings) in a previously healthy young male. Although general expert consensus in the literature recommends approaching NORSE with pharmacologic-induced coma and continuous infusion of IV anesthetic agents, to suppress brain activity and preserve normal brain physiology [26], our patient did not respond and his NORSE became super-refractory.…”

Intravenous methylprednisolone or immunoglobulin for anti-glutamic acid decarboxylase 65 antibody autoimmune encephalitis: which is better?

“…We eliminated many references for the following reasons. First, we excluded studies for which it was difficult to assess the efficacy of methylprednisolone, immunoglobulin or a combination of methylprednisolone and immunoglobulin because patients received immunosuppressive therapy in addition to IVMP or IVIG meanwhile, such as plasma exchange [14][15][16], immunoadsorption [17], azathioprine [18], rituximab [19], mycophenolate mofetil [20] or others [21]. Second, we excluded studies for which we could not obtain the patients' response to IVMP or IVIG or their combination because of the ambiguous descriptions or the inaccessible follow-up information in the original literature [22][23][24][25][26][27].…”