IVIG TREATMENT OF REFRACTORY EPILEPSY ASSOCIATED WITH STIFF-PERSON SYNDROME
Abstract number :
2.033
Submission category :
Year :
2003
Submission ID :
3828
Source :
www.aesnet.org
Presentation date :
12/6/2003 12:00:00 AM
Published date :
Dec 1, 2003, 06:00 AM
Authors :
Yafa Minazad, Said Beydoun, Laura A. Kalayjian, Christi Heck Neurology, Los Angeles County USC Medical Center, University of Southern California Keck School of Medicine, Los Angeles, CA
Intravenous immunoglobulin (IVIG) has been safely used to treat autoimmune diseases such as Guillain-Barre syndrome, Myasethenia gravis, as well as certain refractory epilepsy syndromes such as Rasmussen[rsquo]s encephalitis. Stiff-person syndrome (SPS) may be an autoimmune disease. Approximately 60% of patients with SPS have autoanitbodies to Glutamic Acid Decarboxylase (GAD). GAD is selectively concentrated in neurons secreting gamma amino butyric acid (GABA) and in the pancreatic Beta-cells. SPS is often associated with type I diabetes and less often with epilepsy. Those patients with both SPS and epilepsy are always positive for GAD antibodies. We report a case of SPS, type I diabetes, and refractory epilepsy treated with IVIG.
This is a case report.
NF is a 47-year old woman in excellent health until January 2002 when she experienced her first generalized tonic clonic seizure. She continued to have approximately one seizure a month despite treatment with phenytoin, carbamazepine, and Phenobarbital. She developed a progressive rash to these agents that eventually led to Steven[rsquo]s Johnson Syndrome. She was subsequently placed on gabapentin and clonazepam without benefit. EEG, MRI and MRS were normal. Four months after her epilepsy began, she developed axial rigidity and Type I diabetes. Serum anti-GAD levels were elevated and treatment with IVIG was initiated. She received a total of 2g/Kg in 4 divided doses over 5 days with monthly maintenance doses of IVIG. She has been seizure free for 6 months and has reduced axial rigidity. Anti-GAD antibodies were reduced from 82.9 (U/mL) to 22.4 (U/mL). She remains on insulin for her diabetes
This is the first case of SPS and epilepsy with anti-GAD antibodies successfully treated with IVIG. Olson (2002) reported a case of type I diabetes and epilepsia partialis continua with high anti-GAD antibody titers successfully treated with high-dose steroids, plasmapheresis, and IVIG. Anti-GAD antibodies can be seen in refractory localization-related epilepsy (Petola 2000). Our case further suggests that some refractory epilepsy may be autoimmune related and potentially treated with IVIG.