Abstracts

Rationale: There is a known duality of GAD 65 being associated with both stiff-person syndrome and the pathogenesis in adult onset epilepsy. We describe a case about a patient with a history of positive GAD-65 antibody and stiff-person syndrome (SPS), subsequent development of mesial temporal sclerosis (MTS) with intractable epilepsy who had resolution of one of two distinctive seizure types with partial temporal lobectomy.  Methods: Case report at Mayo Clinic Arizona and review of literature. Results: The patient is a 37 year old male who initially presented with vertigo eleven years ago. His symptoms then progressed to involve right sided ataxia, spasticity, hemiparesis, progressive cognitive decline and eventual discovery of subclinical seizures on EEG localizing to the right hemisphere. Based on his clinical picture and detection of GAD 65 antibodies he was diagnosed with stiff-person syndrome. He was subsequently monitored in the EMU for two spell types: a panic spell and an amnestic spell type. The EMU was able to capture focal seizures from the right temporal lobe. Additionally, MRI brain showed right sided MTS. Therefore a partial right temporal lobectomy was performed with complete resolution of the panic seizure type captured in the EMU admission. Unfortunately, the amnestic events have remained medically refractory. Conclusions: Stiff-person syndrome is a rare disorder typically characterized by rigidity, progressive muscle stiffness, and spasticity of axial muscles. Although the pathogenesis of the disease remains unclear, there has been a positive association between positive antibodies to glutamic acid decarboxylase (GAD-65). Classically patients with SPS do not have any specific neuroimaging findings. However, occasionally this syndrome can be seen in cases of adult onset epilepsy. There have been few reported cases of positive GAD-65 antibodies and epilepsy with hippocampal lesions and mesial temporal sclerosis. Also, there is an established association between positive GAD antibodies and epilepsy. We believe that perhaps autoimmunity with positive GAD-65 antibodies can place one at risk of developing mesial temporal sclerosis. However, although we removed the pathogenic tissue the effect of GAD-65 antibodies seem to be broader, since he still continues to have refractory epilepsy.  Funding: No funding was received in support of this abstract.