Abstracts

Rationale: Status epilepticus is a neurologic emergency and standard protocols exist for rapid initiation of treatment and diagnostic studies. Directing the management of patients with status epilepticus that lasts for several weeks or even several months is less clear. Methods: We retrospectively reviewed seven patients who were treated for status epilepticus lasting longer than one month, with the approval of the Cleveland Clinic Internal Review Board. Results: All patients were previously healthy and presented with some prodromal symptoms prior to the onset of refractory status epilepticus. They were hospitalized in about 6 days (range of 2 to 13) and the first onset of seizure in about 10 days (range of 1 to 90) from the onset of prodrome. The average length of stay in the hospital was 161 days (median of 97, range of 44 to 450). All 7 patients developed focal status epilepticus with or without a variable degree of secondary generalization. The average duration of pharmacologic-induced come was 79.5 days (median of 77.5, range of 6 to 159). Only one patient received immunosuppressive therapy. The average CSF WBC was 135 cells/µl, with lymphocytic predominant (average 73.6%). None of these patients had an infectious organism identified. Brain MRI revealed diffuse atrophy in 66.7%, bilateral hippocampi T2 hyperintensity in 16.7%, unilateral (left) hippocampi atrophy in 16.7%, bilateral basal ganglia enhancement in 16.7%, and bilateral basal ganglia T2 hyperintensity in 16.7%. All 7 patients had a devastated clinical outcome, of which 3 were deceased from ICU complications. Of the remaining patients, they all have major neuropsychiatric sequelae, including cognitive decline, hallucinations, and emotion lability, and severely disabling ICU-related neuropathy and myopathy. Conclusions: We have identified seven cases of explosive onset epilepsy that evolved into chronic status epilepticus, the etiology of these cases is unclear, but is possibly immune mediated. EEG at presentation was focal in all cases reviewed and did not predict outcome. Repeat MRI scans revealed progressive atrophy and evolving T2/Flair hyperintensity in the limbic structures and basal ganglia. We found significant psychiatric, cognitive and physical morbidity associated with chronic status epilepticus. A standardized proposed protocol that utilizes bio-markers, testing for a panel of immune related substrates and serial neuro-imaging (including MRI, PET and SPECT) would help to understand this pathology further in a prospective study.