A 46-year-old man had an episode of loss of awareness while driving home. He was found in a cul de sac by his neighbor and was acting confused. He was brought to the emergency department; while there, he started having recurrent episodes of goose bumps (goose flesh) involving half of his body associated with a “wavelike” sensation that would typically begin in the lower extremities and spread upward. He also had some speech difficulty. Physical and neurologic examinations were unremarkable, except for mild cognitive deficits.
Video electroencephalographic monitoring showed frequent independent left and right temporal ictal and interictal discharges. Magnetic resonance imaging of the brain showed fluid-attenuated inversion recovery hyperintensity in the bilateral hippocampi. Cerebrospinal fluid analysis showed a mildly increased total protein concentration but no supernumerary oligoclonal bands and normal nucleated cell count and immunoglobulin G index. Serum autoimmune epilepsy evaluation was remarkable for leucine-rich, glioma-inactivated protein 1-immunoglobulin G seropositivity.
The patient was diagnosed with leucine-rich, glioma-inactivated protein 1- immunoglobulin G antibody–associated autoimmune seizures presenting with pilomotor seizures.
Before autoimmune work-up, the patient had been treated with a gradual escalation of antiseizure medications. The seizures continued. He was subsequently started on intravenous methylprednisolone. He was seizure free and was transitioned to an oral prednisone taper. He was subsequently started on a 6-week regimen of intravenous immunoglobulin. He was also started on mycophenolate mofetil.
On follow-up clinic visits, the patient had no recurrence of seizures and disclosed no cognitive dysfunction except for mild inattention. Three years after the patient’s initial episode, the antiseizure medications and mycophenolate mofetil were gradually tapered, without recurrence.
Leucine-rich, glioma-inactivated protein 1-immunoglobulin G–associated autoimmunity is typically seen among older patients (>50 years), more commonly men. Symptoms commonly include seizures and cognitive dysfunction, presenting as memory loss and disorientation.
Beneficial response to mycophenolate mofetil by patients with autoimmune hepatitis who have failed standard therapy, is predicted by older age and lower immunoglobulin G and INR levels
