Abstracts

Rationale: This case report explores a rare epilepsy trigger in a patient with multiple positive antibodies associated with multiple neurologic conditions. Methods: Case Report at Dartmouth-Hitchcock Medical Center and Review of the English Language Literature. Results: This is a 39 year old woman who first presented with seizures at the age of 19 years.  She has focal left and right temporal seizures with loss of awareness, occasional secondary generalization. Triggers include stress, menstruation, and certain types of music, such as songs by the singer Norah Jones. Other symptoms include chronic migraine, bilateral lower extremity numbness, left shoulder girdle and back muscle stiffness, pain, and spasms, as well as intermittent diplopia and generalized weakness that worsens with exertion. Neurologic exam reveals mild diffuse weakness, increased tone throughout (left greater than right), mildly impaired coordination bilaterally (left worse than right), and hyperreflexia on the left. She has sensory findings consistent with a diffuse peripheral neuropathy. Her gait is stiff, ataxic and antalgic.MRI brain initially showed a T2/FLAIR signal abnormality in the left hippocampus that resolved on follow-up scans.  An interictal SPECT scan showed hypoperfusion on the right. MRIs have also shown a stable linear T2/FLAIR signal alteration within the superior left cerebellum of unclear significance that has persisted over 20 years. Video-EEG monitoring, nearly 20 years after initial onset, demonstrated independent bitemporal seizure origins. CSF analyses have repeatedly shown mildly elevated protein levels. Serum and CSF paraneoplastic autoimmune studies have been positive for antibodies against glutamic acid decarboxylase-65 (GAD65) in serum and CSF, thyroperoxidase in serum, and acetylcholine receptor (ACh-R) binding antibodies and ACh-R modulating antibodies in serum. See Table 1 for summary. MRI and CT scans of her chest, abdomen and pelvis, gynecological ultrasounds, and GI endoscopies have repeatedly shown no evidence of underlying malignancy.Her seizures have proven to be medically refractory to management with multiple classes of antiepileptic drugs, and she has been treated with combinations of immunosuppressant therapies including intravenous immunoglobulin, azathioprine, corticosteroids, rituximab, and cyclophosphamide. Her clinical syndrome is consistent with post-inflammatory limbic encephalitis with Stiff Person Syndrome and possible Myasthenia Gravis. Conclusions: This case report supports previously reported findings that musicogenic reflex epilepsy arises from the temporal lobe (Tayah et al. Epilepsia. 2006 47(8):1402-6) and may be associated with multiple paraneoplastic autoimmune antibodies, specifically those against GAD65 (Falip et al. Acta Neurol Scand. 2018 137(2):272-276), thyroperoxidase, and ACh-Rs, with no evidence of a primary malignancy. Patients presenting with musicogenic seizures should be evaluated for the presence of paraneoplastic autoimmune antibodies so as not to delay potential treatment with immunosuppressant therapy. Funding: None