Abstracts

Rationale: Steroid Responsive Encephalopathy associated with Autoimmune Thyroiditis (SREAT), also termed Hashimoto encephalopathy (HE), is a rare immune-mediated clinically heterogeneous disease known to affect all age groups including geriatric population and is more common among females. It consists of encephalopathy, epileptic seizures, cognitive dysfunction and variable neuropsychiatric manifestations. Therapeutic options include steroid therapy; prognosis is variable ranging from seizure freedom with complete recovery, relapsing-remitting course to long-term cognitive deficits. We present a case of HE causing cluster of atypical automotor epileptic seizures. To the best of our knowledge, such a complex semiology of “Ictal scream” with visual hallucinations, urinary and fecal urge incontinence has never been reported before.Methods: 66 years old right handed Vietnamese female with past medical history of hypertension and hypothyroidism presented with altered mental status over 4 weeks and new onset diabetes mellitus DM (Blood glucose 600, hemoglobin A1C 13.6). Patient has been having episodes of screaming incomprehensible words followed by mouth and hand automatisms lasting up to 15 seconds, also had visual hallucinations of seeing people standing in the room, occasional fecal and urinary urge incontinence.Results: On exam interictally she was oriented to self only, withdrawn, afebrile, vitals stable, comprehensive workup for traumatic, toxic-metabolic, neoplastic, autoimmune disorders including SLE, infectious conditions like HIV, CJD were negative except for anthyroid antibodies. Pink appearing CSF showed lyphocytic pleocytosis, elevated protein 90, glucose 107, neucleated cells 67, RBC 29000, xanthochromia, negative for oligoclonal bands and serum showed elevated antimicrosomal antibodies (507) and antithyroglobulin antibodies (77), TSH (5), normal FT4, FT3 and prolactin 7.1. Also had hyponatremia corrected for hyperglycemia (123), hypokalemia 3.3, Lactate 6.6. MRI brain with without contrast, MRA head without contrast and EEG findings are attached as images. A diagnosis of SREAT was established, and the patient improved rapidly on prednisone, levothyroxine and valproate therapy. DM was treated with insulin and electrolytes replenished. Patient had reduced ictal episodes from multiple times a day to 2-3 times a week and after nearly 8 months she is seizure free with return to baseline mental status.Conclusions: Early diagnosis and treatment of SREAT with immune suppression is crucial and can lead to rapid clinical improvement. In steroid non-responders other immunomodulatory therapies like IVIG or plasmapheresis should be tried. The pathophysiology of this disorder is unclear but neuroinflammation and electrolyte disorders as highlighted in this case could be potential explanations. Atypical presentations of this rare disorder should be reported to enrich the literature and enhance the understanding of this complex disease. Reference: Epilepsy Associated with Systemic Autoimmune Disorders, O Devinsky et al. Epilepsy Curr. 2013 Mar-April