S2777 A Rare Case of Progressive Encephalomyelitis With Rigidity and Myoclonus Related to Hepatitis E Virus

Abstract

Introduction: We present an unusual case of progressive encephalomyelitis with rigidity and myoclonus (PERM) caused by hepatitis E viral illness. Case Description/Methods: A 42-year-old male with a past medical history of left nephrectomy donation, hypertension, and chronic kidney disease presents with sudden onset of numbness of his tongue. He complains of intermittent dizziness (positional), hearing changes (including voices and music) as well as double/blurry vision, global fatigue, and visual hallucinations. The patient denies any extremity weakness, difficulty swallowing, or headache. No facial droop is noted, but right eye droop is noted. The patient is ambulating with a steady gait. The patient has elevated liver enzymes (AST 178, ALT 616) with elevated alkaline phosphatase (ALP 323). The patient has no recent exposures or ingestion. He is diagnosed with PERM following the onset of symptoms managed with high-dose steroid therapy and plasmapheresis. Symptoms persisted with dysphagia requiring feeding tube placement, tracheostomy, along with progressively worsening transaminase levels with ALP 742, AST 342, ALT 1,062. Hepatology was consulted with negative autoimmune work-up, hepatitis B and C screening. Hepatitis E IgM was obtained and found to be positive. A liver biopsy was obtained showing mild portal tract inflammation with ductal reaction, focal pericentral macrophages, and hepatocyte dropout reflective of viral hepatitis. He was recommended to start ribavirin 200 mg twice daily given persistently elevated transaminase levels. Repeat evaluation with HEV testing after two months of therapy was negative. Treatment for the PERM included steroids, intravenous immunoglobulin, plasmapheresis, and rituximab with improvement in symptoms. Most recent liver enzymes were normal on ribavirin treatment. Discussion: PERM is a rare disorder characterized by muscular rigidity, muscle spasms, abnormal posture, and is caused by inflammation in the brainstem and spinal cord. PERM can be diagnosed clinically or if the patient is positive for anti-HEV IgM and has symptomatology characteristic of stiff-person plus syndrome. Symptoms can be explained by the interruption of glycinergic synaptic transmission in the spinal cord and brainstem. Antibodies present against glycine receptors in the serum and CSF suggest antibody-mediated pathogenesis, possibly responsive to immunotherapy. Ribavirin is commonly prescribed for HEV clearance and amelioration of HEV-associated neurologic symptoms.