A Rare Case of CIDP Associated with Autoimmune Hepatitis

Introduction: Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is an immune-mediated
(molecular mimicry) Peripheral nerve demyelinating disorder. It has been associated with anti-ganglioside antibodies and rarely Lupus, Sjogren’s syndrome, etc. Only one case report shows autoimmune hepatitis associated with CIDP and another with Acute Inflammatory Demyelinating Polyradiculoneuropathy. We present an extremely rare case of CIDP associated with autoimmune liver disease. Objective: To report on a patient with autoimmune hepatitis who developed CIDP. Methods: Retrospective review of patient medical records. Clinical Data: This is a 34-year-old female with history of ethanol abuse and cirrhosis who initially presented with acute (2 weeks) on chronic (2-3 years) bilateral lower extremity progressive numbness and weakness. Initial exam showed proximal and distal lower extremity weakness and hyperesthesia. She was bedridden but her arms were strong. Lab showed normal Cerebrospinal fluid study, increased bilirubin and elevated liver enzymes, elevated anti-smooth muscle antibody, elevated erythrocyte sedimentation rate and, hypergammaglobulinemia. Nerve conduction studies (NCS) suggested axonal neuropathy, and length dependent motor peripheral neuropathy. Sural nerve biopsy suggested loss of large and medium-sized myelinated axons. Thereafter, patient developed upper extremities weakness. Repeat NCS showed conduction blocks. The diagnosis of CIDP was made. Patient was started on IVIG, prednisone, and CellCept. Liver biopsy showed steatohepatitis and atypical hepatocytes. Two weeks after starting the treatment, her liver panel normalized. Patient stayed in rehab for a few months, continued on previously mentioned treatment, and had significant motor improvement. However, after discharge, for one year patient stopped follow-up and treatment. During which she worsened clinically and had deranged liver function. Conclusion: Although rare, In-line with the aforementioned case reports, our patient suggests anti-smooth muscle antibody could be associated with CIDP. The timing of the NCS can be crucial to determine the nature of the neuropathy.