PO199 An autoimmune cranial and peripheral polyneuropathy with myositis

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Abstract

Introduction

An unusual case of autoimmune, relapsing-remitting, polyneuropathy with myositis. No definitive treatment pathways are outlined for such atypical presentations.

Case

A 69 year old man presented in 2006 with diplopia, facial weakness, bulbar dysfunction, profound sensory motor neuropathy (distally more than proximally), and hyporeflexia. Blood at the time did not reveal any major abnormalities. Electrophysiology confirmed severe sensory and motor axonal polyneuropathies. Sural nerve biopsy was non-specific, whereas muscle biopsy specifically showed peri-vascular inflammatory cells. Following significant improvement with intra-venous methylprednisolone, he returned to a functional independent life. More debilitating similar episodes recurred six and nine years later with involvement of cranial nerves 2–7, 9–12, accompanied by predominantly upper limb weakness and hyporeflexia. Post-contrast neural MR and CSF constituents remained normal (negative antiganglioside antibodies). Electrophysiology confirmed progressive axonal peripheral polyneuropathy. Liver biopsy ruled out cirrhosis/autoimmune hepatitis. In 2016, repeat Ro antibodies were positive, with3-year history of Raynaud’s phenomenon. Patient was treated with immunoglobulin, intravenous Methylprednisolone, followed by oral steroids. Subsequently, intravenous Cyclophosphamide then Mycophenolate with ongoing tapered steroids resulted in good response, despite persistent muscle wasting without fasciculations.

Conclusion

We report a unique case of relapsing immune-mediated cranial and peripheral neuropathy with myositits, responsive to aggressive and sustained immunosuppressive therapy.

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