A case of chronic and progressive distal symmetric type CIDP with subacute exacerbation: Focus on clinical pattern of CIDP

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is classified into typical type that is characterized by proximal weakness and atypical type that include demyelinating acquired distal symmetric (DADS) type. DADS type CIDP is characterized by slowly progressive clinical course, motor and sensory involvement dominant in the leg and resistance to therapy. We report the case of a 72 year-old man with DADS type CIDP, who have dysesthesia and weakness dominant in distal of limbs. Disease progression was resistant to therapy for prednisolone and intravenous immunoglobulin, but slowly progressive for 4 years. Subacute exacerbation that was accompanied by proximal weakness was occurred at 4 years later from onset. Nerve conduction study revealed markedly prolonged distal latencies and slowing of conduction velocities. Double filtration plasmapheresis improved symptom, after oral prednisolone kept remission. This case had characteristic of DADS type CIDP at the onset, but changed into typical CIDP also for the therapy in the clinical course. This case indicate the multiplicity of CIDP.