Development of weakness in patients with chronic inflammatory demyelinating polyneuropathy and only sensory symptoms at presentation: A long-term fellow-up study

This long-term follow-up study examined patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and only sensory symptoms at first pres entation, with emphasis on the development of motor symptoms and long-term. disability. From all CIDP patients referred to our Department between 1987 and 1995, seven had only sensory symptoms at first clinical presentation. These were investigated according to a standard protocol, including a quant ified clinical neurological examination and nerve conduction studies. The m ean duration of the disease before weakness developed was 3.1 years, but va ried considerably (0.8-6.3 years). At follow-up, weakness developed in five patients and persisted in three of them. Five patients were nor seriously incapacitated by their disease (Rankin 1 or 2), four of them being in remis sion now and one showing a very slow progression of disease. Two patients w ere moderately disabled (Rankin 3); one had severe persistent sensory ataxi a and only weakness during relapses and one had stepwise progression and mo derate weakness. Motor nerve conduction studies revealed that the most nota ble worsening in the entire group of patients was a decrease in distal comp ound muscle action potential amplitudes, indicating the development of dist al conduction block or axonal degeneration. These findings shaw that CIDP w ith only sensory symptoms is a transient clinical stage that precedes the a ppearance of weakness in about 70% of patients. The long-term prognosis doe s not differ from that of patients with CIDP who have weakness at the begin ning of the disease.