Diagnostic and management difficulties of chronic inflammatory demyelinating polyradiculoneuropathy

OBJECTIVES: To describe the pattern of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and evaluate its local diagnostic and management practices. To define factors responsible for the delay in reaching a diagnosis and initiating treatment. METHODS: Patients with the diagnosis of CIDP attending King Khalid University Hospital, Riyadh, Saudi Arabia between 1986 and 2006 were retrospectively studied, in relation to diagnosis and management. Diagnosis was reassessed, and patients included in view of American Academy of Neurology as well as Latov's criteria. Available patients were reevaluated and prospectively followed up until the end of the study. RESULTS: Twenty-two patients were included (18 males and 4 females, 4.5:1). Age at onset range was 3-70 years (mean of 33 years). Diagnosis in 80% of patients was delayed from 6 months to 10 years (mean of 2.5 years). No case was diagnosed before referral. The course was progressive in 53% and relapsing in 47%. Most patients made significant initial improvement, though less than expected. Two patients with long delay in diagnosis (7.5 and 10 years) showed no improvement. CONCLUSION: Diagnosis of CIDP is frequently delayed, with a deleterious effect on response to treatment. This is related to some degree to the lack of awareness among general physicians, which needs to be corrected. Treatment was also hindered by patients` suboptimal compliance, which could be improved by better education. Management is not standardized, and this could be improved by establishing up-to-date treatment guidelines.