Clinical and Epidemiological Profile of a Brazilian’s cohort of patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy (P2.4-019)

Objective: To analyze the clinico-epidemiological profile of patients with Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a neuromuscular center of a University Hospital in Rio de Janeiro. Background: CIDP is an acquired disorder of peripheral nerves, which is immunologically based. It’s a rare disease and the diagnosis is difficult due to the heterogeneity of the clinical variants. Design/Methods: A retrospective study was performed between March and August 2018, based on 404 patients from the outpatient clinic of neuromuscular diseases, followed up in the last 35 years. We have reviewed charts and drawn up an evaluation card with identification, neurological examination, time from onset of symptoms to diagnosis, evolution, clinical grade score (CGS) and electroneuromyography. CIDP’s criteria used were from the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS). Results: Fourty-six patients had suspected chronic immune-mediated demyelinating neuropathy. Thirty-seven had suspected diagnosis of CIDP. Ten didn’t meet criteria: 2 were excluded (diabetic polyneuropathy) and 8 were atypical (1 CANOMAD; 1 CISP; 2 predominantly distal motor form; 4 predominantly sensory painful form). Twenty-one patients had defined CIDP; 4, probable; and 2, possible. Twenty-one had defined CIDP (19 typical; 2 multifocal acquired demyelinating sensory and motor neuropathy). Four had probable CIDP (2 typical; 1 focal; 1 predominantly sensory form). The 2 with possible CIDP were typical. The mean age was 52. Eight patients had remission; 12, a relapsing-remitting form; 10, a secondarily progressive form; 2 a progressive primary form and 3 remains under investigation. The mean time between onset of symptoms and the date of diagnosis was 54 months. The mean’s CGS at diagnosis was 2.94 and at the last evaluation was of 2.08. Conclusions: The time between the onset of symptoms and diagnosis is often very long, what impacts the efficacy of treatment. The EFNS/PNS criteria were flawed to identify the atypical forms. Disclosure: Dr. Carvalho has nothing to disclose. Dr. Medeiros has nothing to disclose. Dr. Lessa has nothing to disclose. Dr. Maia has nothing to disclose. Dr. Ramalho Monfredinho has nothing to disclose. Dr. Rodrigues has nothing to disclose. Dr. Abreu has nothing to disclose. Dr. Davidovich has nothing to disclose. Dr. Pupe has nothing to disclose. Dr. Nascimento has nothing to disclose.