Epidemiologic variability of chronic inflammatory demyelinating polyneuropathy with different diagnostic criteria: Study of a UK population
- 18 March 2009
- journal article
- Published by Wiley in Muscle & Nerve
- Vol. 39 (4), 432-438
- https://doi.org/10.1002/mus.21206
Abstract
Epidemiologic data on chronic inflammatory demyelinating polyneuropathy (CIDP) is limited, and previous studies have shown variable results. The frequencies of CIDP subtypes remain unknown. Variations due to use of different diagnostic criteria have not been studied. We examined the prevalence and incidence of CIDP in Leicestershire and Rutland, UK (population 963,600). Prevalence day was 1 May 2008. The prevalence of CIDP fulfilling the 2006 clinical and electrophysiologic European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria was 4.77 per 100,000 (95% confidence interval [CI] 3.49–6.37). Using the 1991 American Academy of Neurology (AAN) criteria, the prevalence was 1.97 per 100,000 in this population (95% CI 1.19–3.08). Lewis–Sumner syndrome was diagnosed in 15.2% of patients, and 23.9% had pure sensory onset. Over 40% required no immunotherapy, and 84.6% of those treated responded. More than 80% of the AAN criteria–negative but EFNS/PNS criteria–positive patients were responsive to treatment. Both sets of criteria were equally likely to identify patients who required therapy. The mean annual incidence rate over the 3 years preceding the prevalence day was 0.70 per 100,000/year using EFNS/PNS criteria (95% CI 0.43–1.08), and 0.35 per 100,000/year using AAN criteria (95% CI 0.17–0.64). We conclude that the AAN criteria may underestimate prevalence and incidence of the disease. The EFNS/PNS criteria provide higher diagnostic sensitivity and are of greater clinical relevance, and they also offer a useful breakdown of the epidemiologic data for CIDP subtypes. Muscle Nerve, 2008Keywords
This publication has 22 references indexed in Scilit:
- Prevalence and incidence rates of chronic inflammatory demyelinating polyneuropathy in the Japanese populationJournal of Neurology, Neurosurgery & Psychiatry, 2008
- Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in ItalyJournal of Neurology, Neurosurgery & Psychiatry, 2007
- Multifocal motor neuropathyNeurology, 2007
- Classifications and treatment responses in chronic immune-mediated demyelinating polyneuropathyNeurology, 2007
- A modified peripheral neuropathy scale: the Overall Neuropathy Limitations ScaleJournal of Neurology, Neurosurgery & Psychiatry, 2006
- Optimizing the use of electrophysiology in the diagnosis of chronic inflammatory demyelinating polyneuropathy: a study of 20 casesJournal of the Peripheral Nervous System, 2005
- Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndromeBrain, 2004
- Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathyMuscle & Nerve, 2004
- Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathyAnnals of Neurology, 2001
- Acquired inflammatory demyelinating polyneuropathies: Clinical and electrodiagnostic featuresMuscle & Nerve, 1989