Motor chronic inflammatory demyelinating polyneuropathy (CIDP) in 17 patients: Clinical characteristics, electrophysiological study, and response to treatment.
Adolescent
Adult
Aged
Aged, 80 and over
Child
Disease Progression
Electromyography
Female
Humans
Italy
Male
Middle Aged
Neural Conduction
/ physiology
Neuroimaging
Polyradiculoneuropathy, Chronic Inflammatory Demyelinating
/ cerebrospinal fluid
Recurrence
Retrospective Studies
Treatment Outcome
Young Adult
cancer
corticosteroid
motor CIDP
motor predominant CIDP
pure motor CIDP
Journal
Journal of the peripheral nervous system : JPNS
ISSN: 1529-8027
Titre abrégé: J Peripher Nerv Syst
Pays: United States
ID NLM: 9704532
Informations de publication
Date de publication:
06 2020
06 2020
Historique:
received:
26
10
2019
revised:
29
04
2020
accepted:
29
04
2020
pubmed:
5
5
2020
medline:
2
6
2021
entrez:
5
5
2020
Statut:
ppublish
Résumé
Motor chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare and poorly described subtype of CIDP. We aimed to study their clinical and electrophysiological characteristics and response to treatment. From a prospective database of CIDP patients, we included patients with definite or probable CIDP with motor signs and without sensory signs/symptoms at diagnosis. Patients were considered to have pure motor CIDP (PM-CIDP) if sensory conductions were normal or to have motor predominant CIDP (MPred-CIDP) if ≥2 sensory nerve action potential amplitudes were abnormal. Among the 700 patients with CIDP, 17 (2%) were included (PM-CIDP n = 7, MPred-CIDP n = 10); 71% were male, median age at onset was 48 years (range: 13-76 years), 47% had an associated inflammatory or infectious disease or neoplasia. At the more severe disease stage, 94% of patients had upper and lower limb weakness, with distal and proximal weakness in 4 limbs for 56% of them. Three-quarters (75%) responded to intravenous immunoglobulins (IVIg) and four of five patients to corticosteroids including three of three patients with MPred-CIDP. The most frequent conduction abnormalities were conduction blocks (CB, 82%) and F-wave abnormalities (88%). During follow up, 4 of 10 MPred-CIDP patients developed mild sensory symptoms; none with PM-CIDP did so. Patients with PM-CIDP had poorer outcome (median ONLS: 4; range: 22-5) compared to MPred-CIDP (2, range: 0-4; P = .03) at last follow up. This study found a progressive clinical course in the majority of patients with motor CIDP as well as frequent associated diseases, CB, and F-wave abnormalities. Corticosteroids might be considered as a therapeutic option in resistant IVIg patients with MPred-CIDP.
Types de publication
Journal Article
Multicenter Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
162-170Informations de copyright
© 2020 Peripheral Nerve Society.
Références
Vallat J-M, Sommer C, Magy L. Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol. 2010;9(4):402-412.
Rabin M, Mutlu G, Stojkovic T, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: search for factors associated with treatment dependence or successful withdrawal. J Neurol Neurosurg Psychiatry. 2014;85(8):901-906.
Van den Bergh PYK, Hadden RDM, Bouche P, et al. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force of the European Federation of Neurological Societies and the Peripher: EFNS/PNS CIDP guideline - first revision. Eur J Neurol. 2010;17(3):356-363.
Gorson KC, Allam G, Ropper AH. Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology. 1997;48(2):321-328.
Busby M, Donaghy M. Chronic dysimmune neuropathy. J Neurol. 2003;250(6):714-724.
Mahdi-Rogers M, Hughes RAC. Epidemiology of chronic inflammatory neuropathies in Southeast England. Eur J Neurol. 2014;21(1):28-33.
Doneddu PE, Cocito D, Manganelli F, et al. Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database. J Neurol Neurosurg Psychiatry. 2019;90(2):125-132.
Ikeda S, Koike H, Nishi R, et al. Clinicopathological characteristics of subtypes of chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Neurosurg Psychiatry. 2019;90:988-996.
Sabatelli M, Madia F, Mignogna T, Lippi G, Quaranta L, Tonali P. Pure motor chronic inflammatory demyelinating polyneuropathy. J Neurol. 2001;248(9):772-777.
Kimura A, Sakurai T, Koumura A, et al. Motor-dominant chronic inflammatory demyelinating polyneuropathy. J Neurol. 2010;257(4):621-629.
Nobile-Orazio E. Chronic inflammatory demyelinating polyradiculoneuropathy and variants: where we are and where we should go. J Peripher Nerv Syst. 2014;19(1):2-13.
Querol L, Rojas-Garcia R, Casasnovas C, et al. Long-term outcome in chronic inflammatory demyelinating polyneuropathy patients treated with intravenous immunoglobulin: a retrospective study: long-term effectiveness of IVIg in CIDP. Muscle Nerve. 2013;48(6):870-876.
Viala K, Maisonobe T, Stojkovic T, et al. A current view of the diagnosis, clinical variants, response to treatment and prognosis of chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst. 2010;15(1):50-56.
Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of multifocal motor neuropathy. Report of a Joint Task Force of the European Federation of Neurological Societies and the peripheral nerve society-first revision. J Peripher Nerv Syst. 2010;15(4):295-301.
Viala K. Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome. Brain. 2004;127(9):2010-2017.
Koutlidis RM, Ayrignac X, Pradat P-F, et al. Segmental somatosensory-evoked potentials as a diagnostic tool in chronic inflammatory demyelinating polyneuropathies, and other sensory neuropathies. Neurophysiol Clin. 2014;44(3):267-280.
Viala K, Behin A, Maisonobe T, et al. Neuropathy in lymphoma: a relationship between the pattern of neuropathy, type of lymphoma and prognosis? J Neurol Neurosurg Psychiat. 2008;79(7):778-782.
Rajabally YA, Attarian S. Chronic inflammatory demyelinating polyneuropathy and malignancy: a systematic review: CIDP and malignancy: a review. Muscle Nerve. 2018;57(6):875-883.
Mostoufizadeh S, Souri M, de Seze J. A case of paraneoplastic demyelinating motor polyneuropathy. Case Rep Neurol. 2012;4(1):71-76.
van Dijk GW, Notermans NC, Franssen H, Wokke JH. Development of weakness in patients with chronic inflammatory demyelinating polyneuropathy and only sensory symptoms at presentation: a long-term follow-up study. J Neurol. 1999;246(12):1134-1139.
Molenaar DS, van Doorn PA, Vermeulen M. Pulsed high dose dexamethasone treatment in chronic inflammatory demyelinating polyneuropathy: a pilot study. J Neurol Neurosurg Psychiatry. 1997;62(4):388-390.
Lievens I, Fournier E, Viala K, Maisonobe T, Bouche P, Léger J-M. Neuropathie motrice multifocale: existe-t-il une altération de la conduction sensitive au long cours ? Une étude rétrospective chez 21 patients. Rev Neurol. 2009;165(3):243-248.
Echaniz-Laguna A, Degos B, Mohr M, Kessler R, Urban-Kraemer E, Tranchant C. A study of three patients with amyotrophic lateral sclerosis and a polyneuropathy resembling CIDP. Muscle Nerve. 2006;33(3):356-362.
Rajabally YA, Jacob S. Chronic inflammatory demyelinating polyneuropathy-like disorder associated with amyotrophic lateral sclerosis. Muscle Nerve. 2008;38(1):855-860.
Ahdab R, Créange A, Saint-Val C, Farhat W-H, Lefaucheur J-P. Rapidly progressive amyotrophic lateral sclerosis initially masquerading as a demyelinating neuropathy. Neurophysiol Clin. 2013;43(3):181-187.