Top

Published in:

Open Access 01-12-2015 | Case report

Pure sensory chronic inflammatory polyneuropathy: rapid deterioration after steroid treatment

Authors: Elisabeth Chroni, Dimitra Veltsista, Evangelia Gavanozi, Tavitha Vlachou, Panagiotis Polychronopoulos, Panagiotis Papathanasopoulos

Published in: BMC Neurology | Issue 1/2015

Abstract

Background

Chronic inflammatory demyelinating polyneuropathy (CIDP) as a pure sensory variant is rarely encountered. Therefore the best treatment option is hard to define.

Case presentations

We reported two middle-aged patients of Caucasian origin, one female and one male, who over a period of several months presented limbs and gait ataxia. Clinical and neurophysiological examination revealed only sensory abnormalities. A diagnosis of atypical CIDP was suggested, considering the elevated CSF protein level and the presence of anti-gangliosides antibodies. Ten and 15 days respectively after initiation of prednisolone treatment both patients experienced exacerbation of sensory symptoms and emerging of muscle weakness. Steroids were then substituted by rituximab in the first patient and intravenous immunoglobulin in the second patient resulting in gradual decrement of symptoms and signs. Two-year follow-up showed no further deterioration.

Conclusion

Caution should be exercised when treating cases of pure sensory polyneuropathy with high dose steroids since an unfavorable outcome is possible.

Notermans NC, Wokke JH, Franssen H, van Der Graaf Y, Vermeulen M, van Den Berg LH, et al. Chronic idiopathic polyneuropathy presenting in middle or old age: a clinical and electrophysiological study of 75 patients. Journal of Neurol Neurosurg Psychiatry. 1993;56:1066–71.CrossRef

Pasnoor M, Dimachkie MM, Barohn RJ. Cryptogenic sensory polyneuropathy. Neurol Clin. 2013;31(2):463–76.CrossRefPubMedPubMedCentral

Oh SJ, Joy JL, Kuruoglu R. “Chronic sensory demyelinating neuropathy”: chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy. J Neurol Neurosurg Psychiatry. 1992;5(8):677–80.CrossRef

Sinnreich M, Klein CJ, Daube JR, Engelstad J, Spinner RJ, Dyck PJ. Chronic immune sensory polyradiculopathy: a possibly treatable sensory ataxia. Neurology. 2004;63(9):1662–9.CrossRefPubMed

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):1134–9.CrossRefPubMed

Rajabally YA, Narasimhan M. Characteristics and correlates of sensory function in chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2010;297(1–2):11–4.CrossRefPubMed

Ayrignac X, Viala K, Koutlidis RM, Taïeb G, Stojkoyic T, Musset L, et al. Sensory chronic inflammatory demyelinating polyneuropathy: an under-recognized entity? Muscle Nerve. 2013;48(5):727–32.CrossRefPubMed

Graham RC, Hughes RA. A modified peripheral neuropathy scale: the Overall Neuropathy Limitations Scale. J Neurol Neurosurg Psychiatry. 2006;77(8):973–6.CrossRefPubMedPubMedCentral

Dyck PJ, Davies JL, Litchy WJ, O’Brien PC. Longitudinal assessment of diabetic polyneuropathy using a composite score in the Rochester Diabetic Neuropathy Study cohort. Neurology. 1997;49(1):229–39.CrossRefPubMed

10.

Bril V. NIS-LL: the primary measurement scale for clinical trial endpoints in diabetic peripheral neuropathy. Eur Neurol. 1999;41 Suppl 1:8–13.CrossRefPubMed

11.

European Federation of Neurological Societies/Peripheral Nerve Society, Member of Task Force, Van den Bergh PY, Hadden RD, Bouche P, European Federation of Neurological Societies/Peripheral Nerve Society, et al. Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - first revision. Eur J Neurol. 2010;17(3):356–63.CrossRef

12.

Vedeler CA, Farbu E, Mellgren SI. Chronic inflammatory demyelinating polyneuropathy (CIDP). Acta Neurol Scand. 2013;127 suppl 196:48–51.CrossRef

13.

Camdessanché JP, Jousserand G, Franques J, Pouget J, Delmont E, Créabge A, et al. French CIDP study group: a clinical pattern-based etiological diagnostic strategy for sensory neuronopathies: a French collaborative study. J Peripher Nerv Syst. 2012;17(3):331–40.CrossRefPubMed

14.

Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al. European study group on classification criteria for Sjögren’s syndrome. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by American-European consensus group. Ann Rheum Dis. 2002;61(6):554–8.CrossRefPubMedPubMedCentral

15.

Chin RL, Latov N, Sander HW, Hays AP, Croul SE, Magda P, et al. Sensory CIDP presenting as cryptogenic sensory polyneuropathy. J Peripher Nerv Syst. 2004;9(3):132–7.CrossRefPubMed

16.

Bragg JA, Benatar MG. Sensory nerve conduction slowing is a specific marker for CIDP. Muscle Nerve. 2008;38(6):1599–603.CrossRefPubMed

17.

Ohkoshi N, Harada K, Nagata H, Yato M, Shoji S. Ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy: clinical features and pathological study of the sural nerves. Eur Neurol. 2001;45(4):241–8.CrossRefPubMed

18.

Vallat JM, Sommer C, Magy L. Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol. 2010;9(4):402–12.CrossRefPubMed

19.

Rajabally YA, Wong SL. Chronic inflammatory pure sensory polyradiculoneuropathy: a rare CIDP variant with unusual electrophysiology. J Clin Neuromuscul Dis. 2012;13(3):149–52.CrossRefPubMed

20.

Attarian S, Boucraut J, Hubert AM, Uzenot D, Delmont E, Verschueren A, et al. Chronic ataxic neuropathies associated with anti-GD1b IgM antibodies: response to IVIg therapy. J Neurol Neurosurg Psychiatry. 2010;81(1):61–4.CrossRefPubMed

21.

Donaghy M, Mills KR, Boniface SJ, Simmons J, Wright I, Gregson N, et al. Pure motor demyelinating neuropathy : deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry. 1994;57:778–83.CrossRefPubMedPubMedCentral

22.

Larue S, Dashi F, Demeret S, Léger J-M. Two patients with CIDP deteriorated following plasma exchange. J Peripher Nerv System. 2008;13:307–9.CrossRef

23.

van Schaik IN, Eftimov F, van Doorn PA, Brusse E, van den Berg LH, van der Pol WL, et al. Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial. Lancet Neurol. 2010;9(3):245–53.CrossRefPubMed

24.

Nobile-Orazio E, Cocito D, Jann S, Uncini A, Beghi E, Messina P, et al. Intravenous immunoglobulin versus intravenous methylprednisolone for chronic inflammatory demyelinating polyradiculoneuropathy: a randomised controlled trial. Lancet Neurol. 2012;11(6):493–502.CrossRefPubMed

25.

Sabatelli M, Madia F, Mignogna T, Lippi G, Quaranta L, Tonali P. Pure motor chronic inflammatory demyelinating polyneuropathy. J Neurol. 2001;248(9):772–7.CrossRefPubMed

Title: Pure sensory chronic inflammatory polyneuropathy: rapid deterioration after steroid treatment
Authors: Elisabeth Chroni
Dimitra Veltsista
Evangelia Gavanozi
Tavitha Vlachou
Panagiotis Polychronopoulos
Panagiotis Papathanasopoulos
Publication date: 01-12-2015
Publisher: BioMed Central
Published in: BMC Neurology / Issue 1/2015
Electronic ISSN: 1471-2377
DOI: https://doi.org/10.1186/s12883-015-0291-7

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Pure sensory chronic inflammatory polyneuropathy: rapid deterioration after steroid treatment

Abstract

Background

Case presentations

Conclusion

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

Background

Case presentations

Conclusion

Please log in to get access to this content

Other articles of this Issue 1/2015

The MS@Work study: a 3-year prospective observational study on factors involved with work participation in patients with relapsing-remitting Multiple Sclerosis

A patient develops transient unique cerebral and cerebellar lesions after unruptured aneurysm coiling

Ceruloplasmin activity and iron chelation treatment of patients with Parkinson’s disease

Diagnosing moyamoya syndrome using ultrasound - a case report

May clinical neurophysiology help to predict the recovery of neurological early rehabilitation patients?

Multifocal leukoencephalopathy in cocaine users: a report of two cases and review of the literature