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Neurology India
Medknow Publications on behalf of the Neurological Society of India
ISSN: 0028-3886 EISSN: 1998-4022
Vol. 59, Num. 3, 2011, pp. 476-478

Neurology India, Vol. 59, No. 3, May-June, 2011, pp. 476-478

Letter to Editor

Sjögren's syndrome with chronic inflammatory demyelinating polyneuropathy

Wen-Sou Lin1, Yaw-Don Hsu2

1 Department of Neurology, Kaohsiung Armed Forces General Hospital, Taipei, Taiwan
2 Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan

Correspondence Address: Yaw-Don Hsu Tri-Service General Hospital, National Defense Medical Center, Taipei Taiwan

Code Number: ni11140

PMID: 21743193

DOI: 10.4103/0028-3886.82754


Neurologic manifestation of Sjφgren's syndrome (SS) can affect both peripheral and central nervous systems. [1],[2] Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an extremely rare manifestation of SS. We present a case of SS with CIDP.

An 83-year-old woman presented with gradual limb weakness, predominantly in lower limbs, of 2 months duration. She also complained of dry eyes and dry mouth which make her frequent water drink and use of tear substitutes to reduce discomfort in the eyes. On examination, there was reduced muscle bulk of all the four limbs and symmetrical motor weakness (2/5 grade) of all the four limbs, more pronounced distally. Deep tendon reflexes were sluggish and the ankle jerks were absent. Plantar responses were bilaterally flexor. There was a sensory deficit distally in the sock and glove distribution. Nerve conduction study showed prolonged distal latencies, reduced motor conduction velocity, and low amplitude of compound motor action potentials (CMAPs) in the upper and lower limb nerves. Sensory nerve testing showed moderately prolonged distal latencies, reduced conduction velocities and low amplitude of sensory nerve action potentials (SNAPs). F-wave latencies were prolonged in the ulnar, median, peroneal, and tibial nerves [Table - 1]. The electrodiagnostic findings fulfilled the criteria of Inflammatory Neuropathy Cause and Treatment (INCAT) for the diagnosis of CIDP. [3] Tests for collagen profile showed the following: antinuclear antibodies [anti-nuclear antibody 1: >1280 arbitrary units (AU), speckled pattern, normal reference limit <1:40 AU] and extractable antinuclear antibodies [ENA; anti-Ro/SSA antibody >240.0 U/ml and anti-LA antibody was 2.1 U/ml (normal reference limit: Positive >10, negative <7 U/ml)]. Schirmer's test was positive showing 4 mm of bilateral tearing, and salivary gland scintigraphy revealed mild dysfunction. Based on the clinical features and the laboratory findings, she was diagnosed as a case of SS. [4] Other laboratory data including hepatitis B surface antigen, anti-hepatitis C virus IgG, anti-human immunodeficiency virus IgG, lymphoma, paraproteinemia, thyroid stimulation hormone (TSH), anti-RNP, anti-SM, anti-Jo-1, anti-SCL70, anti-double stranded DNA antibodies, anti-cardiolipin antibody, and rheumatoid factors were all negative. She was started on oral prednisolone 60 mg daily (1 mg/kg). There was a significant improvement of muscle strength (grade 4/5) in the following two weeks, and at 1-month follow-up, she had complete recovery of motor weakness. She had also improvement of dry mouth and dry eyes.

CIDP can occur in association with hepatitis C, inflammatory bowel disease, lymphoma, monoclonal gammopathy of undetermined significance (MGUS), human immunodeficiency virus infection or acquired immunodeficiency syndrome, organ transplant, and connective tissue disorders. [5],[6] The reported prevalence of CIDP in connective tissue diseases is 20.8%; [6] however, there are no specific epidemiologic data concerning CIDP in SS. CIDP as the presenting feature of SS is extremely rare in clinical practice and our patient demonstrates the occurrence of the two diseases concurrently. The exact relationship of pathogenesis between SS and CIDP is unknown. CIDP may share the same pathogenesis including vasculitis and humoral- and/or cellular-mediated immune response.[1] Sensory ataxic neuropathy (sensory neuronopathy) is the most common neurological manifestation of SS [1] and almost 81% patients with SS present with neurologic manifestations before the diagnosis of SS is established.[2] Severe disability is unusual unless there is CNS involvement. [2]

Patients with CIDP respond well to steroid therapy [5] and early steroid treatment limits the demyelination process. It will also be important to develop strategies to protect axons from degeneration and to enhance regeneration. [7] The response to steroid or immunosuppressive therapy of patients of SS with neurological manifestations is unpredictable and at least 30% of patients do not respond to conventional therapies. [3],[8] Our patients illustrate that those having SS with CIDP could recover completely if treated early. Furthermore, it is important to do a systemic screening in patients with CIDP for other immune-mediated disorders.


1.Lafitte C. Neurologic manifestations of primary Gougerot-Sjogren syndrome. Rev Neurol (Paris) 1998;154:658-73.  Back to cited text no. 1    
2.Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjogren syndrome: A study of 82 patients. Medicine (Baltimore) 2004;83:280-91.  Back to cited text no. 2    
3.Hughes R, Bensa S, Willison H, Van den Bergh P, Comi G, Illa I, et al. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 2001;50:195-201.  Back to cited text no. 3    
4.Vitali C, Bombardieri S, Moutsopoulos HM, Coll J, Gerli R, Hatron PY, et al. Assessment of the European classification criteria for Sjögren's syndrome in a series of clinically defined cases: Results of a prospective multicentre study. Ann Rheum Dis 1996;55:116-21.  Back to cited text no. 4    
5.Vallat JM, Sommer C, Magy L. Chronic inflammatory demyelinating polyradiculoneuropathy: Diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol 2010;9:402-12.  Back to cited text no. 5    
6.San-Juan OD, Castro-Macias JL. Chronic inflammatory demyelinating polyradiculoneuropathy. 10 years' experience in a Mexican centre. Rev Neurol 2008;46:656-9.  Back to cited text no. 6    
7.Magy L, Vallat JM. Evidence-based treatment of chronic immune-mediated neuropathies. Expert Opin Pharmacother 2009;10:1741-54.  Back to cited text no. 7    
8.Hughes RA, Gabriel CM, Gregson NA, Smith KJ. Treatment of inflammatory neuropathy. Mult Scler 1997;3:88-92.  Back to cited text no. 8    

Copyright 2011 - Neurology India

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