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Sjögren syndrome in childhood mimicking pediatric multiple sclerosis

1 Department of Pediatric Neurology, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyon, Turkey
2 Department of Pediatric Neurology, Faculty of Medicine, Izmir Katip Celebi University, Izmir, Turkey
3 Department of Radiology, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyon, Turkey
4 Department of Radiology, Izmir Tepecik Training and Research Hospital, Izmir, Turkey

Date of Submission09-Jun-2021
Date of Decision01-Feb-2022
Date of Acceptance24-Jul-2021
Date of Web Publication30-Jan-2023

Correspondence Address:
Dilek Cavusoglu,
Department of Pediatric Neurology, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyon
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_130_21



The differential diagnosis of pediatric multiple sclerosis (MS) presents a wide spectrum of diseases. Sjögren syndrome (SS) is also a rare cause of this disease. Neurological involvement in SS ranges from peripheral neuropathies to the central nervous system (CNS). We report the case of a 5-year-old boy with a possible diagnosis with SS based on positive minor salivary gland biopsy findings and positive anti-smooth muscle antibodies, also fulfilling the 2017 McDonald criteria. If a patient is younger and meets the MS criteria clinically and radiologically, it is suggested to carefully examine the CNS demyelination in a patient for autoimmune diseases, particularly SS.

Keywords: Children, multiple sclerosis, Sjögren syndrome

How to cite this URL:
Cavusoglu D, O Dundar N, Gencpinar P, Kaya F, Oztekin O. Sjögren syndrome in childhood mimicking pediatric multiple sclerosis. J Pediatr Neurosci [Epub ahead of print] [cited 2023 Jun 3]. Available from: https://www.pediatricneurosciences.com/preprintarticle.asp?id=368795

   Introduction Top

Sjögren syndrome (SS) is an autoimmune inflammatory disease characterized by lymphocytic infiltration of both salivary and lacrimal glands and frequently associated with antinuclear antibodies, to the anti-SSA (Ro) or anti-SSB (La).[1] SS mostly presents with dry eyes and mouth known as sicca symptoms.[2] Additionally, extraglandular symptoms such as neurological involvement can occur.[1],[2] We report a case of possible SS that reflects demyelinating properties supporting MS in respect of clinical presentation and neuroimaging.

   Case Report Top

A 5-year-old boy was presented with acute weakness in the right upper and lower extremities. Besides, he had manifested dysarthria 2 months earlier. His neurologic examinations suggested increased deep tendon reflexes, with a positive Babinski reflex on the right side. Brain magnetic resonance imaging (MRI) revealed hyperintense lesions on T2-weighted and fluid-attenuated inversion recovery images, involving the genu and splenium of the corpus callosum, periventricular white matter, and the subcortical area of the superior frontal gyrus with the left anterior of the medulla oblongata with gadolinium enhancement on T1-weighted images [Figure 1] and [Figure 2]. Cervical spine MRI showed a non-contrast millimeter-sized lesion at the proximal level of the C2 cord area. Furthermore, thoracic and lumbar spine MRI showed normal. Cerebrospinal fluid (CSF) examination revealed normal levels of glucose and protein. Furthermore, an oligoclonal band (Type 2) and elevated IgG index (1.01) were determined. Antibodies to aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies were negative. Screenings for viral markers, amino acids in plasma and urine, serum ammonia, lactic acid, pyruvic acid, urine organic acids, acyl-carnitine profile, carnitine, very-long-chain fatty acid, immunoglobulins, lymphocyte subgroups, and lysosomal enzymes were found normal. Pediatric-onset MS was primarily considered based on clinical and laboratory tests and neuroimaging findings. Moreover, positive anti-smooth muscle antibodies (ASMAs) (1:1000) were also noticed. He was tested negative for antinuclear antibody, anti-SSA/Ro, anti-SSB/La, and anti-dsDNA. The erythrocyte sedimentation rate showed 21 mm (range, 0–10 mm/h). The patient was investigated for possible SS due to his younger age and the presence of a high level of ASMA. Thereafter, a minor salivary gland biopsy was performed. The histological examination revealed more than one focus/4 mm² (according to grade 4 in Chisholm and Mason classification). His Schirmer test was negative. Then, he was primarily treated for 3 days with intravenous methylprednisolone 30 mg/kg/day, which was then continued with intravenous immune globulin (IVIG) 1 g/kg/day for 2 days once a month due to these ongoing tests for 3 months. After the pulse steroid treatment, the patient showed clinical recovery. Additionally, the performing MRI revealed the resolution of imaging findings following steroid and IVIG therapy. Finally, IVIG was discontinued, and azathioprine was started with the addition of results supporting SS. Thereafter, no clinical attack was noticed; his neurological examination was normal for 13 months.
Figure 1: (A and B) Brain magnetic resonance imaging revealed hyperintense lesions on T2-weighted and FLAIR images, involving the genu and splenium of the corpus callosum, periventricular white matter, and the subcortical area of the superior frontal gyrus

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Figure 2: (A and B) Axial and sagittal T1-weighted images showed gadolinium enhancement in the left anterior of the medulla oblongata (arrow)

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   Discussion Top

The patient discussed in this case report had possible SS with the hallmarks of central nervous system (CNS) demyelination. The clinical attacks, MRI lesions, and CSF-specific oligoclonal bands fulfilled the 2017 McDonald criteria.[3] However, the 2017 McDonald criteria had a lower positive predictive value for children under 11 years of age as in the 2010 criteria, so it is suggested to carefully apply the criteria in young children like the current 5-year-old boy case.[4] Several diseases can mimic MS with having a relapsing-remitting clinical process and also meeting the dissemination in space and dissemination in time criteria or both [Table 1].[5]
Table 1: Some diseases may mimic multiple sclerosis with dissemination of lesions in space (DIS) or dissemination in time (DIT) or both

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So far, no studies have reported a certain incidence/prevalence of SS in children. It shows a low incidence of SS in childhood.[2] Thus, the neurological involvement of SS presents uncommon as case reports. These reports show neurological manifestations of weakness, paresthesia, headache, dizziness, hemiparesis, seizure, polyradiculoneuritis, and meningoencephalitis, which resulted in quadriparesis, facial diplegia, vertical diplopia, “walking sideways,” leg numbness, dysarthria, ataxia, optic neuritis, urinary incontinence, bilateral lower extremity paresis, a sensory deficit, and changes in mental status. Additionally, reports of these patients showed neuroimaging abnormalities of varying degrees in the cerebrum, cerebellum, brainstem, and spine.[2]

The diagnosis criteria of SS in adults include ocular and oral symptoms, autoantibodies as anti-SSA (Ro) or anti-SSB (La), and positive minor salivary gland biopsy findings (focal lymphocytic sialadenitis and focus score of ≥1 foci/4 mm² in labial salivary gland biopsy samples).[1],[6] But, there is a lack of consensus for the diagnosis of SS in the pediatric population.[1] In the present case report, a highly elevated ASMA level and a minor salivary gland biopsy result consistent with grade 4 according to Chisholm and Mason’s classification supported a possible diagnosis of SS. Although anti-Ro/SSA and anti-La/SSB autoantibodies are the most common autoantibodies observed in SS, several autoantibodies have been observed in the serum of patients with SS like ASMA.[7] The prevalence of ASMA in SS patient’s sera has ranged from 6.5% to 62%. Furthermore, ASMAs are identified as related to type 1 autoimmune hepatitis, but this condition seems to have no clinical value.[8],[9] In addition, the present patient did not show any clinical features connected with hepatitis. Moreover, the pediatric population mostly lacks sicca symptoms similar to our case.

Most of the studies on adults have revealed the presence of diagnosed SS with MS-like involvement.[2] First, in childhood studies, Ohtsuka et al.[10] reported about a 9-year-old girl diagnosed with SS with clinical and neuroimaging mimicking MS, but her CSF IgG and myelin basic protein levels showed a remarkable increase but an absence of oligoclonal bands. The CNS demyelination can be explained by a rare feature of SS.

   Conclusion Top

The present case showed the manifestations of CNS demyelination with MS-like exhibition and possible SS findings. We can enounce that the patient can fulfill the diagnosis criteria of SS in the follow-up after some time due to his younger age. Moreover, the present case report highlights a possible SS by mimicking MS. Although neurological manifestations are uncommon in the pediatric population with SS, the broad differential diagnosis and high clinical suspicion, in especially younger age and having positive autoantibodies, are necessary to make the accurate diagnosis include SS.

Financial support and sponsorship

The author(s) received no financial support for the research, authorship, and/or publication of this article.

Conflicts of interest

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article

   References Top

Pohl D, Benseler S. Systemic inflammatory and autoimmune disorders. Handb Clin Neurol 2013;112:1243-52.  Back to cited text no. 1
Matsui Y, Takenouchi T, Narabayashi A, Ohara K, Nakahara T, Takahashi T. Childhood Sjögren syndrome presenting as acute brainstem encephalitis. Brain Dev 2016;38:158-62.  Back to cited text no. 2
Langille MM, Rutatangwa A, Francisco C. Pediatric multiple sclerosis: A review. Adv Pediatr 2019;66:209-29.  Back to cited text no. 3
Fadda G, Brown RA, Longoni G, Castro DA, O’Mahony J, Verhey LH, et al; Canadian Pediatric Demyelinating Disease Network. MRI and laboratory features and the performance of international criteria in the diagnosis of multiple sclerosis in children and adolescents: A prospective cohort study. Lancet Child Adolesc Health 2018;2:191-204.  Back to cited text no. 4
Rocha AJ, Littig IA, Nunes RH, Tilbery CP. Central nervous system infectious diseases mimicking multiple sclerosis: Recognizing distinguishable features using MRI. Arq Neuropsiquiatr 2013;71:738-46.  Back to cited text no. 5
Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, et al; International Sjögren’s Syndrome Criteria Working Group. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for primary Sjögren’s syndrome: A consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol 2017;69:35-45.  Back to cited text no. 6
Tzioufas AG, Tatouli IP, Moutsopoulos HM. Autoantibodies in Sjögren’s syndrome: Clinical presentation and regulatory mechanisms. Presse Med 2012;41:e451-60.  Back to cited text no. 7
Csepregi A, Szodoray P, Zeher M. Do autoantibodies predict autoimmune liver disease in primary Sjögren’s syndrome? Data of 180 patients upon a 5 year follow-up. Scand J Immunol 2002;56:623-9.  Back to cited text no. 8
Lindgren S, Manthorpe R, Eriksson S. Autoimmune liver disease in patients with primary Sjögren’s syndrome. J Hepatol 1994;20:354-8.  Back to cited text no. 9
Ohtsuka T, Saito Y, Hasegawa M, Tatsuno M, Takita S, Arita M, et al. Central nervous system disease in a child with primary Sjögren syndrome. J Pediatr 1995;127:961-3.  Back to cited text no. 10


  [Figure 1], [Figure 2]

  [Table 1]


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