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CASE REPORT |
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| Ahead of print
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Sjögren syndrome in childhood mimicking pediatric multiple sclerosis
Dilek Cavusoglu1, Nihal O Dundar2, Pinar Gencpinar2, Furkan Kaya3, Ozgur Oztekin4
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 Submission | 09-Jun-2021 |
| Date of Decision | 01-Feb-2022 |
| Date of Acceptance | 24-Jul-2021 |
| Date of Web Publication | 30-Jan-2023 |
Correspondence Address:
Dilek Cavusoglu,
Department of Pediatric Neurology, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyon
Turkey
 Source of Support: None, Conflict of Interest: None DOI: 10.4103/jpn.JPN_130_21
 Abstract | | |
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
| Introduction | |  |
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 | | |
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 | | |
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 | | |
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
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[Figure 1], [Figure 2]
[Table 1]
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