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Abstract

We describe a case of pediatric Sjögren's syndrome with progressive neurologic involvement. At age 4 years, she had been diagnosed with Melkersson-Rosenthal syndrome. After being stable with facial diplegia and swelling for 5 years, she acutely presented with diplopia, vertigo, and ataxia. Cranial magnetic resonance imaging (MRI) showed a left dorsal midbrain lesion. Serologic and histopathologic findings confirmed primary Sjögren's syndrome. She responded well to intravenous methylprednisolone, with subsequent clinical improvement and MRI resolution. This report reviews the pediatric literature and underscores the importance of considering Sjögren's syndrome in a child with unexplained facial weakness and in the differential diagnosis of pediatric stroke. (J Child Neurol 2001;16:683-685).

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References

Bloch KJ , Buchanan WW , Wohl MJ , Bunim JJ : Sjögren's syndrome: A clinical, pathological, and serological study of sixty-two cases. Medicine 1965;44:187-231.

Fox RI , Howell FV , Bone RC , Michelson P. : Primary Sjögren syndrome: Clinical and immunopathologic features. Semin Arthritis Rheum 1984;14:77-105.

Anaya JM , Ogawa N. , Talal N. : Sjögren's syndrome in childhood. J Rheumatol 1995;22:1152-1158.

Streeto JM , Watters FB : Melkersson's syndrome: Multiple recurrences of Bell's palsy and episodic facial edema. N Engl J Med 1964;271:308-309.

Greene RM , Rogers RS : Melkersson-Rosenthal syndrome: A review of 36 patients. JAm Acad Dermatol 1989;21:1263-1270.

Vitali C. , Bombardieri S. , Moutsopoulos HM , et al: Assessment of the European classification criteria for Sjögren's syndrome in a series of clinically defined cases: Results of a prospective multicenter study. Ann Rheum Dis 1996;55:116-121.

Alexander EL : Neurologic disease in Sjögren's syndrome: Mononuclear inflammatory vasculopathy affecting central/peripheral nervous system and muscle. Rheum Dis Clin North Am 1993;19:869-908.

Fox RI , Tomwall J. , Michelson P. : Current issues in the diagnosis and treatment of Sjögren's syndrome. Curr Opin Rheumatol 1999;11:364-371.

Fox RI , Kang H. : Pathogenesis of Sjögren's syndrome. Rheum Dis Clin North Am 1992;18:517-538.

10.

Mellgren SI , Conn DL , Stevens JC , Dyck PJ : Peripheral neuropathy in primary Sjögren's syndrome. Neurology 1989;39:390-394.

11.

Alexander EL : Central nervous system disease in Sjögren's syndrome: New insights into immunopathogenesis. Rheum Dis Clin North Am 1992;18:637-672.

12.

Moutsopoulos HM , Sarmas JH , Talal N. : Is central nervous system involvement a systemic manifestation of primary Sjögren's syndrome? Rheum Dis Clin North Am 1993;19:909-912.

13.

Alexander EL , Beall SS , Gordon B. , et al: Magnetic resonance imaging of cerebral lesions in patients with the Sjögren syndrome. Ann Intern Med 1988;108:815-823.

14.

Berman JL , Kashii S. , Trachtman MS , Burde RM : Optic neuropathy and central nervous system disease secondary to Sjögren's syndrome in a child. Ophthalmology 1990;97:1606-1609.

15.

DeGuzman M. , Fishman MA , Lewis RA , et al: Chronic neurologic disease with visual, gait, and bladder problems in a male teenager. JPediatr 1998;132:742-747.

16.

Gerraty RP , McKelvie PA , Byrne E. : Aseptic meningoencephalitis in primary Sjögren's syndrome: Response to plasmapheresis and absence of CNS vasculitis at autopsy. Acta Neurol Scand 1993;88:309-311.

17.

Ohtsuka T. , Saito Y. , Hasegawa M. , et al: Central nervous system disease in a child with primary Sjögren syndrome. J Pediatr 1995;127:961-963.

18.

Uchigara T. , Yoshida S. , Tsukagoshi H. : Bilateral facial paresis with Sjögren's syndrome . J Neurol 1989;236:186.

19.

Keane JR : Bilateral seventh nerve palsy: Analysis of 43 cases and review of the literature . Neurology 1994;44:1198-1202.

20.

Sjögren H. : Zur Kenntnis der Keratoconjunctivitis sicca. II. Allgemeine Symptomalogie und Aetiologie. Acta Ophthalmol 1935;13:1-39.

Central Nervous System Sjögren's Syndrome in a Child: Case Report and Review of the Literature

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