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Myelitis and Sjogren's Syndrome.
Sjogren's syndrome is a rare cause of myelitis
Objectives:
To present a case of longitudinally extensive transverse myelitis (LETM) in presence of SSA- Sjogren antibodies
Methods:
33 years old Female with history of Hashimoto thyroiditis and Shingles in childhood presenting with bilateral legs numbness and tingling, and subsequent bilateral lower extremities weakness and urinary retention. Neurological exam revealed symmetric proximal weakness of both legs with preserved reflexes and sensory level to mid-thoracic spine. Magnetic Resonance Imaging (MRI) of the spine showed hyperintense spinal cord lesion from C4-T1 without enhancement post-gadolinium administration. MRI brain showed demyelinating lesions perpendicular to right lateral ventricle and within the pons and ventral medulla, and an enhancing lesion in white matter of right parietal lobe. CSF showed lymphocytic pleocytosis and elevated protein.Patient was started on antibiotics/antiviral and IVIG; high-dose solumedrol was then added. She was transitioned to oral steroids and azathioprine after completion of five days course of pulse-therapy with steroids and IVIG. The extensive laboratory workup showed negative Aquaporine-4 antibodies (Aquap-4 Ab), no oligoclonal bands, normal IgG index, negative angiotensin-converting enzyme, negative human T- lymphotropic viruses I/II antibodies, negative antinuclear antibody (ANA), no antibodies to myeloperoxidase, and elevated myelin basic protein and SSA- Sjogren antibodies. Aquap-4 Ab was rechecked on outpatient basis, and was again negative. Patient continued with clinical improvement. Repeat MRI of C/T spine and brain showed resolution of all lesions. We suggest that LETM in this case was associated with Sjogren’s syndrome (SS) without systemic manifestations.
Results: The prevalence of myelitis in patients with Sjogren’s syndrome is unknown, but is possibly less than 1% (Vincent,2003). We searched relevant literature of the reported myelitis cases associated with Sjogren’s syndrome, and found several cases, presenting with acute myelitis with or without systemic manifestations of SS and lack of Aquap-4 antibodies, similarly to our patient. Our case is another example of an acute LETM and evidence of demyelinating brain lesions in the absence of typical serological findings, and in presence of SSA- Sjogren antibodies, with good response to immunosuppressive therapy.
Conclusions: Sjogren’s syndrome is a rare cause of myelitis,that needs to be further investigated.