Objective: This study was to clarify the neuropathological findings of acute encephalomyelopathy with Sjögren's syndrome.
Methods: We examined an autopsied case of acute encephalomyelopathy with Sjögren's syndrome.
Case report: A 40-year-old woman developed acute myelopathy and brainstem dysfunction. Magnetic resonance imaging (MRI) revealed high-intensity lesions on T2-weighted axial images (T2WI) in the medulla oblongata and cervical spinal cord. We established a diagnosis of Sjögren's syndrome (SjS) according to the European Community criteria. The patient was treated with intravenous methylprednisolone (500 mg/day) for three days, followed by oral prednisolone. Although her neurological symptoms improved, her general condition deteriorated after the onset of acute colonic pseudo-obstruction and she died of multiple organ failure associated with hemophagocytosis.
Results: Autopsy showed atrophy of the secretory glands and an accumulation of lymphocytes around the ducts, confirming the diagnosis of Sjögren's syndrome. Neuropathological examination revealed multifocal lesions in the cervical spinal cord and medulla, along with scattered perivascular lymphocytic infiltration. In addition, there was demyelination, spongy change and axonal swelling in the white matter, but no remarkable vasculitic changes were seen in the central nervous system.
Conclusion: Although the steroid therapy may have had a significant influence, the main pathological finding in this case was not vasculitis, but rather axonal degeneration with spongy change and axonal swelling.