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  • Title: [A case of primary Sjögren's syndrome with acute transverse myelopathy and polyneuropathy as the initial manifestations].
    Author: Yanagihara C, Nakaji K, Nishimura Y.
    Journal: Rinsho Shinkeigaku; 2001 Jan; 41(1):50-5. PubMed ID: 11433768.
    Abstract:
    We report a case of primary Sjögren's syndrome with acute transverse myelopathy and polyneuropathy as the initial manifestations. A 72-year-old man developed acute right hemiparesis and his symptoms deteriorated to quadriparesis in four days. A cervical spinal MRI showed an extensive intraparenchymal lesion with high T2-weighted signal intensity, gadolinium enhancement, and cord swelling. An electromyographic study and a sural nerve biopsy showed severe axonal degeneration. The patient complained of thirst and a salivary gland biopsy revealed inflammatory changes, while salivary gland scintigraphy showed diminished secretion. These findings led to a diagnosis of Sjögren's syndrome. Only one case of Sjögren's syndrome with acute transverse myelopathy as the initial manifestation has been reported. In our case, however, polyneuropathy was observed in addition to transverse myelopathy as the initial manifestation. Even if prominent sicca symptoms are absent, all patients with acute transverse myelopathy and polyneuropathy should be carefully examined with considerations of possible presence of Sjögren's syndrome.
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