[Central and peripheral neurologic lesions in primary Gougerot-Sjögren syndrome. Clinicopathological study of a case]

Abstract

A 51 year old woman presented with cerebellar ataxia and paralysis of conjugate lateral movement of the eyes. She had xerophtalmia and xerostomia. Biopsy of minor salivary glands showed mononuclear infiltrates typical of Sjögren's syndrome. Peripheral neuropathy was of the axonal type, and nerve biopsy showed a vasculitis made of a mononuclear infiltrate with only a few polymorphonuclears. The patient deteriorated despite corticosteroid therapy, plasma exchange and cyclophosphamide. Ciclosporin seemed to be transitorily beneficial. Epileptic seizures (auditory hallucinations) occurred. MRI showed hypersignal in the right temporal lobe. At post-mortem examination, a small ischaemic lesion with a large lymphocytic infiltrate was present in the left putamen. Neuronal loss and gliosis with some rod cells and glial stars were found in the brainstem, dentate nucleus and internal part of the right temporal lobe. Inflammatory changes were limited. The posterior columns of the spinal cord were pale, and some residual nodules of Nageotte were present in the spinal ganglia. These lesions were similar to those seen in encephalomyelitic syndromes associated with carcinoma. Lesions of the peripheral nerves were associated with vasculitis but changes in the central nervous system were morphologically similar to those observed as remote effects of carcinoma.