Open AccessAcute myeloneuropathy: An uncommon presentation of Sjögren′s syndrome
Author(s) -
Rajesh Verma,
Rakesh Lalla,
Tushar Patil,
Vinod Mehta
Publication year - 2013
Publication title -
annals of indian academy of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.427
H-Index - 31
eISSN - 1998-3549
pISSN - 0972-2327
DOI - 10.4103/0972-2327.120462
Subject(s) - medicine , myelopathy , hyperintensity , peripheral neuropathy , magnetic resonance imaging , spinal cord , endoneurium , nerve biopsy , pathology , radiology , anatomy , peripheral nerve , psychiatry , diabetes mellitus , endocrinology
Sjögren's syndrome is associated with central and peripheral nervous system involvement. The peripheral neuropathy is usually a sensory predominant neuropathy or a cranial neuropathy. Myelopathy is usually of focal, subacute, chronic or relapsing type. Acute myeloneuropathy as the predominant manifestation has not been described in the literature. We describe a middle aged woman who presented with an acute onset motor quadriparesis and bladder dysfunction. She had dryness of eyes and mouth for 8 months. Nerve conduction studies revealed motor axonal neuropathy and magnetic resonance imaging of spinal cord showed T2 hyperintensities involving entire cord. Mild perineural fibrosis, focal foamy changes in endoneurium and lymphocytic infiltration were seen in sural nerve biopsy specimen. Patient improved clinically after intravenous methylprednisolone therapy.