Clinical and pathological characteristics of primary Sj(o)gren's syndrome-associated neurological lesions / 中华全科医师杂志

ABSTRACT

Objective To explore clinical and pathological characteristics of neurological lesions in primary Sj(o)gren's syndrome (pSS).Methods Ten patients diagnosed as pSS with nervous system involvement were analyzed retrospectively with autoimmune antibody test,cerebrespinal fluid examination and Schivmer's test,six pSS patients with peripheral nervous system lesions (PNS-pSS) were examined with electromyography and nerve conduction velocity and four pSS patients with central nervous system lesions (CNS-pSS) were examined with cranial MRI to analyze their clinical and pathological characteristics.Salivary gland biopsy was performed for all the ten patients,sural nerve biopsy for those with PNS-pSS and brain biopsy or spinal autopsy for those with CNS-pSS.Results Clinically,six patients with PNS-pSS presented symmetrical sensory-motor peripheral neuropathy or pure sensory peripheral neuropathy and four patients with CNS-pSS presented multiple sclerosis,subacute transverse myelitis,encephalitis,pituitary stroke and acute meningitis with varied lesions in the myelin sheath and axon by electromyography and motor nerve conduction velocity.Cranial MRI showed lesions in the white matter of the brain,spinal cord and abnormal signals in the pituitary.Pathologically,sural nerve biopsy showed typical vasculitis and nonvasculitis characterized as degeneration of the axon and myelin sheath,salivary acinar gland biopsy showed its atrophy and infiltration with lymphocytes,and brain biopsy showed demyelination of the brain and spinal white matter,as well as infiltration of lymphocytes surrounding the veins,typical vasculitis and bleeding and necrosis of the pituitary.Conclusions Both central and peripheral nervous systems can be involved in pSS,with complicated clinical manifestations.Electrophysiology,cerebrospinal fluid tests and histopathological examinations by biopsy are essential in diagnosis for neurological lesions in patients with pSS.Inflammatory reaction of the blood vessels and tissues mediated by cell immunity may be involved in mechanism of its neurological lesions.