The clinical heterogeneity of immune sensory and autonomic neuropathies with (or without) sicca

Abstract

In this issue of Brain , Mori and co-workers from several collaborating Japanese medical schools report on the clinical patterns of neuropathy associated with primary Sjogren's syndrome. The criteria for primary Sjogren's syndrome used were accepted by Japanese and American-European consensus groups: (i) symptoms of dry eyes (keratoconjunctivitis or sicca) or a dry mouth (xerostomia); (ii) objective evidence of keratoconjunctivitis (abnormal Schirmer's or Rose Bengal test) and (iii) evidence of lymphocytic sialoadenitis (e.g. from a lip biopsy) and the presence of antibodies (SS-A or SS-B). Of 92 patients reviewed, mean age ∼60 years, there was a strong female preponderance (76 of 92). The following varieties of neuropathy were recognized: sensory ataxia in 36 (39%); sensory painful in 18 (20%); multiple mononeuropathy in 11 (12%); multiple cranial neuropathies in 5 (5%); trigeminal neuropathy in 15 (17%); autonomic neuropathy in 3 (3%); and radiculoneuropathy in 4 (4%). Although each pattern of neuropathy has already been reported as associated with Sjogren's syndrome, the present series provides the frequency distribution for patterns of neuropathy based on evaluation of a single large cohort of such patients. The authors also provide information on characteristic electrophysiologic alterations, patterns of abnormal sweating and pathology. They emphasize the clinical heterogeneity of neuropathies associated with sicca and provide information on the distribution of lymphocytic inflammation in lip, parotid gland, spinal ganglia and nerve (vascular and perivascular). They suggest differences in response to immunotherapy by variety of neuropathy. Accepting the ideas that neuropathies associated with sicca are clinically heterogenous and that altered immunity is probably involved, not only in Sjogren's syndrome …