Atypical neurologic complications in patients with primary Sjögren's syndrome: report of 4 cases.
BACKGROUND
Neurologic involvement occurs in approximately 25% of patients with primary Sjögren's syndrome. Manifestations are diverse and can affect the entire neuroaxis. Central nervous system dysfunction involves the brain as well as the spinal cord and may recur over time. Due to a variety of presentations, Sjögren's syndrome with neurologic involvement may be difficult to diagnose.


METHODS
We report 4 cases of patients with primary Sjögren's syndrome who presented with atypical neurologic manifestations.


RESULTS
The first case describes a patient with a pseudotumoral lesion. The second patient was a 54-year-old woman suffering from a multiple mononeuropathy. The third case describes a 66-year-old man whose primary Sjögren's syndrome presented as progressive multiple sclerosis, and the fourth case reports a 57-year-old woman patient suffering from myelitis along with progressive cognitive disorders.


CONCLUSIONS
Neurologic impairment in Sjögren's syndrome is probably underestimated and the diagnosis is often delayed. Primary Sjögren's syndrome should be suspected in patients presenting with atypical clinical and radiologic neurologic manifestations.
Michel
Laure
L
Service de Neurologie, Centre Hospitalier Universitaire de Nantes, Hôpital Laennec, Nantes Cedex, France. Laure.michel@univ-nantes.fr
Toulgoat
Frédérique
F
Desal
Hubert
H
Laplaud
David Axel
DA
Magot
Armelle
A
Hamidou
Mohamed
M
Wiertlewski
Sandrine
S
D000368:Aged; D001921:Brain / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D020422:Mononeuropathies / Q000209:etiology* / Q000473:pathology; D009103:Multiple Sclerosis / Q000209:etiology* / Q000473:pathology; D009187:Myelitis / Q000209:etiology* / Q000473:pathology; D011559:Pseudotumor Cerebri / Q000209:etiology* / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000473:pathology