Neurologic Complications Associated with Sjögren’s Disease: Case Reports and Modern Pathogenic Dilemma
Table 2
Synoptic view of cranial nerve involvement in the course of Sjögren’s syndrome (SS).
Cranial nerve(s)
Frequency of involvement (%)
Key points
I
1
Probably underdiagnosed because of the concomitant olfactory dysfunction secondary to mucosal dryness.
II
46
Possible feature of neuromyelitis optica spectrum disorder, associated with SS, otherwise, autoimmune neuritis in patients with possibly misdiagnosed SS.
III, IV, VI
12
Clinically evident as diplopia, this neuritis is generally responsive to steroids.
V
38
Autoimmune damage of the Gasser ganglion could be suspected. Neuritis tends to be recurrent or to stabilize and to be less frequently responsive to steroids than other cranial neuritis.
VII
5
Neuritis isolated or associated with another nerve involvement, with good prognosis.
VIII
3
Neuritis rarely reported as tinnitus, often isolated.
IX, X, XI, XII
4
Transient, often recurrent neuritis episodes, generally responsive to steroids. The IX nerve was always reported in cases of multineuritis, while the XI was never mentioned in literature.
Cranial nerves are named with their Roman numerals. The frequencies of involvement refer to the cases illustrated in Table 1, considering a total of 267 SS patients with cranial neuritis.