Neurologic Complications Associated with Sjögren’s Disease: Case Reports and Modern Pathogenic Dilemma

Abstract

Objectives. Sjögren's syndrome (SS) may be complicated by some neurological manifestations, generally sensory polyneuropathy. Furthermore, involvement of cranial nerves was described as rare complications of SS. Methods. We reported 2 cases: the first one was a 40-year-old woman who developed neuritis of the left optic nerve as presenting symptom few years before the diagnosis of SS; the second was a 54-year-old woman who presented a paralysis of the right phrenic nerve 7 years after the SS onset. An exhaustive review of the literature on patients with cranial or phrenic nerve involvements was also carried out. Results. To the best of our knowledge, our second case represents the first observation of SS-associated phrenic nerve mononeuritis, while optic neuritis represents the most frequent cranial nerve involvement detectable in this connective tissue disease. Trigeminal neuropathy is also frequently reported, whereas neuritis involving the other cranial nerves is quite rare. Conclusions. Cranial nerve injury is a harmful complication of SS, even if less commonly recorded compared to peripheral neuropathy. Neurological manifestations may precede the clinical onset of SS; therefore, in patients with apparently isolated cranial nerve involvement, a correct diagnosis of the underlying SS is often delayed or overlooked entirely; in these instances, standard clinicoserological assessment is recommendable.

The Clinical Characteristics of Primary Sjogren's Syndrome With Neuromyelitis Optica Spectrum Disorder in China

“… 4 However, NMO is now recognized as a spectrum disease that affects other regions of the CNS and includes more diverse clinical presentations because of the identification of a disease-specific autoantibody against aquaporin-4 (AQP4). 5 With increased numbers of emerging pSS patients with neuromyelitis optica spectrum disorder (NMOSD) cases reports, 6 – 8 studies of large Chinese populations have been rare. The aim of the present study was to assess the clinical characteristics, seroimmunological correlations, and risk factors for pSS with NMOSD in a Chinese cohort at a single center.…”

A Rare Neurological Involvement in Sjögren’s Syndrome: Abducens Nerve Palsy

“…The involvement of cranial nerves is relatively rare [2]. In the published literature, isolated abducens nerve palsy has been reported in only 1 patient [3]. The current report presents a case of pSS with the involvement of cranial nerves.…”

“…Cranial neuropathies show symptoms depending on the cranial nerve involved. Among cranial nerves, the trigeminal nerve and optic nerves are the most commonly involved [3]. Visual changes can be quite profound in Sjögren's.…”

Bilateral Carotid-Cavernous Fistulas: An Uncommon Cause of Pituitary Enlargement and Hypopituitarism

“…Anterior pituitary enlargement and palsy of cranial nerve VI in this patient initially raised the possibility of an inflammatory disorder of the pituitary or a pituitary adenoma. Indeed, hypophysitis, particularly granulomatous hypophysitis, can present as a sellar mass associated with cranial nerve palsies due to involvement of the adjacent cavernous sinus [ 29 ] as can occur with other inflammatory disorders of the pituitary including Sjögren's syndrome [ 30 , 31 ], sarcoidosis [ 32 ], Wegener's granulomatosis [ 33 , 34 ], tuberculosis [ 35 ], syphilis [ 36 , 37 ], and fungal infections [ 38 ]. Langerhans histiocytosis, germinoma, and lymphoma may also be associated with similar findings but often associated with central diabetes insipidus [ 39 – 41 ].…”