Neurologic Manifestations in Primary Sjögren Syndrome

Abstract

Neurologic involvement occurs in approximately 20% of patients with primary Sjögren syndrome (SS). However, the diagnosis of SS with neurologic involvement is sometimes difficult, and central nervous system (CNS) manifestations have been described rarely. We conducted the current study to describe the clinical and laboratory features of SS patients with neurologic manifestations and to report their clinical outcome. We retrospectively studied 82 patients (65 women and 17 men) with neurologic manifestations associated with primary SS, as defined by the 2002 American-European criteria. The mean age at neurologic onset was 53 years. Neurologic involvement frequently preceded the diagnosis of SS (81% of patients). Fifty-six patients had CNS disorders, which were mostly focal or multifocal. Twenty-nine patients had spinal cord involvement (acute myelopathy [n = 12], chronic myelopathy [n = 16], or motor neuron disease [n = 1]). Thirty-three patients had brain involvement and 13 patients had optic neuropathy. The disease mimicked relapsing-remitting multiple sclerosis (MS) in 10 patients and primary progressive MS in 13 patients. We also recorded diffuse CNS symptoms: some of the patients presented seizures (n = 7), cognitive dysfunction (n = 9), and encephalopathy (n = 2). Fifty-one patients had peripheral nervous system involvement (PNS). Symmetric axonal sensorimotor polyneuropathy with a predominance of sensory symptoms or pure sensory neuropathy occurred most frequently (n = 28), followed by cranial nerve involvement affecting trigeminal, facial, or cochlear nerves (n = 16). Multiple mononeuropathy (n = 7), myositis (n = 2), and polyradiculoneuropathy (n = 1) were also observed. Thirty percent of patients (all with CNS involvement) had oligoclonal bands. Visual evoked potentials were abnormal in 61% of the patients tested. Fifty-eight patients had magnetic resonance imaging (MRI) of the brain. Of these, 70% presented white matter lesions and 40% met the radiologic criteria for MS. Thirty-nine patients had a spinal cord MRI. Abnormalities were observed only in patients with spinal cord involvement. Among the 29 patients with myelopathy, 75% had T2-weighted hyperintensities. Patients with PNS manifestations had frequent extraglandular complications of SS. Anti-Ro/SSA or anti-La/SSB antibodies were detected in 21% of patients at the diagnosis of SS and in 43% of patients during the follow-up (mean follow-up, 10 yr). Biologic abnormalities were more frequently observed in patients with PNS involvement than in those with CNS involvement (p < 0.01). Fifty-two percent of patients had severe disability, and were more likely to have CNS involvement than PNS involvement (p < 0.001). Treatment by cyclophosphamide allowed a partial recovery or stabilization in patients with myelopathy (92%) or multiple mononeuropathy (100%). The current study underlines the diversity of neurologic complications of SS. The frequency of neurologic manifestations revealing SS and of negative biologic features, especially in the event of CNS invo...

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

“…Transverse myelitis has also been described in patients with pSS [3,82,83], and in a small percentage it may be accompanied by brain MS-like lesions or by optic neuritis making, at times, the distinction from a primary demyelinating disease such as MS or NMO rather difficult [31,80]. The meninges can also be affected, alone or in the context of meningoencephalitis [84], while in some cases a subacute encephalopathy presenting with memory loss, cognitive dysfunction, visual disturbances, and reduced concentration and attention has been noted [80].…”

“…The meninges can also be affected, alone or in the context of meningoencephalitis [84], while in some cases a subacute encephalopathy presenting with memory loss, cognitive dysfunction, visual disturbances, and reduced concentration and attention has been noted [80]. Symptoms such as seizures, headaches, psychiatric disturbances, and cognitive dysfunction have also been described but their true prevalence and association with the underlying syndrome is questionable [3,82]. Isolated cases of optic neuropathy, chorea, and motor neuron syndromes can also exist [82,83], but there does not appear to be any relationship or pathogenetic mechanism with the primary disease.…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…Involvement of the CNS is more likely in patients with lung involvement [201]. Although most of the neurological manifestations of Sjögren's syndrome are not severe enough to warrant ICU admission, acute myelopathy can occur and potentially necessitate ventilatory support [203,204]. Tumefactive brain lesions are also reported in Sjögren's syndrome [205,206].…”

Experience of intravenous immunoglobulin therapy in neuropathy associated with primary Sjögren's syndrome: A national multicentric retrospective study

“…Extraglandular involvement, such as peripheral neuropathy, might also occur. The spectrum of primary SS-related peripheral neuropathy is wide, includ-ing sensorimotor or painful sensory polyneuropathy, sensory ataxic neuropathy (sometimes called ganglionopathy), trigeminal neuropathy, autonomic neuropathy, multiple mononeuropathy, polyradiculoneuropathy, and multiple cranial neuropathy (1)(2)(3)(4). The pathogenic mechanism responsible for most forms of SS-associated neuropathy remains unknown.…”

“…When motor symptoms are present, necrotizing vasculitis diagnosed by neuromuscular biopsy results must be considered and, when present, usually may respond to immunosuppressive therapy (5). However, although it represents the majority of peripheral involvement in SS, peripheral neuropathy associated with another pathogenic mechanism (such as lymphocytic infiltration, loss of neuronal cell bodies, and clinical correspondence to sensory distal neuropathy or ganglionopathy) lacks efficient treatment (1)(2)(3)(4)6). In these cases, the best therapeutic option is not known (6), and corticosteroids, plasmapheresis, and conventional immunosuppressive drugs have been unsuccessful in comparison with their recognized benefit on the other systemic complications of SS (6).…”