Severe sensory neuronopathy responsive to infliximab in primary Sjögren’s syndrome

Caroyer, Jean-Marc; Manto, Mario; Steinfeld, Serge

doi:10.1212/wnl.59.7.1113

Cited by 52 publications

(25 citation statements)

References 7 publications

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“…Several treatment regimens have been applied, however due to the rarity of this disorder randomized controlled trials are lacking. There are reports of the use of intravenous immunoglobulin (IVIg) [30], plasmapheresis [31], D-penicillamine [32], infliximab [33] and interferon-a [34]. There is some preliminary evidence that rituximab treatment, when administered early, may be effective [35,36].…”

Section: Sensory Ataxic Neuropathy/ganglionopathymentioning

confidence: 99%

Peripheral neuropathies in Sjögren’s syndrome: A critical update on clinical features and pathogenetic mechanisms

Pavlakis

Alexopoulos

Kosmidis

et al. 2012

Journal of Autoimmunity

147

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Section: Sensory Ataxic Neuropathy/ganglionopathymentioning

confidence: 99%

Peripheral neuropathies in Sjögren’s syndrome: A critical update on clinical features and pathogenetic mechanisms

Pavlakis

Alexopoulos

Kosmidis

et al. 2012

Journal of Autoimmunity

147

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“…Although results using immunosuppressive agents and plasmapheresis have been disappointing or equivocal, infusions of infliximab, a TNF␣ blocker, at a dosage of 3 mg/kg should be considered for IVIg nonresponders, based on observation of one patient who showed a marked improvement of clinical and neurophysiological deficits. 8 Autologous stem-cell transplantation or immunomodulatory gene transfer in the salivary glands are emerging as therapeutic options. 61 …”

Section: Dysimmune Sensory Ganglionopathiesmentioning

confidence: 99%

Clinical features and pathophysiological basis of sensory neuronopathies (ganglionopathies)

Antoine²,

2004

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Sensory ganglionopathies have a frequent association with neoplastic disorders (paraneoplastic subacute sensory neuronopathy, or SSN) or dysimmune disorders (Sjögren's syndrome, SS; Miller Fisher syndrome; and Bickerstaff's brainstem encephalitis, BBE), with drugs, such as cisplatin or pyridoxine, and with inherited disorders with degeneration of dorsal root ganglion cells. Unsteady gait and pseudoathetoid movements of the hand are the distinctive signs encountered in these disorders. The chronic disorders are characterized by non-length-dependent abnormalities of sensory nerve action potentials (SNAPs) and differ from other sensory neuropathies in showing a global, rather than distal, decrease in SNAP amplitudes. This review focuses on recent advances in defining the mechanisms involved in sensory ganglionopathies. Specific topics include a summary of their clinical features, pathological findings, and immunopathology. In SSN, early diagnosis by the detection of anti-Hu antibodies and early treatment of the cancer gives the best chance of stabilizing the disorder. In SS sensory ganglionitis, response to treatment has been disappointing, but immunomodulating treatments are emerging. The immunological profile common to BBE and Fisher syndrome supports a common pathogenesis. In toxic sensory neuronopathy, no treatment is available. The differential diagnosis involves separating sensory ganglionopathies from other ataxic polyneuropathies, such as infectious neuropathies, sensory neuropathies with various autoantibodies, and the neuropathies seen in celiac disease.

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“…It presents with impaired kinesthetic awareness and proprioception, and leads to severe sensory ataxia with pseudoathetotic hand movements due to involvement of large ganglionic neurons [92]. A small case series reports successful management with IVIg (grade 3D) [93], while treatment with rituximab, plasmapheresis, infliximab, and interferon (IFN)-α were encouraging in other series (grade 3D) [94][95][96][97]. In our experience, this is a difficult entity to treat and quite disabling.…”

Section: Sjögren's Syndromementioning

confidence: 68%

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

et al. 2016

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Neurological involvement is relatively common in the majority of systemic autoimmune diseases and may lead to severe morbidity and mortality, if not promptly treated. Treatment options vary greatly, depending on the underlying systemic pathophysiology and the associated neurological symptoms. Selecting the appropriate therapeutic scheme is further complicated by the lack of definite therapeutic guidelines, the necessity to differentiate primary neurological syndromes from those related to the underlying systemic disease, and to sort out adverse neurological manifestations caused by immunosuppressants or the biological agents used to treat the primary disease. Immunotherapy is a sine qua non for treating most, if not all, neurological conditions presenting in the context of systemic autoimmunity. Specific agents include classical immune modulators such as corticosteroids, cyclophosphamide, intravenous immunoglobulin, and plasma exchange, as well as numerous biological therapies, for example antitumor necrosis factor agents and monoclonal antibodies that target various immune pathways such as B cells, cytokines, and co-stimulatory molecules. However, experience regarding the use of these agents in neurological complications of systemic diseases is mainly empirical or based on small uncontrolled studies and case series. The aim of this review is to present the state-of-the-art therapies applied in various neurological manifestations encountered in the context of systemic autoimmune diseases; evaluate all treatment options on the basis of existing guidelines; and compliment these data with our personal experience derived from a large number of patients.

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Severe sensory neuronopathy responsive to infliximab in primary Sjögren’s syndrome

Cited by 52 publications

References 7 publications

Peripheral neuropathies in Sjögren’s syndrome: A critical update on clinical features and pathogenetic mechanisms

Peripheral neuropathies in Sjögren’s syndrome: A critical update on clinical features and pathogenetic mechanisms

Clinical features and pathophysiological basis of sensory neuronopathies (ganglionopathies)

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

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