Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjögren’s syndrome

Takahashi, Yuya; Takata, Tadayuki; Hoshino, M; Sakurai, M; Kanazawa, I

doi:10.1212/01.wnl.0000046680.47883.7d

Cited by 103 publications

(65 citation statements)

References 8 publications

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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: 70%

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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Section: Sjögren's Syndromementioning

confidence: 70%

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

et al. 2016

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“…Some authors have suggested that the clinical benefit of IVIG therapy may appear rapidly after the first or second infusion in ataxic sensory neuropathy (10,13). Consequently, further studies are necessary to investigate the optimal number of IVIG courses necessary to definitively assess the efficacy or the failure of the treatment, as well as to know how long to continue the treatment.…”

Section: Discussionmentioning

confidence: 99%

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

Rist

Sellam

Hachulla

et al. 2011

Arthritis Care & Research

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Objective. Sjö gren's syndrome (SS)-related peripheral neuropathy is responsible for disability, but no treatment has been shown to improve its outcome. In some cases, intravenous immunoglobulin (IVIG) therapy has been associated with some benefit. In this study, we investigated the effectiveness of IVIG in SS-related peripheral neuropathy. Methods. We assessed the efficacy and tolerance of IVIG in 19 patients with primary SS-related neuropathy without any necrotizing vasculitis in a retrospective national multicentric study. The evaluation of the response was assessed using the disability Modified Rankin Scale (MRS) and a global evaluation by the practitioner. Results. Eight patients (42%) exhibited a decrease of the MRS score corresponding to a clinical improvement, 10 patients (52%) exhibited a stable MRS score, and 1 patient (6%) showed an increase of MRS score. According to the global evaluation by the practitioner, 9 (47%) of the 19 patients were improved, 6 patients (31%) were stable, and 4 patients (21%) worsened. All the patients with sensorimotor (n ‫؍‬ 5) or nonataxic sensory neuropathy (n ‫؍‬ 4) were improved or stabilized. However, among the patients with ataxic neuropathy (n ‫؍‬ 9), only 2 improved and 4 worsened. Ten of the 13 patients treated with corticosteroids could have had the prednisone dosage decreased from 15 mg/day (range 7.5-60) to 10 mg/day (range 5-20) with IVIG. Only 1 patient stopped the treatment after 1 dose because of a minor side effect and lack of initial efficacy. Conclusion. IVIG may be useful in the treatment of SS-associated sensorimotor neuropathies or nonataxic sensory neuropathy without any necrotizing vasculitis. The benefit of such therapy in the SS-related ataxic neuropathy seems less clear.

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“…We Di f f u s e p u n c t a t e s i a l e c t a s i s i s s e e n i n t h e l e f t p a r o t i d g l a n (Fig. 3) (9,10). In contrast to methylprednisolone pulse therapy and plasma exchange, IVIG has multiple mechanisms of action, including suppression of the opsonin effect, neutralization of cytokines, and reduced production of antibodies (11) …”

Section: A Wide Variety Of Neurological Complications Are Described Imentioning

confidence: 92%

Upper Motor Neuron Syndrome Associated with Subclinical Sjoegren's Syndrome

et al. 2008

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Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjögren’s syndrome

Cited by 103 publications

References 8 publications

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

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

Upper Motor Neuron Syndrome Associated with Subclinical Sjoegren's Syndrome

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