Upper Motor Neuron Syndrome Associated with Subclinical Sjoegren's Syndrome

Hagiwara, Koichi; Murai, Hiroyuki; Ochi, Hirofumi; Osoegawa, Manabu; Sekiya, Hiroshi; Ohyagi, Yasumasa; Kira, Jun Ichi

doi:10.2169/internalmedicine.47.0846

Cited by 16 publications

(11 citation statements)

References 10 publications

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“…The symptoms of both patients were alleviated following treatment with high-dose corticosteroids. In addition, in 2008, Hagiwara et al 11 presented two patients with primary lateral sclerosis-like upper motor neuron disease with accompanying subclinical Sj€ ogren's syndrome. Both patients showed significant neurological improvement after an initial course of intravenous immunoglobulin therapy.…”

Section: Discussionmentioning

confidence: 99%

Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report

et al. 2020

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Sjögren’s syndrome is an autoimmune disease that can affect multiple systems. Sjögren’s syndrome with motor neuron disease is rarely reported. Herein, we describe a patient with rapidly progressive motor neuron disease secondary to Sjögren’s syndrome. A 42-year-old woman was admitted to our hospital with a 2-month history of progressive limb weakness. Neurological assessment revealed fasciculation in the lower limbs and amyotrophy in the bilateral supraspinatus, interosseous, and thenar muscles. Serological examination and labial gland biopsy revealed Sjögren’s syndrome. In addition, electromyography demonstrated neurogenic damage to the upper and lower limbs. The patient received a short course of high-dose corticosteroids, intravenous immunoglobulins, and immunosuppressant treatment, including a weekly dose of 0.4 g cyclophosphamide and a daily dose of 0.2 g hydroxychloroquine. However, the patient’s limb weakness was further aggravated and her respiratory function was compromised. Electromyography re-examination demonstrated extensive neurogenic damage, and she was diagnosed with Sjögren’s syndrome with motor neuron disease. The patient died of respiratory failure after 2 months. We suggest that more effective maintenance treatments should be sought. Further investigation is required to elucidate the association between autoimmune motor neuron disease and Sjögren’s syndrome.

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Section: Discussionmentioning

confidence: 99%

Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report

et al. 2020

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“…Liao Qin et al [5] reported 4 cases of amyotrophic lateral sclerosis with Sjögren's syndrome. Hagiwara et al [6] reported two cases of motor neuron damage-based ALS with subclinical Sjögren's syndrome. Salachas [7] reported three Sjogren's syndrome patients with motor neuron disease, two of whom met the proposed or probable ALS, and the other was eligible for primary lateral sclerosis in 1988.…”

Section: Discussionmentioning

confidence: 99%

“…As Salachas [7] reported, 2 of 3 patients whose neurological symptoms were shortterm relieved after glucocorticoid treatment. While Hagiwara et al [6] reported the treatment with methylprednisolone pulse therapy failed to improve patients' neurological symptoms, other immunomodulating therapies, including plasma exchange and cyclophosphamide pulse therapy still couldn't stop the symptoms progressing. But intravenous immunoglobulin therapy (IVIG) with a dose of 0.4 g/kg per day for 5 consecutive days significantly improved patients' weakness and gait disorder, and the improvement presented within 3-4 days of initiating therapy.…”

Section: Discussionmentioning

confidence: 99%

Treatment and Rehabilitation of Motor Neuron Syndrome Associated with Sjögren's Syndrome: A Case Report

Haixia¹,

Du²

2017

J Speech Pathol Ther

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We report a patient with motor neuron syndrome accompanying Sjögren's syndrome. The patient showed progressive quadriparesis and slurred speech, but neither sensory involvement nor detrusor dysfunction was noted. EMG displayed upper and lower motor neuron damage. Although sicca symptom was not too obvious, parotid gland and submandibular gland ultrasound showed diffuse lesions. The Laboratory tests also displayed markedly elevated levels of Anti-SSA and anti-Ro 52 antibody. After a course of intravenous immunoglobulin therapy and 3 weeks of comprehensive rehabilitation, the patient showed significant improvements in terms of speech, muscle strength, trunk balance, posture control and ability of daily life. We suggested that the possibility of autoimmune-mediated motor neuron damage should be considered, when patients with motor neuron syndrome accompanying Sjögren's syndrome. And they may benefit from intravenous immunoglobulin therapy and appropriate rehabilitation.

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“…Neuropathies associated with SjS may be underreported as they can convalesce prior to sicca symptoms or without the patient realizing they have sicca symptoms, which in either case generally removes the necessity to perform further testing [ 55 ]. However, misdiagnosis is common due to these reasons [ 56 – 60 ] and may prevent an appropriate course of treatment. Recognizing the extent to which neuropathies are present in pSjS may help both neurologists and rheumatologists correctly diagnose and treat this aspect of the disease.…”

Section: Discussionmentioning

confidence: 99%

Beyond the Glands: An in-Depth Perspective of Neuropathy in Sjögren’s Syndrome

Voigt

Sukumaran

Nguyen³

2014

Rheumatology (Sunnyvale)

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Primary Sjögren’s Syndrome (pSjS) is an autoimmune disease characterized by sicca (xerophthalmia or xerostomia) symptoms, anti-SS-A (Ro) or anti-SS-B (La) autoantibodies, and lymphocytic infiltrates in the exocrine glands. Disease incidence is estimated to be 0.1–3% of the general population with 0.4–3.1 million individuals in the US with women being nine times more likely to be afflicted with SjS than men. The frequency continues to rise accompanied with the multi-factorial etiology making it a challenging disease to manage and treat. Treatment of this disease remains problematic due to the lack of therapeutic treatments relying on replacement therapies such as artificial saliva and eye lubricants or immunosuppressive agents. To further complicate the management of the disease, there are number of multi-systemic manifestations specifically peripheral neuropathy associated with later stage of disease onset. Increasingly, there is mounting evidence that suggests the involvement of central nervous system. It remains to be determined the underlying cause and effect of the dysregulated immune response and the neuropathy associated with SjS. In this review, we provided an in-depth look at key neurological dysfunctions documented to occur in pSjS. Specifically, we discussed the prevalence, symptomology, and current treatments.

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Upper Motor Neuron Syndrome Associated with Subclinical Sjoegren's Syndrome

Abstract: Abstract

Cited by 16 publications

References 10 publications

Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report

Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report

Treatment and Rehabilitation of Motor Neuron Syndrome Associated with Sjögren's Syndrome: A Case Report

Beyond the Glands: An in-Depth Perspective of Neuropathy in Sjögren’s Syndrome

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