Neurologic involvement in scleroderma: A systematic review

Amaral, Tiago Nardi; Peres, Fernando Augusto; Lapa, Aline Tamires; Marques-Neto, João Francisco; Appenzeller, Simone

doi:10.1016/j.semarthrit.2013.05.002

Cited by 195 publications

(187 citation statements)

References 200 publications

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“…Clinical and laboratory studies have revealed that increased vascular permeability secondary to perivascular inflammatory cell infiltration plays an essential role in the pathogenesis of the disease, and that vascular structures are the main target (5). Perivascular infiltration, gliosis, thickening of vessel walls, thrombus and focal parenchymal necrosis were observed in biopsies of nine patients (6). Although all forms of scleroderma are similar in pathophysiology, they show clinical differences in terms of organ involvement.…”

Section: Introductionmentioning

confidence: 89%

Transient Gyral Enhancement After Epileptic Seizure in a Patient with CREST Syndrome

Açıkgöz¹,

Demirel²,

Güdül³

et al. 2017

tnd

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Limited scleroderma, also termed CREST syndrome (calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia), is a form of scleroderma which is an autoimmune connective tissue disease. Neurological involvement is less common than other system involvements in scleroderma. Epileptic seizure is a rare neurological complication. Gyral enhancement is not an expected finding during disease. Gyral enhancement is contrast enhancement of superficial brain parenchyma and it usually occurs as a result of vascular, inflammatory, infectious and neoplastic processes. A-sixty-seven-year old female patient, who was diagnosed as CREST syndrome for three years was admitted to our hospital. She had an epileptic seizure three days ago and behavioral changes after the seizure. There was not epilepsy diagnosis in her medical history. In neurological examination; she was apathic and deep tendon reflexes were hyperactive, Hoffman's and Babinski reflexes were positive on the right side of her body. In laboratory studies; anti-nuclear antibody was positive at 1/1000 and anti-centromere antibody was positive. On contrast-enhanced cranial magnetic resonance imaging (MRI); gyral enhancement was observed in the left parieto-occipital region. Cerebrospinal fluid tests were within normal limits. Electroencephalography demonstrated diffuse cerebral dysfunction. We administrated antiepileptic drug to the patient during hospitalization. On the follow up, apathy and restriction of the cooperation were improved. Epileptic seizure did not recur. After ten days, in the control cranial MRI, gyral enhancement was reduced significantly. Neurological involvement in CREST syndrome is rare and epileptic seizures have been appeared in a limited number in the literature. Inflammatory and vascular processes can cause gyral enhancement, but also it must be kept in mind that gyral enhancement can be observed transiently after epileptic seizure.Keywords: CREST syndrome, epileptic seizure, gyral enhancement Sınırlı skleroderma; otoimmün bağ dokusu hastalığı olan sklerodermanın bir formudur ve CREST sendromu (kalsinozis kutis, Raynaud fenomeni, özefageal dismotilite, sklerodaktili ve telenjiektazi) olarak da adlandırılır. Sklerodermada nörolojik tutulum diğer sistem tutulumlarına göre daha az görülür. Epileptik nöbet ise nadir görülen nörolojik komplikasyonlardandır. Hastalıkta giral kontrastlanma beklenen bir bulgu değildir. Giral kontrastlanma, beyin parankiminin yüzeyel kontrast tutulumudur ve genellikle vasküler, enflamatuvar, enfeksiyöz ve neoplastik süreçler sonucu ortaya çıkar. Üç yıldır CREST sendromu tanısı olan 67 yaşında kadın hasta hastanemize başvurdu. Üç gün önce epileptik nöbet geçirmişti ve nöbet sonrasında davranış değişiklikleri mevcuttu. Özgeçmişinde epileptik nöbet hikayesi yoktu. Nörolojik muayenesinde; apatikti ve kooperasyonu kısıtlı idi. Derin tendon refleksleri yaygın olarak artmıştı, sağda Hoffman ve Babinski refleksleri pozitifti. Laboratuvar incelemelerinde anti-nükleer antikor pozitifti (1/1000) ve...

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Section: Introductionmentioning

confidence: 89%

Transient Gyral Enhancement After Epileptic Seizure in a Patient with CREST Syndrome

Açıkgöz¹,

Demirel²,

Güdül³

et al. 2017

tnd

View full text Add to dashboard Cite

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“…Other associated symptoms are psychiatric disturbances and depression [107]. Brain lesions include focal subcortical calcifications, meningocortical alterations, white matter lesions, and gray matter atrophy [2,108].…”

Section: Sclerodermamentioning

confidence: 99%

“…More complex manifestations, such as cognitive dysfunction, headaches, myokymias, dystonia, and CNS vasculitis can occur, but mainly in the context of Parry Romberg syndrome (or hemifacial atrophy), which is considered a Bcoup de sabrev ariant [2,109]. Treatment remains empirical, and immunotherapy with steroids, cyclophosphamide, MTX, ciclosporin, IFN-γ, and Dpenicillamine is proposed by some studies but mainly for the treatment of aggressive skin and bone destruction (grade 3D) [107,110,111]. The effect of the abovementioned treatments in neurological involvement remains to be clarified.…”

Section: Sclerodermamentioning

confidence: 99%

“…Similarly to pSS, multiple mononeuropathies, peripheral sensorimotor neuropathy, autonomic nervous system dysfunction, and cranial neuropathies (mainly trigeminal) have been described in patients with systemic and localized scleroderma [107]. Proposed immunotherapies remain empirical like those in pSS, and include steroids, cyclophosphamide, AZA mycophenolate mofetil, and IVIg, based on a case-by-case evaluation (grade 3D) [107].…”

Section: Sclerodermamentioning

confidence: 99%

“…Proposed immunotherapies remain empirical like those in pSS, and include steroids, cyclophosphamide, AZA mycophenolate mofetil, and IVIg, based on a case-by-case evaluation (grade 3D) [107].…”

Section: Sclerodermamentioning

confidence: 99%

See 2 more Smart Citations

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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Morphoea (Localized Scleroderma)

Orteu

2016

Rook's Textbook of Dermatology, Ninth Edition

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Morphoea encompasses a group of related conditions characterized by varying degrees of sclerosis, fibrosis and atrophy in the skin and subcutaneous tissues, sometimes extending deeply into the muscle, bone, eye and brain. Extracutaneous manifestations occur in up to 25% of cases but in contrast to systemic sclerosis (SSc), no internal organ fibrosis or vascular changes occur. Antinuclear antibody positivity is common but the specific autoantibodies seen in SSc are rarely present. There is no increased mortality, but substantial morbidity may occur as a result of joint contractures, facial and limb asymmetry, extracutaneous manifestations and the psychological impact of the condition. Although previously considered self‐limiting, there is now emerging evidence that a protracted, relapsing–remitting course may be common. The key to successful treatment is the involvement of a multidisciplinary team and the initiation of therapy during the active inflammatory stage, before significant damage has occurred. Topical and phototherapies are appropriate in superficial forms of morphoea, whilst a majority of linear, generalized and deep forms require systemic therapy.

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Neurologic involvement in scleroderma: A systematic review

Cited by 195 publications

References 200 publications

Transient Gyral Enhancement After Epileptic Seizure in a Patient with CREST Syndrome

Transient Gyral Enhancement After Epileptic Seizure in a Patient with CREST Syndrome

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

Morphoea (Localized Scleroderma)

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