Neurologic Manifestations of Progressive Systemic Sclerosis

Averbuch‐Heller, Lea; Steiner, Israel; Abramsky, Oded

doi:10.1001/archneur.1992.00530360094024

Cited by 116 publications

(57 citation statements)

References 16 publications

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“…The involvement of cerebral vessels would therefore also be expected. The infrequent incidence of clinical central nervous system disease has been attributed by some to the paucity of connective tissue in the brain with sparseness of media and adventitia in cerebral arteries [5].…”

Section: Discussionmentioning

confidence: 99%

Cerebral Aneurysms in Scleroderma

Kaku¹,

Kouda²,

Yoshimura³

et al. 2004

Cerebrovasc Dis

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Section: Discussionmentioning

confidence: 99%

Cerebral Aneurysms in Scleroderma

Kaku¹,

Kouda²,

Yoshimura³

et al. 2004

Cerebrovasc Dis

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“…In the patient of Hauser et al [15]in whom the disease started at the age of 15 years with inflammatory skin changes following Blaschko’s lines on the trunk, evolved within 17 years, at first morphea and then pigmented atrophies indistinguishable from atrophoderma of Moulin. However, in spite of a possible similarity and overlaps, the pathogenic mechanism of these two disorders is quite different since – in contrast to atrophoderma of Moulin – scleroderma is distributed segmentally and/or related to the various levels of the nervous system [16, 17, 18]. …”

Section: Discussionmentioning

confidence: 99%

Primary Atrophic Profound Linear Scleroderma

Błaszczyk¹,

Krysicka-Janiger²,

Jabłońska³

2000

Dermatology

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We present 3 unusual cases of deep linear, primary atrophic scleroderma, not preceded by inflammatory reaction and sclerosis, involving the subcutis and deeper tissues. These cases differ in the course and prognosis from typical profound scleroderma since they do not lead to disfiguration and crippling deformities. In contrast to the atrophies left after regression of morphea or linear scleroderma, they do not involve the dermis, which does not show discoloration or changes in texture. Infiltrates in the endomysium, involvement of deeper tissues and the progressive character of the disease argue for atypical primary atrophic profound scleroderma. The coexistence in one case of primary facial hemiatrophy appears to indicate also its relationship with primary linear atrophies of the limbs.

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“…Cerebrovascular involvement often seems quiescent in scleroderma but might contribute to the onset of CNS disorders during low-flow states, as described in 1 patient, 3 or via vasospastic phenomena, revealed by serial angiography in a case report. 10 Increased vasospasm has been demonstrated in the main internal organs (heart, kidney, and lung) in scleroderma 11 and might explain why some middle-aged scleroderma patients have otherwise unexplained transient ischemic attacks with focal neurological defects 3,14,15 or transient global amnesia, 16 both of which were observed in our patients. In addition, various psychological disorders have been reported in scleroderma patients 15,[17][18][19] and may be partly due to organic brain involvement; indeed, it has recently been shown that patients with basal ganglia calcification frequently have neuropsychological alterations.…”

Section: Discussionmentioning

confidence: 69%

Intracerebral Calcification in Systemic Sclerosis

Héron

Hernigou

Châtellier

et al. 1999

Stroke

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Background and Purpose-Advanced cerebrovascular wall calcification was recently observed at autopsy in 2 patients with systemic sclerosis. To further investigate this issue, we conducted a prospective CT study of scleroderma patients to detect intracerebral calcification. Methods-Thirty-seven consecutive patients with systemic sclerosis underwent unenhanced brain CT. Images were blindly interpreted, together with those of 2 age-matched (Ϯ1 year) and sex-matched control subjects per patient. Results-Intracerebral calcification was found in 12 patients (32.4%) and 7 controls (9.5%) (Pϭ0.006). Among the patients, intracerebral calcification was associated with the duration of Raynaud's phenomenon (Pϭ0.005) and not with age (Pϭ0.086). Conclusions-Intracerebral calcification is closely associated with scleroderma, which suggests that scleroderma causes primary cerebrovascular changes. (Stroke. 1999;30:2183-2185.)Key Words: basal ganglia Ⅲ calcification Ⅲ scleroderma, systemic Ⅲ tomography, x-ray computed S mall-artery changes have been observed in nearly every organ in scleroderma. 1 One exception is the brain, where the distinction between primary lesions and lesions secondary to arterial hypertension and renal disease could not be established in the few available pathological reports. 2 We recently described 2 patients with scleroderma, central nervous system (CNS) manifestations, and autopsy evidence of small cerebral artery wall calcification, mainly in the basal ganglia. 3 Both patients also had mild basal ganglia calcification on brain CT, which is the most frequently observed CT abnormality in the brain of patients with cerebral lupus. 4 Because of the young age of the 2 patients and the absence of other known causes of cerebrovascular calcification, we postulated that the latter were primarily related to scleroderma. 3 To confirm this we conducted a prospective CT study to detect intracerebral calcification in patients with systemic sclerosis. Subjects and Methods SubjectsBetween October 1997 and June 1998, 37 consecutive scleroderma patients hospitalized in our department, mainly for elective investigations, underwent unenhanced brain CT. There were 31 women and 6 men, aged 21 to 78 years (meanϮSD 54Ϯ14 years). The main characteristics of the study population are shown in the Table. Sixteen patients (43%) had diffuse scleroderma according to the American Rheumatism Association (ARA) criteria, 5 and 21 (57%) had the limited form of systemic sclerosis. Among the ARA diagnostic criteria: all the patients had Raynaud's phenomenon, 68% had sclerodactyly, 49% had a history of digital ulceration, 35% had pulmonary fibrosis, and 16% had cutaneous sclerosis proximal to the knees and/or elbows. Screening for antinuclear antibodies was positive in all 37 patients, at a dilution of 1/500 or more in 32 (86%). MethodsBrain CT studies were performed with an electron beam CT (Evolution, Siemens Inc) with 6-mm unenhanced contiguous slices through the whole brain. Between January 1997 and June 1998, 1240 patients underwent br...

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Neurologic Manifestations of Progressive Systemic Sclerosis

Cited by 116 publications

References 16 publications

Cerebral Aneurysms in Scleroderma

Cerebral Aneurysms in Scleroderma

Primary Atrophic Profound Linear Scleroderma

Intracerebral Calcification in Systemic Sclerosis

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