Intracerebral Calcification in Systemic Sclerosis

Abstract

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...

Linear Scleroderma en coup de sabre With Associated Neurologic Abnormalities

“…4,8 Neuroradiologic abnormalities including cerebral atrophy, whitematter lesions, intraparenchymal calcification, meningeocortical alterations, and skull atrophy have all been described in the literature. 4,6,7,9,18 Resolution and recurrence of radiologic lesions may correlate clinically with neurologic disease activity. 4,9 Just as imaging may be abnormal in patients without neurologic symptoms, normal imaging can be seen despite the presence of neurologic disease as demonstrated by 2 of our patients as well as the case of a patient with facial and oculomotor nerve palsy and grand mal seizures described by Gambichler et al 19 The lack of specific and/or sensitive radiologic findings for the associated neurologic disease contributes to the difficulty in counseling these patients regarding prognosis.…”

Mood and anxiety disorders in systemic sclerosis patients

“…Les épisodes de MDE actuels étaient plus fréquents chez les patients hospitalisés que chez les autres (28 % vs 10 %, p = 0,02). Des troubles anxieux spécifiques étaient mis en évidence chez 37 [28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44][45][46][47] patients. Moins de 50 % des patients qui avaient des troubles de l'humeur avaient un traitement psychotrope.…”

Atteintes neuropsychiatriques au cours de la sclérodermie systémique

“…Si ces symptômes sont le plus souvent interprétés comme la conséquence de la chronicité de la maladie, de la diminution de l'espérance de vie, du handicap physique et de la douleur[35,38], il faut raisonnablement se poser la question du rôle d'une atteinte organique spécifique du SNC dans le déve-loppement de ces symptômes, à l'instar des troubles neuropsychiatriques observés au cours du neurolupus. Par exemple, les calcifications cérébrales, témoins de l'atteinte vasculaire, pourraient être à l'origine de troubles neuropsychologiques spécifiques liés à leur localisation dans les ganglions de la base, impliqués dans les émotions et dans certaines fonctions cognitives[16]. De plus, chez une femme sclérodermique de 42 ans, une symptomatologie à type de délire paranoïde avec désorientation a régressé avec un traitement par corticoïdes, suggérant un support organique à cette symptomatologie[40] (même si dans ce cas, les anomalies intracérébrales pouvant être responsables de la symptomatologie psychiatrique n'ont pas été documentées).…”