Neurological involvement in systemic sclerosis

• Published in: Revista clínica espanõla Vol. 203; no. 8; p. 373
• Main Authors: Campello Morer, I, Velilla Marco, J, Hortells Aznar, J L, Almárcegui Lafita, C, Barrena Caballo, R, Oliveros Juste, A
• Format: Journal Article
• Language: Spanish
• Published: Spain 01-08-2003
• Subjects: Adult; Aged; Diagnostic Techniques, Neurological; Female; Humans; Male; Middle Aged; Nervous System Diseases - epidemiology; Nervous System Diseases - etiology; Nervous System Diseases - pathology; Peripheral Nervous System Diseases - diagnosis; Peripheral Nervous System Diseases - epidemiology; Peripheral Nervous System Diseases - etiology; Prevalence; Risk Factors; Scleroderma, Systemic - complications; Scleroderma, Systemic - diagnosis; Scleroderma, Systemic - epidemiology
• ISSN: 0014-2565
• DOI: 10.1016/S0014-2565(03)71296-X

Systemic sclerosis (SS) is recognized as the connective tissue disease which less frequently presents neurological complications; in recent studies it is demonstrated, however, that neurological involvement in SS is more frequent of what it had been assumed.
Clinical neurological exploration was done in 26 patients with definitive SS; an electroneurogram was carried out in 23 cases in order to determine the prevalence of central neurological pathology and of peripheral neuropathy, to define its characteristics, and to investigate possible associations with clinical parameters and with autoimmunity.
23 cases (88%) were females and 3 cases (12%) males; the median age was 57.5 12.0 (SD) years, while the median age to the diagnosis was 51.3 12.3 (SD) years and the median period of natural history of disease was 6.2 3.1 years. Seven patients (26.9%) showed involvement of the CNS, being the headache and the neuropsychiatric manifestations the most common conditions (11.5%). Peripheral neuropathy prevalence was 39.1% (9 cases); according to the distribution of the injury, the polyneuropathy prevailed in 30.4% of cases. With regard to the functional selectivity, the sensitive-motor forms were most frequent (55.6%); according to the most involved structure, the axonal neuropathy was most common (44.4%).Discussion. The possible pathogenic mechanisms of the neurological pathology in this disease are discussed.