ADRENOMYELONEUROPATHY, A COMMON-CAUSE OF ADDISONS-DISEASE

Citation
Jr. Schafer et al., ADRENOMYELONEUROPATHY, A COMMON-CAUSE OF ADDISONS-DISEASE, Deutsche Medizinische Wochenschrift, 119(10), 1994, pp. 327-331
Citations number
29
Categorie Soggetti
Medicine, General & Internal
Volume
119
Issue
10
Year of publication
1994
Pages
327 - 331
Database
ISI
SICI code
Abstract
Adrenomyeloneuropathy (AMN) is a >>milder form<< of adrenoleukodystrop hy with a X-linked inheritance. Abnormal catabolism of the very long-c hain fatty acids (VLCFA) results in Addison's disease and spastic para paresis. The VLCFA concentration was measured in 23 of 26 patients wit h Addison's disease (mean age 48.5 [20-75] years) being treated at the University Hospital Marburg during May, 1991. The concentration was e levated in four of the 12 men with the disease, while it was within no rmal limits in the 11 women. Only two patients had paraparesis-like ne urological deficits. This finding suggests that AMN is not as rare as has been supposed. It is recommended that the concentration of VLCFA b e measured in all patients with Addison's disease, because an increase could have important consequences.