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  • Title: [Adrenomyeloneuropathy. A frequent cause of Addison's disease].
    Author: Schäfer JR, Ehlenz K, Steinmetz A, Pilz C, Hunneman DH, Baerwald C, von Wichert P, Kaffarnik H.
    Journal: Dtsch Med Wochenschr; 1994 Mar 11; 119(10):327-31. PubMed ID: 8125035.
    Abstract:
    Adrenomyeloneuropathy (AMN) is a "milder form" of adrenoleukodystrophy with a X-linked inheritance. Abnormal catabolism of the very long-chain fatty acids (VLCFA) results in Addison's disease and spastic paraparesis. The VLCFA concentration was measured in 23 of 26 patients with Addison's disease (mean age 48.5 [20-75] years) being treated at the University Hospital Marburg during May, 1991. The concentration was elevated in four of the 12 men with the disease, while it was within normal limits in the 11 women. Only two patients had paraparesis-like neurological deficits. This finding suggests that AMN is not as rare as has been supposed. It is recommended that the concentration of VLCFA be measured in all patients with Addison's disease, because an increase could have important consequences.
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