[Adrenomyeloneuropathy. A frequent cause of Addison's disease].
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.
Schäfer
J R
JR
Abteilung für Endokrinologie und Stoffwechsel sowie Medizinische Poliklinik, Universität Marburg, sowie Universitäts-Kinderklinik, Göttingen.
Ehlenz
K
K
Steinmetz
A
A
Pilz
C
C
Hunneman
D H
DH
Baerwald
C
C
von Wichert
P
P
Kaffarnik
H
H
D000224:Addison Disease / Q000209:etiology*; D000326:Adrenoleukodystrophy / Q000150:complications* / Q000235:genetics / Q000378:metabolism; D000328:Adult; D000368:Aged; D005227:Fatty Acids / Q000097:blood / Q000378:metabolism; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009128:Muscle Spasticity / Q000209:etiology; D010243:Paralysis / Q000209:etiology; D012737:Sex Factors
D005227:Fatty Acids
Adrenomyeloneuropathie. Eine häufige Ursache des Morbus Addison.