" /> X-linked adrenoleukodystrophy - CISMeF





Preferred Label : X-linked adrenoleukodystrophy;

ICD-11 definition : X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disease characterized by progressive demyelinisation of the central nervous system (CNS) (brain and/or spinal cord) and peripheral adrenal insufficiency. The cerebral forms of juvenile X-ALD (45% of the cases) affect previously healthy 5 to 12 year-old boys. The adult form, adrenomyeloneuropathy (AMN), is characterized by the onset of spastic paraparesia between 20 and 45 years of age, associated with gait disturbances, urinary disorders and sexual dysfunction.;

ICD-11 synonym : Schilder-Addison complex;

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X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disease characterized by progressive demyelinisation of the central nervous system (CNS) (brain and/or spinal cord) and peripheral adrenal insufficiency. The cerebral forms of juvenile X-ALD (45% of the cases) affect previously healthy 5 to 12 year-old boys. The adult form, adrenomyeloneuropathy (AMN), is characterized by the onset of spastic paraparesia between 20 and 45 years of age, associated with gait disturbances, urinary disorders and sexual dysfunction.

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18/05/2024


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