Disease Information for Schilder disease/Adrenoleukodystrophy: Definition

  • Inherited disease of CNS degeneration with Adrenal insufficiency in male infants and children; schilders disease synonyms are: Adrenoleukodystrophy;ALD; AMN

    Addison Disease with Cerebral Sclerosis; Addison-Schilder Disease ; Adrenomyeloneuropathy Adult Onset ALD;

    Bronze Schilder"s Disease ; Encephalitis Periaxialis Diffusa; Flatau-Schilder Disease; Melanodermic Leukodystrophy; Myelinoclastic Diffuse Sclerosis; Schilder Disease; Schilder Encephalitis; Siewerling-Creutzfeldt Disease; Sudanophilic Leukodystrophy, ADL.

    Onset Subdivisions:Childhood Adrenoleukodystrophy(X-linked recessive) And Neonatal Adrenoleukodystrophy (autosomal recessive); Discussion: Adrenoleukodystrophy is a rare inherited metabolic disorder characterized by the loss of the fatty covering (myelin sheath) on nerve fibers within the brain (cerebral demyelination) and the progressive degeneration of the adrenal gland (adrenal atrophy); Adrenoleukodystrophy that is inherited as an X-linked genetic trait may begin in childhood or adulthood; However, Adrenoleukodystrophy that is inherited as an autosomal recessive genetic trait typically begins during infancy (neonatal period)[NORD 2005].