(MIM 272100)

(diffuse periaxial  encephalitis,  diffuse myelinoclastic sclerosis, Addison-Schilder disease, sudanophilic leucodystrophy, sudanophilic cerebral sclerosis)

Very rare (12/20,000). Form of adrenoleukodystrophy (see adrenoleukodystrophy). Gradual onset of asymmetric zones of demyelination in the cerebral white matter. Signs and symptoms depend on the location of these lesions: blindness or cortical deafness, behavioural disorders, dementia, spastic hemiplegia or paraplegia, pseudobulbar palsy. Death usually occurs within two years; however, certain forms dramatically respond to steroid therapy if the diagnosis is made early. A form is associated with adrenal insufficiency: these children have a tanned skin (Addison-Schilder).

Anesthetic implications:

risk of pulmonary inhalation, risk of airway obstruction. Epilepsy. Hemodynamic instability in case of spastic paraplegia. Provide a so called stress dose of hydrocortisone at the induction of anesthesia even in the absence of clinical signs of adrenal insufficiency.

References : 

• Tobias JD. 
  Anaesthetic considerations for the child with leukodystrophy. 
  Can J Anaesth 1992; 39:394-7.
•  Lyon G, Evrard P. 
  Neuropédiatrie 2ème édition, Masson et cie, Paris 2000, p 328.

Updated: September 2018