Wolfram-type syndrome

[MIM 614 296] 

Very rare. Autosomal dominant transmission of a mutation of the WFS1 gene (4p16.1). Other autosomal recessive mutations of the same gene  produce Wolfram type 1 syndrome (see this term).


Rare endocrine disease characterized by the triad:


The only neurological abnormalities reported in rare cases are psychiatric (anxiety, depression, hallucinations and sleep disorders). The patients do not show endocrine or cardiac signs, on the contrary to what is observed in Wolfram syndrome.


Anesthetic implications:

glycemia control, hearing-impaired patient, management of a mitochondrial cytopathy



References :

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Updated: May 2021