A to Z of syndromes

A-Z of syndromes: Leigh syndrome

How to recognise and support children who have this rare neurological condition.

Leigh syndrome is a rare inherited neuro-metabolic disorder affecting the central nervous system. It is named after Archibald Denis Leigh, a British neuropsychiatrist, who is credited with first describing the condition.

Children with Leigh syndrome have a sub-acute necrotising encephalomyelopathy which occurs early in life. Sometimes symptoms emerge following an acute event such as an infection or a surgical procedure. It has a poor prognosis.

There are different inheritance patterns and although it is most commonly inherited through an autosomal recessive pattern, there are other modes of inheritance and genetic changes are complex.

There is cell death in the brain tissue and these losses result in the characteristic lesions seen on the scans of


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