Leigh's subacute necrotizing encephalomyelopathy manifesting as spasmus nutans
L. A. Sedwick, R. M. Burde and F. J. Hodges 3rd
Subacute necrotizing encephalomyelopathy (SNE), or Leigh's disease, is an
unusual neurologic disorder that is associated with nystagmus, strabismus,
and optic atrophy in the majority of cases, and is most often described in
infants. The defect currently thought to account for SNE is the presence of
an inhibitor factor in the thiamine pathway. We saw a patient whose eye
findings initially were believed to represent spasmus nutans, but whose
clinical course, computed tomographic findings, and autopsy findings were
typical of SNE.
