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October 1977

Ophthalmoplegia and Ondine's Curse

Author Affiliations

From the Neurology Service and the Charles S. Kubik Laboratory for Neuropathology, James Homer Wright Pathology Laboratories, Massachusetts General Hospital, and the Departments of Neurology-Neuropathology and Pathology, Harvard Medical School, Boston.

Arch Ophthalmol. 1977;95(10):1790-1793. doi:10.1001/archopht.1977.04450100092009

• Ocular abnormalities and psychomotor difficulties were prominent in two unrelated children; in addition, the older child had respiratory irregularity during sleep. The pathologic findings included lesions of the optic nerve in the case with available material and established the diagnosis of Leigh's subacute necrotizing encephalopathy. This disorder is thought to result from inhibition of a thiamine-dependent enzymatic process and may be modified by greatly increased thiamine intake. Suspicion of the diagnosis in a child with ophthalmoplegia or other ocular abnormalities may lead to earlier recognition and more successful treatment of the disease.

(Arch Ophthalmol 95:1790-1793, 1977)