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May 1974

Subacute Necrotizing Encephalomyelopathy: Rapidly Fatal Course of Leigh Disease in a 5-year-old Child

Author Affiliations

Bronx, NY
From the departments of neurology and pediatrics (Drs. Eisengart and Rose), and pathology (Dr. Powers), Albert Einstein College of Medicine of Yeshiva University, Bronx, NY.

Am J Dis Child. 1974;127(5):730-732. doi:10.1001/archpedi.1974.02110240116018

A previously normal, 5-year-old boy developed a rapidly progressive neurological illness three days following diagnosis and treatment of a streptococcal pharyngitis. He had quadriparesis, then developed pseudobulbar palsy and respiratory failure, and died eight weeks later. At postmortem examination, the neuropathological findings were diagnostic of subacute necrotizing encephalomyelopathy, a disease primarily of infancy, which when present in older children almost always follows the course of a chronic neurological illness.

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