Phenotypic continuum in neuronopathic Gaucher disease: an intermediate phenotype between type 2 and type 3.

Goker-Alpan, Ozlem; Schiffmann, Raphael; Park, Joseph K; Stubblefield, Barbara K; Tayebi, Nahid; Sidransky, Ellen · J Pediatr · 2003

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Abstract

Neuronopathic Gaucher disease, classically divided into two types, can have a continuum of phenotypes, often defying categorization. Nine children had an intermediate phenotype characterized by a delayed age of onset but rapidly progressive neurological disease, including refractory seizures and oculomotor abnormalities. There was genotypic heterogeneity among these patients.

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