\ɡɹˈiːnfiːld dɪzˈiːz], \ɡɹˈiːnfiːld dɪzˈiːz], \ɡ_ɹ_ˈiː_n_f_iː_l_d d_ɪ_z_ˈiː_z]\
Definitions of GREENFIELD DISEASE
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An autosomal recessive lysosomal storage disease caused by a deficiency of CEREBROSIDE-SULFATASE leading to an accumulation of cerebroside sulfate in the nervous system and other organs. Pathologic features include diffuse demyelination and metachromatically-staining granules within glial cells, macrophages, and free in tissue. Clinical subtypes include late infantile, juvenile, and adult forms. The late infantile form presents at 12-18 months of age with progressive psychomotor retardation, ATAXIA, spasticity, and OPTIC ATROPHY. The juvenile form has onset at 4-12 years and features behavioral alterations and ataxia followed by spasticity, DEMENTIA, and visual loss. The adult form presents in the second decade or later with psychiatric manifestations, gait difficulties, and less often as a peripheral neuropathy. (From Menkes, Textbook of Child Neurology, 5th ed, pp192-5)
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Word of the day
Dorsal Root Ganglia
- ganglia located on dorsal roots within vertebral column. ganglion cells are pseudounipolar. single primary bifurcates sending peripheral process to carry sensory information from the periphery and a central branch which relays that spinal cord or brain.