juvenile spinal muscular atrophy

juvenile spinal muscular atrophy,

a disorder beginning in childhood in which progressive degeneration of anterior horn and medullary nerve cells leads to skeletal muscle wasting. The condition usually begins in the legs and pelvis. Also called Wohlfart-Kugelberg-Welander disease.

---

atrophy [at´ro-fe]

1. decrease in size of a normally developed organ or tissue; see also wasting.

2. to undergo or cause such a decrease. adj., adj atroph´ic.

acute yellow atrophy massive hepatic necrosis.

circumscribed cerebral atrophy pick's disease.

disuse atrophy atrophy of a tissue or organ as a result of inactivity or diminished function.

gyrate atrophy of choroid and retina a rare hereditary, slowly progressive atrophy of the choroid and pigment epithelium of the retina; inherited as an autosomal recessive trait.

juvenile spinal muscular atrophy Kugelberg-Welander syndrome.

Leber's optic atrophy Leber's optic neuropathy.

lobar atrophy pick's disease.

myelopathic muscular atrophy muscular atrophy due to lesion of the spinal cord, as in spinal muscular atrophy.

olivopontocerebellar atrophy any of a group of progressive hereditary disorders involving degeneration of the cerebellar cortex, middle peduncles, ventral pontine surface, and olivary nuclei. They occur in the young to middle-aged and are characterized by ataxia, dysarthria, and tremors similar to those of parkinsonism.

peroneal atrophy (peroneal muscular atrophy) progressive neuromuscular atrophy.

progressive neuromuscular atrophy hereditary muscular atrophy beginning in the muscles supplied by the fibular (peroneal) nerves, progressing slowly to involve the muscles of the hands and arms. Called also peroneal or peroneal muscular atrophy and Charcot-Marie-Tooth disease.

senile atrophy the natural atrophy of tissues and organs occurring with advancing age.

spinal muscular atrophy progressive degeneration of the motor cells of the spinal cord, beginning usually in the small muscles of the hands, but in some cases (scapulohumeral type) in the upper arm and shoulder muscles, and progressing slowly to the leg muscles. Called also Aran-Duchenne disease, Cruveilhier's disease, and Duchenne's disease.

subacute yellow atrophy submassive necrosis of the liver associated with broad zones of necrosis, due to viral, toxic, or drug-induced hepatitis; it may have an acute course with death from liver failure occurring after several weeks, or clinical recovery may be associated with regeneration of the parenchymal cells.