Duchenne-Aran disease


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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.

a·my·o·tro·phic lat·er·al scle·ro·sis (ALS),

a fatal degenerative disease involving the corticobulbar, corticospinal, and spinal motor neurons, manifested by progressive weakness and wasting of muscles innervated by the affected neurons; fasciculations and cramps commonly occur. The disorder is 90-95% sporadic in nature (although a number of cases are inherited as an autosomal dominant trait [MIM*105400]), affects adults (typically, older adults), and usually is fatal within 2-5 years of onset. It is in the most common subgroup of motor neuron diseases, and the only one manifested by a combination of upper and lower abnormalities. Variants include: progressive bulbar palsy, in which isolated or predominant lower brainstem motor involvement occurs; primary lateral sclerosis, in which only upper motor neuron abnormalities are seen; and progressive spinal muscle atrophy, in which only lower motor neuron dysfunction is noted. See also entries under Henry Louis Gehrig

Duchenne-Aran disease

[do̅o̅shen′äräN′]
Etymology: Guillaume B.A. Duchenne, French neurologist, 1806-1875; François A. Aran, French physician, 1817-1861
muscular atrophy caused by degeneration of the anterior horn cells of the spinal cord, primarily affecting the upper extremities. Chronic muscle wasting and weakness first appear in the hands and advance progressively to the arms and shoulders, eventually affecting the legs and other body areas. Several conditions may lead to this disease, such as the injection of toxins.

amy·o·tro·phic lat·er·al scle·ro·sis

(ALS) (ā-mīō-trōfik latĕr-ăl skler-ōsis)
A disease of the motor tracts of the lateral columns and anterior horns of the spinal cord, causing progressive muscular atrophy, hyperactive deep tendon reflexes, fibrillary twitching, and spastic irritability of muscles; associated with a defect in superoxide dismutase.
Synonym(s): Aran-Duchenne disease, Charcot disease, Cruveilhier disease, Duchenne-Aran disease, Lou Gehrig disease, progressive muscular atrophy.

Duchenne,

Guillaume B.A., French neurologist, 1806-1875.
Aran-Duchenne disease - Synonym(s): Lou Gehrig disease
Aran-Duchenne dystrophy - Synonym(s): Lou Gehrig disease
Duchenne-Aran disease - Synonym(s): Lou Gehrig disease
Duchenne attitude - paralysis of trapezius resulting in shoulder lowering on external rotation.
Duchenne disease - Synonym(s): (1) Duchenne dystrophy; - (2) progressive bulbar paralysis
Duchenne dystrophy - the most common childhood muscular dystrophy, with onset usually before age 6. Synonym(s): childhood muscular dystrophy; Duchenne disease (1); Duchenne-Griesinger disease; pseudohypertrophic muscular dystrophy
Duchenne-Erb paralysis - Synonym(s): Erb palsy
Duchenne-Erb syndrome - Synonym(s): Erb palsy
Duchenne-Griesinger disease - Synonym(s): Duchenne dystrophy
Duchenne paralysis - brachial birth palsy in which there is paralysis of upper arm and shoulder girdle muscles due to lesion of upper trunk of brachial plexus or roots of fifth and sixth cervical roots.
Duchenne sign - falling in of the epigastrium during inspiration in paralysis of the diaphragm.
Duchenne syndrome - subacute or chronic anterior spinal paralysis combined with multiple neuritis.
Erb-Duchenne paralysis - Synonym(s): Erb palsy

Aran,

François, French physician, 1817-1861.
Aran-Duchenne disease - Synonym(s): Lou Gehrig disease
Aran-Duchenne dystrophy - Synonym(s): Lou Gehrig disease
Duchenne-Aran disease - Synonym(s): Lou Gehrig disease

Gehrig,

Henry Louis, U.S. baseball player for the New York Yankees, 1903-1941, victim of Lou Gehrig disease.
Lou Gehrig disease - a disease of the motor tracts of the lateral columns and anterior horns of the spinal cord, causing progressive muscular atrophy. Synonym(s): amyotrophic lateral sclerosis; Aran-Duchenne disease; Aran-Duchenne dystrophy; Charcot disease; Cruveilhier disease; Cruveilhier palsy; Duchenne-Aran disease
Duchenne-Aran disease; progressive muscular atrophy presentation of motor neurone disease characterized by progressive atrophy of spinal cord anterior horn cells, with inexorable, slowly progressive, wasting and paralysis of limb and trunk muscles, presenting initially as weakness and wasting of small muscles of one hand, marked muscle fasciculation, hyper- or hyporeflexia (depending on anterior horn cell involvement)
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