A disease in which the spinal muscles atrophy, making the muscles of the shoulders, arms and legs weak. In its most severe form, infants are born floppy, have feeding and breathing problems and rarely live more than two or three years.
Werdnig-Hoffmann disease (also known as infantile spinal muscular atrophy Type I, or SMA I) is a rare progressive neuromuscular degenerative disorder of infancy. SMA I is characterized by the wasting of the skeletal muscles caused by progressive deterioration of the motor nuclei within the lower brain stem and of the anterior horn cells of the spinal cord.
Genetic disease (autosomal recessive disease) in which degeneration of spinal and brain nerve cells leads to progressive atrophy of skeletal muscles. Symptoms include flaccid paralysis, lack of sucking ability in the infant, lack of muscle tone, and absence of normal reflexes. Death from respiratory complications usually occurs in early childhood.
Werdnig-Hoffmann disease, also known as infantile spinal muscular atrophy, is an extremely uncommon inherited disorder of the nervous system that primarily impacts infants. This condition falls under the category of motor neuron diseases, affecting the nerve cells in the spinal cord responsible for controlling muscle movement.
Severe muscle weakness and paralysis become evident within the initial few months of life, and unfortunately, children affected by this condition seldom live past the age of three.
A cure for the disease does not currently exist. Instead, the focus of treatment is on providing as much comfort as possible to the affected infant.