Werdnig-hoffman Disease (Noun)
Meaning
Autosomal recessive disease in which the degeneration of spinal nerve cells and brain nerve cells leads to atrophy of skeletal muscles and flaccid paralysis; death usually occurs in early childhood.
Classification
Nouns denoting natural processes.
Examples
- Werdnig-Hoffman disease, also known as spinal muscular atrophy type 1, is caused by mutations in the survival motor neuron 1 gene.
- This infant was diagnosed with Werdnig-Hoffman disease after showing signs of muscle weakness and difficulty breathing.
- Werdnig-Hoffman disease is characterized by the degeneration of motor neurons in the spinal cord and brainstem, leading to progressive muscle atrophy and paralysis.
- The prognosis for children with Werdnig-Hoffman disease is poor, with most not surviving past early childhood.
- Researchers are working to develop treatments for Werdnig-Hoffman disease, such as gene therapy, that may slow or halt disease progression.