Presence of what neuromuscular disorder appears on a radiograph as a classic bell-shaped chest wall

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Spinal muscular atrophy type 1 (SMA1), also known as Werdnig-Hoffmann disease, is a severe form of spinal muscular atrophy that presents early in life. It is characterized by muscle weakness and hypotonia resulting from the degeneration and loss of the anterior horn cells in the spinal cord and the brainstem nuclei. This leads to a "bell-shaped" chest due to the diaphragmatic breathing associated with the disease, as the intercostal muscles are weak and the diaphragm becomes the primary muscle for respiration. This distinctive chest shape can be observed on a radiograph.

Presence of What Neuromuscular Disorder Appears on a Radiograph as a Classic