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Acute Werdnig-Hoffmann disease is heredofamilial degeneration of anterior horn cells in the spinal
cord and of motor nuclei in the brain stem, which is clinically characterized by fasciculations of the
tongue, paradoxical respiration, bell shaped thorax, frog-leg posture and intact mentality.
We experienced an 8 month old female infant with acute Werdnig-Hoffmann disease.
We report the case with brief review of some related literatures. |