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Werding Hoffman Disease
Disease is inherited as an autosomal due to primary degeneration and atrophy
of AHC and motor nuclei of brain stem → lower motor neuron weakness.
It is characterized by marked hypotonia, sluggish fetal movement, and
fasciculation of tongue.
The child is alert. Feeding behaviour and cry are poor. Deep tendon reflexes
are absent.
Muscle biopsy shows neurogenic type of atrophy or the muscle spindles are
atrophied in groups.
Death occurs by 2-4 years of age.
Treatment : supportive
