Progressive Bulbar Paralysis of Childhood (Fazio-Londe Disease)
Fazio-Londe disease, or progressive bulbar paralysis of childhood, is a progressive bulbar paralysis that is probably genetically transmitted. This is a disorder of bulbar motor neurons.
Patients present with cranial nerve findings, including ptosis, facial weakness, dysphagia, normal hearing, and respiratory stridor. They may show hyperreflexia. Dominant transmission is rare. One group with recessive inheritance had early onset in infancy and rapid progression, with death from respiratory failure less than two years from the age of onset. Another group with recessive inheritance shows later onset (3 to 12 years), less respiratory involvement but slowly progressive dysarthria, dysphagia, and facial weakness. These patients may have progressive motor neuron disease with primary involvement of the anterior horn cells in the cervical and upper thoracic core segments. In addition, there may be widespread degenerative changes in the brainstem. Cranial nerve VII is almost always affected. These patients develop dysphagia secondary to cranial nerve XII involvement. The nuclei of cranial nerves III, IV, VI, and X may also be involved; however, clinical impairment of extraocular movement is rare.
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