Progressive bulbar paralysis of childhood (Fazio-Londe disease) with deafness. Case report with clinicopathologic correlation. 1982

S Rosemberg, and C L Lancellotti, and F Arita, and C Campos, and N P de Castro

UI MeSH Term Description Entries
D008526 Medulla Oblongata The lower portion of the BRAIN STEM. It is inferior to the PONS and anterior to the CEREBELLUM. Medulla oblongata serves as a relay station between the brain and the spinal cord, and contains centers for regulating respiratory, vasomotor, cardiac, and reflex activities. Accessory Cuneate Nucleus,Ambiguous Nucleus,Arcuate Nucleus of the Medulla,Arcuate Nucleus-1,External Cuneate Nucleus,Lateral Cuneate Nucleus,Nucleus Ambiguus,Ambiguus, Nucleus,Arcuate Nucleus 1,Arcuate Nucleus-1s,Cuneate Nucleus, Accessory,Cuneate Nucleus, External,Cuneate Nucleus, Lateral,Medulla Oblongatas,Nucleus, Accessory Cuneate,Nucleus, Ambiguous,Nucleus, External Cuneate,Nucleus, Lateral Cuneate
D009046 Motor Neurons Neurons which activate MUSCLE CELLS. Neurons, Motor,Alpha Motorneurons,Motoneurons,Motor Neurons, Alpha,Neurons, Alpha Motor,Alpha Motor Neuron,Alpha Motor Neurons,Alpha Motorneuron,Motoneuron,Motor Neuron,Motor Neuron, Alpha,Motorneuron, Alpha,Motorneurons, Alpha,Neuron, Alpha Motor,Neuron, Motor
D009434 Neural Pathways Neural tracts connecting one part of the nervous system with another. Neural Interconnections,Interconnection, Neural,Interconnections, Neural,Neural Interconnection,Neural Pathway,Pathway, Neural,Pathways, Neural
D010244 Bulbar Palsy, Progressive A motor neuron disease marked by progressive weakness of the muscles innervated by cranial nerves of the lower brain stem. Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. The adult form of the disease is marked initially by bulbar weakness which progresses to involve motor neurons throughout the neuroaxis. Eventually this condition may become indistinguishable from AMYOTROPHIC LATERAL SCLEROSIS. Fazio-Londe syndrome is an inherited form of this illness which occurs in children and young adults. (Adams et al., Principles of Neurology, 6th ed, p1091; Brain 1992 Dec;115(Pt 6):1889-1900) Fazio-Londe Disease,Fazio-Londe Syndrome,Fazio-Londe's Disease,Fazio-Londe's Syndrome,Paralysis, Bulbar,Bulbar Palsy,Bulbar Palsy, Progressive, Of Childhood,Childhood Progressive Bulbar Palsy,Progressive Bulbar Palsy of Childhood,Bulbar Palsies,Bulbar Palsies, Progressive,Bulbar Paralyses,Bulbar Paralysis,Disease, Fazio-Londe,Disease, Fazio-Londe's,Fazio Londe Disease,Fazio Londe Syndrome,Fazio Londe's Disease,Fazio Londe's Syndrome,Palsies, Bulbar,Palsies, Progressive Bulbar,Palsy, Bulbar,Palsy, Progressive Bulbar,Progressive Bulbar Palsies,Syndrome, Fazio-Londe,Syndrome, Fazio-Londe's
D002531 Cerebellum The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills. Cerebella,Corpus Cerebelli,Parencephalon,Cerebellums,Parencephalons
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003391 Cranial Nerves Twelve pairs of nerves that carry general afferent, visceral afferent, special afferent, somatic efferent, and autonomic efferent fibers. Cranial Nerve,Nerve, Cranial,Nerves, Cranial
D003638 Deafness A general term for the complete loss of the ability to hear from both ears. Deafness Permanent,Hearing Loss Permanent,Prelingual Deafness,Deaf Mutism,Deaf-Mutism,Deafness, Acquired,Hearing Loss, Complete,Hearing Loss, Extreme,Acquired Deafness,Complete Hearing Loss,Deafness, Prelingual,Extreme Hearing Loss,Permanent, Deafness,Permanent, Hearing Loss,Permanents, Deafness
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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