[Progressive bulbopontine paralysis in children]. 1988

P Beauvais, and A Roubergue, and T B de Villemeur, and J M Richardet
Service de Pédiatrie, Neurologie et Pathologie du développement, Hôpital Trousseau, Paris.

A case of pons-bulbar palsy in a 10 year-old girl is reported. The authors insist on the fact that motor cranial nerve nuclear dysfunction was isolated, without any pyramidal tract disorder. They also emphasize the slow course of the disease, which after one year of quick progression was stable for the next 5 years. Nineteen identical cases were found in the literature. From this nosographic group, one must exclude patients with associated lateral spinal tract dysfunction: these patients must be discussed with the juvenile or infantile amyotrophic lateral sclerosis group. The heterogeneity of the pons-bulbar paralysis group is also underlined: the course may be quickly fatal or, on the contrary, protracted for a long time; genetic transmission is also quite variable.

UI MeSH Term Description Entries
D008297 Male Males
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
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013997 Time Factors Elements of limited time intervals, contributing to particular results or situations. Time Series,Factor, Time,Time Factor

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