RESUMO
Six cases of a comparatively rare motor neuron disease are described. Essential features of this syndrome include (1) X-linked inheritance; (2) adult onset in the fourth to fifth decades; (3) slow progression; (4) predominant proximal and bulbar muscle involvement; and (5) absence of sensory or pyramidal tract signs. The previously reported finding of gynecomastia was absent, whereas longitudinal midline furrowing of the tongue was present in only one case. Electromyography in five patients revealed neurogenic changes. Muscle biopsies in two patients showed fiber type grouping with type I fiber predominance. The coexistence of this form of motor neuron disease and diabetes mellitus is prominent in family 2. It is important to recognize that these patients have a chronic, slowly progressive illness. The prognosis for longevity is good, although severe disability is inevitable. Management includes reassurance, supportive therapy, genetic counseling, and periodic testing for diabetes.
Assuntos
Cromossomos , Neurônios Motores , Doenças Neuromusculares/genética , Doenças da Medula Espinal/genética , Diabetes Mellitus/genética , Eletromiografia , Ligação Genética , Ginecomastia/genética , Humanos , Masculino , Pessoa de Meia-Idade , Neurônios Motores/fisiologia , Condução Nervosa , Linhagem , Língua Fissurada/genéticaRESUMO
Relationships were examined between prolactin concentrations in bovine milk and various environmental and lactational variables. Prolactin was quantified by radioimmunoassay in 1.316 milk samples from two experiments. Environmental temperatures preceding milking, stage of lactation, daily miik yield, and dominance rank of the cow were correlated significantly with milk prolactin concentration. Stepwise multiple regression analysis was used to determine, in order of importance, the variables having significant independent effects on milk prolactin. Ambient temperature extremes, high and low, exerted the greatest effects and were each associated with elevated concentrations of prolactin. In Experiment I,conducted throughout the year, the variable representing maximum temperature preceding milking accounted for 14.3% of the variation in milk prolactin. In Experiment II, conducted during late fall and early winter, the minimum temperature preceding milking accounted for 21.1% of the variation. Although earlier stages of lactation and larger daily milk yields were associated with higher prolactin concentrations, inclusion of lactation stage in the stepwise regression model tended to eliminate almost all the variance of prolactinpreviously associated with daily yield. Dominance measurements indicated that more submissive cows had higher milk prolactin. Individual cows tended to have characteristic prolactin, but this tendency was eliminated by statistical adjustment of the data for environmental and lactational variables.
