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861.
Distal spinal muscular atrophy is a rare lower motor neuron disorder that may be difficult to distinguish clinically from type II Charcot-Marie-Tooth disease. We report on clinical and pathologic findings in 13 members of a four-generation extended family with autosomal dominant distal spinal muscular atrophy. The patients developed a slowly progressive lower motor neuron disorder involving mainly the distal lower extremities; onset was from the second to fourth decades. Electromyography and muscle biopsy findings were indicative of motor denervation. Combined silver/cholinesterase/immunocytochemical staining of intramuscular nerve revealed abundant collateral axonal branching in mild disease but marked loss of terminal motor endplate innervation in the more severe state, suggesting decreased growth of motor axon collaterals with disease progression. Multipoint DNA linkage analysis showed that this family's disorder is not linked to the chromosome 5q11.2-13.3 spinal muscular atrophy locus.  相似文献   
862.
The interaction of betacyclodextrin with ampicillin, methicillin and phenytoin has been investigated. All three drugs, (in the unionized form) interact strongly with betacyclodextrin. It is suggested that the interaction of all three drugs with betacyclodextrin could perhaps be exploited to pharmaceutical advantage. For ampicillin it is possible that the complex may have an improved bioavailability and a reduction in the incidence of gastro-intestinal side effects in comparision with the uncomplexed drug. For methicillin the significant extension of the hydrolytic half life for the complex makes the oral route a possibility for this drug, which is normally given intravenously because of stability problems. The interaction of phenytoin with betacyclodextrin produces a complex with a substantially enhanced solubility likely to give greatly improved bioavailability and a reduction in intra and inter subject blood level variation.  相似文献   
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With the aid of a polygraphical study of day sleep in infants with cerebral palsy it was possible to demonstrate in many cases changes of qualitative characteristics of the stages of sleep with disorders of normal dynamics in the EEG, motor and vegetative signs. The authors detected a certain correlation between the degree of such changes and the severity of the clinical symptomatology, as well as a possibility of changes in expression of the pathology of sleep at different stages of the disease, sometimes with an increase of this pathology.  相似文献   
867.
Studies have been made on thermal denaturation of rhodopsin in hotoreceptive membranes of retinal rods of some Japan Sea fishes--Podthecus sp., Enophrys diceraus, Myoxocephalus stelleri, Pleurogrammus monopterygius, Sebastichthys trivittatus, Pheumatophorus japonicus, Gadus morhua, Theragia chaloogramma, Eleginus gracilis and Lepidopsetta herzensteini. It was shown that visual pigments of the species studied significantly differ in their thermostability, whereas with respect to their spectral properties they are rather similar (absorbtion maxima lie near 500 nm). Positive correlation between the thermostability level of rhodopsins and the environmental temperature of the species was found.  相似文献   
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Until recently parity has been the traditional method of data protection. Parity, however, has several drawbacks: it masks out even bit errors, it cannot locate the bit in error and beyond a certain buswidth it can become inefficient. These factors have led to the development of a new breed of devices for data protection. Error detection and correction circuits (EDSs or EDACs) are today's response to the growing need for greater data reliability. They afford a greater level of protection than that offered by parity, by using a higher order error correction code (modified Hamming code).  相似文献   
870.
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