摘要:

Responses evoked over the scalp and the neck by median nerve (or finger I) stimulation were concurrently recorded in 10 subjects. It was found that the first component of the cortical SEP consists of a small amplitude polyphasic wave (S wave) which could be recorded bilaterally upon unilateral stimulation. The polarity of the S wave varied according to the reference electrode position, at variance with the P15component which remained constantly positive. It is therefore correct to assume that different generators are responsible for these two potentials.The synchrony between the S wave and the cervical response, which is largely spinal in origin, as well as some pertinent experimental data, suggest that the S wave is a far field reflection of activity generated mainly in the cervical dorsal columns. These findings might be relevant to the diagnosis of neurological disorders.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02881.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

β‐Galactosidase activity was investigated in one case of juvenile GM1‐gangliosidosis. This patient exhibited normal activity of the neutral form of β‐galactosidase (measured as β‐glucosidase activity) and normal pH curve of residual acid β‐galactosidase activity in leucocytes and fibroblasts. A shift towards more neutral pH optimum was seen in the β‐galactosidase enzyme occurring in serum.The communication also presents a study of the relationship of the different β‐galactosidases in human liver using isolated urine oligosaccharide from this patient as a β‐galactoside substrate. The other natural β‐galactoside substrates used in this investigation were different oligosaccharides, one glycopeptide and ceramide‐β‐galactoside. The β‐galactosidase forms with acidic pH optimum towards synthetic substrate (A forms) exhibit activity towards the natural substrate (except ceramide‐β‐galactoside). The “neutral”β‐galactosidase with broad substrate specificity (B form) which includes β‐glucosides had no activity towards the natural substrates used.It could also be shown that the activity towards ceramide‐β‐galactoside was a third

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02882.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

The maximum sensory conduction velocity was measured along the articular branch of the suprascapular nerve. The potential was evoked by stimulating in the perception area of the nerve above the glenohumeral joint and recorded by an electrode near the suprascapular nerve in the supraclavicular fossa. The maximum conduction velocity averaged 42 m/s, SD 4.6 m/s (20 normal subjects); it decreased slightly with age. When correcting for the error in distance arising from the measurement by obstetric calipers (20%), the maximum conduction velocity averaged 50 m/s. The peak‐to‐peak amplitude of the main component of the sensory potential was 0.8 μV, SD 0.5 μV.In one of seven patients with a pain syndrome in the shoulder secondary to a brachial plexus neuropathy the velocity and the amplitude of the sensory potential was diminished. In five other patients with pain in the region of the shoulder the conduction velocity was normal but the amplitude was dimin

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02883.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Fifty‐nine diabetic patients participated in a double‐blind study in order to evaluate the efficiency of myoinositol to improve the function of peripheral nerves.Myoinositol in the amounts given was not able to change motor conduction velocity or vibratory perception threshold. No change in retinopathy and several biochemical parameters was obser

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02884.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

In a large family five affected males belonging to four different kinships exhibited a muscle wasting of varying degree and with a predominantly proximal distribution. The index case had a facio‐scapulo‐humeral and peroneal muscular atrophy, whereas one of his cousins suffered a more generalized involvement starting in infancy and similar to Werdnig‐Hoffman disease. The other affected family members had only slight changes. The index case and his affected brother had a positive Babinski sign.In enzyme histochemical preparations, specimens from the index case showed small group atrophy of type 2 fibers along with pseudomyopathic changes (whorled and coiled fibers, splitting) of type 1 fibers. Similar findings were observed in his cousin. Ultrastructural investigation gave no further information.Since all patients were males and the offspring of unaffected sisters, an X‐chromosomal mode of transmission is proposed for this

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02885.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY