Biomedical subjects
Publications and source records attributed to N Hyman.
A case of posterior spinal ligament rupture associated with a general anaesthetic for a laparoscopic cholecystectomy is reported. The role of the general anaesthetic in this case is discussed and a review of the literature is presented.
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OBJECTIVE: To define a safe and effective dose of Dysport for treating hip adductor spasticity. METHODS: Patients with definite or probable multiple sclerosis, and disabling spasticity affecting the hip adductor muscles of both legs, were randomised to one of four treatment groups. Dysport (500, 1000, or 1500 Units), or placebo was administered by intramuscular injection to these muscles. Patients were assessed at entry, and 2, 4 (primary analysis time-point), 8, and 12 weeks post-treatment. RESULTS: A total of 74 patients were recruited. Treatment groups were generally well matched at entry. The primary efficacy variables-passive hip abduction and distance between the knees-improved for all groups. The improvement in distance between the knees for the 1500 Unit group was significantly greater than placebo (p = 0.02). Spasm frequency was reduced in all groups, but muscle tone was reduced in the Dysport groups only. Pain was reduced in all groups, but improvements in hygiene scores were evident only in the 1000 Unit and 1500 Unit groups. Duration of benefit was significantly longer than placebo for all Dysport groups (p<0.05). Adverse events were reported by 32/58 (55%) Dysport patients, and by 10/16 (63%) placebo patients. Compared with the two lower dose groups, twice as many adverse events were reported by the 1500 Unit group (2.7/patient). The incidence of muscle weakness was higher for the 1500 Unit group (36%) than for placebo (6%). The response to treatment was considered positive by two thirds of the patients in the 500 Unit group, and by about half the patients in the other groups. CONCLUSION: Dysport reduced the degree of hip adductor spasticity associated with multiple sclerosis, and this benefit was evident despite the concomitant use of oral antispasticity medication and analgesics. Although evidence for a dose response effect was not statistically significant, there was a clear trend towards greater efficacy and duration of effect with higher doses of Dysport. Dysport treatment was well tolerated, with no major side effects seen at doses up to 1500 Units. The optimal dose for hip adductor spasticity seems to be 500-1000 Units, divided between both legs.
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BACKGROUND: Most anorectal fistulas may be safely and reliably treated by fistulotomy. However, certain complex fistulas (e.g., rectovaginal fistulas, high transsphincteric tracts, Crohn's disease) are not well suited to this technique. Few satisfactory alternatives exist. The aim of this study was to assess the utility of endoanal advancement flap repair for these difficult fistulas. METHODS: Thirty-three consecutive patients underwent endoanal advancement flap repair of a complex anorectal fistula. Patients were followed up via a prospective database. Demographic information, the presence of previous fistula surgery, and surgical complications were noted. Patients were closely followed up until healing of the fistula or treatment failure was noted. RESULTS: The overall initial healing rate was 81% (27 of 33). However, 3 patients with perianal Crohn's disease ultimately developed a recurrent fistula. There were no major complications and two minor urinary complications. No patient required hospital readmission, and there were no new problems with fecal incontinence. No patient required a colostomy. CONCLUSION: Endoanal advancement flap repair is effective in a variety of difficult, complicated anorectal fistulas. Since the morbidity is quite low, it should be attempted prior to fecal diversion, when possible, in these settings.
Anorectal abscesses and fistulas are seen commonly in the primary care practice. An abscess forms as the result of obstruction of an anal gland, with resulting retrograde infection. An anal fistula simply represents the chronic phase of a perianal abscess. The history generally is diagnostic, and special studies usually are not required. Treatment is surgical, with good results.
Intellectual, psychological and functional outcomes were evaluated in a consecutive series of 20 Parkinsonian patients who had unilateral (UPVP) or simultaneous bilateral posteroventral pallidotomy (BPVP) using Image Fusion and Stereoplan (Radionics Inc., Boston, Mass., USA) with stimulation for lesion localization. Comprehensive baseline and 3-month postoperative neuropsychological and neurological assessment protocols were administered together with questionnaire measures of functional disability, quality of life and psychological symptomatology. Changes in patients' clinical presentation and scores on psychometric tests, questionnaires and observational rating scales were then examined. We observed no new neuropsychiatric sequelae directly related to pallidotomy. Cognitive sequelae were restricted to selective reductions in categorical verbal fluency following UPVP (P < 0.001) and BPVP (P < 0.01) and a reduction in phonemic verbal fluency following BPVP (P < 0.01); these changes were not reported subjectively. A fall in diadochokinetic rates (P < 0.01) and some subjective reports of a worsening in pre-existing dysarthria, hypophonia and hypersalivation/drooling following BPVP also suggested changes in speech motor apparatus; however, these changes did not have significant functional consequences. There was one case of more generalized cognitive impairment following BPVP. We also observed significant symptomatic improvement on neurological rating scales; following UPVP, Total Unified Parkinson's Disease Rating Scale (UPDRS) scores improved by 27% (P < 0.01) and following BPVP the improvement was 53% (P < 0.05). Patients' perceptions of reduced postoperative functional disability and improvements in 'quality of life' also achieved statistical significance on a number of both physical and psychosocial questionnaire subscales.
We quantified antibodies to botulinum A (anti-BTx) by immunoprecipitation of 125I-BTx. We tested seven bioassay-positive sera and 68 coded samples, including 18 from patients who had ceased to respond to BTx treatment. Compared with values from healthy control subjects and 42 neurologic control subjects, all bioassay-positive sera were positive (range, 258 to 2,809 pM) and 49 of 50 patients who continued to respond to BTx were negative (<130 pM). This simple, specific, sensitive, and quantitative assay should prove helpful in the investigation of BTx resistance.
With the introduction of the antispasticity agent, tizanidine hydrochloride (Zanaflex), physicians have requested information about the optimal way to switch appropriate patients from baclofen to tizanidine. A group of neurologists and rehabilitation specialists with a particular interest in spasticity was therefore asked to draw up a suitable approach to changing treatment.
OBJECTIVE: To assess the validity and responsiveness of a questionnaire to assess health-related quality of life in Parkinson's disease (PD)--the 39-item Parkinson's Disease Questionnaire (PDQ-39)--and to report problems experienced by patients by means of the questionnaire. METHODS: Patients completed the PDQ-39 and the SF-36 at baseline and 4 months later. At the same assessments, neurologists rated patients with Hoehn and Yahr and Columbia Scales. RESULTS: Evidence for validity of the new questionnaire was observed by agreement of scores with clinical scales at both assessments. Evidence for responsiveness of scales assessing physical function, particularly mobility and activities of daily living, was observed from significant paired t tests for differences between scores at baseline and follow-up, and correlations with patients' retrospective judgments and changes in the SF-36 summary scores. However, there were no significant associations with changes in neurologists' clinical scores. Patients most frequently reported problems of physical function in the PDQ-39. Scores for several dimensions of the PDQ-39 were significantly more favorable than those reported by nonclinic samples of patients with PD. CONCLUSIONS: The PDQ-39 has validity for use among patients attending neurological clinics for treatment of PD. There is also some evidence of responsiveness. The questionnaire identifies problems that are important to patients and that appear to be more commonly experienced by nonclinic attenders.
OBJECTIVES: to briefly outline the development and validation of the Parkinson's Disease Questionnaire (PDQ-39) and then to provide evidence for the use of the measure as either a profile of health status scores or a single index figure. DESIGN: the PDQ-39 was administered in two surveys: a postal survey of patients registered with local branches of the Parkinson's Disease Society of Great Britain (n = 405) and a survey of patients attending neurology clinics for treatment for Parkinson's disease (n = 146). Data from the eight dimensions of the PDQ-39 were factor-analysed. This produced a single factor on the data from both surveys. OUTCOME MEASURES: the eight dimensions of the PDQ-39 and the new single index score-the Parkinson's disease summary index (PDSI), together with clinical assessments (the Columbia rating scale and the Hoehn and Yahr staging score). RESULTS: in the postal survey 227 patients returned questionnaires (58.2%). AH 146 patients approached in the clinic sample agreed to take part. Higher-order principal-components factor analysis was undertaken on the eight dimensions of the PDQ-39 and produced one factor on both datasets. Consequently it was decided that the scores of the eight domains could be summed to produce a single index figure. The psychometric properties of this index were explored using reliability tests and tests of construct validity. The newly derived single index was found to be both internally reliable and valid. DISCUSSION: data from the PDQ-39 can be presented either in profile form or as a single index figure. The profile should be of value in studies aimed at determining the impact of treatment regimes upon particular aspects of functioning and well-being in patients with Parkinson's disease, while the PDSI will provide a summary score of the impact of the illness on functioning and well-being and will be of use in the evaluation of the overall effect of different treatments. Furthermore, the PDSI reduces the number of statistical comparisons and hence the role of chance when exploring data from the PDQ-39.
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BACKGROUND AND PURPOSE: Responses to changes in intraluminal pressure of isolated human pial arteries (200 to 1200 microns i.d.) obtained from patients undergoing neurosurgery were measured. METHODS: The vessels were cannulated and pressurized (60 mm Hg); vascular diameter and intraluminal pressure were recorded simultaneously. After spontaneous development of steady state tone, intraluminal pressure was changed to both higher and lower levels in random sequence. RESULTS: Human pial arteries exhibited myogenic responses and maintained their diameter over the pressure range of 20 to 100 mm Hg. The level of myogenic tone observed at 30 mm Hg did not vary significantly with artery diameter. In contrast, at 60 and 90 mm Hg, the extent of myogenic tone increased as the diameter decreased (up to 70% to 80% of maximum in 200-microns i.d. arteries). The arteries contracted to KCl 30 mmol/L, norepinephrine 1 mumol/L, and vasopressin 0.1 mumol/L and relaxed to acetylcholine 3 mumol/L. The extent of these responses did not vary with the diameter of the artery. Arterial distensibility, represented by the slope of the tangent of the passive pressure-diameter curve at lower pressures (5 to 50 mm Hg), increased as arteries became smaller. This is consistent with the possibility that the level of myogenic tone is related to vessel distensibility. Human omental arteries of comparable size did not develop myogenic tone but contracted to KCl and norepinephrine and relaxed to acetylcholine to an extent similar to pial arteries. CONCLUSIONS: There is a specific gradient of myogenic responsiveness in human pial arteries that varies inversely with their diameter. This tone does not develop in all vascular beds. These levels of tone in the pial circulation would be expected to be of profound functional significance by allowing blood flow to vary widely.
The neurological features of 13 patients with primary hypogammaglobulinaemia are described. Seven patients had X-linked agammaglobulinaemia (XLA) and six had common variable immunodeficiency (CVID). Three clinical pictures emerged: (i) a progressive myelopathy (one case); (ii) a myelopathy progressing to an encephalopathy (four cases); (iii) a pure encephalopathy (eight cases). In four patients the encephalopathy was temporarily reversible; the relationship of this to immunoglobulin therapy is unclear. Additional features occurred in some patients. Three had retinopathy interpreted as retinitis pigmentosa, in one of whom the retinopathy resolved. Two patients had a sensori-neural hearing loss and three had features of dermatomyositis; a variable pleocytosis was found in the CSF of nine patients. Imaging revealed atrophic changes in the cerebral hemispheres in eight cases. Ten patients have died, 1-11 years after the onset of the CNS manifestations, and in four autopsies were obtained. Two patients had encephalopathy, one with XLA had evidence of end-stage encephalitis and the other with CVID had a multi-focal leucoencephalopathy. The other two with XLA had leptomeningitis without evidence of encephalitis. Enteroviral infection is probably an important cause of neurological disease in these patients as CSF from seven patients was either positive by polymerase chain reaction (PCR) or by culture for enteroviruses. Other possible mechanisms are discussed.