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We report the clinical and pathological findings of the unusual combination of two idiopathic central nervous system diseases, multiple sclerosis and amyotrophic lateral sclerosis in a 56 year old physician with a twenty-seven year history of a disease initially characterized by relapses and remissions, followed by an eight year quiescent period. During the last year of life there was rapid deterioration with development of generalized weakness, atrophy, weight loss and fasciculations of body and tongue, and associated difficulty with swallowing and sudden respiratory failure. The autopsy confirmed characteristic “burned out” plaques of multiple sclerosis and anterior horn cell and axonal degeneration of amyotrophic lateral sclerosis.
This study presents the clinical characteristics of 8 victims of multiple sclerosis from the hamlet of Henribourg, Saskatchewan with a population of less than 75 people. A diligent victim of the disease had observed that six female classmates from the early 1940's had later developed multiple sclerosis. Two male military personnel who had also resided briefly in close proximity, during the same common exposure time, also later developed multiple sclerosis. The mean onset time of developing the disease after leaving the area was 20 years. This cluster-focus suggests a common exposure to an environmental factor or a common infective agent in the etiology of multiple sclerosis.
Objective: To report the occurrence, clinical characteristics and genealogical analysis of multiple sclerosis in the Hutterites of North-Western United States and Western Canada. Background: The incidence of multiple sclerosis is reported to be lower or rare in certain ethnic groups and genetic isolates and was previously observed to be absent in the Hutterite population. Methods: After long-term surveillance, six patients were identified and clinical examinations and laboratory investigations including VER and MRI were completed. Results: The six cases included two brothers, two first cousins, male and female, another male and female, all representing two of the three endogamous groups of Hutterites, are linked to two common ancestors through lines of descent dating to 1723. The individual pedigrees were analyzed from extensive genealogical records covering eight generations. Conclusion: The incidence of multiple sclerosis in Hutterites is low in a high risk area of North America. A specific mode of inheritance pattern has not been established and a common founder effect may play a role in the development of multiple sclerosis. The genetic contribution of the Hutterites seems greater than previously recognized.
Although severe traumatic brain injury (sTBI) is a devastating condition with tremendous public health implications, the epidemiology of this disease has not previously been described in Canada. We sought to define the incidence, risk factors and outcome of patients suffering sTBI in a large Canadian region.
A population-based surveillance cohort design was utilized to identify all Calgary Heath Region residents who were victims of trauma with an injury severity score ³12. Subsequent application of a specific sTBI case definition defined the final cohort.
The annual incidence of sTBI was 11.4 per 100,000 population. The incidence of sTBI was significantly higher for males as compared to females [17.1 vs. 5.9 per 100,000; relative risk (RR) = 2.91, 95% confidence interval; 2.17, 3.94; p<0.0001]. There was a striking increase in the annual age specific population incidence of sTBI observed among those older than 74 years of age. The relative risk among the highest risk group of elderly (>85 years) males as compared to the lowest risk female group (50-64 years) was 19.78 (95% CI; 6.27, 62.3; p<0.0001). One hundred and eight patients died prior to hospital discharge for a mortality rate of 5.1 per 100,000 per year.
Severe traumatic brain injury is common among residents of the Calgary Health Region and is associated with a high mortality rate. Males and the elderly are at the highest risk for acquiring sTBI and may represent target groups for preventive efforts.
Les lÉsions cÉrÉbrales par traumatisme crânien sÉvère sont frÉquentes parmi les rÉsidents de la rÉgion sanitaire de Calgary et sont associÉes à une mortalitÉ ÉlevÉe. Les hommes et les personnes âgÉes sont les groupes les plus à risque et constituent des groupes cibles pour les interventions à visÉe prÉventive.
A population-based prevalent group of 150 clinical definite patients ascertained on 1 January 1977, in Saskatoon, Saskatchewan, was followed for 30 years.
To outline the clinical characteristics, determine the levels of disability at 15, 25, 35, 40, and 45 years after onset, to estimate the survival after onset and life expectancy.
Clinical records were maintained, and the cohort reviewed each decade for 30 years. The disability levels according to the Kurtzke Extended Disability Status Scale were recorded and survival times were estimated. SPSS and Kaplan-Meier methods were used for analysis.
On prevalence day, 1 January 1977, there were 48(32%) men and 102(68%) women, with an average age of onset of 32.2±10 years and 28.4±8.6 years. The average duration of disease was 15.7 years. On 1 January 2007, 39(26%) patients were living, 105(70%) deceased, and 6(4%) were missing The disability levels recorded in 1977 and 2007, at 15 and 45 years after onset, were mild (EDSS≤2.5), 33.3% and 8.0%; moderate (EDSS3-5.5), 17.3% and 2.7%; severe (EDSS6-7.5), 6.6% and 4.7%; maximum (EDSS8-9.5), 22.7% and 10.7%. The median survival time after onset was 33 (95% CI: 27.3-38.6) years for men and 38 (95% CI: 34.1-41.9) years for women. The median duration of life was 68.9 years for men and 69.5 years for women, and a decreased life expectancy of 7.7 and 12.8 years.
Multiple sclerosis is a progressive neurological disorder and long-term survival is associated with moderate to severe disability and decreased life expectancy.
Background: Cognitive dysfunction is a common complaint associated with obstructive hydrocephalus. The purpose of this study was to determine the effect of endoscopic third ventriculostomy (ETV) on the neuropsychological outcome in patients presenting with cognitive decline and obstructive hydrocephalus. Methods: A retrospective review of patients who underwent ETV at the University of Calgary and had both pre and post operative neuropsychological testing, was completed. Presenting clinical features, etiology of hydrocephalus and ventricle size utilizing frontal occipital horn ratio was obtained. Outcomes and complications of the ETV were recorded. Detailed measures of intelligence, attention and concentration, executive function, visual and verbal memory, language functions and fine motor skills were completed. Post treatment change was determined utilizing Reliable Change Index. Results: A total of 13 patients were identified. Etiology of the hydrocephalus was aqueductal stenosis in 8 and tectal glioma in 4. The majority of patients (11 of 13, 85%) demonstrated cognitive dysfunction at the borderline (≤1 SD) or impairment level (≤1.5 SDs) in at least one domain. Nine patients (69%) showed reliable improvement in at least one cognitive domain. The greatest improvement was seen with visual memory (42%). One quarter to one third of patients demonstrated improvement on tests of intelligence quotient, verbal memory, attention and concentration, and executive function. Two patients declined in executive functioning. Ventricle size improved in eight patients. Conclusions: ETV is a safe effective procedure, capable of producing reliable improvements in cognitive dysfunction with hydrocephalus. Patients with cognitive complaints alone may benefit from ETV.
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