End Stage Clinical Features and Cause of Death of Behavioral Variant Frontotemporal Dementia and Young-Onset Alzheimer's Disease.

BACKGROUND: Limited literature exists regarding the clinical features of end stage behavioral variant frontotemporal dementia (bvFTD). This data is indispensable to inform and prepare family members as well as professional caregivers for the expected disease course and to anticipate with drug-based and non-pharmacological treatment strategies. OBJECTIVE: The aim of the present study was to describe end stage bvFTD in a broad explorative manner and to subsequently evaluate similarities and dissimilarities with the end stage of the most prevalent form of young-onset dementia, Alzheimer's disease (yoAD). METHODS: We analyzed medical files on patients, using a mixed model of qualitative and quantitative approaches. Included were previously deceased patients with probable bvFTD and probable yoAD. End stage was defined as the last 6 months prior to death. Primary outcome measures comprised somatic, neurological, and psychiatric symptoms and the secondary outcome measure was cause of death. RESULTS: Out of 89 patients, a total of 30 patients were included (bvFTD; n = 12, yoAD; n = 18). Overall, the end stages of bvFTD and yoAD were characterized by a broad spectrum of clinical symptoms including severe autonomic dysfunction and an increased muscle tone. Patients with bvFTD displayed more mutism compared with yoAD while compulsiveness was only present in bvFTD. CONCLUSION: Our study describes the full clinical spectrum of end stage bvFTD and yoAD. In this study, symptoms extend far beyond the initial behavioral and cognitive features. By taking both somatic, psychiatric, and neurological features into account, family members and professional caregivers may anticipate (non) pharmacological treatment.

The Impact of Intrathecal Baclofen Therapy on Health-related Quality of Life for Children with Marked Hypertonia.

Purpose: To investigate the effects of intrathecal baclofen therapy (ITB) on health-related quality of life for children with cerebral palsy and neurological conditions. Method: This study is part of a longitudinal, multicentre audit. The primary outcome measure, the Caregiver Priorities and Child Health Index of Life with Disabilities, was completed at baseline, 6 and 12 months post ITB implant. Results: Forty subjects with cerebral palsy and other neurological conditions demonstrated significant improvement in aspects of health-related quality of life following ITB therapy, mean change 42.3 (SD 14.9) at baseline to 53.3 (SD 14.7) at 12 months (p< .001). Conclusion: Evidence to demonstrate the utility of ITB in pediatric populations beyond spasticity and dystonia reduction is limited. Our findings suggest that ITB improves aspects of quality of life, comfort, and ease of caregiving in children with cerebral palsy and other neurological conditions.

Intrathecal baclofen therapy in children: an analysis of individualized goals.

AIM: To determine whether intrathecal baclofen (ITB) therapy improves performance and performance satisfaction in goal areas identified by patients' parents. METHOD: This study formed part of an ongoing multicentre national audit involving six paediatric ITB pump implant centres across Australia. The Canadian Occupational Performance Measure was the primary outcome measure utilized at baseline, 6 months, and 12 months after pump implants in paediatric patients receiving ITB therapy for the first time between 31st December 2009 and 31st December 2014. RESULTS: Twenty-five children had goals identified (mean age 11y 1mo), 19 had a diagnosis of cerebral palsy and 22 were at Gross Motor Function Classification System level IV, V, or equivalent. Strong evidence for an improvement in goal performance (2.33, 95% CI 1.70, 2.96, p<0.001) and performance satisfaction scores (3.08, 95% CI 2.28, 3.88, p<0.001) were demonstrated at 6 months, compared to baseline. The differences were clinically significant and were sustained to 12 months. INTERPRETATION: ITB therapy in paediatric patients with hypertonia results in clinically significant improvements in average performance and performance satisfaction scores. WHAT THIS PAPER ADDS: The most commonly identified goals of parents of children treated with intrathecal (ITB) therapy were: improving ease of dressing, positioning, and transfers. ITB therapy is effective in improving performance and performance satisfaction in children with hypertonia. Score improvements are mainly evident within the first 6 months of therapy.

Muscle hypertonia after permanent focal cerebral ischemia in rats: a qualitative and quantitative behavioral and electrophysiological study.

OBJECTIVES: To examine the level of peripheral muscle resistance after cerebral ischemia. METHODS: A total of 326 healthy male Sprague-Dawley rats were used in the present experiments. We used a modified method to establish peripheral muscle resistance in rat model of stroke, and qualified the recovery of motor functional deficits by behavioral measures and quantified the level of peripheral muscle resistance by electrophysiological test. RESULTS: Neurological score started to go up from day 0, achieved its peak on day 3 (1.49 ± 0.56) and kept at a high level within 10 days after surgery. Compared with 1 day before surgery, both the turn score in corner test and asymmetry score in cylinder test were increased significantly on day 3, day 6 and day 9 after surgery (p < 0.01). On day 6 and day 9 after surgery, the Hmax:Mmax ratio of hemiplegic side of middle cerebral artery occlusion (MCAO) rats was obviously higher than the same side in healthy rat (p < 0.01) and the ratio on the contralateral side of MCAO rats (p < 0.05). CONCLUSIONS: There is a progressive increase in peripheral muscle resistance on day 6 to day 9 after surgery in a rat model of postischemic stroke.

[Dynamic bladder neck stenosis as a cause of psychogenic mictrition discomforts and mictrition disorders]. / Dynamische Blasenhalsstenose als Ursache psychogener Miktionsbeschwerden und Miktionsstörungen.

Symptoms of an "overactive bladder" can be psychological and in many cases are considered to be the result of psychogenic muscular tension of the pelvic floor causing a "dynamic stenosis" of the bladder neck. Psychogenic muscular tension can have its origin in psychic trauma during bladder training at the age of 1-3 years, leading to an obsessive-compulsive personality structure. A tendency to muscular tension, especially in the pelvic region, as well as to disorders of the lower bowl can be found in afflicted persons. Psychological defence and avoidance reactions, problems with contact and security, sexual abuse in childhood, as well as stress, anxiety, disappointment and anger can be the result of psychic trauma. Typical triggers involve psychological actualisation or compression. Because of the tendency to muscular tension with many psychogenic symptoms of voiding dysfunction, dispensing of medication, physical muscle relaxant measures show good therapeutic results.

Clinical efficacy of tolterodine with or without a simplified pelvic floor exercise regimen.

OBJECTIVES: To investigate whether the combination of tolterodine plus (Tp) a simple pelvic floor muscle exercise (PFME) program would provide improved treatment benefits compared with tolterodine alone (Ta) in patients with symptoms of overactive bladder (OAB). METHODS: After a 1-2 week run-in period, 480 patients with symptoms of urinary frequency (> or =8 micturitions/24 hr), urgency, and urge incontinence (> or =1 episode/24 hr), were randomized to receive tolterodine 2 mg bid with or without a simple PFME program for 24 weeks in this multinational study. Treatment efficacy was assessed by comparing the change from baseline in 3-day micturition diary recordings. RESULTS: After 24 weeks' treatment, in the Ta group the urgency episodes reduced from mean of 4.1 to 1.5 (83% reduction) while in the Tp group the urgency episodes reduced from 4.2 to 2.1 (78.7% reduction). Mean incontinence episodes per day decreased from 3.21 (standard deviation (SD) 3.4) to 0.95 (SD 1.9) in Ta group and from 3.44 (SD 3.4) to 1.25 (SD 2.7) in the Tp group. Similarly, the number of micturition/24 hr were significantly reduced, from 12.78 to 9.20 (27.3% reduction) in the Ta group and from 11.87 to 9.29 (23% reduction) in the Tp group. There was an improvement in the patients' perception of urinary symptoms in 85.9% of patients on Ta and 81.7% patients on Tp PFME. There were no statistically significant differences between the groups with regard to any of the outcome parameters. CONCLUSIONS: Tolterodine therapy for 24 weeks results in significant improvement in urgency, frequency, and incontinence, however, no additional benefit was demonstrated for a simple PFME program.

Effects of aging on generic SF-36 quality of life measurements in Hong Kong Chinese women with urinary incontinence.

BACKGROUND: To evaluate the relationship between aging, urodynamic diagnosis and generic quality of life measurement in Chinese women with urinary incontinence. METHODS: A total of 170 women presented to a university teaching hospital with urinary incontinence were recruited. The women completed the medical outcome survey, short form-36 (SF-36), immediately before they had the urodynamic investigation. The women were classified into either (1) genuine stress incontinence (n = 94) or (2) detrusor instability (n = 76). The relationships between aging, the transformed subscale scores, the population standardized component summary scores of the SF-36, and the two groups of women were studied using simultaneous multiple regression analysis. RESULTS: There was no significant difference in the age distribution between the two groups of patients (median age: 47 vs. 48, p = 0.18). However, aging causes significant impairment in the physical functioning (p < 0.001), role physical (p = 0.002) and bodily pain (p = 0.047) domains as well as the physical component scale (p < 0.001) of generic SF-36 quality of life measurements. There was no significant difference in the transformed subscale scores and the component summary scores with respect to different urodynamic diagnoses after adjustment for the effect of age. CONCLUSIONS: Aging causes significant deterioration on physical performance for Chinese women suffering from urinary incontinence. The generic SF-36 questionnaire was unable to detect a significant difference in quality of life measurement between women suffering from genuine stress incontinence and detrusor instability.

[Painful hypertonia of the calves. The role of the level of arousal apropos of 25 new cases]. / Hypertonie douloureuse des mollets. Rôle du niveau de vigilance à propos de 25 nouveaux cas.

Role of the level of alertness. Based upon 25 new cases. The author, after referring to the leg muscle bed syndrome associated with exercise, reports the frequency of painful spasticity of the calves, occurring initially at rest and in particular at night in the form of a restless legs syndrome, then secondarily during the day with varying degrees of persistence at rest and during exercise. This problem frequently has a lateral dominance and appears to be induced by self-maintained functional muscular spasticity of the gastrocnemii and soleus, explained by a study. It is frequently attributed to a venous etiology. This study based upon 25 cases enabled definition of the diagnosis and showed the probable links between EMG hyperactivity and a high level of alertness indicative of a predisposing situation.