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Motor Neurone Disease (motor + neurone_disease)

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Medical Sciences	100%

Selected Abstracts

Nutritional factors associated with survival following enteral tube feeding in patients with motor neurone disease

JOURNAL OF HUMAN NUTRITION & DIETETICS, Issue 4 2010
A. Rio
Abstract Background: Motor neurone disease (MND) is a progressive neurodegenerative disease leading to limb weakness, wasting and respiratory failure. Prolonged poor nutritional intake causes fatigue, weight loss and malnutrition. Consequently, disease progression requires decisions to be made regarding enteral tube feeding. The present study aimed to investigate the survival, nutritional status and complications in patients with MND treated with enteral tube feeding. Methods: A retrospective case note review was performed to identify patients diagnosed with MND who were treated with enteral tube feeding. A total of 159 consecutive cases were identified suitable for analysis. Patients were treated with percutaneous endoscopic gastrostomy (PEG), radiologically inserted gastrostomy (RIG) or nasogastric feeding tube (NGT). Nutritional status was assessed by body mass index (BMI) and % weight loss (% WL). Serious complications arising from tube insertion and prescribed daily energy intake were both recorded. Results: Median survival from disease onset was 842 days [interquartile range (IQR) 573,1263]. Median time from disease onset to feeding tube was PEG 521 days (IQR 443,1032), RIG 633 days (IQR 496,1039) and NGT 427 days (IQR 77,781) (P = 0.28). Median survival from tube placement was PEG 200 (IQR 106,546) days, RIG 216 (IQR 83,383) days and NGT 28 (IQR 14,107) days. Survival between gastrostomy and NGT treated patients was significant (P , 0.001). Analysis of serious complications by nutritional status was BMI (P = 0.347) and % WL (P = 0.489). Conclusions: Nutritional factors associated with reduced survival were weight loss, malnutrition and severe dysphagia. Serious complications were not related to nutritional status but to method of tube insertion. There was no difference in survival between PEG and RIG treated patients. [source]

Misrepresentations about palliative options and prognosis in motor neurone disease: some legal considerations

JOURNAL OF EVALUATION IN CLINICAL PRACTICE, Issue 1 2005
Charles Foster MA(Cantab) Vet MB MRCVS
Abstract If euthanasia were legalized, clinicians would be under a duty to explain to patients requesting euthanasia what the prognosis and palliative options were. Anecdotal evidence suggests that some patients suffering from motor neurone disease may request euthanasia because of a fear of choking to death. The literature indicates that with competent palliative care this is unlikely to occur. It is assumed, for the purposes of argument, that responsible clinicians would accordingly reassure patients that such a fear was unwarranted, and that such a reassurance would cause patients for whom choking was the main concern either to withdraw a request for euthanasia or not to make it in the first place. The legal consequences of both negligent and deliberate failure to represent the true prognostic and palliative situation are discussed. In the case of a deliberate failure, with the intention to induce the patient to consent to euthanasia, it is suggested that a verdict of murder would be ethically right. It is argued that such a failure is best regarded as an omission. The English law currently does not countenance the possibility of murder by omission. It is suggested that the distinction between acts and omissions, while sometimes convenient, can sometimes produce injustice, and that the distinction should not be allowed to prevent conviction for murder where this is clearly appropriate. [source]

Nutritional factors associated with survival following enteral tube feeding in patients with motor neurone disease

Olfaction in neurodegenerative disorder

MOVEMENT DISORDERS, Issue 4 2003
Christopher Hawkes MD
Abstract There has been an increase of interest in olfactory dysfunction since it was realised that anosmia was a common feature of idiopathic Parkinson's disease (PD) and Alzheimer-type dementia (AD). It is an intriguing possibility that the first sign of a disorder hitherto regarded as one of movement or cognition may be that of disturbed smell sense. In this review of PD, parkinsonian syndromes, essential tremor, AD, motor neurone disease (MND) and Huntington's chorea (HC) the following observations are made: 1) olfactory dysfunction is frequent and often severe in PD and AD; 2) normal smell identification in PD is rare and should prompt review of diagnosis unless the patient is female with tremor-dominant disease; 3) anosmia in suspected progressive supranuclear palsy and corticobasal degeneration is atypical and should likewise provoke diagnostic review; 4) hyposmia is an early feature of PD and AD and may precede motor and cognitive signs respectively; 5) subjects with anosmia and one ApoE-4 allele have an approximate 5-fold increased risk of later AD; 6) impaired smell sense is seen in some patients at 50% risk of parkinsonism; 7) smell testing in HC and MND where abnormality may be found, is not likely to be of clinical value; and 8) biopsy of olfactory nasal neurons shows non-specific changes in PD and AD and at present will ot aid diagnosis. © 2003 Movement Disorder Society [source]

Embryonic stem cells and prospects for their use in regenerative medicine approaches to motor neurone disease

NEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 5 2007
Y. A. Christou
Human embryonic stem cells are pluripotent cells with the potential to differentiate into any cell type in the presence of appropriate stimulatory factors and environmental cues. Their broad developmental potential has led to valuable insights into the principles of developmental and cell biology and to the proposed use of human embryonic stem cells or their differentiated progeny in regenerative medicine. This review focuses on the prospects for the use of embryonic stem cells in cell-based therapy for motor neurone disease or amyotrophic lateral sclerosis, a progressive neurodegenerative disease that specifically affects upper and lower motor neurones and leads ultimately to death from respiratory failure. Stem cell-derived motor neurones could conceivably be used to replace the degenerated cells, to provide authentic substrates for drug development and screening and for furthering our understanding of disease mechanisms. However, to reliably and accurately culture motor neurones, the complex pathways by which differentiation occurs in vivo must be understood and reiterated in vitro by embryonic stem cells. Here we discuss the need for new therapeutic strategies in the treatment of motor neurone disease, the developmental processes that result in motor neurone formation in vivo, a number of experimental approaches to motor neurone production in vitro and recent progress in the application of stem cells to the treatment and understanding of motor neurone disease. [source]

Pathogenesis and molecular targeted therapy of spinal and bulbar muscular atrophy

NEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 2 2007
H. Adachi
Spinal and bulbar muscular atrophy (SBMA) or Kennedy's disease is a motor neurone disease characterized by muscle atrophy, weakness, contraction fasciculations and bulbar involvement. SBMA mainly affects males, while females are usually asymptomatic. SBMA is caused by expansion of a polyglutamine (polyQ)-encoding CAG trinucleotide repeat in the androgen receptor (AR) gene. AR belongs to the heat shock protein 90 (Hsp90) client protein family. The histopathologic hallmarks of SBMA are diffuse nuclear accumulation and nuclear inclusions of the mutant AR with expanded polyQ in residual motor neurones in the brainstem and spinal cord as well as in some other visceral organs. There is increasing evidence that the ligand of AR and molecular chaperones play a crucial role in the pathogenesis of SBMA. The success of androgen deprivation therapy in SBMA mouse models has been translated into clinical trials. In addition, elucidation of its pathophysiology using animal models has led to the development of disease-modifying drugs, that is, Hsp90 inhibitor and Hsp inducer, which inhibit the pathogenic process of neuronal degeneration. SBMA is a slowly progressive disease by nature. The degree of nuclear accumulation of mutant AR in scrotal skin epithelial cells was correlated with that in spinal motor neurones in autopsy specimens; therefore, the results of scrotal skin biopsy may be used to assess the efficacy of therapeutic trials. Clinical and pathological parameters that reflect the pathogenic process of SBMA should be extensively investigated. [source]

Phenytoin toxicity masquerading as motor neurone disease

ANAESTHESIA, Issue 3 2009
T. Veenith
No abstract is available for this article. [source]

Nutritional advice and treatment by dietitians to patients with amyotrophic lateral sclerosis/motor neurone disease: a survey of current practice in England, Wales, Northern Ireland and Canada

JOURNAL OF HUMAN NUTRITION & DIETETICS, Issue 1 2007
A. Rio
Abstract Background, The management of amyotrophic lateral sclerosis/motor neurone disease (ALS/MND) has shifted from an attitude of nihilism to treatments that prolong survival and offer hope. Nutrition is an integral component of ALS/MND care requiring coordination among acute and community multi-disciplinary teams (MDT). Evidence-based nutrition guidelines exist for this patient group but their use among dietitians is unknown. The aim of this study was to survey the knowledge, practice and guideline use of dietitians working in ALS/MND centres/clinics across England, Wales, Northern Ireland (EWNI) and Canada. Method, Dietetic contact details were obtained from the Motor Neurone Disease Association (MNDA) and the ALS Society of Canada (ALSSC) websites. Telephone interviews were conducted with 23 dietitians using a standardized questionnaire. Results, Multi-disciplinary team membership was high (78%). Only 22% dietitians had >4-years experience in ALS/MND care. Dietitians reported using body weight, percentage weight loss (PWL) and body mass index (BMI) to assess nutritional status. Equations used to estimate energy and protein requirements differed. Most frequent dietary advice was high calorie, texture modification and prescription nutritional supplements. Artificial nutrition and hydration (ANH) was discussed when patients developed dysphagia, energy intake was inadequate, weight loss of 10% or forced vital capacity (FVC) was reduced. A percutaneous endoscopic gastrostomy (PEG) service was available at all clinics/centres. Conclusion, Nutritional assessment techniques and dietary advice should be standardized. Dietetic collaboration at national and international level is recommended to reduce professional isolation. Training and support in ALS/MND nutrition should be made available as part of post-dietetic registration. Further dietetic research is required to stimulate nutritional care. [source]