June 2007

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2: Lancet. 2007 Jun 16;369(9578):2031-41 

Motor Neurone Disease Care and Research Centre, Royal Preston Hospital, Fulwood, Preston PR2 9HT, UK. Douglas.Mitchell@lthtr.nhs.uk

Amyotrophic lateral sclerosis (known in the UK as motor neuron disease) is a devastating illness with uncertain pathogenesis\. In this Seminar, we review its natural history, clinical features, diagnostic criteria, variant and mimic syndromes, genetic forms, and epidemiology\. Several hypotheses about causes of the disorder are discussed, such as excitotoxicity and oxidant stress, and we review past and present putative disease-modifying treatments\. Disease-management strategies, from telling the patient about their illness to end-of-life decisions and palliative care, are presented\. We review options for control of the main symptoms of amyotrophic lateral sclerosis--including dysphagia, dysarthria, respiratory distress, pain, and psychological disorders--and care in the terminal phase\. The need for good psychosocial and spiritual care of patients and families is emphasised\. We conclude with an overview of some current major issues and future prospects, ranging from the search for disease markers to challenging developments such as stem-cell and gene therapy.

PMID: 17574095 [PubMed - indexed for MEDLINE]


1: Neurology. 2007 Jun 5; 68(23): 2046-7; author reply 2047 

Comment on:Neurology. 2007 Jan 2;68(1):76-7. 

PMID: 17548561 [PubMed - indexed for MEDLINE]

4: Neurology. 2007 Jun 5;68(23):2003-7.  

School of Physical Therapy, University of Saskatchewan, Saskatoon, SK, Canada. vanina.dalbello-haas@usask.ca

OBJECTIVE: To determine the effects of resistance exercise on function, fatigue, and quality of life in individuals with ALS\. METHODS: Subjects with a diagnosis of clinically definite, probable, or laboratory-supported ALS, forced vital capacity (FVC) of 90% predicted or greater, and an ALS Functional Rating Scale (ALSFRS) score of 30 or greater were randomly assigned to a resistance exercise group that received a home exercise program consisting of daily stretching and resistance exercises three times weekly or to a usual care group, who performed only the daily stretching exercises\. ALSFRS, the Fatigue Severity Scale (FSS), and Short Form-36 (SF-36) were completed at baseline and monthly for 6 months\. FVC and maximum voluntary isometric contraction (MVIC) were monitored monthly throughout the study\. RESULTS: Of 33 subjects screened, 27 were randomly assigned (resistance = 13; usual care = 14)\. Eight resistance exercise subjects and 10 usual care subjects completed the trial\. At 6 months, the resistance exercise group had significantly higher ALSFRS and SF-36 physical function subscale scores\. No adverse events related to the intervention occurred, MVIC and FVC indicated no negative effects, and less decline in leg strength measured by MVIC was found in the resistance exercise group\. CONCLUSION: Our study, although small, showed that the resistance exercise group had significantly better function, as measured by total ALS Functional Rating Scale and upper and lower extremity subscale scores, and quality of life without adverse effects as compared with subjects receiving usual care.

PMID: 17548549 [PubMed - indexed for MEDLINE]