spinal muscular atrophy (sma) is a genetic disorder which affect nervous system and is characterized with progressive distal motor neuron weakness. the survival motor neuron (smn) protein level reduces in patients with sma. two different genes code survival motor neuron protein in human genome. skeletal and intercostal muscles denervation lead to weakness, hypotony, hyporeflexia, respiratory fa...

Clinical signs of upper motor neuron involvement are an essential observation to support the diagnosis of amyotrophic lateral sclerosis. However, clinical signs of upper motor neuron can be difficult to elicit in patients with motor neuron disease. One postulated reason for this problem is the presence of marked limb weakness and amyotrophy in motor neuron disease. This has been observed in pat...

OBJECTIVE To review evidences on the management of patients with motor neuron disease. DATA SOURCES PubMed literature searches from January 1982 up to January 2011. STUDY SELECTION Key words for literature search were "motor neuron disease review (MND)". Only the articles which concentrated on the ventilation, nutrition, cognitive or multidisciplinary approaches for motor neuron disease wer...

While motor neuron diseases are currently incurable, induced pluripotent stem cell research has uncovered some disease-relevant phenotypes. We will discuss strategies to model different aspects of motor neuron disease and the specific neurons involved in the disease. We will then describe recent progress to investigate common forms of motor neuron disease: amyotrophic lateral sclerosis, heredit...

Trace elements were estimated in the spinal cord, liver and bone of five patients dying of motor neuron disease and five control subjects dying of non-neurological disease. The content of selenium in cord and liver and the cord manganese level were significantly increased in the motor neuron disease patients. These findings are discussed in terms of the possible aetiology of motor neuron disease.

objective(s) motor deficit and neuron degeneration is seen after nerve transection. the aim of this study is to determine whether a poled polyvinelidene fluoride (pvdf) tube with other supportive strategies can protect the neuronal morphology and motor function after sciatic nerve transaction in rats. materials and methods after transection of the left sciatic nerve in 60 male wistar rats (200-...

Linked statistics from hospital records and death certificates were used to study the incidence of and mortality from motor neuron disease in a defined English population. The incidence of motor neuron disease, measured as first-admission rates for the disease, was studied from 1963 to 1985 and death certificates for the patients admitted to hospital were obtained to the end of 1990. The averag...