Amiotrophic lateral sclerosis

This is a case report of autopsy findings for a male Japanese patient who presented with progressive gait disturbance and dysarthria. Neurological examination at the age of 61 years revealed pseudobulbar palsy and upper motor neuron disorder. The patient appeared unaware of his illness. Electrophysiological examination showed lower neuron damage. The patient was diagnosed with amyotrophic lateral sclerosis characterized by lower motor neuron damage. He died of pneumonia 2 years and 3 months after diagnosis...

Melatonin is an indolamine synthesized and secreted by the pineal gland along with other extrapineal sources including immune system cells, the brain, skin and the gastrointestinal tract. Growing interest in this compound as a potential therapeutic agent in several diseases stems from its pleiotropic effects. Thus, melatonin plays a key role in various physiological activities that include regulation of circadian rhythms, immune responses, the oxidative process, apoptosis or mitochondrial homeostasis. Most of these processes are altered during inflammatory pathologies, among which neurodegenerative and bowel diseases stand out...

Although the prevalence of neurodegenerative diseases is increasing as a consequence of the growing aging population, the exact pathophysiological mechanisms leading to these diseases remains obscure. Multiple sclerosis (MS), an autoimmune disease of the central nervous system and the most frequent cause of disability among young people after traumatic brain injury, is characterized by inflammatory/demyelinating and neurodegenerative processes that occurr earlier in life. The ability to make an early diagnosis of MS with the support of conventional MRI techniques, provides the opportunity to study neurodegeneration and the underlying pathophysiological processes in earlier stages than in classical neurodegenerative diseases...

An efficient alternative channel for communication without overt speech and hand movements is important to increase the quality of life for patients suffering from Amiotrophic Lateral Sclerosis or other illnesses that prevent correct limb and facial muscular responses. Often, such diseases leave the ocular movements preserved for a relatively long time. The aim of this study is to present a new approach for the hybrid system which is based on the recognition of electrooculogram (EOG) and electroencephalogram (EEG) measurements for efficient communication and control...

Psychosocial support, education and self-management are important complements of rehabilitation programs. A central concept in self-management is self-efficacy, which refers to oneself confidence in reaching a desired goal. The General Perceived Self-Efficacy scale (GSE), developed to measure self-efficacy at the broadest level, could be useful in the rehabilitation setting, in order to assess patients' self-management difficulties as well as to design specific interventions for specific diseases. Aim of this work is to verify the GSE Italian version psychometric properties applied to the rehabilitation setting...

Glutamic acid (Glu) is the major excitatory neurotransmitter in the mammalian central nervous system (CNS) where it is involved in the physiological regulation of different processes. It has been well established that excessive endogenous Glu is associated with many acute and chronic neurodegenerative disorders such as cerebral ischaemia, epilepsy, amiotrophic lateral sclerosis, Parkinson's, and Alzheimer's disease. These data have consequently added great impetus to the research in this field. In fact, many Glu receptor antagonists acting at the N-methyl-D-aspartic acid (NMDA), 2-amino-3-(3-hydroxy-5-methylisoxazol-4-yl)propionic acid (AMPA), and/or kainic acid (KA) receptors have been developed as research tools and potential therapeutic agents...

Glutamic acid (Glu) is the major excitatory neurotransmitter in the mammalian central nervous system (CNS) where it is involved in the physiological regulation of different processes. It has been well established that excessive endogenous Glu is associated with many acute and chronic neurodegenerative disorders such as cerebral ischemia, epilepsy, amiotrophic lateral sclerosis (ALS), Parkinson's and Alzheimer's diseases. In addition to the classical competitive glutamate receptor (GluR) antagonists, much effort has been directed toward the development of many different non-competitive antagonists of these receptors and, among them, compounds blocking the glycine site on the NMDA receptor complex (Gly/NMDA) have been widely investigated...

No abstract text is available yet for this article.

Two groups of tick-borne encephalitis (TBE) virus strains were studied: Group 1, 5 strains isolated from patients with chronic TBE with progressive course, Group 2, 13 strains isolated from residents of an endemic locality, with chronic diseases of the CNS (amiotrophic lateral sclerosis, epidemic encephalitis, polyoencephalomyelitis, syringomyelia, etc.). Strains of both groups belong to two serotypes of TBE virus: mid-Siberian and Transbaikal (synonym Aina/1448) and eastern. Group 1 strains were heterogeneous in their virulence, immunogenic and surface properties of the virions...

Recent data suggest that amiotrophic lateral sclerosis (ALS) could be the result of motoneuron damage induced by endogenous or exogenous excitotoxins, and especially by excitatory amino acids (EAA). Three main sources support this hypothesis: 1) The induction of experimental models of motor neuron disease by 2 excitotoxins (BOAA and BMAA). 2) Evidence of disordered glutamate metabolism in ALS. 3) Data suggesting that EAAs may be a factor in the pathogenesis of other degenerative neurologic diseases (Huntington disease and Alzheimer disease)...