Objective(s): Cell therapy has provided clinical applications to the treatment of motor neuron diseases. The current obstacle in stem cell therapy is to direct differentiation of stem cells into neurons in the neurodegenerative disorders. Biomaterial scaffolds can improve cell differentiation and are widely used in translational medicine and tissue engineering. The aim...

behçet’s ‎ disease is a multisystem vasculitis. its neurological involvement mostly includes parenchymal and non-parenchymal central nervous system manifestations. peripheral nervous system presentations are rare. a 32-yr-old male patient who fulfilled the international study group criteria for behçet’s disease, referred to our center with walking difficulty and repeated falling downs. neurolog...

Background: Spinal muscular atrophy (SMA) is a genetic motor neuron disease caused by mutations in the SMN1 (Survival Motor Neuron) gene, which leads to hypotonia and muscle weakness with high mortality related respiratory involvement. Gene therapy (GT) (onasemnogeno aberpavovec) for SMA, through an adeno-associated viral vector 9 (AAV9) was recently approved our country, but its safety efficac...

Neurologists in the 19th century recognized that muscle weakness could be due to primary disorders of muscle or secondary to loss of neuromuscular integrity, as happens when peripheral nerves are cut or when motor neurons degenerate. Furthermore, it was observed that there are forms of motor neuron degeneration which selectively affect upper motor neurons or lower motor neurons. A combination o...

Hadjikoutis and Wiles draw attention to a number of circumstances in which the serum chloride and bicarbonate can be misleading. They do not mention that they will also be misleading if they are measured when hypercapnia is increasing or decreasing. An exacerbation of hypercapnia will take 4 – 6 days to raise the serum bicarbonate and lower the serum chloride to new steady levels. 1 How long it...

Motor neuron disease (MND) represents one ofthe major unsolved problems in neurology. The literature is characterized by numerous speculative theories, mostly derived by analogy with other recognized causes of motor neuron death such as poliomyelitis, or from toxic exposures such as lead poisoning. However, analogous reasoning of this type has not, so far, given insights into MND itself, or res...

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...

BACKGROUND Frontotemporal lobar degeneration with motor neuron disease (FTLD-MND) is a pathological entity characterized by motor neuron degeneration and frontotemporal lobar degeneration. The ability to detect the clinical signs of dementia and motor neuron disease in pathologically confirmed FTLD-MND has not been assessed. OBJECTIVES To determine if all cases of pathologically confirmed FTL...