science revisited pathogenesis Evidence for disease pathogenesis is derived from the animal model of GBS, named experimental allergic neuritis, which is caused by a combination of T-cell-mediated immunity to myelin…
The diabetic and nondiabetic radiculoplexus neuropathies are now commonly included in the spectrum of nonsystemic/localized variants of vasculitis associated with neuropathy. The typical presentation of radiculoplexus neuropathy includes that of…
science revisited Spinal bulbar muscular atrophy (Kennedy’s disease) is an X-linked inherited disease caused by a trinucleotide expansion in the androgen receptor gene. The abnormal protein forms aggregates that are…
science revisited The primary source of energy for resting muscle is derived from fatty acid oxidation (FAO). At rest, glucose utilization accounts for 10–15% of total oxygen consumption and both…
caution! Although muscle disease itself leads to morbidity and even mortality, involvement of other organ systems is the usual ultimate cause of death in most patients. Consequently, when appropriate, involvement…
science revisited mechanisms linked to the development of diabetic polyneuropathy Excessive flux of polyols (sugar alcohols) such as sorbitol, which alter Schwann cell and neuronal function Disease of small blood…
tips and tricks Patients unable to walk 5 m (Hughes scale score ≥3) require intensive monitoring. Patients with autonomic dysregulation or who fail the “20/30/40” test should be monitored in…
science revisited The underlying pathomechanism of sporadic ALS remains elusive. A complex interplay of genetic and environmental factors is likely involved in the etiology of motor neuron degeneration. Some risk…
Disease severity is associated with age at onset, with the most severe forms of the disease presenting during infancy as first described in the late nineteenth century by Werdnig and…
