potentials and twitch force were measured in muscle strips obtained from patients and controls. Of the 36 patients, 25 presented with chronic muscle weakness of varying degrees, up to wheelchair-dependence. The weakness was associated with intracellular Na overload and edema. Older patients revealed a vacuolar myopathy or a progressive muscular dystrophy. Weakness, intracellular Na overload and...

BACKGROUND Inspiratory muscle weakness is a recognised cause of unexplained dyspnoea. It may be suggested by the finding of a low static inspiratory mouth pressure (MIP), but MIP is a difficult test to perform, with a wide normal range; a low MIP may also occur if the patient has not properly performed the manoeuvre. Further investigation conventionally requires balloon catheters to obtain oeso...

MYOPATHIES Clinical Features Myopathies are diseases that primarily affect the muscles and are usually characterized clinically by weakness, fatigue, or stiffness (myotonia). They are usually characterized by symmetrical proximal muscle weakness and wasting, normal sensation, and normal stretch reflexes. More than one-half of muscle bulk must be lost before functional weakness can be demonstrat...

Disruption of dystrophin or the sarcoglycans within the dystrophin protein complex leads to muscular dystro-phy. The dystrophin complex is enriched in myofibers and concentrated into costameres, rib-like structures in the plasma membrane. In the absence of dystrophin, the plasma membrane becomes fragile with reduced stiffness and increased leakiness, and exposing dystro-phin-deficient muscle to...

Motor neurone disease (MND) is a devastating and progressive neurological disorder in which degeneration of motor neurones results in weakness and wasting of the dependent muscles. The prevalence of MND is approximately five per 100 000 and approximately 30% of patients have primary bulbar symptoms. Half of the patients die within 36 months of experiencing the first symptom. Presentation with v...

muscle diseases constitute a large variety of both acquired and hereditary disorders that can affect muscle structure, metabolism, or the function of the muscle channel. a successful clinical approach to a patient with a suspected myopathy is based on a thorough medical history and neurological examination. associated clinical symptoms such as myoglobinuria, contractures, myotonia, cardiac dise...

potentials and twitch force were measured in muscle strips obtained from patients and controls. Of the 36 patients, 25 presented with chronic muscle weakness of varying degrees, up to wheelchair-dependence. The weakness was associated with intracellular Na overload and edema. Older patients revealed a vacuolar myopathy or a progressive muscular dystrophy. Weakness, intracellular Na overload and...

potentials and twitch force were measured in muscle strips obtained from patients and controls. Of the 36 patients, 25 presented with chronic muscle weakness of varying degrees, up to wheelchair-dependence. The weakness was associated with intracellular Na overload and edema. Older patients revealed a vacuolar myopathy or a progressive muscular dystrophy. Weakness, intracellular Na overload and...

S arcoidosis is a multisystem granulomatous disorder that may involve many organs. Beside organ specific symptoms, non-specific constitutional complaints such as fatigue and general weakness are frequent. The granuloma formation in sarcoidosis is driven by an array of cytokines such as tumour necrosis factor (TNF)-a, interleukin (IL)-6, interferon (INF)-c and others. High circulating levels of ...