By Albert P. Galdi M.D. (auth.)

Neurologists and non-neurologists alike can not forget about illnesses of the neuromuscular method. The previous dogma that those problems are either unusual and untreatable has misplaced its validity. fresh technological advances have enabled us to check extra accurately muscle and nerve anatomy, body structure and biochem­ istry. due to this growth, we're now spotting new neuromuscular di­ seases in addition to diagnosing extra sophisticated situations of myasthenia gravis, myotonia, and metabolic myopathies. remedy of the neuromuscular illnesses has additionally un­ dergone dramatic switch in keeping with new discoveries within the fields of immunology and pharmacology. now not are myotonia, periodic paralysis, and malignant hyperthermia untreatable scientific curiosities. now not are situations of steroid-un­ responsive myositis given up as hopeless. due to most of these advances, non-sur­ gical physicians and particularly neurologists needs to replace their wisdom regard­ ing the neuromuscular problems. This publication, it really is was hoping, might help such clinicians in facing this activity. Emphasis has been put on the prognosis and administration of those problems instead of on their pathophysiology. The extra unusual ailments and people of doubtful lifestyles were passed over purposely and left to the bigger and extra encyclopedic reference works. bankruptcy 1 discusses the medical signs and symptoms of muscle sickness and is designed to allow the reader to formulate a differential prognosis at the foundation of the patient's historical past and actual examination.

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L! ~m i tochon drion ~ ~ -_. ;( '" '" / '" '" '" H ZO NE I· . I "" ""~ th ick filament (myosin) _1_ -------1-- - - -======±====~~======~~====±=====~~~: _ Zime I· A BAND I BAND contracted sarcomere Figure 2·2. The sarcomere . thin fil ament (actin , troponin tropomyosin) and Laboratory Aids for Diagnosing Neuromuscular Disease 21 Electrodiagnostic testing is a useful supplement to the clinical neuromuscular examination. The basic electrical examination (ENMG) consists of two parts: (1) measurement of nerve conduction velocities, and (2) needle electromyography.

On the other hand, when the motor nerve is stimulated by a nerve action potential (NAP), numerous quanta of ACh are liberated. Via similar permeability changes described above, a much greater change in potential is produced, called an end plate potential (EPP). , of sufficient magnitude to change the membrane potential from -80 mV to -55 mV), triggers the generation of a muscle action potential (MAP) (Figure 3-1). The muscle action potential is propagated along the muscle fiber without decay of its amplitude, and is transmitted to the interior of the fiber by means of an elaborate system of membrane infoldings called the transverse tubules.

A decremental response at low rates (l-3/sec) of nerve stimulation, and 3. marked facilitation of the response at high rates of stimulation (20-50/ sec) and after 10 seconds the maximal muscle contraction (see Figure 3-2). , abducton minimi digiti muscle) in all cases, although on clinical grounds, features are evident only in proximal muscles. It is important to note that in most centers, neuromuscular transmission studies are not done as part of Muscle Disease 46 Figure 3-2. Characteristic EMG in the Eaton-Lambert syndrome.

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