generate barcode in c#.net DISEASES OF THE PERIPHERAL NERVES in Microsoft Office

Print QR Code in Microsoft Office DISEASES OF THE PERIPHERAL NERVES

DISEASES OF THE PERIPHERAL NERVES
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solute neutrophil count recovers Individual reports of successful treatment by autologous stem-cell transplantation after high-dose chemotherapy have appeared (Vermuelen and van Oers) This may become an option in severe and treatment-resistant cases We have no explanation for the remarkable improvement and continued good health of a few of our patients after a severe toxic bacterial infection (Ropper) One of the problems most dif cult to address is a lack of clear clinical measurements to guide retreatment with IVIG and plasma exchange and even the proper adjustment of the steroid dose Often one may be in uenced by the patient s fear of losing any ground and even slight changes in sensory or motor symptoms It has been stated that patients with discrete relapses have a better prognosis than those with a progressive course In McCombe s series, 73 percent were said to have eventually recovered but the long-term outcome has generally been poor In a small number of patients, less than 10 percent, has the disease nally burned out Unexplained remission occurs occasionally Multifocal Motor Neuropathy and Multifocal Conduction Block Several polyneuropathies that share many of the features of CIDP have been delineated on the basis of some unique clinical or electrophysiologic attribute These include particularly multifocal motor neuropathy and multifocal conduction block The latter seems to be a type of CIDP with the main feature being a block to sensory and motor conduction across focal sites in a limited number of nerves as described below In multifocal motor neuropathy, there are multiple sites of block in motor nerves alone, without change in sensory conduction The distinction between these two entities has been somewhat blurred by the nding of mild but consistent demyelination in sensory nerve biopsies from patients with multifocal motor neuropathy (Corse et al) While these conditions may perhaps be considered for the moment as types of CIDP, there is a high concordance of multifocal motor neuropathy with a particular IgM antibody, antiGM1, directed against a ganglioside component of peripheral myelin (Pestronk et al) For this reason, some view this illness as belonging to the class of paraproteinemic neuropathies (see earlier and Simmons et al), and it is distinctive enough clinically to be categorized separately Its importance as a clinical entity lies in the similarity of the clinical picture to a purely lower motor neuron type of amyotrophic lateral sclerosis and, unlike ALS, its responsiveness to treatment Multifocal motor neuropathy predomiantes in men It usually begins with an acute to subacute motor mononeuropathy, manifest, for example, as weakness of the wrist or foot drop, and is often joined insidiously by another focal motor palsy The process is painless, unlike vasculitic mononeuritis multiplex, involves the nerve incompletely, and, in its usual form, is unaccompanied by any sensory symptoms such as paresthesias or numbness Despite the initially demyelinating character of the disorder, there is almost always atrophy of the weakened muscle within months, and there may be a few fasciculations, thus reminiscent of ALS Nevertheless, the weakness tends to be out of proportion to atrophy Usually, the tendon re ex is lost or muted in an affected region, but for unexplained reasons, some patients have one or more brisk re exes Our experience has been that this latter re ex change does not reach the point of appearing pathologic and that clonus and Babinski signs are categorically not part of the illness When there is an association with sensory symptoms or sensory loss and there is slowing of sensory conduction in regions of
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motor conduction block, the acronym MADSAM (Multifocal Acquired Demyelinating Sensory and Motor Neuropathy) has been used, but the disorder while similar to multifocal conduction block, more resembles CIDP This also conforms to what has been called Lewis-Sumner syndrome based on the original description by these authors and their colleagues of subacute, painless asymmetric, distal multiple mononeuropathies The ulnar and median nerves were involved and there was motor conduction block and sensory slowing in affected nerves Curiously, two of their ve original patients had optic neuritis, a feature not reported by others The disease is not connected to antibodies against GM1 Treatment For multifocal motor conduction block, IVIG infusions have been highly effective, albeit temporarily, in over half of patients Some authoritative clinicians favor the early addition of cyclophosphamide, 1 g/m2 monthly for 6 months in treatmentresistant cases or when the frequency of infusions is unsustainable Other immune-modulating drugs such as rituximab are being tried There is no response to corticosteroids The MADSAM illness responds similarly to CIDP in most instances Uremic Polyneuropathy Polyneuropathy is among the most common complications of chronic renal failure Robson estimated that it complicates end-stage renal failure in two-thirds of patients who are about to begin dialysis therapy Bolton s gures are much the same; 70 percent of his patients being dialyzed regularly had uremic polyneuropathy and in 30 percent it was moderate or severe in degree As in the original description by Asbury, Victor, and Adams, the neuropathy takes the form of a painless, progressive, symmetrical sensorimotor paralysis of the legs and then of the arms In some patients, the neuropathy begins with burning dysesthesias of the feet or with sensations of creeping, crawling, and itching of the legs and thighs, which tend to be worse at night and are relieved by movement ( restless legs syndrome, page 339) Renal failure accompanied by diabetes may give rise to a particularly severe form The combination of muscle weakness and atrophy, are exia, sensory loss, and the graduated distally predominant distribution of the neurologic de cit in the limbs leaves little doubt about the neuropathic nature of the disorder Usually the neuropathy evolves slowly over many months, at times more subacutely Infrequent instances of a more acute sensorimotor polyneuropathy that have been reported occur mainly in diabetic patients receiving peritoneal dialysis as discussed earlier on (see page 1128; Ropper; Asbury et al) Also, a rare uremic polymyositis with hypophosphatemia has been described (Layzer) The neuropathy has been observed with all types of chronic kidney disease More important to the development of chronic neuropathy than the nature of the renal lesion are the duration and severity of the renal failure and symptomatic uremia With long-term hemodialysis, the neuropathic symptoms and signs stabilize but improve in relatively few patients In fact, rapid hemodialysis may occasionally worsen the polyneuropathy temporarily Peritoneal dialysis appears to be more successful than hemodialysis in improving the neuropathy but this observation has not been rmly established Complete recovery, occurring over a period of 6 to 12 months, usually follows successful renal transplantation for the reasons given later The pathologic ndings are those of a nonspeci c axonal degeneration In rapidly progressive cases, there is a tendency for the large bers to be affected; this is evident particularly on electrophysiologic testing which shows slowing of nerve conduction ve-
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