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portion began as acute GBS but continued to progress or relapsed in the following months (Gorson et al, 1997) Other comprehensive accounts have been given by Barohn, Cornblath, Dyck, and Hughes and their associates All of these studies delimited the time course of CIDP to encompass progression for longer than 8 or 12 weeks The term subacute demyelinating neuropathy has been used to describe a Guillain-Barre illness that progresses for only 4 to 8 weeks and is responsive to corticosteroids Also recognized is the frequency (up to one quarter of the patients in some series, less often in our experience) with which there was a systemic condition such as paraproteinemia, lymphoma, an undifferentiated reactive adenopathy, or lupus in association with an in ammatory demyelinating polyneuropathy These associations create problems in nosology that can be reconciled by labeling a given instance as CIDP with paraproteinemia, CIDP with lupus, etc, thus separating such cases from the idiopathic variety These symptomatic in ammatory polyneuropathies respond to corticosteroids, albeit unpredictably, and to treatment of the underlying disease The status of a predominantly axonal polyneuropathy that clinically simulates CIDP and responds to some extent to the same immunomodulating treatments has been described by Uncini et al and by Gorson and Ropper The present authors have the impression that it is an immune-mediated neuropathy comparable to CIDP but obviously with preferential destruction of axons rather than of myelin Its frequency as a cause of acquired polyneuropathy is not known but we see several new cases every year Laboratory Features The CSF protein is elevated in 80 percent of patients with CIDP, typically in the range of 75 to 200 mg/dL In rare instances there is papilledema and a pseudotumor syndrome in relation to extremely high levels of CSF protein (usually 1000 mg/dL) Elevation of the CSF gamma globulin fraction and a mild lymphocytic pleocytosis are reportedly found in 10 percent of patients (more consistently in those that are HIV-seropositive), a considerably higher percentage than in our series In biopsy material (sural nerve), half of cases are found to have interstitial and perivascular in ltrates of in ammatory cells, although one expects that most nerves would show these changes if a suf cient number could be sampled In other biopsy specimens there is only demyelination, or in cases of long-standing, severe depletion of all nerve bers As in GBS, the demyelination appears to be effected by T cells and macrophages within the endoneurium and perineurium The loss of myelinated bers is variable and many of the remaining bers are seen to be undergoing wallerian degeneration or show changes of segmental demyelination or demyelination-remyelination So-called onion-bulb formations are conspicuous in the recurrent and relapsing cases The few complete autopsy studies have shown only minimal or patchy in ammation and a considerable degree of axonal damage, probably re ecting the long duration of illness before examination The presence of endoneurial and subperineurial edema has been emphasized by Prineas and McLeod Treatment Several well-conducted trials have shown that there is short-term bene t from the intravenous infusion of high doses of gamma globulin (2 g/kg in divided infusions over 4 or 5 days) More than half of our patients have responded to this treatment, albeit for only several weeks or months, after which the infusions must be repeated A desire to spare patients the side effects of inde nite prednisone administration (see later) make this mode of therapy a reasonable alternative Some patients have been treated with repeated infusions for almost 10 years without ill effects Pa-.

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RDLC reports, created by the Visual Studio ReportViewer control based on Report Definition Language Client Side, are local reports and completely run in local ...
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tients who require treatment at such short intervals as to be impractical have bene ted from the addition of small doses of prednisone or of an immunosuppressive drug as described below The main drawbacks of IVIG are its expense and the several hours required for its infusion Rare instances of nephrotic syndrome, aseptic meningitis, serum sickness, thrombolic arterial occlusion, including stroke and hypotension have been reported, particularly if the infusion is too rapid Half of patients with CIDP also respond well to plasma exchanges In a prospective double-blind trial, Dyck and colleagues found that plasma exchange administered twice weekly for 3 weeks had a bene cial effect on both neurologic disability and nerve conduction The response to plasma exchange in our patients has been comparable to that obtained with IVIG and with steroids, but it has become clear that some patients respond to one type of treatment and not the other The bene cial effects in most patients begin to subside in 10 to 21 days, or even less In some cases the response lasts longer, as found by Dyck and in the series by Hahn et al For these reasons we prefer to try plasma exchange or immune globulin before committing a patient to long-term treatment with prednisone The relative ease of administering IVIG favors its use rst, followed by a series of plasma exchanges if there is no improvement When there is a clear response, three or four brief series of plasma exchanges or repeated infusions of immune globulin often suf ce to bring the patient to an improved level of function These treatments can be supplemented by small doses of prednisone when frequent infusions or exchanges become impractical It has been our experience that in about one-third of cases, IVIG and plasma exchange may cease to have clinical bene t after repeated use for one or more years Corticosteroids were formerly the mainstay of therapy, but most patients become dependent on the medication and correspondingly suffer side effects The general approach has shifted to utilizing these medications as a third type of treatment after plasma exchange or IVIG, and as an adjunct to one of the previously mentioned treatments The usual regimen begins with 60 to 80 mg of prednisone daily that is tapered over months to the lowest effective dose, typically 25 to 40 mg A small number of patients will have no response to corticosteroids within the rst 1 or 2 months but will do so if treatment is continued The drug produces tremor or may exaggerate the tremor caused by the neuropathy Should a sustained trial of prednisone therapy prove unsuccessful, a course of azathioprine (for at least 3 months), 3 mg/ kg in a single daily dose, has been recommended (Dalakas and Engel), but as mentioned, a controlled trial has failed to show bene t from this combination and we have had little success with it When the preceding measures prove unsatisfactory, cyclophosphamide or mycophenolate can be added, or another similar immunosuppressive medication, but we have been unable to draw any rm conclusions as to the effectiveness of these combined regimens Some patients who have failed to bene t from the aforementioned treatments have improved in response to the administration of -interferon (Gorson et al), and Vallat has shown in a small trial that -interferon may be similarly successful in a small group of patients who failed other therapies A larger trial is being conducted in order to determine if adjunctive treatment with -interferon allows an increase in the interval between infusions of IVIG Highdose cyclophosphamide has proven helpful in several cases under our care, although it has as often failed (see Brannagan et al) The usual regimen is 50 mg/kg IV daily for 4 days followed by granulocyte-stimulating factor beginning on the tenth day until the ab-.

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