immune-mediated and cidp

Norman Latov, M.D.,Ph.D.

Autoimmune mechanisms are implicated in several chronic neuropathic syndromes that are amenable to immune therapy (Table I). Collectively, these neuropathies are relatively common; Barohn et al (1998) reported that approximately 13% of consecutive patients with neuropathy seen at their institution had an immune mediated neuropathy, and Verghese et al (2001) found that 6% of their elderly neuropathy patients had a demyelinating inflammatory etiology. M practice, however, many of the autoimmune neuropathies are difficult to diagnose, due to a lack of generally accepted clinical diagnostic criteria, or availability of reliable serological tests. Consequently, many patients with autoimmune neuropathies are diagnosed as having "idiopathic neuropathy" instead, and left untreated despite progression of their disease. (Click here to download entire article)


Diagnosis and Treatment of Chronic Immune-mediated Neuropathies
Norman Latov, M.D., Ph.D. & Kenneth C. Gorson, M.D. & Thomas H. Brannagan, III M.D. & Roy L. Freeman, M.D. & Slobodan Apostolski, M.D. & Alan R. Berger, M.D. & T Walter G. Bradley, D.M, F.R.C.P. & Chiara Briani, M.D. & Vera Bril, M.D. Neil A. Busts, M.D. & Didier P. Giros, M.D. & Marinos C. Dalakas, M.D. & Peter D. Donofrio, M.D. &  P. James B. Dyck, M.D. John D. England, M.D. & Morris A. Fisher, M.D. & David N. Herrmann, M.D. & Daniel L. Menkes, M.D. & Zarife Sahenk, M.D. Howard W. Sander, M.D. & WilliamJ. Triggs, M.D.& Jean Michel Vallat, M.D.

The chronic autoimmune neuropathies are a diverse group of syndromes that result from immune-mediated damage to the peripheral nerves. Our understanding of these disorders has evolved through clinŽical observations and empiric therapeutic interventions, that were confirmed by independent investigators over the years. For many of these disorders, there are no definitive diagnostic tests, and only a few or no controlled therapeutic trials. Consequently, the diagnoses may be missed and the patients remain untreated. The Medical Advisory Board of the Neuropathy Association therefore reviewed the existing literature regarding the diagnosis and treatment of the immune-mediated neuropathies, with the aim of summarizing and presenting the information in a concise form, so as to help physicians recognize these disorders and decide on the most appropriate therapy. (Click here to download entire article)


Immune-Mediated Autonomic Neuropathies
Roy Freeman, M.D.

Autonomic nerve fibers are affected in most generalized peripheral neuropathies. While this involvement is often mild or subclinical, there are a group of peripheral neuropathies in which the small or unmyelinated fibers are selectively or prominently targeted. While most generalized peripheral polyneuropathies are accompanied by clinical or subclinical autonomic dysfunction, there are a group of peripheral neuropathies in which the small or unmyelinated fibers are selectively targeted. In these neuropathies, autonomic dysfunction is the primary manifestation. A constellation of signs and symptoms occur from impairment of cardiovascular, gastrointestinal, urogenital, thermoregulatory, sudomotor and pupillomotor autonomic function. (Click here to download entire article)


Guillain Barre Syndrome and Its Variants
Alan R. Berger, M.D.

Guillain Barre Syndrome most commonly characterized by some combination of limb paresthesias, generalized weakness, and areflexia. Pathogenesis of GBS not yet fully understood and current thinking is that GBS may not be a single disease, but a variety of acute neuropathies with a number of related immune-mediated pathogenetic mechanisms. Most common immunopathologic finding: endoneurial inflammation in spinal nerves roots, distal nerve segments, or around potential nerve entrapment sites. Target antigens appear to be common to the axon, myelin sheath, or both. The exact antigens, the precipitating event, and the resultant mechanism of injury somewhat unclear. (Click here to download entire article)


Vasculitic Neuropathy
Jose R. Carlo, M.D., F.A.A.N.

The vasculitides are a group of heterogeneous disorders which present with a variable and complex clinical picture. Debates over clinical versus pathological approaches to classification abound in the literature, all these, with recognized limitations given the variable clinical presentations and the overlap between the recognized diagnostic entities. Peripheral neuropathy is an important, and often the presenting clinical feature of the vasculidities. Its recognition can be critical to attain an early diagnosis in these disorders where the ultimate outcome can be greatly influenced by early therapeutic intervention. (Click here to download entire article)


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