Neuropathy demyelinating

Seroconversion neuropathy Inflammatory demyelinating neuropathy Early pre-AIDS Acute Generalized systemic dlness, mononeuropathies Immune dysfunction... 

Acute inflammatory demyelinating polyradiculopathy Early pre-AIDS Acute Motor to sensory signs NCS show demyelinating features Immune dysfunction complement/macrophage-mediated demyelinating neuropathy... 

Opportunistic infection, eg. CMV Progressive polyradiculopathy AIDS Acute, subacute Multiple, asymmetric mononeuropathies, usually painful CMV infection, Schwann cell infection demyelinating neuropathy... 

Diphtheria causes a demyelinative neuropathy. Coryne-bacterium diphtheriae colonizes the pharynx or open wounds, and secretes a protein exotoxin. The B subunit of this exotoxin binds to plasma membranes and facilitates entry into cytosol of the A subunit, which catalyzes ADP-ribosylation, and inactivation of an elongation factor required for protein synthesis. Cardiac muscle and Schwann cells are particularly susceptible to this toxin, and hence patients with diphtheria develop cardiomyopathy and demyelinative polyneuropathy [20]. While diphtheria is now uncommon because of childhood immunization against C. diphtheriae, the disruption in preventative medicine programs caused by disintegration of the Soviet Union was followed by a substantial incidence of diphtheritic polyneuropathy in Russia. 

Once the monoclonal antibodies reacting with complex glycolipids were shown to occur frequently in patients with neuropathy in association with gammopathy, antibodies were demonstrated in some patients with GBS and other forms of inflammatory demyelinating neuropathy [21]. 

Kieseier, B. C., Dalakas, M. C. and Hartung, H. P. Immune mechanisms in chronic inflammatory demyelinating neuropathy. Neurology 59, S7-S12, 2002. 

In four patients with a pure motor demyelinating neuropathy treated with oral prednisolone (60 mg/day) motor function rapidly deteriorated within 4 weeks of starting prednisolone (SEDA-19, 375 54). Intravenous immunoglobulin some months later in two of them produced clear improvement in strength and motor nerve conduction. 

Demyelinating neuropathies have been reported in some individuals [32]. 

Antoine JC, Mosnier JF, Honnorat J, Convers P, Absi L, Lapras J, et al. Paraneoplastic demyelinating neuropathy, subacute sensory neuropathy, and anti-Hu antibodies Clinicopathological study of an autopsy case. Muscle Nerve 1998 21(7) 850-857. 

Latov N, Renaud S. Effector mechanisms in anti-MAG antibody-mediated and other demyelinating neuropathies. J Neurol Sci 2004 220(1-2) 127-129. 

The neuron and the Schwann cell are the principal cell types in the PNS. There are great morphological, biochemical, and functional differences between neurons and Schwann cells, and this is reflected in the considerable variation in their vulnerability to toxic injury. Some toxic neuropathies are characterized primarily by injury of the neuron, its axon, or its terminal, as evidenced by the presence of axonal degeneration in peripheral nerve, while other toxic neuropathies are characterized primarily by Schwann cell injury, as evidenced by the presence of demyelination. Those neuropathies characterized by axonal injury are often categorized as axonal neuropathies, whereas those characterized by demyelination are categorized as demyelinating neuropathies. ... 

Peters RA (1936) The biochemical lesion in vitamin Bi deficiency, application of modern biochemical analysis in its diagnosis. Lancet 1 1161-1164 Puri V, Chaudhry N, Tatke M, Prakash V (2005) Isolated vitamin E deficiency with demyelinating neuropathy. Muscle Nerve 32(2) 230-235... 

As noted in the preceding section, Cx32 is a ubiquitous protein that is also found in CNS myelin. Its presence in Schwann cells was discovered when Cx32 mutations were associated with Charcot-Marie-Tooth disease of the CMTX type. CMTX is an X-linked demyelinating neuropathy. The molecule is located in the paranodes. There are subtle anomalies of the myelin sheath and the Ranvier node (Hahn et al.,... 

Martini R, Toyka KV (2004) Immune-mediated components of hereditary demyelinating neuropathies Lessons from animal models and patients. Lancet Neurol 3 457-465. 

Kieseier BC, Tani M, Mahad D, Oka N, Ho T, Woodroofe N, Griffin JW, Toyka KV, Ransohoff RA, Hartung HP (2002b) Chemokines and chemokine receptors in inflammatory demyelinating neuropathies A central role for IP-10. Brain 125 823-834. 

Donaghy M, Mills KR, Boniface SJ, Simmons J, Wright 1, Gregson N, Jacobs J. Pure motor demyelinating neuropathy deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry 1994 57(7) 778-83. 

FIGURE 20.61 Locations of T lymphocytes among sural nerve biopsy specimens from 13 cases of chronic inflammatory demyelinat-ing polyneuropathy (CIDP) and 22 cases of Guillain-Barre syndrome (CBS). Percentages positive are as reported in Schmidt B, Toyka KV, Keifer R, et al. Inflammatory infiltrates in sural nerve biopsies in Guillain-Barre syndrome and chronic inflammatory demyelinating neuropathy. Muscle Nerve. 1996 19 474-487. 

Schmidt B, Toyka KV, Kiefer R, et al. Inflammatory infiltrates in strral nerve biopsies in Guillain-Barre syndrome and chronic inflammatory demyelinating neuropathy. Muscle Nerve. 1996 19 474-487. 

Recently a new copper transport defect was described by Buchmann et al. [16] in a patient with symptoms of a demyelinating neuropathy, chronic intestinal pseudo-obstruction, osteoporosis, testicular failure, retinal degeneration and a cardiomyopathy with a tortuous aorta. In this patient, copper could not be incorporated into ceruloplasmin. The symptoms started at age 10 and the patient died at 21 years. These findings were clearly different from Wilson s and Menkes diseases. 

Nervous system Demyelinating neuropathy is a rare adverse reaction to anti-TNF therapy, including adalimumab, etanercept, and infliximab improvement usually cxxurs after drug interruption and/or in association with usual treatments for demyelinating neuropathies [88 ]. 

Dalakas MC, Rakocevic G, Salajegheh M, Dambrosia JM, Hahn AF, Raju R, McElroy B. Placebo-controlled trial of rituximab in IgM anti-myelin-associated glycoprotein antibody demyelinating neuropathy. Aim Neurol 2009 65 286-93. 

Nervous system Demyelinating disorders provoked by mAbs are said to affect 0.02-0.2% of treated patients. Three patients with chronic lymphocytic anaemia developed acute inflammatory demyelinating neuropathy while being treated with alemtuzumab [90 ]. All three showed a good clinical response to treatment with intravenous immunoglobulin. 

Nervous system A number of different demyelinating neuropathies have been associated with the use of infliximab. A 64-year-old man treated with infliximab for psoriasis vulgaris 1 month later developed subacute sensory polyradiculopathy that appeared to be immxme mediated judging by the detection of anti-ganglioside antibodies [147 ]. 

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