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Electrophysiological examination for signs of demyelination
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Electrodiagnostic and CSF criteria fulfilled and no evidence of another cause of polyneuropathy
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Signs of demyelination in sural-nerve biopsy specimen and no evidence or other cause of polyneuropathy
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Start treatment for chronic inflammatory demyelinating polyneuropathy
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Figure 12 1 Diagnostic approach Algorithm of diagnostic procedures If a patient presents with a history of symptoms suggestive of chronic in ammatory demyelinating polyneuropathy of 2 months duration or more, we perform nerve-conduction studies for signs of demyelination including partial conduction block, reduced motor-nerve CV, prolonged distal latency of the motor nerve, and the absence of F waves or a prolonged F-wave latency to differentiate between predominantly demyelinating and axonal disease of peripheral nerves We also use laboratory tests including cell-count and protein studies of cerebrospinal uid (CSF) to evaluate supportive criteria and to rule out other causes If these causes have been ruled out and electrodiagnostic and supportive CSF criteria are ful lled, patients may begin long-term immunomodulating/ immunosuppressive therapy A sural nerve is helpful if diagnosis is not clear (With permission from Koller N Engl J Med 2005;352(13):1343 1356, Fig 2, p 1349)
abnormal, indicating proximal demyelination of the sensory roots Cranial neuropathies can lead to mild facial weakness, ophthalmoparesis, dysarthria, dysphagia, hearing loss, or vertigo1,3,21,22,38,39 A rare presentation is neck extensor weakness leading to dropped head syndrome40 Papilledema may be seen in a few patients, particularly those with a CIDP-line neuropathy related to POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) Respiratory insuf ciency secondary to intercostal muscle and diaphragm is rare in idiopathic CIDP, and development should also make one consider POEMS21,22,41 As POEMS is frequently associated with an osteosclerotic myeloma, a skeletal survey and scans for focal plasmacytomas or lymphoma should be performed
As many as 3% of patients with CIDP develop evidence of central nervous system (CNS) demyelination clinically, electrophysiologically (evoked potential studies), or by magnetic resonance imaging (MRI) scans42 48 Attacks of CNS demyelination can precede or follow the onset of CIDP or may be entirely asymptomatic lesions It is unclear if these patients have multiple sclerosis or a distinct immunologic disorder of the CNS Several medical conditions, particularly other autoimmune disease, HIV infections, and immunoproliferative disorders/malignancies, are associated with CIDP or a CIDPlike neuropathy (Table 12 4)1,13,36,49 55 Rarely, patients with diabetes mellitus develop a CIDP-like neuropathy with symmetric proximal and distal extremity weakness, elevated CSF protein, and demyelinating features on the NCS and nerve biopsies56 60 These patients may have an unusual form of diabetic neuropathy, incidental
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TABLE 12 2 AMERICAN ACADEMY OF NEUROLOGY RESEARCH CRITERIA FOR DIAGNOSIS OF CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY (CIDP)
I Clinical A Mandatory 1 Progressive or relapsing motor and sensory, rarely only motor or sensory, dysfunction of more than one limb of a peripheral nerve nature, developing over at least 2 months 2 Hypo or are exia This will usually involve all four limbs B Supportive 1 Large- ber sensory loss predominates over small- ber sensory loss C Exclusion 1 Mutilation of hands or feet, retinitis pigmentosa, ichthyosis, appropriate history of drug or toxic exposure known to cause a similar peripheral neuropathy, or family history of a genetically based peripheral neuropathy 2 Sensory level 3 Unequivocal sphincter disturbance II Physiologic studies A Mandatory Nerve conduction studies including studies of proximal nerve segments in which the predominant process is demyelination Must have three of four: 1 Reduction in conduction velocity (CV) in two or more motor nerves: a <80% of lower limit of normal (LLN) if amplitude >80% of LLN b <70% of LLN if amplitude <80% of LLN 2 Partial conduction block or abnormal temporal dispersion in one or more motor nerves: peroneal nerve between ankle and below bular head, median nerve between wrist and elbow, or ulnar nerve between wrist and below elbow Criteria suggestive of partial conduction block: <15% change in duration between proximal and distal sites and >20% drop in negative-peak ( p) area or peak-to-peak (p p) amplitude between proximal and distal sites Criteria for abnormal temporal dispersion and possible conduction block: >15% change in duration between proximal and distal sites and >20% drop in -p area or p p amplitude between proximal and distal sites These criteria are only suggestive of partial conduction block, as these are derived from studies of normal individuals Additional studies, such as stimulation across short segments or recordings of individual motor unit potentials, are required for con rmation 3 Prolonged distal latencies in two or more nerves: a >125% of upper limit of normal (ULN) if amplitude >80% of LLN b >150% of ULN if amplitude <80% of LLN 4 Absent F waves or prolonged minimum F-wave latencies (10 15 trials) in two or more nerves: a >120% of ULN if amplitude >80% of LLN b >150% of ULN if amplitude <80% of LLN B Supportive 1 Reduction in sensory CV <80% of LLN 2 Absent H re exes III Pathologic features A Mandatory 1 Nerve biopsy showing unequivocal evidence of demyelination and remyelination 2 Demyelination by either electron microscopy (>5 bers) or teased ber studies (>12% of 50 teased bers, minimum of four internodes each, demonstrating demyelination/remyelination) B Supportive 1 Subperineurial or endoneurial edema 2 Mononuclear cell in ltration 3 Onion-bulb formation 4 Prominent variation in the degree of demyelination between fascicles C Exclusion 1 Vasculitis, neuro lamentous swollen axons, amyloid deposits, or intracytoplasmic inclusions in Schwann cells or macrophages, indicating adrenoleukodystrophy, metachromatic leukodystrophy, globoid cell leukodystrophy, or other evidence of speci c pathology IV CSF studies A Mandatory 1 Cell count <10/mm3 if HIV seronegative or <50/mm3 if HIV seropositive 2 Negative VDRL B Supportive 1 Elevated protein
Diagnostic categories for research purposes: DEFINITE: Clinical A and C, Physiology A, Pathology A and C, and CSF A PROBABLE: Clinical A and C, Physiology A, and CSF A POSSIBLE: Clinical A and C and Physiology A Laboratory studies: Depending on the results of the laboratory tests, those patients meeting the criteria above will be classi ed into the groups listed below The following studies are suggested: CBC, ESR, SMA 6/12, CK, ANA, thyroid functions, serum and urine immunoglobulin studies (to include either immuno xation electrophoresis or immunoelectrophoresis), and HIV and hepatitis serology The list of laboratory studies is not comprehensive For instance, in certain clinical circumstances other studies may be indicated, such as phytanic acid, long-chain fatty acids, porphyrins, urine heavy metals, and alpha-lipoprotein CSF, cerebrospinal uid With permission from Cornblath DR, Asbury AK, Albers JW, et al: Research criteria for diagnosis of chronic in ammatory demyelinating polyneuropathy (CIDP) Neurology 1991;41:617 618 Download at BoykmaCom
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