Chronic Inflammatory Demyelinating Polyneuropathy and Other Immune-Mediated Demyelinating Neuropathies

ABSTRACT

The identification of specific disorders caused by immune-mediated attack on Schwann cells and myelin have both improved and confused our understanding and classification of chronic inflammatory and immune-mediated neuropathies. Some investigators consider all neuropathies that respond to immunosuppressive treatment as chronic inflammatory demyelinating polyneuropathy (CIDP), regardless of whether there is evidence for demyelination. Other investigators rely on a very strict definition of CIDP and do not include variations. The goal of this article is to clarify the spectrum of these disorders and provide a logical classification scheme that has clinical utility and yet allows for new information that might distinguish new variations. Therapeutic strategies are also discussed.

KEYWORDS

CIDP (chronic inflammatory demyelinating polyneuropathy) - myelin-associated glycoprotein - CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, IgM paraproteinemia, cold agglutinins, and disialosyl antibodies) - paraprotein - MMN (multifocal motor neuropathy) - Lewis-Sumner syndrome - multifocal acquired demyelinating sensory and motor (neuropathy) - paraprotein - conduction block

REFERENCES

• 1 Dyck P J, Lais A C, Ohta M et al.. Chronic inflammatory demyelinating polyradiculoneuropathy.  Mayo Clin Proc. 1975;  50 621-637
• 2 Haq R U, Pendlebury W W, Fries T J, Tandan R. Chronic inflammatory demyelinating polyradiculoneuropathy in diabetic patients.  Muscle Nerve. 2003;  27 465-470
• 3 Sharma K R, Cross J, Farronay O, Ayyar D R, Shebert R T, Bradley W G. Demyelinating neuropathy in diabetes mellitus.  Arch Neurol. 2002;  59 758-765
• 4 Gorson K C, Ropper A H, Adelman L S, Weinberg D H. Influence of diabetes mellitus on chronic inflammatory demyelinating polyneuropathy.  Muscle Nerve. 2000;  23 37-43
• 5 Krendel D A, Costigan D A, Hopkins L C. Successful treatment of neuropathies in patients with diabetes mellitus.  Arch Neurol. 1995;  52 1053-1061
• 6 Ad Hoc Subcommittee of the American Academy of Neurology Task Force . Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP).  Neurology. 1991;  41 617-618
• 7 Saperstein D S, Katz J S, Amato A A, Barohn R J. The clinical spectrum of chronic acquired demyelinating polyneuropathy.  Muscle Nerve. 2001;  24 311-324
• 8 Hughes R, Bensa S, Willison H et al.. Randomized controlled trial of intravenous immunoglobulin versus oral prednisone in chronic inflammatory demyelinating polyradiculoneuropathy.  Ann Neurol. 2001;  50 195-201
• 9 Magda P, Latov N, Brannagan III T H, Weimer L H, Chin R L, Sander H W. Comparison of electrodiagnostic abnormalities and criteria in a cohort of patients with chronic inflammatory demyelinating polyneuropathy.  Arch Neurol. 2003;  60 1755-1759
• 10 Gorson K G, Allam G, Ropper A H. Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy.  Neurology. 1997;  48 321-328
• 11 Rotta F T, Sussman A T, Bradley W G, Ram Ayyar D, Sharma K R, Shebert R T. The spectrum of chronic inflammatory demyelinating polyneuropathy.  J Neurol Sci. 2000;  173 129-139
• 12 Kaku D A, England J D, Sumner A J. Distal accentuation of conduction slowing in polyneuropathy associated with antibodies to myelin-associated glycoprotein and sulphated glucuronyl paraglobiside.  Brain. 1994;  117 941-947
• 13 Katz J S, Saperstein D S, Gronseth G, Amato A A, Barohn R J. Distal acquired demyelinating symmetric neuropathy.  Neurology. 2000;  54 615-620
• 14 Willison H J, O’Leary C P, Veitch J et al.. The clinical and laboratory features of chronic sensory ataxic neuropathy with anti-diasialosyl IgM antibodies.  Brain. 2001;  124 1968-1977
• 15 Willison H J, Yuki N. Peripheral neuropathies and anti-glycolipid antibodies.  Brain. 2002;  125 2591-2625
• 16 Lopate G, Choksi R, Pestronk A. Severe sensory ataxia and demyelination polyneuropathy with IgM anti-GM2 and GalNAc-GD1A antibodies.  Muscle Nerve. 2002;  25 828-836
• 17 Soubrier M J, Dubost J J, Sauvezie B JM. POEMS syndrome: a study of 25 cases and a review of the literature.  Am J Med. 1994;  97 543-553
• 18 Katz J S, Barohn R J, Kojan S et al.. Axonal multifocal motor neuropathy without conduction block or other features of demyelination.  Neurology. 2002;  58 615-620
• 19 Parry G J, Clarke S. Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease.  Muscle Nerve. 1988;  11 103-107
• 20 Pestronk A, Cornblath D R, Ilyas A A et al.. A treatable multifocal neuropathy with antibodies to GM1 ganglioside.  Ann Neurol. 1988;  24 73-78
• 21 Chad D A, Hammer K, Sargent J. Slow resolution of multifocal weakness and fasciculation: a reversible motor neuron syndrome.  Neurology. 1982;  32 958-964
• 22 Engel W K, Hopkins L C, Rosenberg B J. Fasciculating progressive muscular atrophy (F-PMA) remarkably responsive to antidysimmune treatment (ADIT): a possible clue to more ordinary ALS?.  Neurology. 1985;  335(suppl 1b) 72
• 23 Freddo L, Yu R K, Latov N et al.. Gangliosides GM1 and GD1b are antigens for IgM M-protein in a patient with motor neuron disease.  Neurology. 1986;  36 454-458
• 24 Roth G, Rohr J, Magistris M R, Oschner F. Motor neuropathy with proximal multifocal persistent block, fasciculations and myokymia. Evolution to tetraplegia.  Eur Neurol. 1986;  25 416-423
• 25 Bouche P, Moulonguet A, Ben Younes-Chennnoufi A et al.. Multifocal motor neuropathy with conduction block: a study of 24 patients.  J Neurol Neurosurg Psychiatry. 1995;  59 38-44
• 26 Comi G, Amadio S, Galardi G, Fazio R, Nemni R. Clinical and neurophysiological assessment of immunoglobulin therapy in five patients with multifocal motor neuropathy.  J Neurol Neurosurg Psychiatry. 1994;  57(suppl) 35-37
• 27 Leger J-M, Younes-Chennoufi A B, Chassande B et al.. Human immunoglobulin treatment of multifocal motor neuropathy and polyneuropathy associated with monoclonal gammopathy.  J Neurol Neurosurg Psychiatry. 1994;  57(suppl) 46-49
• 28 Lange D J, Trojaberg W, Latov N et al.. Multifocal motor neuropathy with conduction block: is it a distinct clinical entity?.  Neurology. 1992;  42 497-505
• 29 Oh S J, Kim D E, Kuruguolu H R. What is the best diagnostic index of conduction block and temporal dispersion?.  Muscle Nerve. 1994;  17 489-493
• 30 Chaudhry V, Cornblath D R, Griffin J W et al.. Multifocal motor neuropathy: electrodiagnostic features.  Muscle Nerve. 1994;  17 198-205
• 31 American Association of Electrodiagnostic Medicine . Consensus criteria for the diagnosis of partial conduction block.  Muscle Nerve. 1999;  22 S225-S229
• 32 Katz J S, Wolfe G I, Bryan W W, Jackson C E, Amato A A, Barohn R J. Electrophysiologic findings in multifocal motor neuropathy.  Neurology. 1997;  48 700-707
• 33 Pakiam A S, Parry G J. Multifocal motor neuropathy without overt conduction block.  Muscle Nerve. 1998;  21 243-245
• 34 Weimer L H, Grewal R P, Lange D J. Electrophysiologic abnormalities other than conduction block in multifocal motor neuropathy.  Muscle Nerve. 1994;  9 A1089
• 35 Van den Berg L H, Franssen H, Van Doorn P A, Wokke J H. Intravenous immunoglobulin treatment in lower motor neuron disease associated with highly raised anti-GM1 antibodies.  J Neurol Neurosurg Psychiatry. 1997;  63 674-677
• 36 Azulay J P, Blin O, Pouget J et al.. Intravenous immunoglobulin treatment in patients with motor neuron syndromes associated with anti-GM1 antibodies: a double-blind, placebo controlled study.  Neurology. 1994;  44 429-432
• 37 Tsai C P, Lin K P, Liao K K et al.. Immunosuppressive treatment in lower motor neuron syndrome with autoantibodies against GM1.  Eur Neurol. 1993;  33 446-449
• 38 Olney R K, Lewis R A, Putnam T D, Campellone Jr J V. Consensus criteria for the diagnosis of multifocal motor neuropathy.  Muscle Nerve. 2003;  27 117-121
• 39 Kaji R, Shibasaki H, Kimura J. Multifocal demyelinating motor neuropathy: cranial nerve involvement and immunoglobulin therapy.  Neurology. 1992;  42 506-509
• 40 Kaji R, Oka N, Tsuji T et al.. Pathological findings at the site of conduction block in multifocal motor neuropathy.  Ann Neurol. 1993;  33 152-158
• 41 Kaji R, Kimura J. Facts and fallacies on anti-GM1 antibodies: physiology of motor neuropathies.  Brain. 1999;  122 797-798
• 42 Ogawa-Goto K, Funamoto N, Abe T et al.. Different ceramide compositions of gangliosides between human motor and sensory nerves.  J Neurochem. 1990;  55 1486-1493
• 43 Sheikh K A, Deerinck T J, Ellisman M H, Griffin J W. The distribution of ganglioside-like moieties in peripheral nerves.  Brain. 1999;  122 449-460
• 44 Takigawa T, Yasuda H, Kikkawa R, Shigeta Y, Saida T, Kitasato H. Antibodies against GM1 ganglioside affect K+ and Na+ currents in isolated rat myelinated nerve fibers.  Ann Neurol. 1995;  37 436-442
• 45 Waxman S G. Sodium channel blockade by antibodies: a new mechanism of neurological disease?.  Ann Neurol. 1995;  37 421-423
• 46 Pestronk A, Choksi R. Multifocal motor neuropathy. Serum IgM anti-GM1 ganglioside antibodies in most patients detected using covalent linkage of GM1 to ELISA plates.  Neurology. 1997;  49 1289-1292
• 47 Van Schaik I N, Bossuyt P M, Brand A, Vermeulen M. Diagnostic value of GM1 antibodies in motor neuron disorders and neuropathies: a meta-analysis.  Neurology. 1995;  45 1570-1577
• 48 Carpo M, Allaria S, Scarlato G, Nobile-Orazio E. Anti-GM1 IgM antibodies in multifocal motor neuropathy: slightly improved detection with covalink ELISA technique.  Neurology. 1999;  53 2206-2207
• 49 Santoro M, Uncini A, Corbo M et al.. Experimental conduction block induced by serum from a patient with anti-GM1 antibodies.  Ann Neurol. 1992;  31 385-390
• 50 Van den Berg L H, Kerkhoff H, Oey P L et al.. Treatment of multifocal motor neuropathy with high dose intravenous immunoglobulin: a double blind, placebo controlled study.  J Neurol Neurosurg Psychiatry. 1995;  59 248-252
• 51 Federico P, Zochodne D W, Hahn A F, Brown W F, Feasby T E. Multifocal motor neuropathy improved by IVIg. Randomized, double-blind, placebo-controlled study.  Neurology. 2000;  55 1256-1262
• 52 Léger J-M, Chassande B, Musset L, Meininger V, Bouche P, Bauman N. Intravenous immunoglobulin therapy in multifocal motor neuropathy: a double-blind placebo-controlled study.  Brain. 2001;  124 145-153
• 53 Van den Berg L H, Franssen H, Wokke J HJ. The long term effect of intravenous immunoglobulin treatment in multifocal motor neuropathy.  Brain. 1998;  121 421-428
• 54 Azulay J-Ph, Rihet P, Pouget J et al.. Long term follow up of multifocal motor neuropathy with conduction block under treatment.  J Neurol Neurosurg Psychiatry. 1997;  62 391-394
• 55 Meucci N, Cappellari A, Barbieri S, Scarlato G, Nobile-Orazio E. Long term effect of intravenous immunoglobulins and oral cyclophosphamide in multifocal motor neuropathy.  J Neurol Neurosurg Psychiatry. 1997;  63 765-769
• 56 Van den Berg-Vos R M, Franssen H, Wokke J HJ, Van den Berg L H. Multifocal motor neuropathy: long-term clinical and electrophysiological assessment of intravenous immunoglobulin maintenance treatment.  Brain. 2002;  125 1875-1886
• 57 Nobile-Orazio E, Meucci N, Carpo M et al.. Multifocal motor neuropathy: clinical and immunological features and response to IVIg in relation to the presence and degree of motor conduction block.  J Neurol Neurosurg Psychiatry. 2002;  72 761-766
• 58 Van den Berg-Vos R M, Franssen H, Wokke J HJ, Van Es H V, Van den Berg L H. Multifocal motor neuropathy: diagnostic criteria that predict the response to immunoglobulin.  Ann Neurol. 2000;  48 919-926
• 59 Van den Berg-Vos R M, Franssen H, Visser J et al.. Disease severity in multifocal motor neuropathy and its association with the response to immunoglobulin treatment.  J Neurol. 2002;  249 330-336
• 60 Pestronk A, Lopate G, Kornberg A J et al.. Distal lower motor neuron syndrome with high-titer serum IgM anti-GM1 antibodies: improvement following immunotherapy with monthly plasma exchange and intravenous cyclophosphamide.  Neurology. 1994;  44 2027-2031
• 61 Pestronk A. Multifocal motor neuropathy: diagnosis and treatment.  Neurology. 1998;  51(suppl 5) S22-S24
• 62 Lewis R A, Sumner A J, Brown M J, Asbury A K. Multifocal demyelinating neuropathy with persistent conduction block.  Neurology. 1982;  32 958-962
• 63 Saperstein D S, Amato A A, Wolfe G I et al.. Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome.  Muscle Nerve. 1999;  22 560-566
• 64 Van den Berg-Vos R M, Van den Berg L H, Franssen H et al.. Multifocal inflammatory demyelinating neuropathy: a distinct clinical entity?.  Neurology. 2000;  54 26-32
• 65 Oh S J, Claussen G C, Kim D S. Motor and sensory demyelinating mononeuropathy multiplex (multifocal motor and sensory demyelinating neuropathy): a separate entity or a variant of chronic inflammatory demyelinating polyneuropathy.  J Peripher Nerv Syst. 1997;  2 362-369
• 66 Viala K, Renié L, Maisonobe T et al.. Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome.  Brain. 2004;  127 2010-2017
• 67 Nikhar N J, Lewis R A. Multifocal sensorimotor demyelinating neuropathy with persistent conduction block is distinct from multifocal motor neuropathy.  Neurology. 1998;  50(suppl) A206
• 68 Parry G J. Are multifocal motor neuropathy and Lewis-Sumner syndrome distinct nosologic entities?.  Muscle Nerve. 1999;  22 557-559
• 69 Lewis R A. Relationship of multifocal motor neuropathy and Lewis-Sumner syndrome.  Muscle Nerve. 1999;  23 1738-1739
• 70 Barhon R J, Kissel J T, Warmolts J R, Mendell J R. Chronic inflammatory demyelinating polyradiculoneuropathy.  Arch Neurol. 1989;  46 878-884