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Klinische Neurophysiologie 2003; 34(3): 111-118
DOI: 10.1055/s-2003-42251
Übersicht
© Georg Thieme Verlag Stuttgart · New York

Chronisch inflammatorische demyelinisierende Polyneuropathie - elektrophysiologische Diagnostik

Chronic Inflammatory Demyelinating Polyneuropathy - Electrophysiological DiagnosedH.  Grehl1 , A.  Jaspert2
  • 1Neurologische Klinik, Evangelisches und Johanniter-Klinikum Duisburg
  • 2Klinik für Neurologie und Klinische Neurophysiologie, Alfried-Krupp-Krankenhaus, Essen
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Publication History

Publication Date:
18 September 2003 (online)

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Zusammenfassung

Als immunvermittelte und damit prinzipiell gut behandelbare Polyneuropathie sollte die CIDP möglichst frühzeitig und sicher diagnostiziert werden. Die typische klinische Symptomatik besteht in progredienten oder schubförmigen, oft proximal-betonten, symmetrischen, vorwiegend motorischen peripheren Defiziten. Grundsätzlich basiert die Diagnosesicherung aber auf elektrophysiologischen Parametern. Während entsprechend der diagnostischen Kriterien der „American Academy of Neurology” eine signifikante Demyelinisierung in zahlreichen Nerven mittels verschiedener Parameter nachgewiesen werden muss, sollte - aufgrund atypischer Sonderformen der CIDP mit mehr umschriebenen, asymmetrischen oder distal-betonten klinischen Defiziten - mittlerweile besonderer Wert auf den Nachweis umschriebener Entmarkungen wie Leitungsblock oder fokale temporale Dispersion gelegt werden. Sind gerade zu Beginn der Erkrankung die Läsionen nur sehr umschrieben, kann die proximale Nervenstimulation zum Nachweis fokaler Entmarkungen im Bereich von Plexus oder Wurzel von besonderer Bedeutung sein. Von der idiopathischen CIDP abgegrenzt werden sollten symptomatische Formen wie die CIDP bei monoklonalen Gammopathien, da sich hier häufig therapeutische Besonderheiten ergeben. Therapeutisch kommen bei der CIDP neben den drei Basistherapieverfahren (Kortison, hoch dosierte Immunglobuline, Plasmapherese) auch verschiedene klassische immunsuppressive Medikamente in Betracht. Zur Therapiekontrolle sollte neben einer validierten klinischen Befunderhebung auch der elektrophysiologische Verlauf mit besonderer Berücksichtigung der Leitungsblockdiagnostik herangezogen werden.

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune and treatable disease. Therefore, CIDP should be diagnosed as early as possible. Typical clinical symptoms of CIDP are progressive or stepwise, often proximal, symmetrical deficits with predilection of motor nerves. Diagnosis of CIDP is predominantly based on electrophysiological criteria. According to the diagnostic criteria of the American Academy of Neurology, significant demyelination must be demonstrated in multiple nerves by different parameters. However, most acquired demyelinating polyneuropathies with more focal, asymmetrical or distally accentuated clinical deficits are also considered to be variants of CIDP. Therefore, diagnostic evidence of focal demyelination with conduction block or focal temporal dispersion is more important. In early CIDP, focal demyelination might be limited to proximal nerve segments as plexus or dorsal roots and proximal nerve stimulation can be necessary to demonstrate focal demyelinating lesions. Symptomatic forms of CIDP as in monoclonal gammopathy should be differentiated from idiopathic CIDP because of differences in treatment response. Therapy of CIDP comprises three alternative basic regimens (steroids, immunoglobulins, plasmapheresis). In non-responders, different immunosuppressive agents can be applied. Treatment response should be evaluated by standardised and scored clinical examinations as well as electrophysiological tests including conduction block studies.

Key words

Chronic inflammatory demyelinating polyneuropathy - CIDP criteria - conduction block - temporal dispersion - monoclonal gammopathy

Literatur

  • 1 Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force . Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP).  Neurology. 1991;  41 617-618
    PubMedGoogle Scholar
  • 2 Lunn M P, Manji H, Choudhary P P, Hughes R AC, Thomas P K. Chronic inflammatory demyelinating polyradiculoneuropathy: a prevalence study in southeast England.  J Neurol Neurosurg Psychiatry. 1999;  66 677-680
    CrossrefPubMedGoogle Scholar
  • 3 McLeod J F, Pollard J D, Macaskill P, Mohamed A, Spring P, Khurana V. Prevalence of chronic inflammatory demyelinating polyneuropathy in New South Wales, Australia.  Ann Neurol. 1999;  46 910-913
    CrossrefPubMedGoogle Scholar
  • 4 Mygland A, Monstadt P. Chronic polyneuropathies in Vest-Agder, Norway.  Eur J Neurol. 2001;  8 157-165
    CrossrefPubMedGoogle Scholar
  • 5 Barohn R J. Approach to peripheral neuropathy and neuronopathy.  Semin Neurol. 1998;  18 7-18
    PubMedGoogle Scholar
  • 6 Dyck P J, Oviatt K F, Lambert E H. Intensive evaluation of referred unclassified neuropathies yields improved diagnosis.  Ann Neurol. 1981;  10 222-226
    CrossrefPubMedGoogle Scholar
  • 7 Bromberg M B, Feldman E L, Albers J W. Chronic inflammatory demyelinating polyradiculoneuropathy: comparison of patients with and without an associated monoclonal gammopathy.  Neurology. 1992;  42 1157-1163
    PubMedGoogle Scholar
  • 8 Eurelings M, Notermans N C, Donk N WCJ van de, Lokhorst H M. Risk factors for hematological malignancy in polyneuropathy associated with monoclonal gammopathy.  Muscle Nerve. 2001;  24 1295-1302
    PubMedGoogle Scholar
  • 9 Gorson K C, 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
    CrossrefPubMedGoogle Scholar
  • 10 Latov N. Prognosis of neuropathy with monoclonal gammopathy.  Muscle Nerve. 2000;  23 150-152
    PubMedGoogle Scholar
  • 11 Simmons Z, Albers J W, Bromberg M B, Feldman E L. Long-term follow-up of patients with chronic inflammatory demyelinating polyradiculoneuropathy, without and with monoclonal gammopathy.  Brain. 1995;  118 359-368
    CrossrefPubMedGoogle Scholar
  • 12 Katz J S, Saperstein D S, Gronseth G, Amato A A, Barohn R J. Distal acquired demyelinating symmetric neuropathy.  Neurology. 2000;  54 615-620
    CrossrefPubMedGoogle Scholar
  • 13 Berg-Vos R M van den, Berg L H van den, Franssen H, Vermeulen M, Witkamp T D, Jansen G H van, Es H W van, Kerkhoff H, Wokke J HJ. Multifocal inflammatory demyelinating neuropathy: A distinct clinical entity?.  Neurology. 2000;  54 26-32
    CrossrefPubMedGoogle Scholar
  • 14 Saperstein D S, Katz J S, Amato A A, Barohn R J. Clinical spectrum of chronic acquired demyelinating polyneuropathies.  Muscle Nerve. 2001;  24 311-324
    CrossrefPubMedGoogle Scholar
  • 15 Lewis R A, Sumner A J, Brown M J, Asbury A K. Multifocal demyelinating neuropathy with persistent conduction block.  Neurology. 1982;  32 958-964
    CrossrefPubMedGoogle Scholar
  • 16 Thomas P K, Claus D, Jaspert A, Workman J M, King R H, Larner A J, Anderson M, Emerson J A, Ferguson I T. Focal upper limb demyelinating neuropathy.  Brain. 1996;  119 765-774
    PubMedGoogle Scholar
  • 17 Krarup C. Nerve conduction studies in selected peripheral nerve disorders.  Curr Opin Neurol. 2002;  15 579-593
    CrossrefPubMedGoogle Scholar
  • 18 Nicolas G, Maisonobe T, Forestier N Le, Léger J-M, Bouche P. Proposed revised electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy.  Muscle Nerve. 2002;  25 26-30
    CrossrefPubMedGoogle Scholar
  • 19 Bromberg M B. Comparison of electrodiagnostic criteria for primary demyelination in chronic polyneuropathy.  Muscle Nerve. 1991;  14 968-976
    CrossrefPubMedGoogle Scholar
  • 20 Barohn R, Kissel J, Warmolts J. Chronic inflammatory polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria.  Arch Neurol. 1989;  46 878-884
    CrossrefPubMedGoogle Scholar
  • 21 Olney R K. Consensus criteria for the diagnosis of partial conduction block.  Muscle Nerve. 1999;  22, Suppl 8 S225-S229
    PubMedGoogle Scholar
  • 22 Latov N. Diagnosis of CIDP.  Neurology. 2002;  59, suppl 6 S2-S6
    PubMedGoogle Scholar
  • 23 Capasso M, Torrieri F, Muzio A Di, Angelis M V De, Lugaresi A, Uncini A. Can electrophysiology differentiate polyneuropathy with anti-MAG/SGPG antibodies from chronic inflammatory demyelinating polyneuropathy?.  Clin Neurophysiol. 2002;  113 346-353
    CrossrefPubMedGoogle Scholar
  • 24 Cocito D, Isoardo G, Ciaramitaro P, Migliaretti G, Pipieri A, Barbero P, Cucci A, Durelli L. Terminal latency index in polyneuropathy with IgM paraproteinemia and anti-MAG antibody.  Muscle Nerve. 2001;  24 1278-1282
    PubMedGoogle Scholar
  • 25 Gorson K C, Weinberg D H, Weinstein R. Treatment Experience in Patients with Anti-Myelin-Associated Glycoprotein Neuropathy.  Muscle Nerve. 2001;  24 778-786
    PubMedGoogle Scholar
  • 26 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 paragloboside.  Brain. 1994;  117 941-947
    PubMedGoogle Scholar
  • 27 Attarian S, Azulay J P, Boucraut J. Terminal latency index and modified F ratio in distinction of chronic demyelinating neuropathies.  Clin Neurophysiol. 2001;  112 457-463
    CrossrefPubMedGoogle Scholar
  • 28 Ashworth N L, Zochodne D W, Hahn A F, Pillay N, Chalk C, Benstead T, Bril V, Feasby T E, Bolton C F. Impact of plasma exchange on indices of demyelination in chronic inflammatory demyelinating polyradiculoneuropathy.  Muscle Nerve. 2000;  23 206-210
    PubMedGoogle Scholar
  • 29 Pollard J D. Chronic inflammatory demyelinating polyradiculoneuropathy.  Curr Opin Neurol. 2002;  15 279-283
    CrossrefPubMedGoogle Scholar
  • 30 Doorn P A van, Garssen M PJ. Treatment of immune neuropathies.  Curr Opin Neurol. 2002;  15 623-631
    CrossrefPubMedGoogle Scholar
  • 31 Koski C L. Therapy of CIDP and related immune-mediated neuropathies.  Neurology. 2002;  59, Suppl 6 S22-S27
    PubMedGoogle Scholar
  • 32 Hughes R, Bensa S, Willison H, Bergh P van den, Comi G, Illa I, Nobile-Orazio E, Doorn P van, Dalakas M, Bojar M, Swan A. Inflammatory Neuropathy Cause and Treatment (INCAT) Group. Randomized Controlled Trial of Intravenous Immunoglobulin Versus Oral Prednisolone in Chronic Inflammatory Demyelinating Polyradiculoneuropathy.  Ann Neurol. 2001;  50 195-201
    CrossrefPubMedGoogle Scholar
  • 33 Hahn A F, Bolton C F, Pillay N, Chalk C, Benstead T, Bril V, Shumak K, Vandervoort M K, Feasby T E. Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy. A double-blind, sham-controlled, cross-over study.  Brain. 1996;  119 1055-1066
    CrossrefPubMedGoogle Scholar
  • 34 Hahn A F, Bolton C F, Zochodne D, Feasby T E. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy. A double-blind, placebo-controlled, cross-over study.  Brain. 1996;  119 1067-1077
    CrossrefPubMedGoogle Scholar
  • 35 Dyck P, O'Brien P, Oviatt K. Combined azathioprine and prednisone in chronic inflammatory demyelinating polyneuropathy.  Neurology. 1985;  35 1173-1176
    CrossrefPubMedGoogle Scholar
  • 36 Doorn P A van, Rossi F, Brand A, Lint M van, Vermeulen M, Kazatchkine M D. On the mechanism of high-dose intravenous immunoglobulin treatment of patients with chronic inflammatory demyelinating polyneuropathy.  J Neuroimmunol. 1990;  29 57-64
    CrossrefPubMedGoogle Scholar
  • 37 Doorn P A van, Brand A, Strengers P F, Meulstee J, Vermeulen M. High-dose intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: a double-blind, placebo-controlled, crossover study (see comments).  Neurology. 1990;  40 209-212
    PubMedGoogle Scholar
  • 38 Doorn P A van, Vermeulen M, Brand A, Mulder P G, Busch H F. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy. Clinical and laboratory characteristics associated with improvement.  Arch Neurol. 1991;  48 217-220
    PubMedGoogle Scholar
  • 39 Doorn P A van. Treatment of patients with chronic inflammatory demyelinating polyneuropathy.  Rev Neurol (Paris). 1996;  152 383-386
    PubMedGoogle Scholar
  • 40 Grehl H, Jaspert A, Claus D, Neundörfer B. Long-term therapy with high-dose intravenous immunoglobulins (IVIG) in inflammatory neuropathies.  European Journal of Neurology. 1997;  4 266-273
    PubMedGoogle Scholar
  • 41 Grehl H, Jaspert A, Claus D, Neundörfer B. Immunglobulintherapie chronisch-entzündlicher Neuropathien.  Nervenarzt. 1996;  67 1003-1010
    CrossrefPubMedGoogle Scholar
  • 42 Dyck P J, Daube J, O'Brien P. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy.  N Engl J Med. 1986;  314 461-465
    CrossrefPubMedGoogle Scholar
  • 43 Dyck P J, Litchy W J, Kratz K M, Suarez G A, Low P A, Pineda A A, Windebank A J, Karnes J L, O'Brien P C. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy.  Ann Neurol. 1994;  36 838-845
    CrossrefPubMedGoogle Scholar
  • 44 Good J L, Chehrenama M, Mayer R F, Koski C L. Pulse cyclophosphamide therapy in chronic inflammatory demyelinating polyneuropathy.  Neurology. 1998;  51 1735-1738
    CrossrefPubMedGoogle Scholar
  • 45 Brannagan T H, Pradhan A, Heiman-Patterson T, Winkelman A C, Styler M J, Topolsky D L, Crilley P A, Schwartzman R J, Brodsky I, Gladstone D E. High-dose cyclophosphamide without stem cell rescue for refractory CIDP.  Neurology. 2002;  58 1856-1858
    CrossrefPubMedGoogle Scholar
  • 46 Barnett M H, Pollard J D, Davies L, McLeod J G. Cyclosporin A in resistant chronic inflammatory demyelinating polyradiculoneuropathy.  Muscle Nerve. 1998;  21 454-460
    CrossrefPubMedGoogle Scholar
  • 47 Mahattanakul W, Crawford T O, Griffin J W, Goldstein J M, Cornblath D R. Treatment of chronic inflammatory demyelinating polyneuropathy with cyclosporin-A.  J Neurol Neurosurg Psychiatry. 1996;  60 185-187
    CrossrefPubMedGoogle Scholar
  • 48 Chaudhry V, Cornblath D R, Griffin J W, O'Brien R, Drachman D B. Mycophenolate mofetil: A safe and promising immunosuppressant in neuromuscular diseases.  Neurology. 2001;  56 94-96
    CrossrefPubMedGoogle Scholar
  • 49 Mowzoon N SA, Bradley W G. Mycophenolate (CellCept) treatment of myasthenia gravis, chronic inflammatory polyneuropathy and inclusion body myositis.  J Neurol Sci. 2001;  185 119-122
    CrossrefPubMedGoogle Scholar
  • 50 Gorson K C, Allam G, Simovic D, Ropper A H. Improvement following interferon-alpha 2A in chronic inflammatory demyelinating polyneuropathy.  Neurology. 1997;  48 777-780
    CrossrefPubMedGoogle Scholar
  • 51 Gorson K C, Ropper A H, Clark B D, Dew R B, Simovic D, Allam G. Treatment of chronic inflammatory demyelinating polyneuropathy with interferon-alpha 2A.  Neurology. 1998;  50 84-87
    CrossrefPubMedGoogle Scholar
  • 52 Lisak R P. Type I Interferons and Chronic Inflammatory Demyelinating Polyneuropathy: Treatment or Cause?.  Muscle Nerve. 2000;  23 307-309
    PubMedGoogle Scholar
  • 53 Mariette X, Chastang C, Clavelou P, Louboutin J-P, Léger J-M, Brouet J-C. A randomised clinical trial comparing interferon-alpha and intravenous immunoglobulin in polyneuropathy associated with monoclonal IgM.  J Neurol Neurosurg Psychiatry. 1997;  63 28-34
    CrossrefPubMedGoogle Scholar
  • 54 Meriggioli M N, Rowin J. Chronic Inflammatory Demyelinating Polyneuropathy after Treatment with Interferon-alpha.  Muscle Nerve. 2000;  23 433-435
    CrossrefPubMedGoogle Scholar
  • 55 Pavesi G, Cattaneo L, Marbini A, Gemignani F, Mancia D. Long-term efficacy of Interferon-alpha in chronic inflammatory demyelinating polyneuropathy.  J Neurol. 2002;  249 777-779
    CrossrefPubMedGoogle Scholar
  • 56 Medical Research Council .Aids to the investigation of peripheral nervous system. Memorandum no. 45. London; Her Majesty's Stationery Office 1976
    Google Scholar
  • 57 Merkies I SJ, Schmitz P I, Meché F GA van der, Doorn P A van. Psychometric evaluation of a new sensory scale in immune-mediated polyneuropathies.  Neurology. 2000;  54 943-949
    PubMedGoogle Scholar
  • 58 Merkies I SJ, Schmitz P I, Meché F GA van der, Samijn J PA, Doorn P A van. Psychometric evaluation of a new handicap scale in immune-mediated polyneuropathies.  Muscle Nerve. 2002;  25 370-377
    PubMedGoogle Scholar

PD Dr. Holger Grehl

Neurologische Klinik Evangelisches und Johanniter-Klinikum

Fahrner Straße 133

47169 Duisburg

Email: holger.grehl@ejk.de

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