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
- 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
- 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
- 4
Mygland A, Monstadt P.
Chronic polyneuropathies in Vest-Agder, Norway.
Eur J Neurol.
2001;
8
157-165
- 5
Barohn R J.
Approach to peripheral neuropathy and neuronopathy.
Semin Neurol.
1998;
18
7-18
- 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
- 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
- 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
- 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
- 10
Latov N.
Prognosis of neuropathy with monoclonal gammopathy.
Muscle Nerve.
2000;
23
150-152
- 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
- 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
- 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
- 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
- 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
- 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
- 17
Krarup C.
Nerve conduction studies in selected peripheral nerve disorders.
Curr Opin Neurol.
2002;
15
579-593
- 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
- 19
Bromberg M B.
Comparison of electrodiagnostic criteria for primary demyelination in chronic polyneuropathy.
Muscle Nerve.
1991;
14
968-976
- 20
Barohn R, Kissel J, Warmolts J.
Chronic inflammatory polyradiculoneuropathy. Clinical characteristics, course, and
recommendations for diagnostic criteria.
Arch Neurol.
1989;
46
878-884
- 21
Olney R K.
Consensus criteria for the diagnosis of partial conduction block.
Muscle Nerve.
1999;
22, Suppl 8
S225-S229
- 22
Latov N.
Diagnosis of CIDP.
Neurology.
2002;
59, suppl 6
S2-S6
- 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
- 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
- 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
- 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
- 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
- 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
- 29
Pollard J D.
Chronic inflammatory demyelinating polyradiculoneuropathy.
Curr Opin Neurol.
2002;
15
279-283
- 30
Doorn P A van, Garssen M PJ.
Treatment of immune neuropathies.
Curr Opin Neurol.
2002;
15
623-631
- 31
Koski C L.
Therapy of CIDP and related immune-mediated neuropathies.
Neurology.
2002;
59, Suppl 6
S22-S27
- 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
- 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
- 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
- 35
Dyck P, O'Brien P, Oviatt K.
Combined azathioprine and prednisone in chronic inflammatory demyelinating polyneuropathy.
Neurology.
1985;
35
1173-1176
- 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
- 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
- 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
- 39
Doorn P A van.
Treatment of patients with chronic inflammatory demyelinating polyneuropathy.
Rev Neurol (Paris).
1996;
152
383-386
- 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
- 41
Grehl H, Jaspert A, Claus D, Neundörfer B.
Immunglobulintherapie chronisch-entzündlicher Neuropathien.
Nervenarzt.
1996;
67
1003-1010
- 42
Dyck P J, Daube J, O'Brien P.
Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy.
N Engl J Med.
1986;
314
461-465
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 52
Lisak R P.
Type I Interferons and Chronic Inflammatory Demyelinating Polyneuropathy: Treatment
or Cause?.
Muscle Nerve.
2000;
23
307-309
- 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
- 54
Meriggioli M N, Rowin J.
Chronic Inflammatory Demyelinating Polyneuropathy after Treatment with Interferon-alpha.
Muscle Nerve.
2000;
23
433-435
- 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
- 56 Medical Research Council .Aids to the investigation of peripheral nervous system.
Memorandum no. 45. London; Her Majesty's Stationery Office 1976
- 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
- 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
PD Dr. Holger Grehl
Neurologische Klinik Evangelisches und Johanniter-Klinikum
Fahrner Straße 133
47169 Duisburg
Email: holger.grehl@ejk.de
