Classic chronic inflammatory demyelinating polyneuropathy (CIDP) is characterized by the occurrence of symmetrical weakness in both proximal and distal muscles that progressively increases for more than two months (setting this condition apart from the Guillain–Barré syndrome, which is self-limited). The condition is associated with impaired sensation, absent or diminished tendon reflexes, an elevated cerebrospinal fluid protein level, demyelinating nerve-conduction studies, and signs of demyelination in nerve-biopsy specimens. The course can be relapsing or chronic and progressive, the former being much more common in young adults.
Diagnostic Criteria for Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
There is general agreement that the following criteria support the diagnosis of the classic form of CIDP:
- Progression over at least two months
- Weakness more than sensory symptoms
- Symmetric involvement of arms and legs
- Proximal muscles involved along with distal muscles
- Reduced deep tendon reflexes throughout
- Increased cerebrospinal fluid protein without pleocytosis
- Nerve conduction evidence of a demyelinating neuropathy
- Nerve biopsy evidence of segmental demyelination with or without inflammation
For the diagnosis of idiopathic CIDP, the Koski criteria require the following:
- Chronic polyneuropathy, progressive for at least eight weeks
- No serum paraprotein and no genetic abnormality and
- Recordable compound muscle action potentials in at least 75 percent of motor nerves and either abnormal distal latency or abnormal motor conduction velocity or abnormal F wave latency in >50 percent of motor nerves
- Symmetric onset or symmetric exam and weakness in all four limbs and proximal weakness in at least one limb.
Electrodiagnostic criteria for CIDP
These criteria are applied by testing the median, ulnar (stimulated below the elbow), peroneal (stimulated below the fibular head), and tibial nerves on one side of the body. During testing, limb temperature should be no less than 33°C at the palm and no less than 30°C at the external malleolus.
At least one of the following demyelinating parameters are necessary:
- >/=50 percent prolongation of motor distal latency above the ULN in two nerves
- >/=30 percent reduction of motor conduction velocity below the LLN in two nerves
- >/=20 percent prolongation of F-wave latency above the ULN in two nerves, or >50 percent if the amplitude of the distal negative peak CMAP is <80 percent of the LLN
- Absence of F-waves in two nerves, if these nerves have amplitudes of distal negative peak CMAPs >/=20 percent of the LLN, plus at least one other demyelinating parameter (meeting any of the definite criteria) in at least one other nerve
- Partial motor conduction block, defined by a >/=50 percent amplitude reduction of the proximal negative peak CMAP relative to distal, if distal negative peak CMAP is >/=20 percent of the LLN, in two nerves, or in one nerve plus at least one other demyelinating parameter (meeting any of the definite criteria) in at least one other nerve
- Abnormal temporal dispersion, defined by a >30 percent duration increase between the proximal and distal negative peak CMAP in at least two nerves
- Distal CMAP duration (interval between onset of the first negative peak and return to baseline of the last negative peak) increase in at least one nerve (median >/=6.6 ms, ulnar >/=6.7 ms, peroneal >/=7.6 ms, tibial >/=8.8 ms) plus at least one other demyelinating parameter (meeting any of the definite criteria) in at least one other nerve
>/=30 percent amplitude reduction of the proximal negative peak CMAP relative to the distal, excluding the posterior tibial nerve, if the distal negative peak CMAP is >/=20 percent of LLN, in two nerves, or in one nerve plus at least one other demyelinating parameter (meeting any of the definite criteria) in at least one other nerve
As in “Definite CIDP” but in only one nerve
CMAP: compound muscle action potential; ULN: upper limit of normal; LLN: lower limit of normal.
- Köller H, Kieseier BC, Jander S, Hartung HP. Chronic inflammatory demyelinating polyneuropathy. N Engl J Med. 2005 Mar 31;352(13):1343-56. [Medline]
- Gorson KC, Ropper AH. Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP): A Review of Clinical Syndromes and Treatment Approaches in Clinical Practice. J Clin Neuromuscul Dis. 2003 Jun;4(4):174-89. [Medline]
- Koski CL, Baumgarten M, Magder LS, Barohn RJ, Goldstein J, Graves M, Gorson K, Hahn AF, Hughes RA, Katz J, Lewis RA, Parry GJ, van Doorn P, Cornblath DR. Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2009 Feb 15;277(1-2):1-8 [Medline]
- Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society–First Revision. J Peripher Nerv Syst. 2010 Mar;15(1):1-9. [Medline]
Created Sep 22, 2011.