The levels of evidence (class I-IV) supporting the recommendations and ratings of recommendations (A-C, Good Practice Point) are defined at the end of the "Major Recommendations" field.
Diagnostic Criteria for Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)
Good Practice Points for defining diagnostic criteria for CIDP:
- Clinical: typical and atypical CIDP (see Table 1 below)
- Electrodiagnostic: definite, probable and possible CIDP (see Table 2 below)
- Supportive: including cerebrospinal fluid (CSF), magnetic resonance imaging (MRI), nerve biopsy and treatment response (see Table 3 below)
- CIDP in association with concomitant diseases (see Table 4 in the original guideline document)
- Categories: definite, probable, and possible CIDP with or without concomitant diseases (see Table 5 below)
Good Practice Points for diagnostic tests:
- Electrodiagnostic tests are recommended in all patients (Good Practice Point)
- CSF, MRI and nerve biopsy should be considered in selected patients (Good Practice Point)
- Concomitant diseases should be considered in all patients but the choice of tests will depend on the clinical circumstances (see Table 6 in the original guideline document).
Table 1. Clinical Diagnostic Criteria
- Inclusion criteria
- Typical CIDP
Chronically progressive, stepwise, or recurrent symmetric proximal and distal weakness and sensory dysfunction of all extremities, developing over at least 2 months; cranial nerves may be affected, and
Absent or reduced tendon reflexes in all extremities
- Atypical CIDP
One of the following, but otherwise as in A (tendon reflexes may be normal in unaffected limbs)
Predominantly distal weakness (distal acquired demyelinating sensory [DADS])
Pure motor or sensory presentations, including chronic sensory immune polyradiculoneuropathy affecting the central process of the primary sensory neuron
Asymmetric presentations (multifocal acquired demyelinating sensory and motor [MADSAM] Lewis–Sumner syndrome Focal presentations (e.g. involvement of the brachial plexus or of one or more peripheral nerves in one upper limb)
Central nervous system involvement (may occur with otherwise typical or other forms of atypical CIDP)
- Exclusion criteria
Diphtheria, drug or toxin exposure likely to have caused the neuropathy
Hereditary demyelinating neuropathy, known or likely because of family history, foot deformity, mutilation of hands or feet, retinitis pigmentosa, ichthyosis, liability to pressure palsy
Presence of sphincter disturbance
Multifocal motor neuropathy
Antibodies to myelin-associated glycoprotein
|
Table 2. Electrodiagnostic Criteria
- Definite: at least one of the following
- At least 50% prolongation of motor distal latency above the upper limit of normal values in two nerves, or
- At least 30% reduction of motor conduction velocity below the lower limit of normal values in two nerves, or
- At least 20% prolongation of F-wave latency above the upper limit of normal values in two nerves (>50% if amplitude of distal negative peak compound muscle action potential [CMAP] <80% of lower limit of normal values), or
- Absence of F-waves in two nerves if these nerves have amplitudes of distal negative peak CMAPs at least 20% of lower limit of normal values + at least one other demyelinating parametera in at least one other nerve, or
- Partial motor conduction block: at least 50% amplitude reduction of the proximal negative peak CMAP relative to distal, if distal negative peak CMAP at least 20% of lower limit of normal values, in two nerves, or in one nerve + at least one other demyelinating parametera in at least one other nerve, or
- Abnormal temporal dispersion (>30% duration increase between the proximal and distal negative peak CMAP) in at least two nerves, or
- Distal CMAP duration (interval between onset of the first negative peak and return to baseline of the last negative peak) of at least 9 ms in at least one nerve + at least one other demyelinating parametera in at least one other nerve
- Probable
At least 30% amplitude reduction of the proximal negative peak CMAP relative to distal, excluding the posterior tibial nerve, if distal negative peak CMAP at least 20% of lower
limit of normal values, in two nerves, or in one nerve + at least one other demyelinating parametera in at least one other nerve
- Possible
As in 'I' but in only one nerve
|
To apply these criteria the median, ulnar (stimulated below the elbow), peroneal (stimulated below the fibular head), and tibial nerves on one side are tested. Temperatures should be maintained to at least 33 degrees C at the palm and 30 degrees C at the external malleolus (Good Practice Points). Further technical details are given in the accompanying web document (http://www.efns.org) and see van den Bergh and Pieret, Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy. Muscle and Nerve 2004; 29: 565-574.
aAny nerve meeting any of the criteria A–G.
Table 3. Supportive Criteria
- Elevated cerebrospinal fluid protein with leucocyte count <10/mm3 (level A recommendation)
- Magnetic Resonance Imaging showing gadolinium enhancement and/or hypertrophy of the cauda equina, lumbosacral or cervical nerve roots, or the brachial or lumbosacral plexuses (level C recommendation)
- Nerve biopsy showing unequivocal evidence of demyelination and/or remyelination in >5 fibres by electron microscopy or in >6 of 50 teased fibres
- Clinical improvement following immunomodulatory treatment (level A recommendation)
|
Table 5. Diagnostic Categories
|
Definite CIDP
Clinical criteria I A or B and II with Electrodiagnostic criteria I; or Probable CIDP + at least one Supportive criterion; or Possible CIDP + at least two Supportive criteria
Probable CIDP
Clinical criteria I A or B and II with Electrodiagnostic criteria II; or Possible CIDP + at least one Supportive criterion
Possible CIDP
Clinical criteria I A or B and II with Electrodiagnostic criteria III CIDP (definite, probable, possible) associated with concomitant diseases
|
Treatment of CIDP
For induction of treatment:
- Intravenous immunoglobulin (IVIg) or corticosteroids should be considered in sensory and motor CIDP in the presence of troublesome symptoms (level B recommendation). The presence of relative contraindications to either treatment should influence the choice (Good Practice Point).
- The advantages and disadvantages should be explained to the patient who should be involved in the decision making (Good Practice Point).
- In pure motor CIDP IVIg should be considered as the initial treatment (Good Practice Point).
- If IVIg and corticosteroids are ineffective plasma exchange (PE) should be considered (level A recommendation).
For maintenance treatment:
- If the first-line treatment is effective continuation should be considered until the maximum benefit has been achieved and then the dose reduced to find the lowest effective maintenance dose (Good Practice Point).
- If the response is inadequate or the maintenance doses of the initial treatment are high, combination treatments or adding an immunosuppressant or immunomodulatory drug may be considered (see Table 7 in the original guideline document) (Good Practice Point).
- Advice about foot care, exercise, diet, driving, and lifestyle management should be considered. Neuropathic pain should be treated with drugs according to EFNS guideline on treatment of neuropathic pain. Depending on the needs of the patient, orthoses, physiotherapy, occupational therapy, psychological support and referral to a rehabilitation specialist should be considered (Good Practice Points).
- Information about patient support groups should be offered to those who would like it (Good Practice Point).
Definitions:
Evidence Classification Scheme for a Diagnostic Measure
Class I: A prospective study in a broad spectrum of persons with the suspected condition, using a "gold standard" for case definition, where the test is applied in a blinded evaluation, and enabling the assessment of appropriate tests of diagnostic accuracy
Class II: A prospective study of a narrow spectrum of persons with the suspected condition, or a well-designed retrospective study of a broad spectrum of persons with an established condition (by "gold standard") compared to a broad spectrum of controls, where test is applied in a blinded evaluation, and enabling the assessment of appropriate tests of diagnostic accuracy
Class III: Evidence provided by a retrospective study where either persons with the established condition or controls are of a narrow spectrum, and where test is applied in a blinded evaluation
Class IV: Any design where test is not applied in blinded evaluation OR evidence provided by expert opinion alone or in descriptive case series (without controls)
Evidence Classification Scheme for a Therapeutic Intervention
Class I: An adequately powered prospective, randomized, controlled clinical trial with masked outcome assessment in a representative population or an adequately powered systematic review of prospective randomized controlled clinical trials with masked outcome assessment in representative populations. The following are required:
- Randomization concealment
- Primary outcome(s) is/are clearly defined
- Exclusion/inclusion criteria are clearly defined
- Adequate accounting for dropouts and crossovers with numbers sufficiently low to have minimal potential for bias
- Relevant baseline characteristics are presented and substantially equivalent among treatment groups or there is appropriate statistical adjustment for differences
Class II: Prospective matched-group cohort study in a representative population with masked outcome assessment that meets a–e above or a randomized, controlled trial in a representative population that lacks one criteria a–e
Class III: All other controlled trials (including well-defined natural history controls or patients serving as own controls) in a representative population, where outcome assessment is independent of patient treatment
Class IV: Evidence from uncontrolled studies, case series, case reports, or expert opinion
Rating of Recommendations for a Diagnostic Measure
Level A rating (established as useful/predictive or not useful/predictive) requires at least one convincing class I study or at least two consistent, convincing class II studies.
Level B rating (established as probably useful/predictive or not useful/predictive) requires at least one convincing class II study or overwhelming class III evidence.
Level C rating (established as possibly useful/predictive or not useful/predictive) requires at least two convincing class III studies.
Rating of Recommendations for a Therapeutic Intervention
Level A rating (established as effective, ineffective, or harmful) requires at least one convincing class I study or at least two consistent, convincing class II studies.
Level B rating (probably effective, ineffective, or harmful) requires at least one convincing class II study or overwhelming class III evidence.
Level C rating (possibly effective, ineffective, or harmful) requires at least two convincing class III studies.
Good Practice Point When only class IV evidence was available but consensus could be reached the Task Force offered advice as good practice points.
