Underdiagnosis and diagnostic delay in chronic inflammatory demyelinating polyneuropathy

European Federation ◽

Delay In Diagnosis ◽

Alternative Diagnosis ◽

Inflammatory Neuropathy ◽

Made In

Abstract Background The frequency and causes of underdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) are uncertain. We aimed to assess the frequency and electroclinical features of pre-referral CIDP underdiagnosis and the duration of delay prior to diagnosis and treatment initiation in a tertiary specialist clinic. Methods We retrospectively investigated 60 consecutive patients attending our Inflammatory Neuropathy Service, between 2015 and 2019, with a final diagnosis of treatment-responsive definite/probable CIDP. We reviewed the clinical and electrophysiological data in light of European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines and determined the frequency, causes and delay in diagnosis of CIDP. Results An initial alternative diagnosis to that of CIDP had been made in 68.3% (41/60) of patients. The commonest alternative diagnosis was of Guillain–Barré syndrome (GBS) in 23.3% (14/60) patients. Non-GBS underdiagnoses (27/60; 45%) mainly consisted of genetic neuropathy (8/27; 29.6%), diabetic neuropathy (5/27; 18.5%) and chronic idiopathic axonal polyneuropathy (4/27; 14.8%). Non-GBS underdiagnoses were predominantly due to non-recognition of proximal weakness (70.4%), multifocal deficits (18.5%) or proprioceptive loss (7.4%). Electrophysiological misinterpretation was contributory to pre-referral non-GBS underdiagnoses of CIDP in 85% of patients. Mean diagnostic delay in patients with non-GBS underdiagnoses of CIDP was of 21.3 months (range 2–132 months). Conclusion Underdiagnosis of CIDP is frequent and may lead to significant diagnostic and treatment delay. We suggest that lack of comprehensive and precise attention to typical electroclinical features of CIDP and its diagnostic criteria at the time of initial evaluation are equally contributory to underdiagnoses.

Fatigue in patients with chronic inflammatory demyelinating polyneuropathy

Neurology neuropsychiatry Psychosomatics ◽

10.14412/2074-2711-2021-1-51-56 ◽

2021 ◽

Vol 13 (1) ◽

pp. 51-56

Author(s):

R. A. Gapeshin ◽

E. R. Barantsevich ◽

D. I. Rudenko ◽

T. R. Stuchevskaya ◽

E. A. Gavrilova ◽

...

Keyword(s):

Psychiatric Illness ◽

Comparison Group ◽

Chronic Inflammatory Demyelinating Polyneuropathy ◽

European Federation ◽

Study Group ◽

Motor Neuropathy ◽

Somatic Diseases ◽

Made In

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a peripheral neuropathy, predominantly motor neuropathy, with a progressive or relapse-remitting course. Fatigue is a condition characterized by a physical or mental feeling of lack of energy or lack of motivation for action, which is often present in patients with CIDP.Objective: to assess the severity of asthenia in CIDP patients.Patients and methods. Examinations were made in 34 inpatients treated for documented CIDP that met the international European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria. A study group included patients with CIDP, whereas a comparison group consisted of volunteers without psychiatric illness, who were compensated for somatic diseases.Results and discussion. In the patients with CIDP, the level of fatigue was found to be much higher than normal. Approximately half of the CIDP patients had obvious asthenia. However, the level of fatigue did not correlate with the severity of the course of CIDP.Conclusion. The findings suggest that fatigue is important in patients with CIDP that should be taken into account in the treatment of these patients.

Optimizing the Use of Outcome Measures in Chronic Inflammatory Demyelinating Polyneuropathy

US Neurology ◽

10.17925/usn.2017.13.01.26 ◽

2017 ◽

Vol 13 (01) ◽

pp. 26 ◽

Cited By ~ 4

Author(s):

Jeffrey A Allen ◽

Deborah F Gelinas ◽

Richard A Lewis ◽

Richard J Nowak ◽

Gil I Wolfe ◽

...

Keyword(s):

Outcome Measures ◽

Chronic Inflammatory Demyelinating Polyneuropathy ◽

Unmet Need ◽

Daily Practice ◽

Inflammatory Demyelinating Polyneuropathy

Disability Score ◽

Sum Score ◽

Inflammatory Neuropathy ◽

The challenges encountered during the assessment of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) are many. Ideally, CIDP outcome measures capture impairments in disability, strength, and sensory dysfunction, and quality of life (QoL). A number of outcome measures have been validated for this purpose. Disability outcomes include the adjusted inflammatory neuropathy cause and treatment (INCAT) disability score, INCAT overall disability sum score (ODSS), and overall neuropathy limitations scale (ONLS). A more sensitive disability score, the inflammatory Rasch-built overall disability scale (I-RODS), has also been validated for use in clinical trials and may better capture clinically meaningful changes in those with CIDP. Strength and sensory impairment can be assessed in a number of ways, including the INCAT sensory subscore (ISS), Medical Research Council sum score, and Martin vigorimeter or Jamar dynamometer grip strength. However, the feasibility of applying and interpreting these measures during routine daily practice has been questioned. Furthermore, these outcome measures may not reflect other factors that can impair QoL in those affected by CIDP, such as pain and fatigue. A valid, reliable, and responsive composite measure that addresses all aspects of impairment faced by patients with CIDP remains an unmet need in clinical practice.

Chronic inflammatory demyelinating polyneuropathy associated with diabetes: a European multicentre comparative reappraisal

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2020-322971 ◽

2020 ◽

Vol 91 (10) ◽

pp. 1100-1104 ◽

Cited By ~ 1

Author(s):

Yusuf A Rajabally ◽

Stojan Peric ◽

Mina Cobeljic ◽

Saadia Afzal ◽

Ivo Bozovic ◽

...

Keyword(s):

General Population ◽

Chronic Inflammatory Demyelinating Polyneuropathy ◽

Diabetes Prevalence ◽

Management Implications ◽

Population Prevalence ◽

Typical Form ◽

Inflammatory Neuropathy ◽

The Uk

IntroductionThe association between chronic inflammatory demyelinating polyneuropathy (CIDP) and diabetes is uncertain despite important diagnostic and management implications.MethodsWe retrospectively analysed two European cohorts, totaling 257 patients with ‘definite’ or ‘probable’ CIDP, from Serbia and Birmingham, UK.ResultsDiabetes was present at CIDP diagnosis in 25/139 (18%) subjects in the Serbian cohort and in 23/118 (19.5%) in the UK cohort. In both cohorts, diabetes prevalence was higher than local general population prevalence rates (RR: 2.09; 95% CI 1.39 to 2.95 and RR: 2.22; 95% CI 1.46 to 3.17, respectively). Considering typical CIDP only, diabetes prevalence was greater than expected in both cohorts (RR: 2.58; 95% CI 1.60 to 3.82 and RR: 2.68; 95% CI 1.71 to 3.87, respectively). CIDP with diabetes occurred later in life than CIDP without diabetes (58.96 years, SD: 11.09 vs 51.71 years, SD: 16.02; p=0.003) and presented more frequently in the typical form than in patients without diabetes (79.2% vs 61.2%; p=0.02). Baseline Inflammatory Neuropathy Cause and Treatment disability scores were similar in patients with and without diabetes (p=0.90). Proportions of treatment responders were similar in both groups (70% vs 74.9%; p=0.65), as were response amplitudes (p=0.87).DiscussionOur results, both for all CIDP and typical CIDP presentations, support a twofold increased relative risk of diabetes compared with the general population. CIDP with diabetes appears to present older and more frequently in the typical form, as compared with CIDP without diabetes. CIDP with diabetes appears similar to CIDP without diabetes in disability levels at diagnosis and probability, as well as amplitude of treatment response.

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

Intravenous Immunoglobulins (IVIG) in Chronic Inflammatory Demyelinating Polyneuropathy ( CIDP ): time to maximal recovery

10.1017/cjn.2015.159 ◽

2015 ◽

Vol 42 (S1) ◽

pp. S34-S34

Author(s):

S Baker ◽

A Opala

Keyword(s):

Treatment Time ◽

Chronic Inflammatory Demyelinating Polyneuropathy ◽

Intravenous Immunoglobulins ◽

Secondary Outcome ◽

Ivig Treatment ◽

European Federation ◽

Changes Over Time ◽

Over Time

Background: The response of Chronic Inflammatory Demyelinating Polyneuropathy ( CIDP ) to Intravenous Immunoglobulins (IVIG) treatment is well established. However, determination whether patients who do not respond to 2 IVIG treatments or those whose condition stabilizes (ICE Trial) would benefit from additional treatments remains unclear. We aim to identify time period required to reach maximal strength gains from IVIG treatment (plateau). Furthermore, we will assess nerve conduction studies (NCS) changes over time with IVIG treatment. This will help in establishing a time course for treatment of CIDP with IVIG to maximize recovery. Methods: We performed a retrospective chart review of 27 patients with CIDP, with diagnosis confirmed by European Federation of Neurological Societies/Peripheral Nerve Society Guidelines (EFNS/PNS). Each patient’s strength response including: grip strength, knee extension, elbow flexion and dorsiflexion (using JAMAR Dynamometer) and NCS changes over time during IVIG treatment were analyzed. The primary outcome is duration of IVIG treatment, in months, required to reach a plateau in strength. Secondary outcome is NCS change including: Terminal Latencies, Conduction Velocities, Compound Sensory and Motor action potentials in nerves of upper and lower extremities over treatment time (emerging trends). Results: Pending (available by April 2015) Conclusion: Pending (available by April 2015)

Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2019-320314 ◽

2019 ◽

Vol 90 (9) ◽

pp. 981-987 ◽

Cited By ~ 24

Author(s):

Helmar Christoph Lehmann ◽

David Burke ◽

Satoshi Kuwabara

Keyword(s):

Chronic Inflammatory Demyelinating Polyneuropathy ◽

Motor Neuropathy ◽

Immune Mediated ◽

Costly Treatment ◽

Relative Rarity ◽

Treatment Responses ◽

Immunomodulating Drugs ◽

Made In

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy typically characterised by symmetrical involvement, and proximal as well as distal muscle weakness (typical CIDP). However, there are several ‘atypical’ subtypes, such as multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome) and ‘distal acquired demyelinating symmetric neuropathy’, possibly having different immunopathogenesis and treatment responses. In the absence of diagnostic and pathogenetic biomarkers, diagnosis and treatment may be difficult, but recent progress has been made in the application of neuroimaging tools demonstrating nerve hypertrophy and in identifying subgroups of patients who harbour antibodies against nodal proteins such as neurofascin and contactin-1. Despite its relative rarity, CIDP represents a significant economic burden, mostly due to costly treatment with immunoglobulin. Recent studies have demonstrated the efficacy of subcutaneous as well as intravenous immunoglobulin as maintenance therapy, and newer immunomodulating drugs can be used in refractory cases. This review provides an overview focusing on advances over the past several years.

Interleukin 8, a Biomarker to Differentiate Guillain-Barré Syndrome From CIDP

Neurology Neuroimmunology & Neuroinflammation ◽

10.1212/nxi.0000000000001031 ◽

2021 ◽

Vol 8 (5) ◽

pp. e1031

Author(s):

Gautier Breville ◽

Agustina M. Lascano ◽

Pascale Roux-Lombard ◽

Nicolas Vuilleumier ◽

Patrice H. Lalive

Keyword(s):

Predictive Value ◽

Chronic Inflammatory Demyelinating Polyneuropathy ◽

Acute Onset ◽

Interleukin 8 ◽

Guillain Barre Syndrome ◽

European Federation ◽

Guillain Barré Syndrome ◽

Guillain Barré

ObjectiveTo determine whether CSF interleukin 8 (IL-8) concentration can help to distinguish Guillain-Barré syndrome (GBS) from chronic inflammatory demyelinating polyneuropathy (CIDP) at the initial stage of the disease.MethodsWe performed retrospective immunoassay of IL-8 in CSF, collected at the University Hospitals of Geneva between 2010 and 2018, from patients diagnosed with GBS (n = 45) and with CIDP (n = 30) according to the Brighton and European Federation of Neurological Societies/Peripheral Nerve Society criteria by a physician blinded to biological results.ResultsCSF IL-8 was higher in GBS (median: 83.9 pg/mL) than in CIDP (41.0 pg/mL) (p < 0.001). Receiver operating characteristic analyses indicated that the optimal IL-8 cutoff was 70 pg/mL. Above this value, patients were more likely to present GBS than CIDP (specificity 96.7%, sensitivity 64.4%, positive predictive value [PPV] 96.7%, and negative predictive value [NPV] 64.4%). Among GBS subcategories, IL-8 was higher in acute inflammatory demyelinating polyneuropathy (AIDP, median: 101.8 pg/mL) than in other GBS variants (median: 53.7 pg/mL). In addition, with CSF IL-8 above 70 pg/mL, patients were more likely to present AIDP than acute-onset CIDP (p < 0.001; specificity 100%, sensitivity 78.8%, PPV 100%, and NPV 46.2%) or other CIDP with nonacute presentation (p < 0.0001; specificity 95.8%, sensitivity 78.8%, PPV 96.3%, and NPV 76.7%).ConclusionCSF IL-8 levels can help to differentiate AIDP variant of GBS from CIDP, including acute-onset CIDP, with high specificity and PPV. This may improve early and appropriate treatment.Classification of EvidenceThis study provides Class II evidence that CSF IL-8 levels accurately distinguish patients with GBS from those with CIDP.

Inflammatory Neuropathy Cause and Treatment (INCAT) Scale for the assessment of disability level in patients with chronic inflammatory demyelinating polyneuropathy: linguocultural ratification in Russia

Neuromuscular Diseases ◽

10.17650/2222-8721-2021-11-4-26-33 ◽

2021 ◽

Vol 11 (4) ◽

pp. 26-33

Author(s):

А. S. Arestova ◽

Е. А. Melnik ◽

A. B. Zaytsev ◽

A. A. Zimin ◽

А. Yakubu ◽

...

Keyword(s):

Disease Progression ◽

Scale Validation ◽

Chronic Inflammatory Demyelinating Polyneuropathy ◽

Objective Response ◽

Standard Protocol ◽

Russian Version ◽

Inflammatory Neuropathy ◽

Disability Level

Background. Chronic inflammatory demyelinating polyneuropathy (CIDP) is a treatable dysimmune polyneuropathy. An objective response for pathogenic therapy is essential in diagnosis and management of CIDP. For proper assessment of patient’s complaints and evaluation of disease progression, it is recommended to use validated scales and questionnaires. The paper presents the results of the first step of Inflammatory Neuropathy Cause and Treatment (INCAT) validation in patients with CIDP.Objective: the development of the Russian version of the INCAT scale and its linguocultural ratification.Materials and methods. 15 patients with definite CIDP (according to EFNS/PNS criteria) were enrolled. Linguocultural ratification was conducted according to the standard protocol.Results. The Russian version of the INCAT scale was developed.Conclusion. We conducted the first stage of INCAT scale validation in patients with CIDP.