Chronic inflammatory demyelinating polyradiculoneuropathy—Diagnostic pitfalls and treatment approach

Stino, Amro; Naddaf, Elie; Dyck, Peter J.

doi:10.1002/mus.27046

Cited by 40 publications

(37 citation statements)

References 94 publications

(114 reference statements)

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“…These are the same pathological findings typically seen in classical CIDP. 1,2 Response to immunotherapy in CISP-plus and CISP patients was also similar with marked improvement of neurological deficits, especially the sensory ataxia.…”

Section: Discussionmentioning

confidence: 80%

“…Although chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) usually presents with weakness, 1 , 2 sensory-predominant forms have been described, 3 , 4 including the recently recognized paranodal neuropathies (contactin-1–associated CIDP). 5 , 6 In 2004, our group described a restricted form of sensory CIDP, chronic immune sensory polyradiculopathy (CISP), characterized by selective involvement of sensory nerve roots.…”

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confidence: 99%

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Expanding the Spectrum of Chronic Immune Sensory Polyradiculopathy

et al. 2021

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Objectives:Sensory loss with normal nerve conduction studies (NCS) from focal sensory root inflammatory demyelination is characteristic of chronic immune sensory polyradiculopathy (CISP). However, non-pure cases involving motor and distal sensory nerves exist (CISP-plus). We hypothesize that CISP-plus and CISP are fundamentally part of the same syndrome through comparison of clinical, neurophysiological and pathological features.Methods:CISP-plus (primary dorsal root with lesser motor and sensory nerve involvement) and CISP cases were retrospectively analyzed (1986-2019).Results:We identified 44 CISP-plus and 28 CISP cases (n=72) with 86% (38/44) of CISP-plus and 79% (22/28) of CISP patients experiencing imbalance. On examination, large fiber sensory loss was present in 98% (43/44) of CISP-plus and 96% (27/28) of CISP. Gait ataxia was evident in 93% (41/44) of CISP-plus and 79% (22/28) of CISP. Mild distal weakness was common in CISP-plus (75%, 33/44). NCS showed mild abnormalities in all CISP-plus and were normal (by definition) in all CISP. Elevated cerebral spinal fluid (CSF) protein, slowing of somatosensory evoked potentials and MRI root enhancement occurred in most CISP-plus and CISP cases. Eleven CISP-plus nerve biopsies showed loss of large myelinated fibers and onion-bulb formation most prominent in rootlet biopsies. Immunotherapy resulted in marked improvement of gait ataxia in 84% (27/32) of CISP-plus and 93% (14/15) of CISP cases with return to normal neurological examination in half (25/46).Conclusion:The recognition of CISP-plus expands the spectrum of CIDP by combining CISP-plus (predominant sensory polyradiculopathy with mild motor and sensory nerve involvement) with pure-CISP (focal sensory polyradiculopathy) together as proximal sensory CIDP.

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Section: Discussionmentioning

confidence: 80%

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confidence: 99%

Expanding the Spectrum of Chronic Immune Sensory Polyradiculopathy

et al. 2021

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“…acetylcholine receptor antibodies in MG and MRI in multiple sclerosis) a specific and sensitive biomarker has been difficult to identify for these conditions, and even more so with (neuro) inflammation not solely occurring in ALS. As a result, therapeutic trials are based primarily on clinical scales and require longer observation to assess efficacy, thereby increasing cost and slowing drug development [22][23][24][25]. The initial hurdle for a diagnostic biomarker is to distinguish between ALS and controls (as the studies in Table 1 were designed to detect), but in practical use the biomarker(s) need to distinguish between ALS and other ALS-mimicking diseases, e.g.…”

Section: Diagnostic Biomarkersmentioning

confidence: 99%

Blood-based biomarkers of inflammation in amyotrophic lateral sclerosis

Staats¹,

Borchelt

Tansey

et al. 2022

Mol Neurodegeneration

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Amyotrophic Lateral Sclerosis (ALS) is a devastating neurodegenerative disease in which many processes are detected including (neuro)inflammation. Many drugs have been tested for ALS in clinical trials but most have failed to reach their primary endpoints. The development and inclusion of different types of biomarkers in diagnosis and clinical trials can assist in determining target engagement of a drug, in distinguishing between ALS and other diseases, and in predicting disease progression rate, drug responsiveness, or an adverse event. Ideally, among other characteristics, a biomarker in ALS correlates highly with a disease process in the central nervous system or with disease progression and is conveniently obtained in a peripheral tissue. Here, we describe the state of biomarkers of inflammation in ALS by focusing on peripherally detectable and cellular responses from blood cells, and provide new (combinatorial) directions for exploration that are now feasible due to technological advancements.

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“…The diagnosis of CIDP is based on the European Federation of Neurological Societies/Peripheral Nerve Society in 2010 (EFNS/PNS) 2010 diagnostic criteria for CIDP [ 1 ]. However, the diagnosis of CIDP can be challenging [ 4 , 5 ]. Intravenous immunoglobulins (IVIg), corticosteroids, and plasma exchange (PE) are considered effective first-line treatments for CIDP [ 6 – 9 ].…”

Section: Introductionmentioning

confidence: 99%

Clinical outcome of CIDP one year after start of treatment: a prospective cohort study

et al. 2021

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Objective To assess clinical outcome in treatment-naive patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods We included adult treatment-naive patients participating in the prospective International CIDP Outcome Study (ICOS) that fulfilled the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria for CIDP. Patients were grouped based on initial treatment with (1) intravenous immunoglobulin (IVIg), (2) corticosteroid monotherapy or (3) IVIg and corticosteroids (combination treatment). Outcome measures included the inflammatory Rasch-built overall disability scale (I-RODS), grip strength, and Medical Research Council (MRC) sum score. Treatment response, treatment status, remissions (improved and untreated), treatment changes, and residual symptoms or deficits were assessed at 1 year. Results Forty patients were included of whom 18 (45%) initially received IVIg, 6 (15%) corticosteroids, and 16 (40%) combination treatment. Improvement on ≥ 1 of the outcome measures was seen in 31 (78%) patients. At 1 year, 19 (48%) patients were still treated and fourteen (36%) patients were in remission. Improvement was seen most frequently in patients started on IVIg (94%) and remission in those started on combination treatment (44%). Differences between groups did not reach statistical significance. Residual symptoms or deficits ranged from 25% for neuropathic pain to 96% for any sensory deficit. Conclusions Improvement was seen in most patients. One year after the start of treatment, more than half of the patients were untreated and around one-third in remission. Residual symptoms and deficits were common regardless of treatment.

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Chronic inflammatory demyelinating polyradiculoneuropathy—Diagnostic pitfalls and treatment approach

Cited by 40 publications

References 94 publications

Expanding the Spectrum of Chronic Immune Sensory Polyradiculopathy

Expanding the Spectrum of Chronic Immune Sensory Polyradiculopathy

Blood-based biomarkers of inflammation in amyotrophic lateral sclerosis

Clinical outcome of CIDP one year after start of treatment: a prospective cohort study

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