Variants of CIDP
DADS variant
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(Diseases can present with different symptoms or variations)
Search Cidpusa web Lewis--Sumner syndrome
LSS was initially defined as a sensory or sensorimotor multineuropathy with persistent motor nerve conduction blocks . According to some reports, this is the most common variant of CIDP . Later, other authors have defined LSS as an asymmetric polyneuropathy , although only a few of them specified the degree of asymmetry necessary for diagnosis and how to quantify it . Even the 2010 EFNS/PNS diagnostic criteria for CIDP defined LSS as an asymmetric polyneuropathy . Then, other authors have eliminated the presence of conduction blocks as a diagnostic criterion . In the Italian CIDP database, where LSS was defined as a multineuropathy, 37.5% of the patients with the typical form had an asymmetric but not multifocal CIDP including 9.5% with a slight asymmetry [one Medical Research Council (MRC) point difference between the two sides] [7▪▪]. These figures are interesting as they show that the asymmetric form of CIDP is much more frequent than expected from a clinical entity that is considered ‘atypical’. It cannot, however, be excluded that, as in vasculitic neuropathies, a certain number of patients with multineuropathic CIDP evolve over time towards an asymmetrical form. Finally, the 2021 EFNS/PNS criteria has defined LSS as a sensory or sensorimotor multineuropathy specifying that its clinical presentation is usually asymmetric
Distal acquired symmetric demyelinating neuropathy In 2000, Katz et al. [1], described DADS as a distal, symmetric, sensory, or sensorimotor neuropathy sparing proximal limb, neck, and facial muscles. In their study, 60% of the patients had an IgM paraprotein and at least 33% of them were positive for anti-MAG (myelin-associated glycoprotein) antibodies [1]. Being an exclusion criterion for CIDP diagnosis, in the subsequent descriptions of DADS, only patients with negative anti-MAG antibodies were included, whereas idiopathic DADS was considered a variant of CIDP. Still, Larue et al.[3] found that 60% of the patients with DADS had a monoclonal gammopathy (40% IgG and 20% IgM). Association between DADS phenotype and IgM paraprotein was also recently confirmed in a study that has investigated the frequency and role of comorbidities in a large cohort of CIDP patients and found that 12.5% of the patients with DADS had an IgM monoclonal gammopathy (versus 5.5% of the patients with typical CIDP) [48▪▪]. According to some studies, DADS is the most common variant of CIDP, with a frequency that ranges from 2 to 15% (Table 1). Although DADS is defined as predominantly distal, the exact proximal to distal gradient of motor and sensory deficits was not specified neither in the 2010 EFNS/PNS guidelines nor in their revision. Disproportionately prolonged motor distal latencies (DL) are seen in at least two nerves. Symmetric, sensory or sensorimotor polyneuropathy starting distally in the LL, without proximal limb–trunk–face involvement (length-dependent fashion). Other possible symptoms include ataxia, neuropathic pain, cramps, fatigue, autonomic symptoms, tremor. UL distal sensory or sensorimotor symptoms and signs occurring later (at least after 1 year from onset). IVIG in the treatment of chronic pain syndromes.
Goebel A, Netal S, Schedel R, Sprotte G.
Klinik fur Anaesthesiologie, University Wurzburg, Wurzburg, Germany.
IVIG was used to treat patients in pain and the results were impressive. Most pain patients have neuropathy related pain disorders. The study is explained below.
Objective. To examine the use of intravenous immunoglobulin (IVIG) in chronic pain. Design. A prospective multiple-dose, open-label cohort study in 130 consecutive patients who suffered from 12 chronic pain syndromes. The largest symptom groups were (number of patients): Fibromyalgia (48); Spinal pain (20); Complex regional pain syndrome (CRPS, 11); Peripheral neuropathic pain (12); and atypical facial pain (11). Results. Overall, 20% of patients had>70% pain relief and 27.7% of patients reported relief between 25% and 70%. Six patients (4.6%) had moderately increased pain levels for a duration of up to 9 weeks. Good relief, of more than 70%, was found in all major symptom groups. Patients with pain of short duration (<2 years) reported high relief rates (33.8% of patients in this group reported relief openface>70%). No serious adverse events were reported. Conclusions. IVIG may be effective in patients suffering from chronic pain.
Management of chronic inflammatory demyelinating polyradiculoneuropathy.
Hughes RA.
Department of Clinical Neurosciences, Guy's, King's and St Thomas' School of Medicine, London, UK.
This review briefly describes current concepts concerning the nosological status, pathogenesis and management of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). CIDP is an uncommon variable disorder of unknown but probably autoimmune aetiology. The commonest form of CIDP causes more or less symmetrical progressive or relapsing weakness affecting proximal and distal muscles. Against this background the review describes the short-term responses to corticosteroids, intravenous immunoglobulin (IVIg) and plasma exchange that have been confirmed in randomised trials. In the absence of better evidence about long-term efficacy, corticosteroids or IVIg are usually favoured because of convenience. Benefit following introduction of azathioprine, cyclophosphamide, cyclosporin, other immunosuppressive agents, and interferon-beta and -alpha has been reported but randomised trials are needed to confirm these benefits. In patients with pure motor CIDP and multifocal motor neuropathy, corticosteroids may cause worsening and IVIg is more likely to be effective. General measures to rehabilitate patients and manage symptoms, including foot drop, weak hands, fatigue and pain, are important.
PMID: 12534332 [PubMed - indexed for MEDLINE] Continue to next page of CIDP variants
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