Treatment of chronic inflammatory demyelinating polyneuropathy

The management of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the main topic of this review. A few comments will also be made about treatment of the demyelinating form of paraproteinaemic demyelinating polyneuropathy (PDN) and of multifocal motor neuropathy (MMN). The review briefly describes the main characteristics of these neuropathies, and examines case series and trials which evaluated the principal therapeutic strategies for CIDP, PDN and MMN, such as intravenous immunoglobulin (IVIg) therapy, steroid treatment, plasma exchange and immunosuppressor administration. Controlled trials demonstrated that IVIg, steroid treatment and plasma exchange are effective in CIDP. For PDN the therapeutic strategies are the same as for idiopathic CIDP, but usually the clinical response is poorer. For MMN, IVIg therapy is definitely the first choice treatment.

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Sommario Lo scopo principale di questa review è di esaminare le strategie terapeutiche nella neuropatia infiammatoria cronica demielineizzante (CIDP). Alcuni commenti vengono fatti anche riguardo il trattamento della forma demielinizzante della neuropatia demielinizzante paraproteinemica (PDN) e della neuropatia motoria multifocale (MMN). La review delinea le principali caratteristiche cliniche delle diverse forme di neuropatia demielinizzante ed esamina i principali studi clinici, controllati e non controllati, che hanno valutato il trattamento deila CIDP e della PDN e MMN con immunoglobuline endovena (IVIg), con la terapia steroidea, la plasmaferesi e farmaci immunosoppressori. Studi controllati hanno dimostrato che le IVIg, to steroide e la plasmaferesi sono efficaci nella CIDP. Per quanto riguarda la PDN le strategic terapeutiche sono analoghe a quelle adottate nella CIDP anche se la risposta clinica è solitamente meno evidente. Infine, nella MMN le IVIg sono sicuramente il trattamento di prima scelta.

     

Treatment of chronic inflammatory demyelinating polyneuropathy

PURPOSE OF REVIEW: Chronic inflammatory demyelinating poly(radiculo)neuropathy (CIDP) is a treatable disorder. There are three proven effective treatments available. Randomized controlled trials have only focused on short-term effects, but most patients need long-term therapy. The most up-to-date treatment options are discussed. Attention is also paid to the use of appropriate assessment scales and treatment of residual findings. RECENT FINDINGS: A Cochrane review is available indicating that intravenous immunoglobulin is an effective treatment. Equal efficacy of intravenous immunoglobulin and steroids was shown during a 6-week treatment period. New open studies indicated possible efficacy for mycophenolate, interferon-beta and etanercept. Combinations of treatment are scarcely studied yet. Some CIDP patients may have a more acute onset of disease since maximum severity is reached within 4-8 weeks, resulting in confusion about the diagnosis. It was shown that severe fatigue can be a major complaint in CIDP patients; a training regimen might partially resolve these problems. SUMMARY: CIDP is a treatable disorder, but most patients need long-term treatment. Intravenous immunoglobulin, steroids and plasma exchange are shown to be effective. It is suggested that other immunomodulatory agents can also be effective, but randomized trials are needed to confirm these benefits. General measures to rehabilitate patients and to manage symptoms like fatigue and other residual findings are important.     

Chronic inflammatory demyelinating polyneuropathy after treatment with interferon-alpha

Treatment with interferon-alpha (IFN-alpha) has been associated with the occurrence of a number of autoimmune disorders. We report a case of chronic inflammatory demyelinating polyneuropathy (CIDP) occurring in a patient with a chronic viral hepatitis C infection who received a novel, long-acting form of IFN-alpha. After withdrawal of the interferon treatment, this patient responded to a single extended course of plasma exchange that resulted in a complete clinical remission of symptoms without relapse.     

Treatment of chronic inflammatory demyelinating polyneuropathy with cyclosporin-A.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune mediated polyneuropathy for which there are effective therapies. However, not all patients improve with these treatments. Eight patients with CIDP, two with IgG monoclonal gammopathies, were treated with cyclosporin-A (3 to 5 mg/kg/day). In three, this treatment was successful. It was unsuccessful in four patients who were resistant to other treatments and in one who had initially received cyclosporin-A. There were no serious side effects. In selected patients with CIDP, cyclosporin-A is a potentially useful treatment.     

Treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin

Chronic inflammatory demyelinating polyneuropathy is an immune-mediated demyelinating peripheral neuropathy usually treated with immunosuppressants. We reviewed our experience treating 15 patients (9 men, 6 women) with intravenous immunoglobulin. Six patients were on other therapies at the time of intravenous immunoglobulin infusions (4, prednisone; 2, prednisone and azathioprine). The dose of intravenous immunoglobulin was either 0.3 or 0.4 gm/kg/day for 4 to 5 days. Transient fever occurred in 1 patient. Subjective improvement in sensory symptoms was reported by almost all patients. Objective improvements in strength or functional tasks occurred in only 3 patients, a man with human immunodeficiency virus infection, a 14-year-old girl, and a woman with an immunoglobulin G kappa paraprotein. Our results suggest that individual patients may respond to intravenous immunoglobulin therapy. A multicenter controlled trial is needed to assess properly the role of intravenous immunoglobulin therapy in patients with chronic inflammatory demyelinating polyneuropathy.     

Chronic inflammatory demyelinating polyneuropathy in a patient with hyperIgEaemia

We herein report the case of a 46 year old man with chronic inflammatory demyelinating polyneuropathy (CIDP) with hyperIgEaemia. The patient presented with bilateral weakness, generalized hyporeflexia, and mild paresthesia of the fingers of both hands. Nerve conduction studies revealed multiple sites of motor conduction block in the absence of sensory abnormalities. Muscle strength increased, as did compound muscle action potential (CMAP) amplitude immediately after the intravenous infusion of immunoglobulin (IVIg). Serum IgE levels also fluctuated in parallel with his relapsing-remitting clinical course. We propose that pure motor CIDP may be immune mediated and suggest that IgE-mediated allergy may be one potential cause of this condition.     

Pathological findings in a patient with ventilatory failure and chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) may cause significant disability, but severe respiratory complications are uncommon. We describe the case of a 49-year-old man with clinical features of CIDP for 5 years who died of respiratory failure. Post-mortem findings of denervation in diaphragm muscle and axonal loss in phrenic nerve are presented. Severe ventilatory failure may occur in CIDP when neuropathy affects the respiratory muscles. Attention to early clinical features of respiratory insufficiency may facilitate the prevention of more severe features of respiratory failure.     

急、慢性炎症性脱髓鞘性多发性神经病神经电生理对比研究

目的 比较分析急性炎症性脱髓鞘性多发性神经病(AIDP)与慢性炎症性脱髓鞘性多发性神经病(CIDP)的电生理表现.方法 收集2011年1月～2013年1月在吉林大学白求恩第一医院神经内科就诊的19例AIDP患者及15例CIDP患者,分析上下肢周围神经传导检查各项指标.结果 AIDP与CIDP均表现为运动传导速度(MCV)减慢、远端潜伏期延长、波幅降低、传导阻滞、F波及H反射异常,但CIDP组MCV减慢明显,与AIDP组存在显著差异,且CIDP组感觉传导检测异常明显,AIDP组感觉神经传导异常少见.结论 AIDP患者主要以周围神经运动纤维受损为主,存在明显的脱髓鞘及轴索的损伤,但周围神经感觉纤维受损不明显.CIDP患者周围神经运动纤维及感觉纤维受损均非常明显,且脱髓鞘程度明显重于AIDP患者.     

Motor-dominant chronic inflammatory demyelinating polyneuropathy

We reviewed the clinical, electrophysiological an laboratory findings, plus the therapeutics and evolution of patients with motor-dominant Chronic inflammatory demyelinating polyneuropathy (CIDP) and compared them with those of other CIDP patients. Among 12 consecutive CIDP patients, we identified five patients with motor-dominant CIDP. The five patients with motor-dominant CIDP initially presented with weakness of the upper limbs. Cervical magnetic resonance imaging (MRI) examinations of the patients with motor-dominant CIDP showed that the most affected lesions are the cervical nerve roots and brachial plexus. The clinical course of these patients was relapsing-remitting, and they improved markedly after treatment by intravenous immunoglobulin (IVIg) infusion or plasmapheresis. However, they did not improve in response to corticosteroid therapy during the acute phase of relapses. The relapses frequently occurred within 2 years, but rarely occurred after that. The score in the modified Rankin disability scale (mRDS) at the last follow-up period was statistically lower for the patients with motor-dominant CIDP than for the other CIDP patients (P < 0.002). The characteristic clinical features, responsiveness to treatment, and prognosis suggest that motor-dominant CIDP is a distinct subtype of CIDP, with a specific immunological background. Repeated IVIg therapy is required to maintain the motor functions of patients with motor-dominant CIDP. We consider that treatment for recurrence prevention as an alternative to IVIg therapy is very important for patients with motor-dominant CIDP.     

Relapsing inflammatory demyelinating polyneuropathy in a diabetic patient

Summary Inflammatory demyelinating polyradiculoneuropathies exhibit well-known ultrastructural lesions of the peripheral nerve, both in acute cases, i.e., Guillain-Barré syndrome, and in relapsing, subacute and chronic cases. We present a case of relapsing inflammatory demyelinating polyradiculoneuropathy in a diabetic patient with a biopsy exhibiting these lesions, as well as a widening of the outermost myelin lamellae in some fibers. Such associated lesions are classic in experimental inflammatory demyelinating polyradiculoneuropathies, but have not been reported in human pathology.     

Immunoglobulins in chronic inflammatory demyelinating polyneuropathy

Neurological Sciences - This article reviews the efficacy and tolerability of intravenous immunoglobulins (IVIg) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), including those...     

Early neurophysiological evolution of chronic inflammatory demyelinating polyneuropathy in a patient with Hashimoto's thyroiditis

A patient with a known history of hypothyroidism due to Hashimoto's thyroiditis presented with a subacute, progressive sensorimotor deficit that affected the upper limbs predominantly. The electrophysiological findings progressively evolved from multifocal motor conduction block to multifocal demyelinating sensory and motor nerve involvement with conduction block, and finally to findings fulfilling the diagnostic criteria of chronic inflammatory demyelinating polyneuropathy (CIDP). The patient did not respond adequately to intravenous immunoglobulin, whereas oral prednisone led to fast and complete recovery. This report discusses the evolution of early findings of CIDP, as well as its coexistence with Hashimoto's thyroiditis.     

Electrophysiology in chronic inflammatory demyelinating polyneuropathy with IGIV

Patients with chronic inflammatory demyelinating polyneuropathy (CIDP) received immune globulin intravenous, 10% caprylate/chromatography purified (IGIV‐C, Gamunex; n = 59) or placebo (n = 58) every 3 weeks for up to 24 weeks (first period) in a randomized, double‐blind, parallel‐group, response‐conditional, crossover study. Motor and sensory nerves were assessed at baseline and endpoint/week 24. A nonsignificant trend toward improvement in the proximal amplitude of the most severely affected motor nerve was observed with IGIV‐C (0.69 ± 1.86 mV) versus placebo (0.47 ± 2.29 mV), and a greater improvement of 1.08 ± 2.15 mV with IGIV‐C versus 0.46 ± 2.03 mV with placebo (P = 0.089) was observed with exclusion of data from Erb's point stimulation. Greater improvements from baseline favoring IGIV‐C were observed for 127/142 electrophysiologic parameters. The averaged motor amplitudes from all motor nerves significantly improved with IGIV‐C versus placebo [treatment difference, 0.62 mV; 95% confidence interval (CI), 0.05, 1.20; P = 0.035], and conduction block decreased significantly (treatment difference, ?5.54%; 95% CI, ?10.43, ?0.64; P = 0.027), particularly in the lower limbs. Overall, the data suggest that IGIV‐C improves electrophysiologic parameters in CIDP. Muscle Nerve, 2009     

High-dose intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy]

We treated two patients with chronic inflammatory demyelinating polyneuropathy (CIDP) with high-dose intravenous immunoglobulin (HIG). The patients received 400 mg/kg of immunoglobulin a day for five days. One patient, who had failed to respond to prednisolone before, was treated with HIG, 18 months after the onset. His motor symptoms resolved immediately after the commencement of HIG. Electrophysiologically, the compound muscle action potentials increased in amplitude in all nerves examined and F wave reappeared in the left median nerve. The electrophysiological changes were compatible with improvement of conduction blocks. This patient had headache and exanthema during the HIG therapy, but they settled after cessation of the infusion. The other patient was administered HIG as an initial treatment, four months after the onset. HIG was of no effect in this case, but he showed remarkable recovery during the following prednisolone therapy. Although corticosteroid therapy is the first choice for CIDP, there are CIDP patients who do not respond to steroid or can not complete the steroid therapy because of adverse effects. HIG is an expectative and recommendable treatment for the steroid resistant CIDP patients.     

Treatment of chronic inflammatory demyelinating polyneuropathy with high-dose intermittent intravenous methylprednisolone

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) causes progressive disability due to weakness but responds to immunomodulating medication, including oral prednisone and intravenous (IV) immunoglobulin (IVIg). However, there is no consensus on initial therapy, and both of these treatments have drawbacks with long-term treatment. OBJECTIVE: To review the efficacy and safety of high-dose, intermittent IV methylprednisolone (IVMP) as initial and long-term maintenance therapy for patients with CIDP. DESIGN: A retrospective medical record review between 1992 and 2003 of outcomes in CIDP, comparing patients in 3 cohorts depending on whether their primary treatment was IVMP, IVIg, or oral immunosuppression with prednisone or cyclosporine. SETTING: Washington University Neuromuscular Disease Center (St Louis, Mo), outpatient and inpatient records. PATIENTS: Patients with clinical and electrophysiologic evidence of CIDP were identified. Of 57 patients, 39 had sufficient data for full analysis. MAIN OUTCOME MEASURES: Quantitative muscle testing with a handheld dynamometer. Medication profiles and adverse effects were also recorded. RESULTS: There was no significant difference in the mean improvement in quantitative muscle testing at 6 months or at the last clinic visit (an average of 4.5 years later) among the 3 groups. Fewer patients treated with oral immunosuppression improved at 6 months, but at the last visit, 81% to 88% improved in all 3 groups. Less weight gain and fewer cushingoid features affected patients treated with IVMP (19%) compared with patients treated with oral prednisone (58%). CONCLUSIONS: Treatment of patients with CIDP using high-dose intermittent IVMP results in improved strength equal to that with IVIg and oral prednisone. The frequency of occurrences of weight gain and cushingoid features with IVMP is less than that with oral prednisone. Intravenous methylprednisolone should be considered for initial and long-term therapy in CIDP when patients have disability due to weakness.