High-Dose Immunosuppression for Refractory CIDP? Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy for which treatment with plasma exchange, intravenous immunoglobulin, or corticosteroids usually is beneficial. However, one third of CIDP patients do not respond to these conventional therapies, and many who respond initially relapse and develop substantial disability after several years. Compelling data suggest that both B and T cells mediate the immune attack on peripheral nerve, thus providing a biologic rationale for using more aggressive immunosuppressive regimens. These researchers report their experience with high-dose cyclophosphamide (200 mg/kg over 4 days) in 4 patients with severe CIDP that was refractory to other therapies. All patients were disabled (Rankin scores of 4 or 5) and had failed numerous trials of standard therapies. Interestingly, 3 patients also had failed to improve after previous treatment with intravenous cyclophosphamide (1 g/m2). All patients' Medical Research Council strength scores and Rankin disability scores improved; 2 patients improved by 3 Rankin grades. Febrile neutropenia and transient renal insufficiency occurred in 2 patients, and another developed line sepsis, but there was no long-term morbidity or mortality. Comment: Current therapies for CIDP are inadequate for many patients. In this study, a 70-kg patient would have received 14 g of cyclophosphamide, only slightly more than the "immunomodulating" dose (1 g/m2) of 1.5 to 2.0 g administered as a pulse monthly for 6 months (a regimen that many centers already have adopted empirically). Therefore, the intensity of immunosuppression may be a more relevant factor than the total cumulative dose or the duration of therapy. The benefits of this approach must be weighed against high risks for systemic infection and for other serious complications. The risk-benefit balance may be in favor of high-dose immunosuppression if patients experience sustained remission and discontinue prednisone or other chronic immune (and potentially toxic) treatments. — Kenneth C. Gorson, MD Dr. Gorson is Associate Professor of Neurology, Tufts University School of Medicine, Boston. Published in Journal Watch Neurology August 22, 2002 For those with multi-focal neuropathy with conduction block the rules for treatment are different. Those patients get worse with steroids and do not do very well with plasma exchange, but do respond well to IVIg. Some patients with CIDP have a para-protein in the blood, which is an abnormal protein produced by the bone marrow. These patients may respond to CIDP treatments, but may also require treatment for the excess para-protein. To conclude, CIDP is a very variable condition. It is auto-immune and is treatable. It is exceptional for CIDP not to respond to at least one of the available drugs. The first choice of treatment is either prednisolone or IVIg. The diagnosis is difficult, much less so than GBS, depending critically on the nerve conduction studies and neuro-physiologies. Trials for immuno-suppression have shown promise. ..........cidpusa

 

No To Shinkei. 2004 May;56(5):421-4.

[High-dose intravenous immunoglobulin in the treatment of sensory ataxic neuropathy with Sjögren's syndrome: a case report]


Taguchi Y, Takashima S, Takata M, Dougu N, Asaoka E, Inoue H.

The Second Department of Internal Medicine, Toyama Medical & Pharmaceutical University, 2630 Sugitani, Toyama 930-0194, Japan.

We report herein a case of sensory ataxic neuropathy with Sjogren's syndrome (SS-SAN) who became dramatically improved in response to high-dose intravenous immunoglobulin treatment (IVIg). An 81-year-old man began to feel numbness in his hands and feet in August 2002. Because he became unsteady and could not do skillfull movement, he was admitted to our hospital in May 2003. On neurological examination, all tendon reflexes were absent. His vibratory and position senses were severely impaired to knees and elbows. Touch, temperature, and pinprick sensations were mildly disturbed in a glove-stocking distribution. Coordination was clumsy in all limbs because of sensory loss. He had gait ataxia with Romberg sign. Nerve conduction study revealed that sensory nerve action potentials were absent. He was diagnosed as having SS-SAN because Schirmer test, Saxon test and both SS-A and SS-B antibodies were positive. Thereafter, Mg, 400 mg/kg daily for 5 days, was administered. His sensory impairment began to improve 2 days after Mg. Subsequently, he could walk steadily without ataxia. It is considered that IVIg may be an effective treatment for SS-SAN.
 

PMID: 15279200 [PubMed - indexed for MEDLINE]

 

Arch Neurol. 2002 May;59(5):751-7.

 
Diabetic demyelinating polyneuropathy responsive to intravenous immunoglobulin therapy.

Sharma KR, Cross J, Ayyar DR, Martinez-Arizala A, Bradley WG.

Department of Neurology, University of Miami School of Medicine, 1150 NW 14th St, Room 603, Miami, FL 33136, USA. ksharma@med.miami.edu

BACKGROUND: There is growing evidence that idiopathic chronic inflammatory demyelinating polyneuropathy (CIDP) and polyneuropathy in patients with diabetes mellitus (DM) that meets the electrophysiological criteria for CIDP (DM-CIDP) have many similarities. OBJECTIVE: To evaluate whether DM-CIDP responds to intravenous immunoglobulin (IVIG) therapy. PATIENTS AND METHODS: Twenty-six patients (mean [SD] age, 64 [8.9] years; age range, 40-80 years) with type 2 DM (n = 25), who met the electrophysiological criteria for CIDP, were given IVIG therapy (400 mg/kg body weight per day for 5 days) in a prospective open-label pilot study. All patients had quantitative evaluation using the Neuropathy Impairment Score at baseline and at the end of 4 weeks from the initiation of IVIG therapy. RESULTS: The mean Neuropathy Impairment Score improved significantly from baseline (mean [SD], 61.5 [26.0] points) to the end of the fourth week (33 [29.6] points; P<.00l). This clinically significant improvement occurred in 21 (80.8%) of the 26 patients. Conduction block occurred in 11 (42.3%) of the 26 patients; improvement in the Neuropathy Impairment Score was more frequent in patients who had a conduction block (11 of 11 patients) than in those who did not (10/15 [66.7%]; P =.03). Adverse reactions to IVIG included reversible renal dysfunction in 3 patients, flulike symptoms in 5, headache in 5, and chest pain and shortness of breath in 1. CONCLUSION: Although IVIG therapy seemed to improve DM-CIDP in this uncontrolled trial, a controlled trial is required for confirmation of our findings.

Publication Types:

• Clinical Trial

PMID: 12020256 [PubMed - indexed for MEDLINE]

 

Curr Treat Options Neurol. 2001 Mar;3(2):139-146.

Diabetic Lumbosacral Polyradiculoneuropathies.

Amato AA, Barohn RJ.

Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA.

The pathogenic basis and treatment of diabetic polyradiculoneuropathy is a source of recent controversy as there may be two or more distinct forms of diabetic polyradiculoplexopathy. We believe that the following two categories of diabetic polyradiculoneuropathy can be made on the basis of clinically differences: 1) the more common asymmetric, painful polyradiculoneuropathy; and 2) the rare symmetric, painless, polyradiculoneuropathy. The asymmetric, painful form (also known as diabetic amyotrophy) may have an autoimmune basis, but the etiology is not clear. The natural history for diabetic amyotrophy is spontaneous improvement. Nevertheless, various immunotherapies (eg, corticosteroids and intravenous immunoglobulin (IVIg) have been tried with subsequent improvement in symptoms. Treatment is reserved only for patients with severe ongoing pain, given the significant side effects of these medications in those patients with diabetes. Prednisone and IVIg may help alleviate the pain associated with diabetic amyotrophy. Relief of pain can help patients begin physical therapy earlier, however, there are no prospective, blinded, controlled studies that demonstrate that these treatments lead to an earlier and better recovery of muscle strength compared with the natural history of the disorder. The symmetric, painless form of diabetic polyradiculoneuropathy may in fact represent chronic inflammatory demyelinating polyneuropathy (CIDP) occurring in a patient with diabetes mellitus (DM). Patients with idiopathic CIDP may improve various immunomodulating therapies, including corticosteroid treatment, plasma exchange (PE), and IVIg. In this regard, patients with the symmetric, painless, proximal diabetic polyradiculoneuropathy may also respond to corticosteroids, plasma exchange, IVIg, azathioprine, or cyclophosphamide. However, as with diabetic amyotrophy, some patients improve spontaneously without treatment. In still other patients, the neuropathy appears unresponsive to immunotherapy. In such patients, this polyradiculoneuropathy might be caused by metabolic dysfunction associated with DM. Unfortunately, from a clinical, laboratory, and electrophysiologic standpoint, it is impossible to distinguish the patients with a symmetric, painless diabetic polyradiculoneuropathy who might respond to therapy. A trial of PE can be useful in identifying patients who might have a polyradiculoplexopathy that is responsive to immunotherapy. If patients respond to PE, they may continue to receive intermittent exchanges or be switched over to prednisone or IVIg.

PMID: 11180751 [PubMed - as supplied by publisher]

 

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