A retrospective study on the efficacy and safety of intraveinous immunoglobulin (Tegeline(®)) in patients with chronic inflammatory demyelinating polyneuropathy.

A retrospective study on the efficacy and safety of intraveinous immunoglobulin (Tegeline(®)) in patients with chronic inflammatory demyelinating polyneuropathy. Presse Med. 2015 Jun 9; Authors: Robert F, Edan G, Nicolas G, Pouget J, Vial C, Antoine JC, Puget S Abstract OBJECTIVE: To assess the efficacy and safety of intraveinous immunoglobulin (IV Ig) in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) at 4, 7 and 12months. METHODS: A national multicenter retrospective study was conducted by LFB Biotehcnologies in patients with CIDP who had received at least one cycle of a 5% polyvalent IV Ig, Tegeline(®), from LFB biomédicaments between 1995 and 2004. The primary endpoint was the efficacy of IV Ig at 4 months, which was defined as the responder rate based on the modified Rankin scale. Several secondary endpoints were assessed: safety and efficacy (i.e., responders according to the investigators' overall assessment of the patients' status) at 4, 7 and 12 months. The analysis was performed at 7 months only (due to missing data for 12 months and few patients). RESULTS: A total of 26 patients were included who had received between 1 and 6 cycles of IV Ig (mean 3±2) with a median follow-up of 9.9 months. The responder rate at 4 months based on the modified Rankin scale was 52% (95% CI 0.313-0.722), whereas the responder rate with placebo reported in the literature (meta-analysis including results from va...
Source: Presse Medicale - Category: Journals (General) Authors: Tags: Presse Med Source Type: research

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Authors: Kuwabara S, Misawa S Abstract Chronic inflammatory demyelinating polyneuropathy (CIDP) is immune-mediated neuropathy defined by clinical progression for more than 2 months, and electrodiagnostic evidence of peripheral nerve demyelination. However, there are several clinical phenotypes, classified into "typical CIDP," and "atypical CIDP" such as "multifocal acquired demyelinating sensory and motor neuropathy (MADSAM)." Typical CIDP is a most common form, characterized by symmetric proximal and distal muscle weakness and motor-dominant manifestation. In typical CIDP, demyel...
Source: Advances in Experimental Medicine and Biology - Category: Research Tags: Adv Exp Med Biol Source Type: research
Source: Infectious Diseases - Category: Infectious Diseases Authors: Source Type: research
Conclusions Our findings confirm previous data on the tight clinico-serological correlation between antibodies to nodal/paranodal proteins and CIDP. Despite the low prevalence, testing for their presence and isotype could ultimately be part of the diagnostic workup in suspected inflammatory demyelinating neuropathy to improve diagnostic accuracy and guide treatment. Classification of evidence This study provides Class III evidence that antibodies to nodal/paranodal proteins identify patients with CIDP (sensitivity 6%, specificity 100%).
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Autoimmune diseases, All Neuromuscular Disease, Peripheral neuropathy, Chronic inflammatory demyelinating polyneuropathy Article Source Type: research
We present a patient who had four episodes of demyelination in the central nervous system as the only manifestation of inherited CD59 deficiency. Relapsing encephalopathy partially responsive to intravenous immunoglobulin and steroid treatments on the background of parental consanguinity suggested an inherited immune dysregulation. Next generation sequencing revealed homozygous mutation in the CD59 gene, confirmed by lack of CD59 expression on flow cytometry. Inherited CD59 deficiency is a rare autosomal recessive condition characterized by chronic hemolysis, recurrent strokes, and relapsing peripheral demyelinating neurop...
Source: Neuropediatrics - Category: Neurology Authors: Tags: Short Communication Source Type: research
Conclusions: Our patients achieved low scores in both the physical and mental aspects of QoL compared with other studies. We found a significant correlation between the physical domain of the SF-36 score and muscle strength of the hand, hip, and foot muscle group and with total manual muscle testing, suggesting that physical disability is the most crucial factor affecting QoL. Also, there was a significant negative association between the duration of the disease and the physical domain.
Source: Journal of Clinical Neuromuscular Disease - Category: Neurology Tags: Original Article Source Type: research
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired neurological disorder characterized clinically by weakness and impaired sensory function evolving over 2  months or more, loss or significant decrease in deep tendon reflexes, and by electrophysiological evidence of peripheral nerve demyelination. Expeditious diagnosis and treatment of CIDP early in the disease course is critical such that irreversible disability can be avoided. Intravenous immunoglo bulin (IVIG) is one first-line and maintenance therapy option for CIDP.
Source: Journal of the Neurological Sciences - Category: Neurology Authors: Source Type: research
ConclusionEven though disability seems to progress in most cases despite the treatments used, our results suggest that an early electrophysiological reduction in sensory nerves could be considered a ‘red flag’ for the prompt initiation of rituximab to try to delay long-term disability.
Source: Journal of Neurology - Category: Neurology Source Type: research
Gangliosides are sialylated glycosphingolipids, highly abundant in our nervous system. Antibodies targeting gangliosides are usually developed as a consequence of molecular mimicry following infections. Antiganglioside antibodies are implicated in many neurological disorders such as acute and chronic polyradiculoneuropathies which includes different variants of Guillain-Barr é syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and multifocal motor neuropathy. Presence of such antibodies in paraneoplastic peripheral neuropathy, neurodegenerative disorders, multiple sclerosis, myasthenia gravis and am...
Source: Journal of the Neurological Sciences - Category: Neurology Authors: Tags: Review Article Source Type: research
Authors: Franques J Abstract Chronic inflammatory demyelinating polyradiculoneuropathies are acquired demyelinating neuropathies belonging to the group of autoimmune neuropathies. Since specific biological markers are present in less than 10% of cases, the diagnosis is based on the clinical and electrophysiological analysis of each patient. Furthermore, a decision-making algorithm ranking all other available paraclinical tools will guide the physician to the diagnosis of atypical forms. In nearly 80% of cases, these dysimmune neuropathies are responsive to first-line treatments, namely intravenous immunoglobulins, ...
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research
Abstract Therapeutic plasma exchange (TPE) has been used as a treatment modality in many autoimmune disorders, including neurological conditions, such as Guillain-Barre syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The American Society for Apheresis (ASFA) publishes its guidelines on the use of therapeutic apheresis every 3 years based on published evidence to assist physicians with both the medical and technical aspects of apheresis consults. The ASFA Guidelines included the use of TPE in both GBS and CIDP as an acceptable first-line therapy, either alone and/or in conjuncti...
Source: Presse Medicale - Category: General Medicine Authors: Tags: Presse Med Source Type: research
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