Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0848771 (neurological disability)
 928 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Of 40 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 28 completed a controlled three-month trial of prednisone. Prednisone was shown to cause a small but significant improvement over no treatment in scored neurological disability, some measures of computer-assisted sensory detection threshold, graded muscle strength, and some attributes of nerve conduction. No subset of patients was more likely than another to be responsive to prednisone; those with a progressive course were as likely to be responsive as recurrent cases. This finding provides further justification for classifying progressive with recurrent cases as CIDP and demonstrates that prednisone treatment should not be withheld from patients with progressive disease.
 ...
PMID:Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. 704 88

Eighteen patients with definite, untreated chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) of chronic progressive (nine patients) or relapsing course (nine patients) were randomized prospectively to receive 10 plasma-exchange (PE) or sham plasma-exchange (SPE) treatments over 4 weeks in a double-blind trial. After a wash-out period of 5 weeks or when they returned to baseline scores, patients were crossed over to the alternate treatments. Neurological function was assessed serially using a quantitative neurological disability score (NDS), a functional clinical grade (CG) and grip strength (GS) measurements. Electrophysiological studies were done at the beginning and end of each treatment. A primary 'intention to treat' analysis showed significant improvement with PE in all clinical outcome measures: NDS by 38 points, P < 0.001; CG by 1.6 points, P < 0.001; GS by +13 kg, P < 0.003 and in selected electrophysiological measurements, sigma proximal CMAP, P < 0.01; sigma motor conduction velocities, P < 0.006; sigma distal motor latencies, P < 0.01. Fifteen patients completed the trial and of those, 12 patients (80%) improved substantially with PE; i.e. five out of seven patients with chronic progressive course and seven out of eight patients with relapsing CIDP improved. There were three drop-outs; one patient lost venous access; one patient suffered a stroke and one patient left the trial to receive open treatment elsewhere. The improvement in motor functions correlated with the electrophysiological data, i.e. with improved motor conduction velocities and reversal of conduction block. Eight of 12 PE responders (66%) relapsed within 7-14 days after stopping PE. All improved with subsequent open label PE; all but two patients required long-term immunosuppressive drug therapy for stabilization. The PE non-responders improved with prednisone. We conclude that PE is a very effective adjuvant therapy for CIDP of both chronic progressive and relapsing course; concurrent immunosuppressive drug treatment is required. Exchange treatments should be given two to three times per week until improvement is established; the treatment frequency should then be tapered over several months.
 ...
PMID:Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy. A double-blind, sham-controlled, cross-over study. 881 70

Thirty patients with definite or probable chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) of chronic progressive (16 patients) or relapsing (14 patients) course were randomly assigned to receive intravenous immunoglobulin (IvIg) 0.4 g per kg body weight or a placebo treatment on 5 consecutive days in a double-blind, cross-over trial. Neurological function was monitored by serial quantitative assessments [neurological disability score (NDS); clinical grade (CG) and grip strength (GS) measurements] and by electrophysiological studies before and after each treatment period. Twenty-five patients completed both treatment periods. A comparison of the observed changes in clinical outcome measures revealed statistically significant differences in favour of IvIg, with (mean +/- SD) improvements in NDS by 24.4 +/- 5.4 points (P < 0.002) in CG by 1 +/- 0.3 points (P < 0.001) in GS by +6.3 +/- 1.7 kg (P < 0.005), whereas scores were unchanged or worse with placebo. A secondary two-groups analysis of the first trial period included all 30 patients; 16 patients had been randomly assigned to IvIg and 14 to placebo treatments. Again significant differences in favour of IvIg were observed in all the clinical end-points: improvement in NDS was 35.6 +/- 25 points (P < 0.0001), in CG it was 1.3 +/- 1.9 points (P < 0.002) and in GS +9.8 +/- 7.7 kg (P < 0.001), whereas all scores worsened with placebo. Of the 30 patients, 19 (63%) improved with IvIg treatments; nine out of 16 patients (56%) with chronic progressive CIDP, and 10 out of 14 patients (71%) with relapsing CIDP (differences were not statistically significant). A placebo response was seen in five patients. Comparison of paired electrophysiological measurements before and 4 weeks after IvIg treatments revealed statistically significant improvements in the summed motor conduction velocities (sigma MCV; P < -0.0001) and in the summed compound muscle action potentials (CMAP) evoked with proximal stimulation (sigma proximal CMAP, P < 0.03) of median, ulnar, peroneal and tibial nerves. Eight of nine IvIg responders with chronic progressive CIDP improved gradually to normal function with a single 5 day course of IvIg; in five of these, small doses of prednisone were prescribed during follow-up. In 10 IvIg responders with relapsing CIDP, improvements lasted a median 6 weeks (range 3-22 weeks) and was reproducible with open label treatments. All 10 patients have been maintained and stabilized with IvIg pulse therapy of 1 g per kg body weight or less, given as a single infusion prior to the expected relapse. A beneficial response to IvIg was found to be most likely in patients with acute relapse or with disease of one year or less. Patients with predominantly sensory signs did not improve.
 ...
PMID:Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy. A double-blind, placebo-controlled, cross-over study. 881 71

We studied the therapeutic characteristics of double filtration plasmapheresis (DFPP) in 14 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The patients were classified into 2 subgroups of the responders (7 patients) and the non-responders (7 patients) to DFPP. The responders to DFPP were designated as those showing the improvement 2 or more grades in measures the activity of daily living by the modified Rankin scale (MRS). All these patients underwent neurological assessment, CSF study, electrophysiological studies at the beginning and end of treatment. Sural nerve biopsy study was performed in 10 cases. Neurological function was assessed serially using a quantitative neurological disability score (NDS). At the beginning of treatment, there were no significant differences in various measurements between the responders and the non-responders except for the frequency of demyelination. In responders, significant improvement was found in mean changes in MRS, NDS, motor nerve conduction velocity, compound muscle action potential, distal motor latency, while in non-responders, all measures remained unchanged or worsened. Muscle wasting was seen in 3/7 responders and 4/7 non-responders, and denervation potentials in needle EMG were seen in 1/7 responders and 3/7 non-responders. Four patients of the responders were classified as chronic relapsing course, and 6 patients of the non-responders as chronic progressive course. We conclude that DFPP was useful for the subgroups of CIDP patients, but the underlying immuno-pathological background that determine the efficacy of plasmapheresis should be elucidated.
 ...
PMID:[Double filtration plasmapheresis (DFPP) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)]. 991 16