Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy.

AIM: The clinical and epidemiological characteristics of chronic inflammatory demyelinating polyneuropathy (CIDP) in an Italian population were assessed. SUBJECTS AND METHODS: All subjects with a diagnosis of demyelinating neuropathy after 1990 in Piemonte and Valle d'Aosta (4,334,225 inhabitants) were considered. The diagnosis of CIDP was based on the research criteria of the American Academy of Neurology. 165 of 294 patients met the diagnostic criteria. RESULTS: The crude prevalence rate was 3.58/100,000 population (95% CI 3.02 to 4.20). At the prevalence day, 76 (49.0%) cases had definite, 67 (43.2%) probable and 12 (7.7%) possible CIDP; disability was mild in 105 (67.7%) cases, moderate in 32 (20.6%) and severe in 18 (11.6%). The course was remitting-relapsing in 40 cases (25.8%), chronic progressive in 96 (61.9%) and monophasic in 19 (12.3%). Considering the 95 patients whose disorder presented in the period 1995-2001, the mean annual crude incidence rate was 0.36/100,000 population (95% CI 0.29 to 0.44), with a male to female ratio of 2.3:1. 14 cases were affected by diabetes mellitus. In multivariate analysis, factors related to severe disability at the prevalence day were: age >60 years; failure of immunomodulating therapies at the time of diagnosis; worse disability at nadir; and chronic course. CONCLUSION: Incidence and prevalence rates of CIDP in Italy were higher than those observed in most previous studies. At the prevalence day, more than 80% of cases had a mild or moderate disability, indicating either a good response to immunomodulating therapy or a tendency of CIDP to have a mild course in most cases.

Ischaemic stroke, factor V Leiden heterozygosity and left atrial thrombosis in sinus rhythm: a case report.

We describe a 63-year-old man in sinus rhythm (SR) with an ischaemic stroke involving basal ganglia region on the right side. The patient was known to be heterozygous for factor V Leiden (FVL) mutation. On diagnostic work-up, no arterial sources of embolism were found. Transoesophageal echocardiography evidenced a left atrial (LA) thrombosis without relevant cardiopathies. LA thrombosis is generally associated to atrial fibrillation, atrial enlargement, mitral valve stenosis and left ventricular dysfunction, whereas mitral regurgitation is considered protective. To our knowledge, this is the first report of cardioembolic stroke related to a LA thrombosis in a patient in SR without risk factors for thrombus formation except for FVL heterozygosity.

Positive effects of tertiary centres for amyotrophic lateral sclerosis on outcome and use of hospital facilities.

OBJECTIVE: To evaluate the effects of tertiary centres for amyotrophic lateral sclerosis (ALS) on ALS outcome and the use of hospital facilities. METHODS: The study was based on the data of an epidemiological, prospective, population-based register on ALS (Piemonte and Valle d'Aosta Register for amyotrophic lateral sclerosis, PARALS). The 221 patients recruited between 1995 and 1996 were prospectively followed up for outcome and use of hospital-based services. RESULTS: In all, 97 patients were followed up by tertiary ALS centres and 124 by general neurological clinics. Patients followed up by tertiary ALS centres were found to be 4 years younger and underwent percutaneous endoscopic gastronomy and non-invasive positive-pressure ventilation more often. Patients followed up by tertiary ALS centres were found to have a considerably longer median survival time (1080 v 775 days), even when stratifying by age, site of onset and respiratory function at diagnosis. In Cox multivariate analysis, attending a tertiary ALS centre was observed to be an independent positive prognostic factor. Moreover, patients attending a tertiary ALS centre were admitted to hospital less often (1.2 v 3.3) and were more frequently admitted for planned interventions. Conversely, patients followed up by general neurological clinics were more frequently admitted for acute events. Also, the hospital stay was considerably shorter for patients attending tertiary ALS centres (5.8 v 12.4 days). CONCLUSIONS: Improved survival was seen in patients with ALS attending tertiary ALS centres, independently from all other known prognostic factors, possibly through a better implementation of supportive treatments. Moreover, because of these centres, the hospitalisation rate was markedly reduced, thus offering a cost-effective service to patients with ALS and to the community as a whole.

Gabapentin vs. amitriptyline in painful diabetic neuropathy: an open-label pilot study.

The objective of this study was to compare the efficacy and tolerability of gabapentin and amitriptyline monotherapy in painful diabetic neuropathy. This was a 12-week, open-label, prospective, randomized trial. Twenty-five type-II diabetic patients with pain attributed to diabetic neuropathy and a minimum score of 2 on a pain intensity scale ranging from 0 (no pain) to 4 (excruciating pain) were randomized to receive either gabapentin, titrated from 1,200 mg/day to a maximum of 2,400 mg/day, or amitriptyline, titrated from 30 mg/day to a maximum of 90 mg/day. Both drugs were titrated over a 4-week period and maintained at the maximum tolerated dose for 8 weeks. The main outcome measures were weekly pain intensity and paresthesia intensity, measured on two categorical scales. Thirteen patients received gabapentin and 12 received amitriptyline. All 25 patients completed the trial. Gabapentin produced greater pain reductions than amitriptyline (mean final scores were 1.9 vs. 1.3 points below baseline scores; P = 0.026). Decreases in paresthesia scores also were in favor of gabapentin (1.8 vs. 0.9 points; P = 0. 004). Adverse events were more frequent in the amitriptyline group than in the gabapentin group: they were reported by 11/12 (92%) and 4/13 (31%) of patients, respectively (P = 0.003). Side effects were the main limiting factor preventing dose escalation. Gabapentin produced greater improvements than amitriptyline in pain and paresthesia associated with diabetic neuropathy. Additionally, gabapentin was better tolerated than amitriptyline. Further controlled trials are needed to confirm these preliminary results.

Factor VII coagulant activity and cholesterol changes in premenopausal women consuming a long-term cholesterol-lowering diet.

We periodically obtained blood samples from mildly hypercholesterolemic, but otherwise healthy, premenopausal women who were recruited to participate in a study of a long-term, cholesterol-lowering diet. All meals were prepared and most meals were consumed in the study center dining facility. Tests performed on blood samples included fibrinogen, cholesterol, factor VII coagulant activity (VIIc), and other measures of factor VII. We found that when women switched from a typical American diet (37% fat, polyunsaturated fatty acid to saturated fatty acid [P/S] ratio 0.5, 300 mg cholesterol/d) to a diet lower in fat and cholesterol (American Heart Association phase 2 diet: 30% fat, P/S ratio of 1, 150 to 200 mg cholesterol/d) and maintained that diet for 20 weeks, their plasma cholesterol levels decreased by approximately 6% after 4 weeks and remained at that level until study termination. Likewise, VIIc decreased by approximately 11% while factor VII antigen, total factor VII activity, and fibrinogen concentration did not change appreciably from baseline values. Our results show that premenopausal women benefit from a diet lower in total and saturated fat by a reduction in blood cholesterol and VIIc. Extrapolation from data on men in the Northwick Park Heart Study indicates that the 11% decrease in VIIc activity would correspond to an approximately 30% decrease in risk of mortality from coronary heart disease.

State of stupor from valproic acid during chronic treatment: case report.

We describe the case of a 26 years old woman in chronic therapy with phenobarbital, carbamazepine, valproic acid (VPA) and clonazepam who showed a hyperammonemic encephalopathy after an increase in dosage of VPA. Similar cases have been reported, but with acute-subacute onset and no correlation with the plasma levels of VPA. Our case suggests the possibility that this toxic effect occurs during chronic treatment too, when the dosage of VPA is increased.

In vivo and in vitro effect of liver on somatomedin generation.

The serum concentration of circulating somatomedins was measured in the blood of healthy donors and subjects with hepatic cirrhosis, and in culture media from in vitro explants of healthy and cirrhotic human liver. Serum levels of somatomedin bioactivity were significantly lower in cirrhotic subjects (0.42 +/- 0.03 U/ml; M +/- SEM) compared with age matched controls (0.99 +/- 0.03 U/ml). Radioreceptor assay of somatomedin concentrations confirmed this reduction in cirrhotic patients (0.89 +/- 0.06 U/ml) compared with controls (1.32 +/- 0.05 U/ml). A parallel reduction in somatomedin circulating binding ability was also observed (99.43 +/- 7.28% in cirrhotic and 123.5% +/- 10.8% in normal subjects). In vitro explants from normal human liver tissue produced a significant increase (0.57 +/- 0.09 U/ml) in somatomedin bioactivity contained in the medium (0.29 +/- 0.06 U/ml), while a decreased bioactivity (0.12 +/- 0.06 U/ml) was observed with explants of cirrhotic livers. These results support a role of liver in the biosynthesis of both somatomedin and somatomedin binding protein.

[Antagonism between focal epilepsy and taurine administered by cortical perfusion]. / Antagonismo fra epilessia focale e taurina somministrata per perfusione corticale

The therapeutic action of taurine cortical perfusion was tested in cats affected with Premarin and cobalt cortical epileptogenic foci. In all animals taurine provoked the disappearance of EEG epileptic abnormalities. In the case of Premarin focus the effect appeared more quickly than in the cobalt one. This different time-course, according to previous reports on the antiepileptic action of the parenteral administration of the amino acid, suggests the hypothesis of a taurine direct inhibitory action against Premarin focus and, on the contrary, a mediated action towards the cobalt's. The latter might be related to the metabolic production of some taurine derivative.