Search

The possible relationship between Guillain-Barré syndrome (GBS) and cancer is still controversial and the existence of a paraneoplastic GBS remains unconfirmed. To better define whether there is a relationship between GBS and malignancy, we compared the observed and the expected number of patients with tumours in a population-based cohort of subjects with GBS. Clinical differences between GBS patients with or without malignancies were analysed. Data were obtained from the Piemonte and Valle d'Aosta Register for GBS (PARGBS) (years 1990-1998). GBS was diagnosed according to NINCDS criteria. The number of expected cases of malignancy in the PARGBS population was calculated using the incidence rate of all types of cancer (ICD codes 140-208) in Piemonte [1985-1987], and in the most important town of this region, that is Turin (years 1993-1997). In the nine-year period, 435 incident patients with GBS were found. Nine of them developed cancer in the six months preceding or following GBS; in seven of them, the diagnosis of cancer and GBS was concomitant. The expected number of malignant tumours was 3.7 (using the incidence in Piemonte) and 3.8 (using the incidence in Turin); therefore, the odds ratios were 2.43 (95 % CI, 1.11-4.62) and 2.37 (95% CI, 1.09-4.50), respectively (p0.01). Although the cases with malignancies were clinically similar to the other cases of GBS observed through the Register, the mortality in GBS patients with cancer was higher and was the final cause of death in two patients affected by severe cancer. These results suggest a possible correlation between some cases of GBS and cancer. However, GBS in cancer patients does not meet all the criteria for paraneoplastic diseases.

The validity of discharge diagnosis of amyotrophic lateral sclerosis (ALS) and of the main procedures performed during hospitalization was assessed using as gold standard the data from the Piemonte and Valle d'Aosta Register for ALS (PARALS), a collaborative population-based registry aimed at determining prospectively the incidence and the factors related to ALS outcome. All patients discharged with ICD code 335.2 (primary and secondary diagnoses) in the period 1995-1996 in Piemonte, Italy, were considered. Out of the 1,049 cases identified, 433 remained after excluding patients not resident in Piemonte and repeated admissions. Of these, 258 had a correct diagnosis of ALS (168 incident and 90 prevalent cases) after a review of clinical records. The sensitivity of discharge diagnoses was 78.9%, and the positive predictive value was 38.8%. The sensitivity for main procedures (percutaneous endoscopic gastrostomy, noninvasive ventilation, and tracheostomy) ranged between 76 and 100%. ICD codes allowed to identify 22 cases that had not been ascertained with other sources. In conclusion, hospital discharge records appear to poorly reflect the incidence of ALS, and can be used only after clinical verification of the diagnosis.

To determine the incidence of ALS in two regions of Northwestern Italy, utilizing a prospective design.The study was performed in Piemonte and Valle d'Aosta (4,418,503 inhabitants) during the period 1995 to 1996. All neurologic departments in the two regions were involved in the study and prospectively collected and followed up ALS cases. Other secondary sources of information were used in order to ensure complete case ascertainment. ALS diagnosis was based on El Escorial criteria. Although all patients with motor neuron disease were enrolled in the follow-up, only probable and definite cases are included in the study.During the study period, 221 cases of ALS were found (120 men and 101 women), corresponding to a mean annual crude incidence rate of 2.5/100,000 population (95% CI 2.2 to 2.9). The rate was higher for men (2.9) than for women (2.3), and increased with age to a peak in the 75 to 79 age group among men and to the 70 to 74 age group among women.Comparing these data to those of epidemiologic studies with a similar prospective design, the incidence rates are similar, despite the large differences in terms of genetics, environment, and socioeconomic background. This finding points to diffuse environmental or genetic factors rather than to a specific exogenous toxin in the pathogenesis of ALS.

A delta and C fibre function has been investigated in 18 patients affected by neuropathies associated with monoclonal gammopathies. Warm, cold and heat pain thresholds have been determined by means of a Somedic Thermotest, operating on the Peltier principle, in a room at constant temperature. Our results indicate that: 1) there is an involvement of A delta and C fibres in these neuropathies that might be difficult to quantitate only on clinical grounds 2) in IgG and IgA patients, all affected by a subclinical or mild axonal neuropathy, small fibres of both types are always involved 3) in IgM anti-MAG positive severe cases, A delta fibre involvement, suggestive of demyelination is evident, while in one anti-MAG negative patient only C fibres are damaged, probably reflecting a different pathogenetic mechanism.

Objective: The authors evaluated the incidence and long-term prognostic factors of Guillain-Barre syndrome (GBS) in a prospective, population-based study. Methods: Patients with GBS diagnosed according to National Institute of Neurological and Communicative Disorders and Stroke criteria in the 2-year period 1995 to 1996 in two Italian regions were prospectively followed up for 2 years after onset of GBS. Results: A total of 120 patients were found, corresponding to a crude annual incidence rate of 1.36/100,000 population (95% CI, 1.13 to 1.63). A total of 7 (5.8%) patients, all but one with axonal or mixed EMG pattern, died acutely within 30 days from the onset of the disease. Acute mortality was due to respiratory involvement and intensive care unit complications. In multivariate analysis, a worse 2-year outcome (Hughes score ≥2) was related to a higher Hughes grade at nadir, axonal or mixed EMG, age ≥50 years, and absence of respiratory infections preceding GBS. The persistence of disability 2 years after the acute phase was related to axonal involvement and a worse status at nadir. Conclusions: After adjustment to US population, the incidence rates for GBS from different countries showed no significant differences. Both acute mortality and long-term disability in GBS seem to be related to an axonal involvement and a Hughes grade ≥2 at nadir.