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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.

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.