The frequency of clinical variants of Guillain-Barré syndrome in Ferrara, Italy.

As the available diagnostic criteria (National Institute of Neurological and Communicative Disorders and Stroke, NINCDS) for Guillain-Barré syndrome (GBS) do not permit inclusion of clinical variants (CV) of GBS, there are few data on their occurrence and few reports of the overall incidence of the disease. A population-based study in the local health district of Ferrara, Italy in 1981-1993 selected cases fulfilling both NINCDS criteria (NINCDS GBS cases) and CV. The incidence of CV was 0.35 per 100,000 person-years (95% CI: 0.15-0.68), 0.32 when age-adjusted to the Italian population. No difference was found between CV and NINCDS GBS for male/female ratio, mean age at onset, elevated CSF protein content, seasonal pattern, or mean time delay from first neurological symptom to maximal severity. A higher frequency of antecedent infections for CV and more frequent serious disease at the nadir time for NINCDS GBS were found. A complete recovery was more frequent for CV than NINCDS GBS, but no difference was found regarding good outcome (defined by a satisfactory recovery and resumption of normal functional life). Since most findings were similar for NINCDS GBS and CV cases, they may have similar underlying pathological mechanisms. When diagnostic criteria for GBS include CV, the overall disease incidence in the Ferrara district increases from 1.87 to 2.21 cases per 100,000 person-years (the contribution of CV to the overall incidence of GBS is 15.7%). The currently available diagnostic criteria for GBS, although useful for field studies, may be too restrictive as they can entail the loss of about 15% of cases.

A prospective study on the predictive value of CSF oligoclonal bands and MRI in acute isolated neurological syndromes for subsequent progression to multiple sclerosis.

A prospective study in patients with a clinical acute isolated brainstem or spinal cord disorder was undertaken. The aim was to evaluate the predictive value of IgG intrathecal synthesis (through the detection of oligoclonal bands in CSF) and MRI lesions at presentation, for the subsequent progression to multiple sclerosis. Forty four patients took part in this study: 22 had a brainstem disorder and 22 a spinal cord disorder. After a mean period of 26 (SD 22) months, 30 patients (68.2%) developed clinically definite multiple sclerosis. The remaining 14 patients were followed up for more than seven years. Twenty six (59.1%) patients had oligoclonal bands in CSF, with a sensitivity of 80.0%, specificity of 85.7%, and a predictive value of 92.2%. Magnetic resonance imaging showed disseminated white matter lesions in 22 patients (50.0%), with a sensitivity of 60.0%, a specificity of 71.4%, and a predictive value of 81.7%. The difference between patients with multiple sclerosis and patients without the disease was statistically significant for the findings of an IgG intrathecal synthesis (P < 0.001). It was only borderline for the MRI findings (P = 0.052). Thus the detection of an intrathecal synthesis at presentation seemed to be a better prognostic indicator of the progression to multiple sclerosis in patients affected by acute isolated brainstem or spinal cord syndromes.