Temporal variation in Guillain-Barré syndrome (GBS) warrants monitoring in certain situations. This study sought to describe a public-health-based GBS surveillance service in Spain and conduct pilot surveillance in the period 1998-1999. Neurologists from 11 hospitals countrywide, serving a population of 3.9 million, reported all patients, ages 20 years or over, admitted to hospital with suspected GBS. Cases that did not belong to the designated hospital catchment area or failed to fulfill diagnostic criteria after follow- up were excluded. Reported monthly incidence was compared against predicted incidence obtained from retrospective data (1985-1997) using a reported method based on 97.5% percentile values. Alarm thresholds for 2000 onwards were obtained by applying the same method to the updated 1985-1999 series. During the 2-year period, 98 GBS cases were reported, yielding an overall age-adjusted incidence of 1.26 per 100 000 population, with a breakdown by sex of 1.83 for males and 0.76 for females. Monthly incidence remained below or was similar to the corresponding threshold limit value. Seasonality with highest incidence in winter was more pronounced in the elderly. Preceding events, mainly respiratory infections, were identified in 71% of patients. Pilot two-year GBS surveillance in Spain resulted neither in alarm nor in preventive measures. Adult GBS incidence in Spain might be monitored by a surveillance system set up at short notice when a possible threat is perceived. A monthly incidence of over 3 per 100 000 person-years in the population aged 20 years or older would exceed threshold values.
Retrospective demographic information and hospital record data were collected for 337 patients resident in Spain who had validated Guillain-Barré syndrome (GBS) diagnoses and clinical onset during the period 1985–1997 and had been admitted to 11 centres, covering a population of 3.9 million. The European age-adjusted GBS incidence per 100,000 for 1985–1997 among the population aged 20 and over was 0.85, with a breakdown of 1.14 in men and 0.58 in women. Incidence increased with age and time, with occasional rises that mimicked outbreaks and occurred at irregular 2- to 4-year intervals, mainly in winter. Spatial variation was modest. Respiratory and gastrointestinal infections respectively constituted 49.3 and 19.3% of recorded preceding events. The 97.5% intercentile limit, obtained from the 1985–1997 monthly incidences using predictions from a Poisson model, was proposed as the threshold value for pilot epidemiological surveillance of GBS in 1998–1999.
In most of the cases previously described, the defect on complex II was suggested by low activity of succinate cytochrome C reductase (SCCR). The clinical pattern of the previous 10 cases is heterogeneous and may be limited to one particular tissue or be of a more general nature. We report a 22‐year‐old‐woman, daughter of consanguineous parents, with generalized muscle weakness, easy fatigability and benign course, who showed a decrease of SCCR activity in mitochondria of muscle fibers. Free carnitine (FC) concentration was decreased in muscle as well. The muscle biopsy showed a mild variation in fiber size, with fiber type I predominance, subsarcolemmal oxidative DPNH accumulations, excess of neutral lipids and abnormally large mitochondria with paracrystalline inclusions. A possible inheritance pattern is discussed. Coenzyme Q10 therapy in this patient induced a significant increase of global MRC index score and a decrease of the turns‐mean amplitude ratio in the automatic analysis of the EMG.
Brain single photon emission computed tomography (SPECT) findings are described in four adult patients with the transient syndrome of headache with neurological deficits and cerebrospinal fluid (CSF) pleocytosis. Focal deficits consisted of right-sided hemisensory changes with or without motor dysphasia or dysarthric speech (n = 4) and confusional episodes (n = 1). All patients had a CSF pleocytosis (with a mean of 309 cells/mm3 on the first spinal tap; range 75 to 590) and an elevated total protein (mean 130.5 mg/dL; range 70 to 193). The EEG showed excessive focal slowing (n = 2). A technetium Tc 99m hexamethyl propylenamine oxime (HMPAO) brain SPECT was performed during a symptom-free period, within 8 and 25 days after the onset of symptoms (n = 4). Three patients showed a decreased tracer uptake in the anterior left hemisphere, topographically consistent with the neurological deficits and EEG slowing. One patient showed no abnormalities. These findings indicate either focally impaired neuronal metabolism or hypoperfusion in regional cerebral blood flow, which could bear some relationship with the clinical features. The possibility that SPECT abnormalities may represent an epiphenomenon was also considered.
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