Door-to-door prevalence survey of epilepsy in three Sicilian municipalities.

A door-to-door prevalence survey of epilepsy was conducted in 3 Sicilian municipalities, as of November 1, 1987. In phase 1, the screening by questionnaire of 24,496 eligible subjects (participation = 92%) identified 544 suspected to have epilepsy. In phase 2, neurological evaluation of the 544 subjects yielded 111 with epilepsy. Of the 111 subjects, 103 (93%) had been previously diagnosed, 68 (61%) were taking antiepileptic medication, and 81 (73%) had active epilepsy. Referring to the 81 subjects with active epilepsy, the seizure type was generalized in 60 (74%), partial in 19 (23%) and undetermined in 2 (3%). The prevalence of active epilepsy (per 1,000 population) was 3.3 overall, 3.5 for men and 3.2 for women. The age-specific patterns for active epilepsy differed by sex, with higher figures for men at younger ages (5-19 years) and older ages (50-99 years). The age-specific prevalence figures for active epilepsy were lower than those from previous Italian surveys.

Parkinson disease survival: a population-based study.

OBJECTIVE: To evaluate whether the survival of patients with Parkinson disease (PD) is shorter than that of the general population. DESIGN: Survival was investigated in a cohort of patients with PD previously identified during a population-based prevalence study (prevalence day, November 1, 1987, reference follow-up date, October 31, 1995). The survival of patients with PD was compared with that of a control sample randomly selected from the same population (2 controls for each case, matched for age, sex, and study municipality). The causes of death in the 2 groups were also compared. Both univariate and multivariate survival analyses were performed to investigate the association with disease-related variables. SETTING: A door-to-door 2-phase prevalence survey performed in 3 Sicilian municipalities. PATIENTS: Fifty-nine patients with PD and 118 controls. RESULTS: Patients with PD showed a high risk of death (relative risk, 2.3; 95% confidence interval, 1.60-3.39). Greater age at November 1, 1987, high Hoehn-Yahr score, and lack of levodopa therapy were associated with a lower survival on univariate analysis. Multivariate analysis confirmed the association between shorter survival among patients with PD and greater age on November 1, 1987. One-way analysis of variance indicated a different effect of levodopa therapy according to age. Multivariate analysis did not confirm this finding. Pneumonia was the cause of death most frequently associated with PD. CONCLUSION: This study indicates that patients with PD have a shorter survival time than the general population.

The efficacy of valproate-lamotrigine comedication in refractory complex partial seizures: evidence for a pharmacodynamic interaction.

PURPOSE: To assess the comparative therapeutic value of valproate (VPA), lamotrigine (LTG), and their combination in patients with complex partial seizures resistant to other established antiepileptic drugs (AEDs). METHODS: After a 3-month prospective baseline, 20 adults with refractory complex partial seizures not exposed previously to VPA and LTG were scheduled to receive three consecutive add-on treatments with VPA, LTG, or their combination, according to an open, response-conditional, crossover design. Each period consisted of a 6- to 12-week dose optimization followed by 3-month evaluation at stabilized serum drug levels. Only patients not responding to one phase proceeded to the next. RESULTS: A >50% reduction in seizure frequency was observed in three of 20 patients given VPA and in four of 17 patients given LTG. Of the remaining 13 patients, four became seizure free, and an additional four experienced seizure reductions of 62-78% when VPA and LTG were given in combination. Mild tremor was observed in three patients receiving VPA and in all patients taking the VPA--LTG combination. In patients responding to combination therapy, optimized dosages and peak serum levels of both VPA and LTG were lower than those during separate administration. CONCLUSIONS: A considerable proportion of patients who failed to respond to VPA and LTG separately improved when the two drugs were combined, although serum levels of both agents were lower during combination therapy. Despite methodologic limitations in the nonrandomized treatment sequence, these findings suggest that VPA and LTG exhibit a favorable pharmacodynamic interaction in patients with refractory partial epilepsy. The dosage of both drugs, however, may need to be reduced to minimize the risk of intolerable side effects.

Stroke incidence and survival in three Sicilian municipalities. Sicilian Neuro-Epidemiologic Study (SNES) Group.

We investigated the incidence of first-ever stroke in three Sicilian municipalities over the years 1984-1987. Case ascertainment involved two approaches: a door-to-door two-phase prevalence survey and a study of death certificates. Only first-ever strokes occurring within the study time interval were included, and diagnoses were based on specified criteria. We found 138 subjects who had experienced a first stroke over 73 488 person-years; the average incidence rate (new cases per 1000 population per year) was 1.9 overall, 1.7 in men, and 2.1 in women. Incidence increased steeply with age and was similar in men and women. Incidence was similar in the three study municipalities. The overall case-fatality rate was 35% at 30 days and 22% at one week. Survival after stroke was similar in men and women but better in younger compared to older stroke patients. Comparisons with previous studies suggest the absence of major geographic variations in stroke incidence within Italy.

Stroke in the young in Sicily: prevalence and clinical features.

Few epidemiological reports focus on the prevalence of stroke in the young population. As part of a neuroepidemiological survey on the total population of three Sicilian municipalities, we assessed the prevalence of stroke in the young, describing the clinical features of each patient. We screened 14,832 subjects younger than 40 years with a door-to-door method and a two-phase study design. Phase 1 consisted of a questionnaire and a brief neurological examination; phase 2 was an extensive evaluation performed by a neurologist. On the prevalence day (November 1, 1987), we ascertained six cases of stroke (prevalence rate, 40.5/100,000). Three were hemorrhagic, one ischemic, and two of uncertain type. Recovery was good in five patients; only one needed aids for walking. Stroke is uncommon in the young, but its prevalence rapidly increases in an age-related fashion. Hemorrhagic lesions are probably more represented in this age-group. Survivors tend to show good motor recovery.

Incidence and lifetime prevalence of Bell's palsy in two Sicilian municipalities. Sicilian Neuro-Epidemiologic Study (SNES) Group.

INTRODUCTION: In a door-to-door two-phase survey of common neurologic diseases conducted in two Sicilian municipalities, we investigated the incidence and the lifetime prevalence of Bell's palsy (BP). MATERIAL & METHODS: During phase I, we administered a screening instrument for facial palsy to 11,901 adult persons. During phase 2, study neurologists using specified diagnostic criteria evaluated those subjects who screened positive. RESULTS: We found 73 subjects who had experienced BP during their life. The lifetime prevalence as of November 1, 1987, was 642.8 cases per 100,000 population age 15 years and above. The prevalence increased with age and was similar in men and women. Eighteen episodes of BP occurred in the three years preceding the prevalence day. The average annual incidence rate was 52.8 new episodes per 100,000 population age 15 years and above. Incidence increased with age. CONCLUSION: Comparison with other incidence studies suggests some geographic variability.

Prevalence of lumbosacral radiculopathy in two Sicilian municipalities. Sicilian Neuro-Epidemiologic Study (SNES) Group.

OBJECTIVES: As part of a door-to-door survey of neurologic diseases, we investigated the prevalence of lumbosacral radiculopathy (LR) in two Sicilian municipalities (N = 14,540, as of November 1, 1987). MATERIAL & METHODS: During phase 1, we administered a brief screening instrument to subjects in the study population. In phase 2, study neurologists using specified diagnostic criteria investigated those subjects who screened positive. Our case finding was restricted to subjects who manifested symptoms of LR in the six months preceding the prevalence day. RESULTS: We found 143 subjects affected by LR (112 definite, 31 possible) yielding a crude prevalence of 9.8 cases per 1,000 population. Age-specific prevalence was generally higher in men; it increased to a peak at age 50-59 years and decreased steadily thereafter. At the peak, prevalence was higher in factory workers, housewives, and clerks compared to workers in other occupations. CONCLUSIONS: LR is a common neurologic disease in the general population.

Prevalence of epilepsy. A door-to-door survey in the Sicilian community of Riposto.

In a door-to-door survey of common neurological disorders in Sicily (SNES Project), we administered a screening symptoms questionnaire and a brief neurological examination to detect epileptic patients. All of the subjects effectively resident in the community of Riposto on 1 November 1987 (prevalence day) were investigated (n = 9956). The subjects with a positive questionnaire or a previous diagnosis of epilepsy were extensively examined by a neurologist and then definitively classified for epilepsy by a panel of senior neurologists. The crude prevalence of active and non-active epilepsy was 3.21/1000; the prevalence of active epilepsy alone was 2.71/1000. Of the 27 active cases, sixteen were affected by generalized tonic-clonic seizures, four by absences and four by complex partial attacks. All except two were being treated with antiepileptic drugs, most with phenobarbital. Our prevalence rate is lower than that reported by most authors, but similar to the rates in other Sicilian communities.

Occupation, education, and Parkinson's disease: a case-control study in an Italian population.

Current epidemiologic data on the association between occupational exposures and Parkinson's disease (PD) are inconsistent. In a case-control study, we investigated the associations between occupation and PD and between education and PD. The cases (n = 62) were those identified in a prevalence survey (door-to-door, two-phase) of three Sicilian municipalities, as a November 1, 1987. We then randomly selected from the general population two controls for each case, matched for age ( +/- year), sex, and municipality (n = 124). Information on current and past occupations and education for cases and controls was obtained during the survey. Subjects who worked for most of their lives as farmers were not at increased risk of PD (OR = 0.6; 95% CI = 0.3-1.3). Neither were subjects who worked for most of their lives in other occupations (e.g., housewives, fishermen, factory workers, salesmen, craftsmen, clerks). PD was not associated with low education. Our findings suggest that farming, as a broad occupational category, does not play a major role in the causation of PD.

Prevalence of cervical spondylotic radiculopathy: a door-to-door survey in a Sicilian municipality.

INTRODUCTION: Because of the limited information on cervical spondylotic radiculopathy, we conducted a door-to-door two-phase survey in a Sicilian municipality. MATERIAL AND METHODS: We first screened for cervical spondylotic radiculopathy among the inhabitants of the municipality: (N = 7653, as of the prevalence day, November 1, 1987). Study neurologists then investigated those subjects suspected to have had a cervical spondylotic radiculopathy. Diagnoses were bases on specified criteria. RESULTS: We found 27 subjects affected by CSR (17 definite, 10 possible). Prevalence (cases per 1000 population) was 3.5 in the total population; it increased to a peak at age 50-59 years, and decreased thereafter. The age-specific prevalence was consistently higher in women. CONCLUSIONS: Comparison with other prevalence studies shows similar age-specific patterns, but different magnitudes, which may partly reflect methodologic differences across studies.

Prevalence of stroke: a door-to-door survey in three Sicilian municipalities. Sicilian Neuroepidemiologic Study (SNES) Group.

As part of a door-to-door survey, we screened for stroke among the inhabitants of three Sicilian municipalities (n = 24,496 as of November 1, 1987). Neurologists then investigated those subjects suspected to have had a stroke. Diagnoses of first-ever strokes were based on specified criteria and were reviewed by an adjudication panel. We found 189 subjects who had experienced at least one completed stroke (180 definite, 9 possible); 15 strokes were hemorrhagic, 71 ischemic, and 103 uncertain. The prevalence (cases/100,000) was 771.6 in the total population and 1,893.6 in those aged 40 years or over. The prevalence increased steeply with age, was higher in men between 60 and 79 years, but was higher in women thereafter. Age-specific figures were similar in the three study municipalities. Although all first-ever strokes had been previously diagnosed, 40% of the subjects had not been hospitalized for this condition.

Sleep features in Tourette's syndrome, neuroacanthocytosis and Huntington's chorea.

Twenty-one patients affected by extrapyramidal disorders were polygraphically recorded during spontaneous nocturnal sleep for two consecutive nights to assess their sleep and movement patterns. The patients (pts) sample included: Gilles de La Tourette syndrome (TS, nine pts), neuroacanthocytosis (NA, six pts) and Hungtington's chorea (HC, six pts). Sleep recording included C3/A2, 01/A2, ROC/LOC, submental EMG, EKG, nasal airflow thoracoabdominal respirogram, bilateral anterior tibialis and other EMGs, in relation to the individual distribution of the abnormal movements. According to our observations, abnormal movements always decreased but never ceased completely during sleep. Sleep efficiency (SE) was nearly always poor with a high percentage of wakefulness after sleep onset (WASO) and increased number of arousals. REM sleep was often reduced and in some cases (3 TS pts) incompletely defined as far as its microstructural aspects. Slow wave sleep (SWS) was reduced in HC, normal in NA, and increased in all TS patients with the exception of the two adult subjects more severely affected, while the percentage of stage 2 was not affected. Spindling was increased in NA, HC and in the two most severely affected adult TS patients.

Epileptic seizures in subjects previously affected by disorders of arousal.

Six patients (3M, 3F, mean age 17.3 yrs) presenting different types of evolution from disorders of arousal to epilepsy are described. All subjects during their childhood had been diagnosed in a sleep center as affected by sleep-walking (three cases) and night terrors (the other three). Successively they developed nocturnal events different from those previously exhibited and consisting of clear epileptic seizures, generalized tonic-clonic in one case and complex partial in five cases. Nocturnal monitoring allowed recognition of clear interictal paroxysmal activity in three patients, while ictal events were recorded in the remaining three. Anticonvulsant treatment (carbamazepine in five patients, phenytoin in one patient) led to the resolution of the ictal events in all cases. The fact that both disorders of arousal and epilepsy are strictly related to sleep and often share common features such as age range of onset and precipitating factors, suggests the existence of common functional substrates identifiable in constitutional maturative and biologic factors. The possible occurrence of seizures in subjects exhibiting parasomnia during their childhood has to be considered in patients with familial history of epilepsy and in all doubtful cases.

Sleep spindles in the initial stages of the vegetative state.

The aim of this study was to look for the presence of spindling in the different stages of remission of the vegetative state to underline all possible correlations with lesional sites, severity of coma and final outcome. The nocturnal polygraphic recordings from 30 patients were examined: 20 (15M, 5F, mean age 31.7 years, range 16-41) had originally suffered a traumatic brain injury, 10 (4M, 6F, mean age 40.5 years, range 24-48) had hypoxic brain lesions. Evidence of spindling, always reduced in density and duration, was found in 44% of these patients, prevalently in the traumatic patients (53.3% versus 30% of hypoxic patients). No subjects in the full stage 0, 0-1 of the vegetative state (apallic syndrome) presented spindling. No significant correlation was found between spindling and the following parameters: gender, the time between the onset of coma and the polygraphic recording, or the site of the lesion.

Effects of the antidepressant drug viloxazine on oxcarbazepine and its hydroxylated metabolites in patients with epilepsy.

The effects of Viloxazine (VLX, 100 mg b.i.d. for 10 days) on the steady-state plasma concentrations of Oxcarbazepine (OXC), its active metabolite 10, 11-dihydro-10-hydroxy-carbazepine (MHD) and the corresponding diol (DHD) were studied in a randomized, double-blind cross-over placebo-controlled trial in 6 epileptic patients stabilized on a fixed dosage of OXC. Administration of VLX resulted in an 11% increase in the plasma concentration of MHD (p = 0.003) associated with a 31% fall in DHD levels (p = 0.0001). Plasma concentrations of unchanged OXC were unaffected by VLX. No changes in seizure frequency nor signs of drug toxicity were observed during the study. Although VLX may inhibit the conversion of MHD to the inactive diol, the interaction is unlikely to be of clinical significance.

Ataxia, mental deterioration, epilepsy in a family with dominant enamel hypoplasia: a variant of Kohlschütter-Tönz syndrome?

We describe 3 sibs, their father, and paternal grandfather with amelogenesis imperfecta. In 2 sibs and the father the defect is associated with a neurological syndrome which has a wide range of phenotypic variability. The proposita has ataxia, EEG abnormalities, moderate dementia, and enamel hypoplasia. This case and the affected relatives are discussed in relation to Kohlschütter-Tönz syndrome and neuroectodermal diseases. The syndrome described here, characterized by the association of a genetic enamel defect and neurological impairment, may be of considerable interest in advancing genetic and clinical knowledge on ectodermal tissues and their development.

Serotoninergic agents in the treatment of Gilles de la Tourette's syndrome.

A preliminary trial with fluoxetine, a 5-HT reuptake inhibitor, was carried out on two young male patients (21 and 32 years old) affected by Gilles de la Tourette syndrome. They both underwent a complete neurological evaluation also including neuroradiological, neurophysiological and neuropsychological assessment. Both patients had already been treated with benzodiazepines and amitriptyline; the older one was also given haloperidol and chlorimipramine with definite, but short-lasting improvement. During hospitalization a therapeutic trial with fluoxetine (20 mg/day in the younger patient and 40 mg/day in the older) in association with chlorimipramine (75 mg/day) was initiated, leading to a significant reduction (at least 50%) of abnormal movements and obsessive-compulsive behaviour. The older patient had no side effects while the 21 year old subject complained of insomnia, urinary retention and anorexia; despite the objective improvement, these side effects led us to modify the therapy after the first month. The favourable action of serotoninergic agents on TS symptoms supports the hypothesis that the multiple tics of the syndrome are motor compulsions.

Prevalence of essential tremor: a door-to-door survey in Terrasini, Sicily. Sicilian Neuro-Epidemiologic Study Group.

As part of a door-to-door neuroepidemiologic survey, we investigated the frequency and distribution of essential tremor (ET) in a Sicilian municipality. During phase 1, we administered a screening instrument for tremor to 7,653 persons residing in Terrasini (Palermo province). During phase 2, neurologists evaluated those subjects who had screened positive. The diagnoses, based on specified clinical criteria, were reviewed to increase reliability across neurologists. We found 31 subjects affected by ET (17 men, 14 women); 11 patients (35.5%) reported a familial aggregation. The prevalence of ET as of November 1, 1987, was 405.1 per 100,000 for the total population, and 1,074.9 per 100,000 for those 40 years old or older. The prevalence increased with advancing age for both sexes and was slightly but consistently higher in men. Comparison with other studies suggests striking geographic variation, which may reflect genetic differences.

Attrition and use of proxy respondents and auxiliary information in the Sicilian Neuroepidemiologic Study.

Two-phase prevalence surveys with screening (phase 1) and examination (phase 2) are useful for some chronic diseases. Attrition, which may bias estimates, occurs in either phase because some eligible subjects die before contact, some refuse to cooperate, some are incapacitated, and some are unreachable. This investigation relates to a survey of neurologic diseases conducted in three municipalities of Sicily (prevalence date, November 1, 1987) and considers the attrition experienced and the use of proxy respondents in phase 1 and auxiliary information in phase 2 to offset, in part, this attrition. Regarding case finding, the salvage effort was more productive for decreased and incapacitated subjects. The age, sex, and household size of the subject were related to phase 1 attrition, but only age was related for all four attrition groups--deceased, refusing, incapacitated, and unreachable subjects. On the basis of information from proxy respondents, the educational levels of refusing and unreachable subjects were compared with those of subjects screened directly. Refusing subjects were less educated, and unreachable subjects were more educated. The proxy respondent performance, as indicated by "don't know" responses, was better with screening items concerning facial paralysis and mouth drooping (and not limb sensory abnormalities or impaired consciousness), better with younger subjects, and worse with refusing or incapacitated subjects.