Biomedical subjects
Publications and source records attributed to A Reggio.
We describe a case of genetic Creutzfeldt-Jakob disease (CJD) with deafness at the onset. We report clinical features, 14-3-3 protein positivity, electroencephalography and brain stem auditory evoked potential abnormalities, and high signal on magnetic resonance imaging in basal ganglia and temporal cortex. Similarities with CJD Heidenhain variant are discussed.
OBJECTIVES: To evaluate central motor conduction to lower limbs in spinocerebellar ataxia type 2 (SCA2). METHODS: Transcranial magnetic stimulation was performed to study the corticospinal tracts of 18 patients with SCA2. RESULTS: Central motor conduction time (CMCT) to lower limbs and thresholds were abnormal in 8 patients (44%); CMCT and thresholds were significantly correlated with disease duration and disability. CONCLUSIONS: Corticospinal tract involvement is more frequent than previously reported in SCA2. Prolonged CMCT and increased threshold should not be used to differentiate between various type of autosomal dominant cerebellar ataxia. Similar to that reported in Friedreich's ataxia, we suggest that examining central motor conduction to the lower limbs may assist in evaluating the progressive steps of neurodegeneration in SCA2.
OBJECTIVE: To compare the monthly distribution of multiple sclerosis (MS) patients' births with that of the Sicilian population. BACKGROUND: Studies on the association between season of birth and risk of MS are scanty and controversial. DESIGN AND METHODS: Archives of the Institute of Neuropsychiatry of the Universities of Palermo and Catania were searched up to 1995 for definite MS patients (McDonald & Halliday criteria). The monthly distribution of MS patients' births (N= 965) was compared with that of the Sicilian population (N= 5,608,307). RESULTS: The distribution of births among MS patients compared with the general population was not different when tested by the chi2 statistic (P> 0.25). The Hewitt's non-parametric test for seasonality showed an excess of births between June and November among MS patients (P=0.004). CONCLUSION: A different pattern of MS patients' births is observed in Sicily and in Northern countries.
We report three patients with isolated unilateral hypoglossal nerve palsy who experienced an excellent outcome. In two patients no cause was found. Our study seems to confirm that the occurrence of benign and idiopathic isolated unilateral palsy of the hypoglossal nerve is more frequent than previously reported. We would like to stress that neuroimaging studies remain mandatory in order to exclude other common causes, such as tumour and spontaneous or traumatic vascular lesions, in which a specific treatment is necessary.
BACKGROUND AND PURPOSE: We carried out a door-to-door survey in rural areas of the Cordillera Province, Santa Cruz Department, Bolivia. A cluster sample of 10 124 inhabitants was selected, and 9955 subjects were screened. The aim was to determine the prevalence of the most common neurological diseases (epilepsy, stroke, parkinsonism, and peripheral neuropathy) in this population. METHODS: We used a modified version of the World Health Organization screening instrument. On screening we found that 1130 subjects tested positive, and 1027 underwent a complete neurological examination. According to the World Health Organization guidelines, we defined stroke as "rapidly developing clinical signs of focal (or global) disturbance of cerebral functions, lasting more than 24 hours or leading to death, with no apparent cause other than that of vascular origin." We considered only first stroke and excluded a possible stroke. RESULTS: We found 16 subjects (cases) who had experienced 1 complete stroke on prevalent day (November 1, 1994). The crude prevalence of stroke was 174/100 000 (322/100 000 age-adjusted to the world standard population) and 663/100 000 in subjects aged >/=35 years. Prevalence was >2-fold higher in men than in women (247/100 000 and 99/100 000, respectively) and increased rapidly with age. Seven cases were hospitalized and received specific treatment. CONCLUSIONS: Our crude prevalence is lower compared with rates from developed countries, probably because of a high case fatality rate. Our findings are comparable with those reported from other surveys carried out in rural developing countries.
Interleukin (IL)-13 is a T-cell derived cytokine closely related to IL-4 that possesses powerful antiinflammatory properties. In this study we have evaluated the blood levels of IL-13 in patients with multiple sclerosis (MS), either with relapsing remitting or secondary chronic progressive (CP) course of the disease, and have also examined the effect of treatment with interferon (IFN)-beta 1b given on alternate days for 10 days both on the serum levels of IL-13 and on the ex vivo secretory capacity of mononuclear cells from MS patients. IL-13 was not detectable in the circulation of MS patients regardless of whether RR MS patients with stable or active disease or those suffering from secondary CP MS were studied. Moreover, circulating levels of IL-13 were not induced by treatment with IFN- beta1b, that was neither capable of modifying the ex vivo IL-13 secretory capacity of peripheral blood mononuclear cells. These data neither anticipate a role for endogenous IL-13 in down-regulating immunoinflammation during MS attacks nor suggest that IFN-betalb treatment exerts its favourable effects on the course of RR MS by augmenting the secretion of this antiinflammatory cytokine.
OBJECTIVE: To carry out a door-to-door survey in rural areas of the Cordillera Province, Santa Cruz Department, Bolivia, to determine the prevalence of neurologic diseases (epilepsy, stroke, parkinsonism, and peripheral neuropathy) in a sample of approximately 10,000 inhabitants. METHODS: A team of nondoctor health workers administered a standard screening instrument for neurologic diseases-a slightly modified version of the World Health Organization protocol. All subjects found positive during the screening underwent a neurologic examination. RESULTS: On screening, the authors found 1,130 positive subjects, of whom 1,027 were then investigated by neurologists. On the basis of the definition proposed by the International League Against Epilepsy, we detected 124 epileptic patients (prevalence, 12.3/1,000), 112 of whom had active epilepsy (prevalence, 11.1/1,000) on the prevalence day (November 1, 1994). Peak age-specific prevalence occurred in the 15 to 24-year age group (20.4/1,000). Sex-specific prevalence was higher in women (13.1/1,000) than men (11.4/1,000). Eighty-nine patients (71.8%) underwent a standard EEG recording. Considering both EEG and clinical data, partial seizures were the most common type (53.2%) based on the classification of the International League Against Epilepsy. The mean age at onset was 20.7 years for partial seizures and 13.6 years for generalized seizures. Only 10.5% of patients had received specific treatment for more than 2 months of their life. CONCLUSION: This report on epilepsy prevalence in Bolivia confirms that epilepsy is a major health problem in rural areas of developing countries.
OBJECTIVES: To evaluate clinical and MRI effects of natural interferon beta treatment in both relapsing-remitting (RR) and secondary-progressive (SP) multiple sclerosis patients. MATERIAL AND METHODS: A double-blind, randomized trial of natural interferon beta (nIFN-beta) in 58 ambulatory patients with RR and 40 with SP multiple sclerosis. Forty-nine patients (29 RR and 20 SP) were treated with intramuscular nIFN-beta6 MIU three times a week for 24 months and 49 control patients were treated with placebo. RESULTS: Primary clinical endpoints were differences in exacerbation rates and proportion of patients remaining exacerbation-free. There were no significant baseline differences between the treated and placebo groups. In the treated RR group a significant reduction in exacerbation rate, an increase in the probability of remaining exacerbation-free, and an improvement in mean EDSS were found at 24 months. MRI activity and total lesion burden were significantly reduced in treated RR patients. In the SP group, nIFN-beta produced a significant reduction in EDSS score, a significant reduction in active lesion number, a marginally significant favourable difference in total lesion burden but no significant effect on the number of gadolinium-enhancing lesions. Side effects were transient and mild in treated patients. CONCLUSIONS: These observations confirm that nIFN-beta is a promising and well-tolerated treatment for either RR or SP MS patients.
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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.
Our aim was to evaluate the utility and effectiveness of a study protocol for neurological involvement in neurological disorders. We studied a sample of 786 patients with neurological disorders followed by the Neurological Department of Catania University and applied to them a four-step diagnostic protocol. Fifty-six per cent of the sample showed urological functional abnormalities; only 78 patients (9.9%) presented with an organic urological disease. Among the vesicosphincteric dysfunctions, bladder hyperreflexia was the most common pattern with remarkable differences between diseases. The present study demonstrated the utility of a standardized urological protocol in the screening and detection of neurological involvement in neurological diseases. Our protocol showed good specificity and reasonable low costs.
The serum levels of TGF-beta1, measured by solid-phase ELISA, were determined to be significantly augmented in patients with both relapsing remitting (RR) and secondary chronic progressive (CP) MS compared with sex- and age-matched healthy controls. Moreover, in RR MS patients, the blood levels of the cytokine were further augmented either during relapses or, in a rapid but reversible fashion, by s.c. injection with 8 million International Units (MIU) IFN-beta1b. Because TGF-beta1 possesses multiple anti-inflammatory activities, we hypothesize that the increase in its circulating levels in RR and CP MS patients might represent an endogenous anti-inflammatory mechanism aimed at counteracting ongoing immunoinflammatory events, and that IFN-beta may further potentiate this natural defensive apparatus.
Cognitive impairment is a frequent feature in multiple sclerosis patients. To assess its evolution in comparison with clinical and neuroradiological evolution, we followed up 57 multiple sclerosis patients over a 3-year period. During this time EDSS deteriorated significantly but not the MRI lesional load nor the cognitive test performance; nevertheless both at the beginning and at the end of the follow-up neuropsychological results showed a significant correlation with both EDSS and lesional load. No clinical or paraclinical features could reliably predict neuropsychological evolution.Copyright Lippincott-Raven Publishers
A door-to-door survey was carried out in rural areas of the Cordillera province, Santa Cruz Department, Bolivia. A cluster sample of 10,124 inhabitants was selected. The aim was to determine the prevalence of the most common neurological diseases (epilepsy, stroke, parkinsonism and peripheral neuropathy) in this population using a modification of the World Health Organization screening instrument. 1,130 subjects screened positive and were then investigated by neurologists. In this paper we describe the background and methods of the survey and the characteristics of the 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.
Interleukin (IL)-1 receptor antagonist (IL-1ra) is a naturally occurring inhibitor of IL-1 which binds to IL-1 receptors without generating immunologic responses. Evidence has recently been provided that the balance between the production of IL-1 and IL-1ra might influence the course of immunoinflammatory diseases such as inflammatory bowel diseases, rheumatoid arthritis (RA) and Lyme arthritis. To assess whether endogenous IL-1ra may also have a role on the course of multiple sclerosis (MS) we presently studied the fluctuation of the serum levels of IL-1ra in patients with relapsing remitting (RR) MS either during remission or exacerbation. Moreover, to evaluate whether the beneficial effect of IFN-beta on the course of MS might also be mediated by an increased production of IL-1ra, we measured the levels of circulating IL-1ra in MS patients prior to and after 6 months of continuous treatment with natural human IFN-beta (6,000,000 IU three times a week for 6 months). Our results demonstrated that, relative to control subjects, IL-1ra serum levels are "normal' during remitting phases of RR MS but significantly elevated either during exacerbations or in response to IFN-beta treatment.
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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.