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Biomedical subjects

F Le Pira

Publications and source records attributed to F Le Pira.

16 recordsLinked to original sources

Cognitive dysfunction in patients with relapsing-remitting multiple sclerosis.

Cognitive dysfunction is considered one of the clinical markers of multiple sclerosis (MS). However, in the literature there are inconsistent reports on the prevalence of cognitive dysfunction, and separate data for the relapsing-remitting (RR) type of the disease are not always presented. In this study, we submitted 461 RRMS patients to a battery of neuropsychological tests to investigate their impairment in various cognitive domains. As a consequence of the exclusion criteria, the sample is not fully representative of the entire population of RRMS patients. In this selected sample, when only the eight scores of a core battery (Mental Deterioration Battery) were considered (with respective cutoffs), it emerged that 31% of the patients were affected by some degree of cognitive deficit. In particular, 15% had mild, 11.2% moderate and 4.8% had severe impairment. Information processing speed was the most frequently impaired area, followed by memory. When two other tests (SDMT and MCST) were added and cognitive domains were considered, it emerged that 39.3% of the patients were impaired in two or more domains. When four subgroups were obtained by means of cluster analysis and then compared, it emerged that information processing speed and memory deficits differentiated the still cognitively unimpaired from the mildly impaired MS patients. Significant associations were found between cognitive and clinical characteristics. However, due to the large sample size, clinically irrelevant relationships may also have emerged. Even with the limitations imposed by the sample selection and the possible underestimation of the prevalence and severity of cognitive dysfunction, these results seem to provide further evidence that information processing speed deficit may be an early and important marker of cognitive impairment in MS patients.

Adult↗

Memory disturbances in migraine with and without aura: a strategy problem?

Cognitive defects in migraine have been reported by several authors. These findings however, are controversial. In this study we carried out an investigation on 14 patients with migraine with aura and 16 with migraine without aura according to the International Headache Society criteria. They were submitted to a comprehensive battery of neuropsychological tests. The patients were compared with a control group not significantly different as to age, sex and education. Migraine subjects showed impaired neuropsychological performances only on some cognitive tests. Both groups of patients did worse than the control group on visuo-spatial memory tasks, while only migraineurs without aura showed significantly impaired verbal memory performances. The memory defects, both on visuo-spatial and on verbal cognitive tasks, could depend on an impaired recall mechanism. These memory difficulties seem related to strategically and organizationally defective aspects of learning.

Adult↗

Identification of SCA2 mutation in cases of spinocerebellar ataxia with no family history in mid-eastern Sicily.

Differential diagnosis between autosomal dominant cerebellar ataxia type I (ADCA I) and idiopathic cerebellar ataxia type P (IDCA-P) is very difficult given only clinical and neuroradiological data. The only certain distinctive characteristic is the presence or absence of family history. We observed 7 patients with late-onset cerebellar ataxia associated with other non-cerebellar signs and without a family history of the disease in which clinical signs were comparable to symptoms found in SCA2. The neuroradiological study showed olivopontocerebellar atrophy in all patients and the presence of hyperintensity of the transverse pontine fibers in 6 patients (85. 6%); molecular analysis showed SCA2 mutations in 2 patients. We also report the case of a patient who was initially considered as IDCA-P but who was later correctly identified as SCA2 with an atypical family history (false IDCA-P), after a genetic mutation was found and following an interview with the mother. Our data suggest that spinocerebellar ataxia syndrome should be defined as idiopathic not only after having excluded the possible symptomatic causes but also in the absence of family history, after having excluded the presence of genetic mutation. We believe that family history, in late-onset spinocerebellar ataxia, cannot be considered as the differential criterion among hereditary (ADCA-I) and non-hereditary (IDCA-P) forms; molecular analysis is required for a correct diagnosis.

Adult↗

Clinical and molecular analysis of 11 Sicilian SCA2 families: influence of gender on age at onset.

Autosomal dominant cerebellar ataxias (ADCAs) are a complex group of slowly progressive neurodegenerative disorders characterized by gait and stance ataxia, dysarthria and other symptoms of nervous system involvement. ADCA type I is the commonest form and is genetically heterogeneous; several loci have been identified. Spinocerebellar ataxia type 2 (SCA2) has been mapped to chromosome 12, with expanded cytosine-adenine-guanine (CAG) repeats being identified as the mutational cause of the disease. We investigated 15 families, all originating from mid-eastern Sicily, with ADCA type I; molecular studies performed in 12 families showed the SCA2 mutation to be present in 11 of them (91.6%) - the highest occurrence so far reported in the literature. The CAG repeat of the affected alleles varied between 34 and 44 repeats. Age at onset and repeat length revealed an inverse correlation. Mean age at onset was 37.32 +/- 16. 74 years, and occurred earlier in males than in females. There were no differences in mean CAG repeat units between the sexes. However, a higher instability of CAG repeats was observed for paternal transmission than for maternal transmission. Age at onset and anticipation were not related to parental transmission. Our data suggest that in SCA2 an unknown sex-linked factor may play a role in the modulation of toxic effects of the polyglutamine tract.

Adolescent↗

Neurobehavioral syndrome induced by H2-receptor blocker withdrawal: possible role of prolactin.

Cimetidine and ranitine are histamine H2-receptor blockers widely used for the treatment of gastric hypersecretion and duodenal pathologies. They are known to induce hyperprolactinemia in humans. Forty-six patients treated with cimetidine or ranitidine who were exhibiting a neurobehavioral syndrome after withdrawal of the drugs were selected. This syndrome was associated with a drop in plasma prolactin levels. The symptoms of this syndrome were greatly improved by restoration of treatment with the same drugs and reappeared when the treatment was again suspended. This syndrome was inhibited in 36 patients by administration of domperidone (30 mg/day), a drug inducing hyperprolactinemia without crossing the blood-brain barrier, as compared with 10 control patients treated with placebo. These results suggest that the drop in prolactin levels occurring when cimetidine and ranitidine are suspended may contribute to the development of this syndrome. Also, the withdrawal of H2-receptor blockers could be included among the possible causes of some neurotic syndromes.

Adult↗

Cervical epidural abscess: serial MRI study.

Cervical epidural abscess (CEA) at first often goes unrecognized and it is to be suspected in patients suffering by spinal ache, root pain, neurological deficit and fever. Staphylococcus aureus is the most frequent etiologic agent. At present MRI is the study of choice for the diagnosis of CEA. We suggest a serial MRI study in close time in at first doubtful cases of CEA. When the responsible organism is not identified and there are also compressive signs of the spinal cord, the treatment of choice is surgical decompression followed by selective antibiotic therapy. The duration of antibiotic therapy may be established not only by clinical judgement, but also by serial MRI study, which gives direct information about disease evolution.

Abscess↗

Hysterical neurosis of the conversion type: therapeutic activity of neuroleptics with different hyperprolactinemic potency.

To show a possible correlation between drug-induced hyperprolactinemia and improvement of hysterical neurosis of the conversion type, we followed 18 patients monitoring clinical somatic and psychic symptoms as well as serum prolactin levels. Six patients were treated with haloperidol and 12 with sulpiride; after 2 months sulpiride was administered at half the daily dose to 6 patients previously treated with the same drug. Clinical evaluation showed that sulpiride treatment led to a greater improvement compared to the haloperidol group. The different effectiveness of treatment could be explained by the different hyperprolactinemic potency. The therapeutic efficacy of neuroleptics suggests also that hyperactivity of dopaminergic transmission is involved in the pathophysiology of hysterical neurotic symptoms.

Adult↗

Magnetic resonance imaging in cervical spinal cord compression.

In patients with cervical spondylotic myelopathy MRI sometimes shows increased signal intensity zones on the T2-weighted images. It has been suggested that these findings carry prognostic significance. We studied 56 subjects with cervical spinal cord compression. Twelve patients showed an increased signal intensity (21.4%) and a prevalence of narrowing of the AP-diameter (62% vs 24%). Furthermore, in this group, there was evidence of a longer mean duration of the symptoms and, in most of the patients, of more serious clinical conditions. The importance of these predisposing factors remains, however, to be clarified since they are also present in some patients without the increased signal intensity.

Adult↗

[Bio-imaging for the study of organic changes in the C.N.S.: comparison between computerized tomography and magnetic resonance].

Several comparative studies have been carried out in order to evaluate the possible diagnostic superiority of Computed Tomography (C.T.) vs. Magnetic Resonance Imaging (M.R.I.) in different diseases of the Central Nervous System. The present investigation is aimed at critically revising this problem also including the study of patients clinical data as a criterion of choice. A sample of 69 subjects was examined. It was seen that in demyelinative as well as in chronic cerebrovascular diseases M.R.I. should be preferred because of its higher sensitivity and specificity. Concerning specifically chronic cerebrovascular diseases, clinical data can have a more significant role in justifying the choice of M.R.I. C.T., and M.R.I. should be considered as complementary rather than competitive in studying cerebral tumors and spinal cord pathology; the planning of surgical operations, needing a complete evaluation of data, which in these cases are very variable, requires the use of both methods.

Central Nervous System Diseases↗

Lifetime prevalence of Bell's palsy in rural Bolivia: a door-to-door survey.

We carried out a door-to-door survey in rural areas of the Cordillera Province, Bolivia, to determine the prevalence of the most common neurological diseases in a sample of about 10,000 inhabitants. A team of non-doctor health workers administered a standard screening instrument for neurological diseases, a slightly modified version of the World Health Organization protocol. All subjects found positive at the screening phase underwent a complete neurological examination. On screening, we found 1,130 positive subjects, of whom 909 were aged 15 years and above. After the neurological examination, we found 52 cases who had experienced Bell's palsy during their life in the population aged 15 years and above. The lifetime prevalence on November 1, 1994 was 11.1/1,000 (95% confidence interval 7.8-14.5) for the population aged 15 years and above. The prevalence was higher in women than in men (13.7 and 8.7/1,000, respectively) and increased with age, reaching a peak in the group aged 65 years or more (31.7/1,000). Only 3 cases (5.8%) had received medical therapy.

Adolescent↗

Leukoaraiosis and lacunar infarcts in ischemic stroke: role of age and vascular risk factors.

To assess the role that age and some vascular risk factors play in the pathogenesis of leukoaraiosis and lacunar infarcts in patients with ischemic stroke, we examined 71 consecutive patients who had undergone magnetic resonance imaging because of clinical suspicion of stroke. We collected data regarding hypertension, diabetes mellitus, cardiac diseases, hypercholesterolemia, and hematocrit, and compared patients with lacunar infarcts to those with cortical or subcortical nonlacunar lesions. Patients were then assigned to one of two age groups, Group A (< or = 66 years), or Group B (> 66 years). We found a significant correlation between the presence and severity of leukoaraiosis and the presence of lacunar infarcts in both groups. In Group A, however, lacunar infarcts were correlated to hematocrit, while in Group B they were correlated to a trend to hypertension. Leukoaraiosis was correlated to hypertension only in Group A. Although we noted a strong correlation between leukoaraiosis and lacunar infarcts suggesting a common small-vessel disease, our data indicate that different pathogenetic mechanisms are involved. We suggest that patients be grouped according to age in future studies on the role that risk factors play in the pathogenesis of leukoaraiosis and lacunar infarcts.

Age Factors↗