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Biomedical subjects

F Girotti

Publications and source records attributed to F Girotti.

At least 55 records · Page 3Linked to original sources

Abnormal MRI signal in the rigid form of Huntington's disease.

Eighteen patients with Huntington's disease (HD) were examined with MRI. Eleven had the common hyperkinetic form, seven had the rigid variant. All seven patients with rigid HD had increased signal intensity in the neostriatum in intermediate and T2-weighted images. Only one hyperkinetic HD had similar findings. In all the other cases, signal abnormalities were questionable or absent. Histological differences that account for differences in signal intensity may therefore lie in the caudate nucleus and putamen. Age of onset may be important, since the rigid patients were younger. Follow up studies may help in understanding these signal differences.

Adolescent↗

Terguride in the treatment of Parkinson disease: preliminary experience.

Terguride, a partial DA-agonist with both dopaminergic and antidopaminergic properties, was tested in 11 PD patients in the "decompensated" phase of the disease, characterized by the presence of dyskinesias and motor fluctuations. Combined treatment of these patients with 1 mg/day of terguride and stabilized doses of levodopa reduced the severity and frequency of dyskinesias and motor fluctuations along with a slight but significant improvement of parkinsonian clinical picture. The "modulatory" effect of terguride on DA receptors, in this experimental conditions, is discussed.

Dyskinesia, Drug-Induced↗

Amyotrophic lateral sclerosis: thyroid and prolactin hormone changes in thyrotropin-releasing hormone therapy.

13 patients with amyotrophic lateral sclerosis (ALS) were treated with intravenous infusion of thyrotropin-releasing hormone (TRH). In 6 patients 2 mg/day of TRH was i.v. given over 2 hours for 10 days. In 7 others 2 mg/day of TRH was continuously infused by means of a pump. An increase of thyroid hormones related to the duration of the treatment was observed. A surprising finding was the onset of prolactin (PRL) response to growth hormone releasing hormone (GHRH), previously absent.

Amyotrophic Lateral Sclerosis↗

Prolactin response to growth hormone-releasing hormone during chronic thyrotropin-releasing hormone infusion in the treatment of amyotrophic lateral sclerosis.

In six patients suffering from amyotrophic lateral sclerosis we evaluated changes of T4, T3, TSH, PRL, and GH during treatment by continuous iv infusion of TRH for at least 15 days. No clinical improvement was detected. A significant rise of thyroid hormone levels was observed, as well as an upward trend of basal TSH levels and no change of basal PRL and GH levels. TRH acute test-induced TSH and PRL responses became blunted. Treatment provoked also the onset of a responsiveness of PRL to GHRH. The reduced TSH and PRL responses to acute TRH test during treatment could be explained by a down-regulation of TRH pituitary receptors. On the contrary, the onset of PRL responsiveness to GHRH is at present without a satisfactory explanation.

Amyotrophic Lateral Sclerosis↗

Variation of therapeutic response in Parkinson's disease: a retrospective study.

A retrospective study of variations of therapeutic response (dyskinesia and on-off phenomenon) in Parkinson's disease was conducted on 278 patients treated with levodopa for at least 6 months and hospitalized at National Neurological Institute "C. Besta" of Milan between 1974 and 1984. Variations of therapeutic response (TRV) were present in 105 of 278 patients; in this group, age at illness onset was significantly lower, while duration of levodopa treatment and also duration of illness were longer than in the group of patients without TRV. Multiple logistic regression analysis showed that the most important variables were age at illness onset and duration of treatment, but they were only modestly predictive. Other factors connected with progression of disease must also contribute to the TRV.

Age Factors↗

Olivopontocerebellar atrophy: MR diagnosis and relationship to multisystem atrophy.

Clinical diagnosis of olivopontocerebellar atrophy (OPCA) must be confirmed by radiologic demonstration of atrophy in an appropriate distribution. OPCA may be associated with degeneration of other systems in multisystem atrophy (MSA). The authors report 23 cases of OPCA, eight of which were associated with MSA. Atrophy involved the cerebellum, pons, and middle cerebellar peduncles in all cases. On intermediate and T2-weighted magnetic resonance (MR) images, abnormal signal intensity was always observed in the transverse pontine fibers, middle cerebellar peduncles, and cerebellum, structures known from pathologic study to degenerate in OPCA. Pyramidal tracts and superior cerebellar peduncles stood out because of their normal signal intensity. Of the eight patients with MSA, four also had variable abnormal signal intensities in the putamen. The authors believe that the combination of atrophy and abnormal signal intensity in the appropriate distribution strongly supports the diagnosis of OPCA. In some cases, MR imaging may demonstrate involvement of different systems, thus confirming the diagnosis of MSA.

Adult↗

Mortality associated with early and late levodopa therapy initiation in Parkinson's disease.

We evaluated the possible influence of early levodopa treatment on the mortality of Parkinson's disease (PD). One hundred forty-five consecutive parkinsonian patients initiated treatment with levodopa between 1970 and 1983. Ninety-eight of those started levodopa therapy 2 or more years after symptom onset, while 47 received levodopa within the 1st 2 years of the disease. At the end of follow-up, in December 1985, 49 patients had died. Mortality was 2.5 times higher among patients who delayed initiation of levodopa therapy 2 or more years than among those who initiated the therapy earlier. Age and disease severity were the most significant predictors of survival after initiation of levodopa treatment. The risk of death was 10% higher every year of age increase and was 2 and 4 times higher, respectively, for patients at Hoehn and Yahr stages II and III than for patients at Hoehn and Yahr stage I. When we controlled for the effect of age and disease severity on mortality, the cumulative death probability was no longer significantly higher among patients who delayed levodopa treatment than among patients treated within 2 years from disease onset. As far as mortality is concerned, the results show that the time of levodopa treatment initiation during PD has no influence and the drug can be introduced as soon as indicated by the severity of the disease progression.

Female↗

Myoclonic epilepsy of Lafora and arylsulphatase A deficiency in the same patient.

Very low levels of arylsulphatase A were found in a young patient with the clinical features of Lafora disease, confirmed by muscle biopsy. The deficiency was shown both in leukocytes and cultured fibroblasts. A cerebroside sulphate loading showed that 93% of [14C]cerebroside sulphate taken up by skin fibroblasts from the patient remained unmetabolized after a 24h pulse, ruling out pseudo-arylsulphatase A deficiency. In the healthy parents and siblings of the patient, biochemical data suggested heterozygosity for arylsulphatase A deficiency. The apparent co-inheritance of arylsulphatase A deficiency and Lafora disease in this family might be the consequence of genetic linkage between the two genes.

Adolescent↗

MRI in internal carotid artery dissection.

We report two cases of internal carotid artery dissection in which MRI unequivocally demonstrated the intramural hematoma. We propose that, whenever the clinical presentation or the angiogram suggest arterial dissection, MRI be done to confirm the diagnosis.

Carotid Artery Diseases↗

A case of Refsum disease with atypical clinical picture in family members.

A typical case of Refsum disease with high phytanic acid plasma levels is described. Two siblings showed some features but not the entire clinical spectrum of the disease. The unusual condition of the patient's father, a presumed heterozygotic carrier with characteristic bone abnormalities and a delayed onset retinopathy, is discussed.

Adult↗

Relationship between motor and cognitive disorders in Huntington's disease.

Akinesia and mental decline appear to be more appropriate criteria than hyperkinesia for the evaluation of the stage and progression of Huntington's disease (HD). In order to establish the relationship between motor and cognitive impairment in the disease, 20 non-demented HD patients were compared with 44 control subjects with respect to motor and cognitive performance. HD patients were significantly impaired in almost all cognitive functions in comparison with controls. Reaction time (RT) and movement time (MT) were considerably slower in HD patients when compared with controls and with patients with parkinsonism. Hyperkinesias did not correlate with cognitive impairment, but there was a good correlation between RT, MT and cognitive functions. Therefore, it seems that akinesia evaluated by RT and MT is an important sign in HD and proceeds at the same rate as mental decay.

Adult↗

Dementia and cognitive impairment in Parkinson's disease.

The frequency of dementia, the clinical characteristics and the pattern of cognitive impairment were studied in 147 unselected Parkinsonian patients. Twenty-one patients (14.28%) were judged to be demented. They had a more severe and widespread cognitive deficit although they were affected particularly in those tests that already discriminated Parkinsonian patients from controls. A direct comparison of Parkinsonian dementia with other types of dementia is needed to validate the concept of subcortical dementia.

Aged↗

Role of motor performance in cognitive processes of parkinsonian patients.

Sixty-seven parkinsonian patients and 44 control subjects were tested for cognitive function and motor performance, using reaction times and movement times. Parkinsonian subjects did significantly worse in tests that required visuoperceptual and perceptual motor abilities, such as Object Assembly, Block Design, and Zazzo's test. Analysis of covariance of test scores showed that Block Design and Object Assembly scores were not significantly different between patients and controls when adjusted for reaction and movement times. Also, reaction and movement times were more delayed in parkinsonian patients than in controls. These results stress the main role of motor dysfunction in visuospatial and perceptual motor impairment.

Cognition↗

Motor and cognitive performances of parkinsonian patients in the on and off phases of the disease.

Twenty-one Parkinsonian patients were tested in on and off phases during chronic levodopa therapy for cognitive function, affective status, and evaluation of motor performance with reaction and movement times. A worsening of mood was observed from the on to the off phase. No variation in cognitive performance was observed from the on to the off phase in spite of evident motor changes. Mood changes during on-off variations may reflect involvement of mesocortical and mesolimbic dopaminergic systems.

Affect↗

Double-blind study of oral gamma-vinyl GABA in the treatment of dystonia.

Six patients with different forms of dystonia were treated with gamma-vinyl GABA, a specific enzyme-activated inhibitor of GABA-transaminase, in a double-blind, placebo-controlled crossover study. gamma-Vinyl GABA therapy, 2 g daily for 2 weeks, was compared with placebo by weekly assessments. There were no consistent changes in three evaluation scores. Agents that augment CNS GABA are unlikely to benefit patients with generalized dystonia.

Administration, Oral↗

Cognitive impairment in Parkinson's disease.

Neuropsychologic studies on Parkinson's disease have shown an impairment of specific cognitive functions and a greater evidence of dementia. Cognitive alterations are related to the gravity of some peculiar motor symptoms of the disease and show a major role of motor disturbances in intellectual disorders. The concept of bradyphrenia and subcortical dementia still seems to be suitable to define the cognitive disorders of Parkinson's disease.

Cognition Disorders↗

Problems arising during chronic treatment with L-dopa for Parkinson's disease: changes in response to treatment.

Some aspects of the problems of long-term L-dopa treatment syndrome are reviewed, with special attention to the changes in response to treatment with dopaminergic agents, specifically end-of-dose deterioration, the on-off phenomenon and hyperkinesia. The various hypotheses for interpreting these are presented, with particular stress on changes in the function of DA-ergic receptors. It is concluded that the on-off phenomenon is probably related to changes in plasma L-dopa levels and to decreased stores of intraneural dopamine.

Corpus Striatum↗