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Biomedical subjects

P Soliveri

Publications and source records attributed to P Soliveri.

At least 37 records · Page 2Linked to original sources

Sensitivity to vecuronium after botulinum toxin administration.

When used to treat focal dystonias, botulinum toxin may cause a transient impairment of neuromuscular transmission in muscles distant from those injected. These systemic effects are not clinically evident, but should not be ignored when patients are exposed to other drugs or conditions that also impair neuromuscular transmission. A patient is described who underwent general anesthesia twice during treatment with botulinum toxin for a long history of blepharospasm. On both occasions, the neuromuscular block produced by vecuronium (0.05 mg kg-1) was monitored in the abductor digiti minimi muscle. Compared with that observed in 24 individuals who were free from neuromuscular problems, the patient's sensitivity to vecuronium was low 90 days after the seventh treatment with toxin and normal 8 days after the ninth. The possibility is considered that repeated treatments with the toxin may cause continuous remodeling of neuromuscular junctions and may cause the patient to develop some tolerance to the action of neuromuscular blockers.

Action Potentials↗

Psychiatric symptoms do not correlate with cognitive decline, motor symptoms, or CAG repeat length in Huntington's disease.

OBJECTIVES: To investigate the hypothesis that psychiatric disturbances in Huntington's disease are related to degree of cognitive or motor compromise and to determine correlations between CAG repeat length within the gene for Huntington's disease and disease severity. DESIGN: Consecutive series of patients with Huntington's disease. SETTING: Neurological specialty hospital. PATIENTS: Seventeen men and 12 women from 24 families. MAIN OUTCOME MEASURES: The Hamilton Psychiatric and Anxiety Rating Scales and Brief Psychiatric Rating Scale were used to assess psychiatric disturbances; Folstein's Quantified Neurological Examination to evaluate motor status; and the Mini-Mental State Examination, Raven Progressive Matrices), Phonemic Verbal Fluency Test, Short Tale Test, Visual Search Test, and Benton's Visual Orientation Line Test to evaluate cognitive function. The length of the CAG repeat sequence in the Huntington's gene was determined by quantitative polymerase chain reaction. RESULTS: Cognitive test scores correlated significantly with each other; of these, results of the Visual Search and Short Tale tests correlated significantly with the Folstein's Quantified Neurological Examination score (P = .05 and P = .03, respectively). Results of the Folstein's Quantified Neurological Examination also correlated with the illness duration and the length of the CAG repeat. Although psychiatric scores correlated significantly among themselves (P < .01), neither cognitive compromise, motor deterioration, nor CAG length were related to the extent of psychiatric compromise. Patients who were depressed when they were examined tended to have a history of psychiatric disorders. CONCLUSIONS: The lack of correlation between disease severity and psychiatric disturbances indicates that psychiatric disorders progress nonlinearly, possibly because of differential degeneration of the striatal-cortical circuits; the possibility that psychiatric disorders are prevalent in certain families with a member who has Huntington's disease is being further investigated. The lack of correlation between CAG length and cognitive and psychiatric variables needs further investigation.

Adult↗

Progression of motor and cognitive impairment in Parkinson's disease.

We performed a longitudinal study (mean follow-up 86.7 months) to evaluate motor and mental deterioration in patients with Parkinson's disease. Of the original 91 patients, only 61 could be re-examined 7 years later and 11 of these had become demented (PD-Dems). PD-Dems were older with worse motor and, obviously, cognitive performance than non-demented parkinsonian patients (PDs). A global cognitive decay index (DI) was calculated for each patient. Based on this, non-demented PDs were further split into 38 stable parkinsonian patients (S-PDs) with DI-30% to +30%, and 10 deteriorated but non-demented parkinsonian patients (D-PDs) with a DI worse than -30% (as had PD-Dems). D-PDs were older and had greater motor impairment than S-PDs but did not differ from PD-Dems on these measures. D-PDs and PD-Dems deteriorated especially in attention, visuospatial and executive ability tests. Ageing seems to be the main predictive factor for mental deterioration.

Adult↗

Maternally inherited hearing loss, ataxia and myoclonus associated with a novel point mutation in mitochondrial tRNASer(UCN) gene.

We report on a new maternally-inherited syndrome characterized by a combination of sensorineural hearing loss, ataxia and myoclonus in a large kindred from Sicily. Hearing loss was the most widespread and sometimes the only symptom found in family members. Sequence analysis of the mitochondrial DNA regions encompassing the tRNA genes revealed the presence of a heteroplasmic insertion at nucleotide position 7472. The insertion adds a seventh cytosine to a six-cytosine run that is part of the mitochondrial tRNASer(UCN) gene. Conformational analysis showed that this mutation is likely to alter the structure of the T psi C loop in the tRNASer(UCN) clover leaf secondary structure. Moreover, the degree of heteroplasmy in blood and muscle was correlated with the clinical phenotype, and homoplasmic mutant hybrids showed decreased complex I activity, low oxygen consumption and high lactic acid output, indicating faulty oxidative phosphorylation. Finally, mutation was absent in 381 unrelated maternal lineages, suggesting specific segregation with the disease. We propose that the C7472 insertion-mutation is pathogenic, and etiologically related to hearing loss and other symptoms that define a novel maternally-inherited clinical entity.

Ataxia↗

Differing patterns of psychiatric impairment in Alzheimer and demented parkinsonian patients.

Psychiatric symptoms were investigated and compared in 95 patients with Alzheimer type dementia (DAT) and in 39 patients with Parkinson's disease with dementia (PD-D). The diagnosis of the dementia and psychiatric disorders was based on DSM III R criteria; dementia stage was assessed using the Clinical Dementia Rating Scale (CDR). PD-D were significantly older than DAT patients. Delirium was more frequent in the advanced stages of both PD-D and DAT, being mainly of the hypoactive type in PD-D and the hyperactive type in DAT. Delusions and hallucinations predominated in the early CDR stages of both illnesses and did not differ between groups; the same was true for depression. The results revealed different psychopathological profiles in DAT and PD-D patients.

Aged↗

Cognitive and motor performance in multiple system atrophy and Parkinson's disease compared.

Nineteen patients with multiple system atrophy (MSA) of striato-nigral degeneration type were tested to examine cognitive and motor performance. Parkinson's disease (PD) patients and healthy subjects served as controls. The MSA and PD patients showed similar cognitive dysfunction and motor impairment, performing poorly in the visuo-spatial organization, the construction tests and motor assessment tests. Movement times were much longer in MSA than PD patients. The association of nigral with putaminal damage may explain the more severe bradykinesia in MSA.

Atrophy↗

Response to L-DOPA in multiple system atrophy.

A poor response to L-DOPA in addition to parkinsonian, cerebellar, and autonomic signs is commonly regarded as indicative of clinical multiple system atrophy (MSA). We compared the motor response to a single oral administration of 250 mg L-DOPA/25 mg carbidopa in eight MSA patients and eight Parkinson's disease (PD) patients with the "on-off" phenomenon, evaluating L-DOPA peripheral pharmacokinetics. Motor response was consistently good in all PD patients, but only four MSA patients had a (moderate) response. Pharmacokinetic parameters did not differ between the groups. The varying extent of putaminal damage could be responsible for the differing motor response to L-DOPA in MSA patients.

Adult↗

Effect of practice on performance of a skilled motor task in patients with Parkinson's disease.

Parkinson's disease leads to a breakdown in the execution of highly practised, skilled movements such as walking and handwriting. The improved execution of skilled movements with practice can be understood as a process of schema learning, the determining of the relevant parameters of the specific movement. The ability of patients with Parkinson's disease and age matched normal control subjects to improve their performance, with practice, on a skilled motor task, doing up buttons, was assessed. The task was assessed on its own and with simultaneous foot tapping. Both groups showed an initial improvement in the task on its own and deterioration in performance when buttoning with foot tapping. The amount of interference, however, decreased with practice, particularly in the patients with a 2 Hz tapping rate. The results suggest that patients with Parkinson's disease are capable of schema learning but require more practice than control subjects to achieve comparable levels of performance. This may be a reflection of the fundamental motor dysfunction of the disease rather than a specific learning deficit.

Adult↗

Early-onset Parkinson's disease.

The study was conducted on 120 patients (76 men and 44 women) affected by idiopathic Parkinson's disease (IPD) responsive to L-dopa and observed for many years. Sixty had clinical onset between the ages of 20-40, representing 10.2% of our PD population; in the others the symptoms began after the 40th birthday. The two groups were matched for sex and length of illness. In all patients a diagnosis of IPD depended on history and clinical and neuroradiological findings. Clinical, pharmacological, evolutive, and epidemiological data were collected on all patients. Thirty-six patients from each group performed motor dexterity tests (reaction time to expected and unexpected stimuli) and cognitive tests (Wechsler Adult Intelligence Scale. Benton, Short tale, and Zazzo's speed and accuracy test). To assess the prevalence of dementia and the severity of psychiatric side effects of L-dopa administration, the 60 patients with early-onset PD were compared with 134 consecutive unselected PD patients. Five percent of early-onset PD patients had a family history of the disorder. Our study showed that early-onset PD does not differ fundamentally from the late-onset form except that the former is characterized by a more rapid establishment of the full-blown parkinsonian clinical picture and deterioration of the therapeutic efficacy of L-dopa, with an earlier appearance of side effects. The results of our neuropsychological investigations suggest that early-onset PD may be a "pure" form of extrapyramidal compromise with exclusively motor manifestations.

Adult↗

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↗

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↗

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↗

Lisuride in Parkinson's disease. 4-year follow-up.

Lisuride at a mean daily dose of 3 mg was given to 48 patients with idiopathic Parkinson's disease. Twenty received lisuride alone (Group A) and 36 received lisuride + L-Dopa + peripheral decarboxylase inhibitors (Group B). Dropouts were due primarily to lack of efficacy in Group A patients and to mental side effects in Group B patients. The patients who remained in the study for the full 4 years showed distinct improvement, which was maintained. Group A patients did not have the on-off phenomenon or abnormal involuntary movements.

Adult↗

Lisuride in de novo parkinsonian patients: a four-year follow-up.

Lisuride at a mean daily dose of 3.2 mg was given to 15 untreated idiopathic Parkinson's disease patients. There were 10 dropouts, due mainly to inefficacy in the first months of therapy. The parkinsonian pattern in the patients who remained in the study for the full 4 years showed distinct improvement, which was maintained for less than 2 years. The patients did not develop "on-off" phenomena or abnormal involuntary movements during follow-up.

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↗