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Biomedical subjects

F Girotti

Publications and source records attributed to F Girotti.

At least 37 records · Page 2Linked to original sources

Magnetic resonance imaging in progressive supranuclear palsy and other parkinsonian disorders.

High field intensity MRI may demonstrate signal abnormalities consistent with deposits of iron or other paramagnetic substances in several extrapyramidal disorders. Hallervorden-Spatz disease was the only disorder widely known to have iron deposits in the pallidum, that are now easily demonstrated in vivo by MRI. However, lower field intensity MRI may also demonstrate characteristic findings. In progressive supranuclear palsy, definite atrophy of the midbrain and of the region around the third ventricle is seen in slightly more than half of the cases. Minimal signal abnormalities are sometimes seen in the periaqueductal region, but MRI studies remain of little help in establishing the diagnosis of the disease. Asymmetric atrophy in the parietal regions is seen in corticobasal degeneration, as expected from pathological studies. Minimal alterations may be seen in the substantia nigra in Parkinson's disease. The most interesting MRI findings are observed in multiple system atrophies. Variable abnormal signal intensities, depending on the field intensity, are visible in the putamen in striatonigral degeneration and in Shy-Drager syndrome; in this latter condition the abnormalities are due to its striatonigral degeneration component. Atrophy of the pons, middle cerebellar peduncles, and cerebellum, and signal abnormalities in a characteristic distribution are visible in olivopontocerebellar atrophy. A combination of these posterior fossa abnormalities and putaminal alterations may confirm the involvement of the cerebellar and extrapyramidal systems in multiple system atrophies.

Atrophy↗

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↗

Sneddon's syndrome: clinical and immunohistochemical findings.

Results of immunological studies on skin biopsies of 5 patients with Sneddon's syndrome are reported. Also studied were coagulation factors and autoantibodies believed to play a role in this syndrome. Hemostasis was normal except for a mild increase of fibrinogen in one subject; lupus anticoagulant (LAC) and anticardiolipin antibodies were negative in all. The skin biopsies ruled out systemic vasculitis and vasculitis in association with connective tissue diseases. Sneddon's syndrome is a peculiar clinicopathological condition, probably with several etiologies, but is distinct from primary antiphospholipid syndrome.

Adult↗

Clinical and magnetic resonance features of the classic and akinetic-rigid variants of Huntington's disease.

We studied 32 patients with confirmed Huntington's disease (HD); six (mean age, 31.7 years) had the akinetic-rigid form and 26 (mean age, 46.1 years) had the classic hyperkinetic form. Clinical examination included a count of abnormal involuntary movements, motor self-sufficiency evaluation by the Physical Disability Rating Scale, cognitive function assessment by the Mini-Mental State examination, and a verbal fluency test. Magnetic resonance imaging permitted measurement of bicaudate diameter, a sensitive indicator of caudate atrophy in HD. Patients with the akinetic-rigid form of HD were younger and had earlier disease onset than those with the classic form of HD. All patients with akinetic-rigid HD (group 1) had striatal hyperintensity on T2-weighted magnetic resonance images; seven patients with classic HD (group 2) had a similar abnormality. Groups 1 and 2 were in fact similar in all other respects, except that the number of abnormal involuntary movements was greater in group 2. Groups 1 and 2 together had significantly younger age at onset, lower Mini-Mental State Examination score, more severe motor disability, worse verbal fluency test result, and greater bicaudate diameter than the 19 patients with classic HD without magnetic resonance signal abnormality (group 3) and appear to be a uniform population, distinct from group 3. The abnormalities on magnetic resonance images indicated greater striatal damage in groups 1 and 2, which could be the neuroanatomic substrate of their greater motor and cognitive compromise.

Adult↗

Adult onset myoclonic Huntington's disease.

A patient with adult onset Huntington's disease (HD) and prominent action myoclonus is described. Neither epileptiform activity nor electroencephalography (EEG) correlates of the movements was found. Unlike the case with most (nonmyoclonic) HD patients, centro-parietal components of somatosensory evoked potentials (SEPs) were well defined and a clear V2 response was found. Treatment with valproic acid greatly reduced myoclonus suggesting that the gamma-aminobutyric acid (GABA) system might be involved in the pathophysiology of myoclonus in HD.

Adult↗

Multiple system atrophy. Clinical and MR observations on 42 cases.

Probable or possible multiple system atrophy (MSA) was diagnosed on strict clinical criteria in 42 patients: 20 with combined parkinsonism and cerebellar ataxia, 9 with striatonigral degeneration (SND) and 13 with olivopontocerebellar atrophy (OPCA). All patients were then studied with 0.5 and/or 1.5 Tesla magnetic resonance (MR) units. MR imaged putaminal abnormalities in all 9 patients with SND and posterior fossa abnormalities consistent with OPCA in all 13 patients with this diagnosis. Of the 20 patients with parkinsonism and cerebellar involvement, classified as probable MSA, 7 presented putaminal abnormalities only, 3 abnormalities consistent with OPCA only and 10 a combination of both. These findings show strong MRI support for the clinical diagnosis of MSA.

Adult↗

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↗

Respiratory chain and mitochondrial DNA in muscle and brain in Parkinson's disease patients.

There are several reports of a defect of complex I in the substantia nigra (SN) of Parkinson's disease (PD) patients. To evaluate whether this is specific to dopaminergic neurons or the phenotypically relevant consequence of a widespread failure of the mitochondrial oxidative phosphorylation (OXPHOS) system, we measured respiratory enzyme activities in muscle homogenates from 16 PD patients and eight age-matched controls, and in muscle isolated mitochondria of six PD patients and six age-matched controls. We found no difference between the PD and control groups. In addition, we detected, by polymerase chain reaction, the mitochondrial DNA (mtDNA) "common deletion" (CD) in muscle specimens of 14 of 17 PD patients, but we obtained similar results in age-matched controls. In both groups, the amount of CD-specific deleted (delta) mtDNA ranged from 0.0% to 0.1%. Our data suggest that PD cannot be attributed to a multisystem decline of mitochondrial OXPHOS, and that lesions of muscle mtDNA in PD are likely due to normal aging. However, there was a remarkable accumulation of delta mtDNA in the SN of a PD patient and an age-matched control, suggesting that the SN is exquisitely sensitive to age-dependent damage of the mitochondrial genome.

Adult↗

MRI in ataxias.

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Ataxia↗

Chronic treatment with L-threonine in amyotrophic lateral sclerosis: a pilot study.

Thirty patients suffering from amyotrophic lateral sclerosis were included in an open therapeutical trial. They were randomized to receive either L-threonine (Thr), a precursor of the inhibitory amino acid glycine, or vitamin B or carnitine. Thirteen patients (9 patients on Thr and 4 control subjects) completed the 1-year trial. No statistical differences were observed between the treated group and the control patients in the decline of the clinical assessment score. Nevertheless, Thr-treated patients complained less frequently of respiratory failure than the control group despite bulbar involvement being more common in the Thr group at entry.

Administration, Oral↗

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↗

Interobserver reliability between neurologists in training of Parkinson's disease rating scales. A multicenter study.

A multicenter study has been conducted to determine the interobserver reproducibility of four of the most frequently used rating scales for Parkinson's disease: the Columbia University Rating Scale (CURS) and the Webster Rating Scale (WRS), both for assessing clinical signs; the Northwestern University Disability Scale (NUDS); and the Hoehn and Yahr staging. Four resident neurologists, inexperienced in the use of the four scales, independently examined 48 parkinsonian patients. The extent to which their assessments agreed was determined by calculating the Cohen k index after the scores had been recodified. The physicians' scores agreed substantially for the CURS and the Hoehn and Yahr scale, while those for the NUDS and the WRS agreed only moderately. Analysis of individual item scores within the scales suggests improvements that would offer greater interobserver consistency.

Disability Evaluation↗

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↗