Spinal cord lesion management in Italy: a 2-year survey.

DESIGN: Prospective 2-year survey from 1 February 1997 to 31 January 1999. OBJECTIVES: To compare the route from injury to rehabilitation, and the outcome of care in a large sample of traumatic (T) and nontraumatic (NT) spinal cord lesion (SCL) patients at their 'first admission'. SETTING: T and NT SCI patients consecutively admitted to 37 SCL centres in Italy. METHOD: Data were recorded on simple, computerised, closed-question forms, which were Centrally collected and analysed. Descriptive and inferential analysis was conducted to define the characteristics and compare the T and NT populations, and to identify correlations among the variables examined: time from the event to admission (TEA); pressure sores (PS) on admission; length of stay (LoS) and destination on discharge. RESULTS: A total of 1014 SCL patients, 67.5% with a lesion of T and 32.5% of NT aetiology were analysed. The subjects in the T group were younger (median 34 versus 58 years), with higher probability of cervical involvement (OR 2.47, CI 1.8-3.4) and completeness of the lesion (OR 3.0, CI 2.3-4.0), shorter median TEA (37 versus 64 days, P<0.0001) and less frequent admission from home (3.6 versus 17.4%) compared to the NT group. TEA and PS on admission were analysed as indicators of the efficacy of the courses from injury to rehabilitation. Longer TEA was reported for people with NT aetiology, admitted to rehabilitation centre (RC), not locally resident, transferred from certain wards and to a lesser degree female subjects and those with complications on admission. PS were associated to completeness of lesion, longer TEA, admission to RC, nonlocal residence and coming from general intensive care units, or general surgery wards. Median LoS was 99 days (mean 116 and range 0-672), and was statistically shorter in the NT group (122 versus 57 median, P<0.00001). Upon discharge, bladder and bowel autonomy were, respectively, obtained in 68.1 and 64.5% of the whole population without significant difference between the T and NT groups. A total of 80.2% of patients were discharged home and the following factors: not living alone, being discharged after longer LoS, having sphincterial autonomy and no PS, were all independent predictors of outcome. CONCLUSION: There are important obstacles in the admission route to rehabilitation facilities, greater for NT, as longer TEA and more complications on admission testify. Moreover, the LoS is shorter for NT population. Our findings suggest that rehabilitation outcome could be improved through an early multidisciplinary approach and better continuity between acute and rehabilitation care, especially for the 'neglected' NT SCL patients.

Superficial siderosis of the central nervous system: a 70-year-old man with ataxia, depression and visual deficits.

BACKGROUND: Superficial siderosis of the central nervous system (SSNS) is caused by cerebral, cerebellar and spinal cord tissue deposition of hemosiderin, often related to repeated episodes of subarachnoid hemorrhage. Typical symptoms include ataxia, sensorineural deafness and dementia. METHODS AND RESULTS: An elderly patient with SSNS presenting with ataxia, depression and severe visual impairment was admitted to the Unit of Geriatrics of the University Hospital of Perugia, Italy. Late diagnosis and the association of symptoms with SSNS prevented the possible surgical treatment of the disease. CONCLUSIONS: Recognition of uncommon clinical variants may facilitate early diagnosis of SSNS and improve therapeutic results.

Neuropsychological characteristics of parkinsonian patients with lateralized motor impairment.

Parkinsonians with predominantly unilateral signs provide an interesting experimental means to evaluate if asymmetric nigro-striatal degeneration may affect neuropsychological functions. The aim of our study was to establish if the side of onset of idiopathic Parkinson's disease, right (PDR) or left (PDL), determines a selective pattern of cognitive performances. Furthermore, we verified if PDR and PDL groups show a different frequency of dementia. PDR and PDL patients with at least seven years of disease duration, matched for age, schooling, severity of extrapyramidal symptomatology and index of lateralization, were evaluated by using an extensive neuropsychological battery aimed at assessing hemispheric cognitive asymmetries. Current side of greater motor impairment was the same as the one affected at the onset of the disease. Only subtle differences in the profile of neuropsychological dysfunction emerged from the comparison of PDR and PDL subjects. Moreover, the number of parkinsonians showing dementia syndrome was the same in both groups. Our results suggest that the side of onset of motor impairment does not significantly influence the cognitive performances in PD. Subcortical anatomic and/or functional asymmetries seem to play a less important role in the intellectual functions than in motor activities.

Neuropsychological follow-up of parkinsonian patients with and without cognitive impairment.

In order to evaluate possible progression in the severity of their cognitive impairment, 34 parkinsonians with intellectual impairment were followed longitudinally for 7 years. Each patient was matched for age, sex, severity and duration of illness, and pharmacological treatment, with a parkinsonian patient without cognitive impairment. Results suggest that cognitive deficits are not static but rather there is a progression in the severity. Furthermore, patients suffering from severe dementia are more likely to die during the follow-up period. The prognosis of Parkinson's disease seems to be changed substantially by the occurrence of dementia. The natural history of parkinsonian dementia does not seem to differ from the history of other forms of dementia with a progressively disabling course leading to a complete loss of autonomy.

Neuropsychological correlates of L-deprenyl therapy in idiopathic parkinsonism.

1. Monoaminergic neurotransmitter systems are known to play an important role in neuropsychological functions and they are impaired in dementia of DAT and PD. 2. L-deprenyl is a monoamine-enhancing drug which at low doses selectively inhibits MAO-B, an enzyme whose brain activity has been reported to increase in normal aging and neurodegenerative dementing disorders. 3. The authors studied the effects of L-deprenyl, 10 mg/day, on several cognitive domains in idiopathic parkinsonians without dementia. Ten out-patients, treated with levodopa plus DDI, were tested before receiving L-deprenyl and retested six months after they had been treated with the drug. A control group of ten parkinsonian out-patients treated with only levodopa plus DDI, matched for age, educational level, severity and duration of extrapyramidal disease, was tested by the same neuropsychological battery and retested after a comparable time interval. 4. Statistically significant changes were noted in the verbal and visuospatial learning performances of PD patients treated with the combination of L-deprenyl and levodopa.

Alzheimer-type dementia and verbal memory performances: influence of selegiline therapy.

In a double blind randomized crossover trial lasting 6 months selegiline, a selective MAO-B inhibitor, was tested against placebo for activity on verbal memory performances in Alzheimer-type dementia (DAT). Verbal memory was assessed with the Rey-Auditory-Verbal Learning Test at the start of treatment, at the time scheduled for crossover (90 days) and at the end of the trial (180 days). The results suggest that selegiline possesses significant activity on some memory parameters, which seems to depend on an improvement both in information processing abilities and in learning strategies at the moment of acquisition.

L-deprenyl therapy improves verbal memory in amnesic Alzheimer patients.

Altered monoaminergic neurotransmission could play an important role in the cognitive dysfunctions typical of dementia of the Alzheimer type (DAT). DAT is not, however, a homogenous phenomenon inasmuch as two forms are distinguishable: early onset (EO) and late onset (LO). Moreover, focal patterns of neuropsychological deterioration fall into various subgroups. According to our hypothesis, DAT patients, who at the onset of the disease mainly manifest memory disorders, also represent a specific subgroup characterized by impaired cortically projecting catecholaminergic pathways. In a 6-month randomized, double-blind, cross-over study versus placebo we analysed the influence of L-deprenyl on the verbal memory of 19 amnesic EO-DAT patients. Verbal memory was assessed by means of the Rey Auditory Verbal Learning Test. The results obtained show significantly better performances for L-deprenyl treated patients in learning and long-term memory skills. We suggest that L-deprenyl, through selective inhibition of MAO-B and by increasing the activity of the catecholaminergic systems, positively influences cognitive functions and behaviour founded on memory efficiency.

[The verbal fluency test for the diagnosis of dementia]. / Il test di fluenza verbale nella diagnosi di demenza.

Two forms of verbal fluency test, phonological (FF) and semantic (FS) sets, have been administered to four groups of demented patients: 11 with Alzheimer-type dementia (DAT), 13 with multi-infarct dementia (MID), 8 with Parkinson-Dementia (P-D) and 11 with adult chronic hydrocephalus (ICA). Patients were matched for age, educational level and neuropsychological impairment pattern. Further, ten neurologically healty subjects were selected as control group. Control subjects result to be different from all other groups in both FF and FS; moreover, FF test results to be more impaired in ICA than in DAT. Furthermore, FF is more impaired than FS in P-D and ICA patients. On the basis of our results, verbal fluency tests might represent an useful instrument to differentiate demented subjects from non-demented ones and within demented groups to characterize the different neuropsychological pattern of the cortical and subcortical type of cognitive deterioration.

Selegiline in the treatment of mild to moderate Alzheimer-type dementia.

Selegiline, an inhibitor of monoamine oxidase B, was tested on patients with mild to moderate dementia of the Alzheimer type. Its efficacy and tolerability were compared with that of phosphatidylserine in a randomized, single-blind, parallel fashion. Forty patients (24 men and 16 women) entered the trial. Selegiline was administered in 10-mg tablets once daily and phosphatidylserine in 100-mg capsules twice daily, both treatments lasting three months. Drug efficacy was assessed at baseline and then each month by means of an extensive battery of neuropsychological tests. The assessment of drug safety was based on monitoring for adverse drug reactions and on routine laboratory tests performed before and after treatment. At the end of the study the selegiline group showed improvements statistically significantly superior to those obtained in the phosphatidylserine group on most of the cognitive areas examined. Furthermore, of particular interest was the discovery, found only in the selegiline group, of an increased degree of autonomy in day-to-day activities. Tolerability was good, the only side effect reported in both groups being slight or moderate nausea, which was severe enough to warrant withdrawal from treatment only in one case, a patient in the selegiline group with a history of gastroduodenitis.

Neuropsychological effects of L-deprenyl in Alzheimer's type dementia.

The monoaminergic neurotransmission defect seen in dementia of the Alzheimer type (DAT) is linked to a known increased activity of type B cerebral monoamine oxidases (MAO-Bs). The use of drugs that are able to block this abnormal activity could therefore be useful in the treatment of some cognitive deficits that characterize DAT. Twenty patients with a clinical diagnosis of DAT and with a slight-moderate mental deterioration were treated with 10 mg/day of L-deprenyl, a selective MAO-B inhibitor, according to a double-blind crossover design vs. placebo. Initial treatment (drug or placebo) was randomly assigned. The patients' cognitive functions were evaluated at baseline and then after 3 and 6 months of treatment with drug or placebo. The patients crossed over treatment after 3 months, without a washout interval. The results of the study show the higher and statistically significant effects of L-deprenyl on memory and attention that seem to be due to an improved function of the monoaminergic systems involved in the process of neuronal degeneration.

[Treatment of progressive supranuclear palsy]. / La terapia della paralisi sopranucleare progressiva.

The neuropharmacological and neurochemical features of Progressive Supranuclear Palsy (PSP) are reviewed, together with the results obtained by various therapeutic trials. PSP is a neurodegenerative disease which often causes Parkinsonian symptoms but dopaminomimetic drugs have given rise to poor improvement, in spite of dopamine decrease observed in PSP patients' nigrostriatal region. The uselessness of L-DOPA therapy in PSP patients may explain the numerous failures encountered in PSP patients misdiagnosed as Parkinsonian. On the basis of the recent discovery of striatal dopaminergic receptor abnormalities and of interaction between various neurotransmitters, the authors suggest some possible therapeutic substances that might improve the outcome of the disease.

[MPTP: a new chapter in the history of Parkinson's disease]. / MPTP: un nuovo capitolo della storia del Parkinson.

Great interest has been recently raised by the discovery of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), a meperidine analogue capable of producing an irreversible Parkinson's disease. On the basis of papers published during the last years, we examined the structural features and the specific mechanism of action of this substance at the level of dopaminergic neurons. Furthermore, the clinical features of the experimental Parkinson model, obtained by means of MPTP inoculation in various animals and their similarities to the analogous human disease are described. We can conclude that the MPTP discovery enhances the hypothesis that Parkinson's disease can be also attributed to toxic factors.

[Therapy of insomnia]. / La terapia dell'insonnia.

The authors review the neurochemical and electrophysiological features of insomnia, together with the results obtained by various substances. The literature data show that the benzodiazepines (BZ) should be administered for short periods of time, in order to avoid addiction and withdrawal symptoms. For this reason, the authors suggest that, before starting a therapy with such substances, an accurate clinical evaluation should be made and a good knowledge of the pharmacokinetics of the various BZ is essential.

Frontal lobe dysfunction in Parkinson's disease: prognostic value for dementia?

The purpose of this longitudinal study was to investigate if the presence of frontal motor deficits in parkinsonians without signs of global intellectual impairment may have a predictive value for the development of a progressive dementing process during the course of the illness. An examination of the higher level of motor organization, using skills thought to depend upon the integrity of the frontal regions, was performed by 30 parkinsonian patients who did not present any signs of general intellectual impairment. According to their performance, as compared with controls, they were divided into two subgroups: those with and those without frontal dysfunctions. After a mean period of 4 years, a second neuropsychological examination was carried out to assess any eventual change of mental status. The results suggest that frontal dysfunctions may be observed several years before the appearance of generalized intellectual impairment and may be considered one of the predictive factors for development of dementia in Parkinson's disease. Careful consideration of these defects during examination of motor abilities may be of value in the clinical management of parkinsonian patients.

Negative evidence of difference between right- and left-handers in interhemispheric transfer of information.

The present study has been designed to investigate the relationship between handedness and callosal function. Based on the previous finding of a greater anatomical connection between the hemispheres in left-handers than in right-handers and in order to test the possibility of greater functional communication, the efficiency of interhemispheric transfer of information has been measured using a finger localization task. Comparison of two groups of male right-handed and left-handed subjects shows no difference in the efficiency of interhemispheric transfer of information.

[2 cases of Binswanger's disease not associated with arterial hypertension: clinical-instrumental and neuropsychological evaluation]. / Su due casi di malattia di Binswanger non associata ad ipertensione arteriosa: valutazione clinico-strumentale e neuropsicologica.

Two cases of subcortical arteriosclerotic encephalopathy (Binswanger's disease) are reported. The two patients lacked a clinical history of hypertension, relevant pathogenetic factor in the development of the small and medium size cerebral arteries atherosclerosis, which is the main pathologic finding of the disease. The two subjects clinically showed a marked intellectual deterioration, together with mood depression and focal neurological signs, that were an expression of the multifocal neurologic involvement. In both cases CT scans evidentiated a mainly periventricular leucoencephalopathy associated, in the first patient, with small multiple ischemic lesions and, in the second, with a unique hypodense area in the centrum semiovale. A review of the literature on the subjects is proposed, together with an attempt of pathogenetic interpretation of our two cases.

Individual differences in cerebral organization: influence of sex and familial sinistrality in the language lateralization of strongly right-handed subjects.

Sixty right-handed subjects, divided into four groups of 15 according to sex and familial sinistrality (FS), performed a test of language lateralization. A verbal-manual dual-task paradigm was employed. Results suggest that the pattern of cerebral organization may differ among right-handers in relation to both sex and FS. However, it is not merely the separate influence of these two factors, but rather their interaction which determines the pattern. It is stressed that identification of individual predictors of language laterality may provide some information on prognosis and management of aphasic patients.