Rivista di patologia nervosa e mentale最新文献

A literature review shows that patients affected by Parkinson's disease present an intellectual impairment more frequently than the comparably aged population. Such impairment has been related to several factors (age, arteriosclerosis, motor difficulties, depression, dopaminergic therapy, cortical and/or subcortical lesions). Parkinsonian dementia may be caused by the extent of the degenerative process. Our own results show that parkinsonian patients with dementia are different from nondemented ones with the following features: a) a more marked bradykinesia b) a more severe extrapyramidal picture c) a progressive unresponsiveness to levodopa in a shorter time. It seems possible that there is a Parkinson syndrome characterized, clinically, by an intellectual impairment with a poor prognosis quoad valetudinem, and, anatomically, with multiple cortical and subcortical lesions. Such syndrome may be a "transition" form between Parkinson disease and senile-presenile dementia.

The Authors describe the result of air encephalography in 5 cases of Progressive Supranuclear Palsy (P.S.P.). In all cases signs of cortical cerebral atrophy were found (with a marked dilatation of the lateral ventricles in two patients). Equally constant were signs of atrophy in the brain stem and cerebellum with involvement of the vermis (in its superior part in four cases and diffused in one case). In three patients there was also subcortical cerebellar atrophy. Among the alterations observed the slight cerebral cortical atrophy does not seem to be due to the pathological process peculiar to P.S.P., but rather to other processes such as senility, vascular alterations etc. Atrophy involving the brainstem and the cerebellum was of interest being a constant finding easily correlated with symptoms and with the site of P.S.P. process. These findings, though without specificity, offer useful data and help to assess the evolution of the disease.

A case of infantile neuroaxonal dystrophy (I.N.A.D.) with late onset is described with protracted course and predominant myoclonic-epileptic symptomatology. A girl of 13 years died in myoclonic-epileptic state. She had suffered from a mild cerebellar deficit, slight intellectual impairment and increasing myoclonic attacks since the age of 5 years. A similar neurological syndrome, beginning at almost the same age, occurred in her younger brother who died at the age of 11 years from acute hepatic failure (without autopsy). Histological examination of the CNS in the girl revealed a diffuse neuroaxonal dystrophy, some areas of spongy degeneration in the cerebral and cerebellar white matter, cortical atrophy of cerebellum accompanied by demyelination of the spinocerebellar tracts, the fasciculus gracilis and the cortico-bulbar tracts. Such histological features are in keeping with those of I.N.A.D. or Seitelberger's disease. The clinical features, however, differ considerably from the latter as well as from Hallervorden-Spatz's disease and seem to belong, instead, to the group of progressive myoclonus epilepsies. In the differential diagnosis of these rare conditions, therefore, also the I.N.A.D. ought to be considered.

In a group of 657 epileptic patients there were 51 presenting with fits even after having reached the age of 60. The onset of seizures dated back to the first 20 years in 15 patients between 20 and 40 years in 15 and between 40 and 60 years in 21. In 67% of the cases it was possible to determine the cause of seizures. The aetiology was unevenly distributed within each group. In the first group (up to 20 years) the P.G.E. forms prevail. In the second (up to 40 years) post traumatic epilepsy and inflammatory processes were predominant, while in the third (up to 60 years) vascular and tumoral pathology seem to be prevalent. 43% of the patients showed paroxysmal abnormalities in the EEG and these were still present even after to age of 60 years in more than fifty per cent. After the age of 60 we found no case of benign epilepsy amenable to complete recovery. Patients older than 60 present epilepsies of mild severity. In symptomatic epilepsies the lesional factors were not subject to evolution and the epileptogenic focuses were stable and persistent. In partial epilepsy there were more cases of complex symptomatology (86%) than cases of elementary symptomatology (14%) The evolution of seizures in old age is considered together with the importance of all factors influencing recurrency.

The diagnostic value of radioisotopic cerebral angioscintigraphy (R.A.) and of Doppler CW (D.C.W.) techniques to identify stenosis of the internal carotid artery in its extracranial course was studied in 97 patients with ischaemic lesions (50 T.I.A. and 47 Complete Stroke). The results of R.A. and D.C.W. were compared with those of contrast carotid-angiography (C.A.). C.A. revealed stenosis above 50% or complete occlusion in 22% of cases, whereas D.C.W. and R.A. showed flow reduction in 27% and 48% of the cases respectively. In T.I.A., C.A. positivity went down to 8%; D.C.W. to 16%; and R.A. to 34%. In "Complete Stroke" positivity was 36% for C.A.; 41% for D.C.W.; and 62% for R.A. There was a high number of false positive findings with D.C.W. (8) but even more with R.A. (27). False negative findings occurred only in two cases with R.A. These data confirm the diagnostic value of these two noninvasive techniques to identify haemodynamically carotid stenosis. The use of both methods can reduce error due to false negativity. The rather marked frequency of false positivity, particularly with reference to R.A. doesn't affect the diagnostic value of the two methods.

Clinico-pathological features are reported in five cases of Creutzfeldt-Jakob disease. In three cases only the brain was examined while in the other two a complete autopsy was performed. In one case a brain biopsy was performed and in other two a piece of cerebral cortex was taken within three hours after death; the brain material was frozen and sent for inoculation in monkeys to Dr. Gajdusek of NINDS-Bethesda, with positive results in two. From the clinical point of view our cases may be classified as follows: two as "classic" type; one as "amaurotic" type; and the last two as "ataxic-amyotrophic" forms according to Alemà and Brion (see text). All five patients were females from 56 to 73 years of age. The duration of the disease ranged from 3 to 7 months. From the histological point of view, in the two "classic" cases the outstanding features was the neuronal loss in all cortical mantle, mainly in the fronto-parietal cortex; furthermore in one there was severe and diffuse spongiform state. In the "amaurotic" case the three types of lesion were mainly localized in the occipital cortex. In the two "ataxic-amyotrophic" forms the neuronal loss was particularly evident in the cerebellar cortex and especially in the granular layer. The Kuru plaques were seen especially in the cerebellum in four of the five cases examined. The aim of our study is to contribute to the epidemiology of this disease.

Two patients with fibromuscular dysplasia of the internal carotid artery have been studied. Our patients' clinical histories suggest that cerebral symptoms were due either to episodic arterial embolization or progressive arterial occlusion. The radiological features were characteristic. Surgery of cervical vessels was performed with benefit in both cases. Histopathology of the carotid arteries showed segmental lesions with alternating marked thickening and thinning of the media and irregular hyperplasia of the intima.

60 patients under 65 years of age with one or more episodes of reversible ischemic attacks lasting less or more than 24 hours (T.I.A. or R.I.N.D.) were studied. Each patient had a complete clinical assessment and an EEG, followed by an arteriogram. 35 patients had an EEG activation with digital carotid compression. EEG abnormalities were present in 26 cases while 34 patients had normal records. No correlation was found between EEG abnormalities and arteriographic alterations. EEG activation by digital carotid compression is a valuable hemodynamic test but cannot necessarily indicate a controlateral carotid artery occlusion.

The Cerebrospinal Fluid (CSF) and the serum of 102 neurological patients was studied in subgroups according to diagnostic criteria. The Blood-Brain-Barrier (BBB) is more severely damaged in cases of cerebral tumors and haemorragic cerebral diseases; synthesis of IgG within the CNS is more pronounced in Multiple Sclerosis (MS) patients in comparison with patients of other groups. The ratio IgG/PT in CSF is very high in MS patients. Considering the degree of increase of the IgG Index and of the ratio IgG/PT in CSF the Authors found the highest values in MS and this is typical of such disease. The degree of increase of the ratio CSF albumin/serum albumin showed highest values in patients with tumors or with haemorragic cerebral diseases.

610 sacrorodiculographic and myelographic examinations were carried out injecting at lumbar level Iopamidol at 200, 300 and 370 mg I/ml concentrations. Immediate and early complications have been noticed in 18,52% of cases, i.e. sensation of pain during contrast injection, headache, nausea, vomit, neck stiffness, photophobia, epilepsy. In sacrorodiculographic examinations, side effects especially occurred by using 370 mg I/ml concentrations; in lumbar and thoracic myelographies, incidence of complications did not statistically differ by uing 300 or 370 mg I/ml concentrations. Side effects have occurred most frequently in cervical myelographies carried out with 370 mg I/ml concentration. Analysis of complications as well as radiographic results suggest to use Iopamidol at 300 mg I/ml concentration in sacrorodiculographics, in lower thoracic myelographies and in studies of narrowed, stenosed or obstructed canals; Iopamidol at 370 mg I/ml concentration may be used in upper thoracic studies and cervical myelographies.