Rivista di patologia nervosa e mentale最新文献

The aim of our research is to check if the combined use of radionuclide angiography and Doppler-sonography leads to correct screening of carotid lesions in the neck, independently from the indexes of reliability of the individual examinations. 125 cerebrovascular patients (for a total of 250 carotid studies) were investigated with radionuclide angiography and Doppler-sonography. 100 of these carotids were also angiographically investigated. On the basis combined angioscintigraphic and Doppler results, these patients have been classified in four groups. When both non-invasive examinations result negative, the angiography shows pathological arteries only in 22% of cases. When only radionuclide angiography is positive, the angiography shows pathological findings in 63% of cases: when only Doppler is positive, the angiography confirmation occurs in 72% of cases. Finally, when both non-invasive tests results positive, there is always arterial pathology in the angiography.

Clinical, EMG, genetic and CT investigations were performed on 16 subjects suffering from Torsion Dystonia. We obtained the following results: i) genetically most cases were sporadic, only two could be considered autosomic dominant; ii) one of the pedigrees, with many patients, confirmed that spasmodic torticollis is not a distinct form from that of Dystonia Muscolorum Deformans; the two forms must be included in one disease called "Torsion Dystonia", as maintained by some Authors; iii) we found that the various drugs employed, rarely produced improvement, although benzodiaze4pines have given some benefit; iv) CT findings were not specific, though useful for differential diagnosis.

Two female patients aged 64 and 54 were studied, affected by a progressive, distal sensori-motor mononeuritis multiplex. A nerve biopsy specimen from the first patient, suffering from chronic active hepatitis without detectable serum hepatitis B surface antigen (HBsAg) or antibody (anti-HBs), showed segmental demyelination and widespread inflammatory and necrotizing vasculitis and perivasculitis. The second patient, affected by a localized, caeseating tuberculous infection of the right kidney, refused to undergo a nerve biopsy. Serum levels of circulating immune complexes, detected by C1q BA binding method were strongly positive in both subjects; activation of the complement system (decreased serum level of C3 and/or C4) was also present. The course of the peripheral neuropathy was progressive and worsened in the first patient whose circulating immune complexes were always detectable. On the contrary the second patient showed improvement after the nephrectomy, which coincided with a quick disappearance of the circulating immune complexes. The findings may suggest in these two patients an immune complex mediated pathogenetic mechanism like the one recently accepted for the peripheral neuropathies in the course of chronic hepatitis B.

The genetical forms of hypertrophic neuropathies, inherited either as recessive or autosomal dominant trait, are classified, according to Dyck (1975), as HMSN type I, III, and IV. Sporadic cases are also reported. We studied three patients, one with autosomal recessive inheritance, and two without family history, who had the following common features: --onset of symptoms before the age to two years; --slowly progressive course; --peroneal muscular atrophy with absent tendon reflexes; --reduction of MCV and SCV; --decreased number of myelinated fibers; --schwannian cell hyperplasia, with onion bulb complexes formation; --absence of aspects of hypomyelination; --increased number of collagen pockets and denervated Schwann-Remak cells or processes. On light microscopy, multilamellated onion bulbs of large size were found in a very high percentage in case 1, while there were either simple in type or in a lower percentage in case 2. In the third, case, onion bulbs were recognized only on electron microscopy. It is known that in the various kinships affected with type I of HMSN, the pathological changes of peripheral nerves differ greatly. Therefore, despite early onset of symptoms and varying degree of severity of nerve changes, all three cases have been classified within the group of HMSN type I. The different severity of nerve damage may suggest the possibility of a genetical heterogeneity in this disorder.

From 1978 to 1980, thirty-one patients aged forty years or less, had cerebral ischemic events as TIA, RIND or complete stroke. These patients have been studied, both clinically and with laboratory tests to assess the most common causes of their disorder. It was found that hypertension, excess smoking, diabetes and disorders of the lipid metabolism are the most common causes of the atherosclerosis, which plays a role in enhancing ischemic cerebro-vascular accidents during youth. The detection as well as the localization of the site of the ischemic lesions was difficult; only in six out of the seventeen patients examined it was possible to show angiographically a stenosis of one cerebral vessel. Comparison between the 31 patients and 31 subjects of the same age without clinical symptoms or neurological signs, showed a significant incidence of causes of atherosclerosis in the control subjects. In view of the limited number of controls it was not possible to predict a clearcut prognosis.

The family of an "affected" subject with limb girdle dystrophy has been studied in order to assess the clinical-hereditary characteristics of the disease and to contribute to its definite genetic features (phenotypical expressiveness of the pathologic gene). The diagnosis of certitude was based on the anamnestic-clinical criteria and instrumental investigations, supported by histological and histochemical studies of the muscles. The clinical, electromyographic and biochemical data made it possible to distinguish the "affected" from the "subclinical" and the healthy subjects. The subjects that, without noticeable symptoms of neuromuscular disorders, showed a slight clinical expressiveness which didn't alter the normal social and working activities, have been defined "subclinical". The modalities of hereditary transmission of this form of muscular dystrophy are considered in the light of the genetics most present trends that are tending to overcome the dominant-recessive dualism. The possibility of a modality of transmission definable as "intermediate inheritance" is proposed. In the case of the examined family the hypothesis that a pathologic recessive autosomic gene gives rise to a clinical expressiveness in heterozygote subjects seems tenable. This situation definable as "incomplete recessive" is rarely found in the limb girdle dystrophy.

The results of a light and electron microscopy study of seven amputation neuromas are presented. The neuromas were removed during surgery for traumatic lesions of limbs. The histological study showed a complete disappearance of the nerve architecture at the neuroma level, where the fibres are collected in small bundles, enveloped in an abnormal perineural sheath, surrounded by an overgrowing connective tissue. In two cases a partial and incomplete reinnervation of the distal stump was found. The ultrastructural study shows abnormalities of the nervous fibers, both of the axon and of the relationships between the axonal part and the Schwann cell. The results of our data in man are compared to nerve regeneration patterns recently investigated in experimental models.

The AA relate a case of a complex malformation of the AICA characterized by aneurysm of the internal auditory artery developed inside the internal acoustic channel associated with an angioma fed by the cerebellar branch of the same artery. During a long period with dizziness, tinnitus, headache in the occipital region, mild sensoneural retrocochlear hearing loss a subarachnoid hemorrhage occurred. The vascular malformations were visualized by a vertebral angiography and the axial projection resulted to be the most important clue. The AA report the case for its rarity and emphasize the vascular origin of certain unexplained kinds of vestibular syndromes in young patients.

A 42 years old man, suffering from pulsating headache, episodes of loss of consciousness and motor difficulty of the right arm, had an angiographic examination, showing a malformation of the right carotid, which connected the external carotid artery with the basilar artery. The AA. have discussed the theories about this condition, trying to find an embryo-genetic explanation. They have evaluated the clinical aspects caused by the abnormality considering mainly its circulatory defect, considering also the possible association with intracranial aneurysm caused by modification of the normal circulation in the brain stem and neck arteries.

The principle that increasing the collection of data through an exchange of clinical experience means scientific progress for psychoanalysis is criticized: this can be achieved only by adopting new theories or paradigms. Freudian metapsychology in an unsatisfactory theory because instead of explaining clinical data, it contrasts them but an exclusively empirical theory would limit psychoanalysis. The Author suggests to adopt and develop a structural paradigm. The concept of structure is utilized at neurophysiological level, at psychological structures level and finally in the structuralistic analysis of psychoanalytical multiple narratives. Three types of theories are considered clinically: descriptive, comprehensive (of multiple aims and meanings) and genetic. The articulation of comprehensive and genetic theories with the hypothetical theory level represented by psychological structures is being sought. This different theoretical approach will also involve differences at technical level and the Author deals with this latter aspect by referring to interpretations, transference dreams and pathology.