Convulsion during lignocaine infiltration.
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Biomedical subjects
Publications and source records attributed to A Fortuna.
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The authors report a case of dilated cardiomyopathy caused by a muscular dystrophy, namely Becker's disease. The peculiarity of this case is the fact that the patient has lived enough time to have clinical manifestations of myocardiopathy because, in general, in this myopathy, the affected males have a short life and the early myocardial involvement is rare. A particular attention is paid to the need of consulting the cardiac patients not forgetting an eventual causal systemic disease and to the crucial importance of the genetic counselling in that kind of diseases.
We report 14 cases of intracranial cavernous angioma, analyzing the clinical features, with special reference to the risk of bleeding, radiological images and treatment in these and in 153 published cases, 167 in all. Cerebral hemorrhage occurred in 44%: typical (intraparenchymal or subarachnoid) in 24.6%, and masked by epilepsy, headache or neurological deficits in 19.2%. In patients with the typical hemorrhagic pattern posthemorrhagic mortality was 12.2%. Of the patients who had a hemorrhage 42.5% were left with more or less disabling neurological deficits, and 16.4% had a rebleed. In discussing treatment we consider four groups of intracranial cavernous angioma: A) symptomatic in a zone of low surgical risk; B) asymptomatic with low surgical risk; C) symptomatic with high surgical risk; D) asymptomatic with high surgical risk. The treatment is surgical, except in the high risk asymptomatic variety, best followed initially with sequential CT scan and MRI and then considered for surgery if the lesion becomes symptomatic, increases in size or presents neuroradiological signs of bleeding.
We report the case of an achondroplastic child in whom severe morphological and postural abnormalities of the cervical vertebrae were coupled with only mild and stable neurological deficits. The pathogenesis of the abnormalities is discussed.
Clinical features and surgical outcome of 16 patients with dermoid or epidermoid cysts on the midline of the posterior cranial fossa are compared. Salient points in the comparison are the younger age, presence of associated malformations and better prognosis of dermoid cyst.
Of the 62 patients with intramedullary spinal cord ependymoma treated surgically at our Neurosurgery Division between January 1951 and December 1990 45 had a follow-up of at least 3 years and the longest 30 years. The 28 conus-cauda equina-filum ependymomas operated during the same period are not considered in this study. An analysis of our cases and of the larger published series shows that favourable prognostic factors, apart of course from total tumour removal, which is now usually possible, are a site below the high cervical segments and a mild pre-operative symptom pattern. Patient age at diagnosis, tumour size and "low dose" (< 40 Gy) radiotherapy seem to have no influence on the prognosis. Aggressive surgical removal is the treatment of choice and also for long-term recurrence.
A rare case of trochlear nerve neurinoma is described. Including this case, the number of reported intracranial tumors arising from the sheaths of the third, fourth, and sixth cranial nerves is 38. By site and relationship to the nerve segment, they fall into three groups: cisternal, cisternocavernous, and cavernous. In cisternal tumors of the third and sixth nerves, paresis of the nerve hosting the tumor is the unique nerve deficit; by contrast, in those of the fourth nerve, paresis of the trochlear nerve can be absent and that of the third nerve present. In the latter tumors, a peculiar ataxic hemiparesis syndrome is produced by midbrain compression. Cisternocavernous neurinomas often cause symptoms of intracranial hypertension, while cavernous neurinomas bring about two clinical features: paresis of one or more nerves of the cavernous sinus and a clinicoradiological orbital apex syndrome. At surgery, generally cisternal neurinomas are totally removed and the nerve source of the tumor identified; in cisternocavernous and cavernous neurinomas, total removal of tumor and identification of the parent nerve have been reported in only half of the cases. In the majority of parasellar neurinomas, clinical differences can be found between those arising from the nerves governing eye movement and those arising from the gasserian ganglion.
Thirty-eight primary tumors of the cervical spine were operated on in the Section of Neurosurgery, Department of Neurological Sciences, "La Sapienza" University of Rome between 1954 and 1988. Of these, 23 were malignant and 15 benign. The aims of surgical treatment in every case were tumor removal, decompression of the spinal cord, and conservation or restoration of vertebral stability. In no case was surgery confined to biopsy. Surgical stabilization was performed in 12 patients. Bone fusion was obtained with autologous bone (iliac crest, fibula) in benign tumors, whereas synthetic material (acrylic and metal) was used in malignant tumors. Past experience and greater awareness of the concrete possibilities of treating these tumors have led us to evolve the following strategy: 1. For aggressive benign tumors (osteoblastoma, giant cell tumors) and for malignancies in patients with fair life expectancy (solitary myeloma, chordoma): radical removal. 2. For benign tumors with low neoplastic potential and for malignant tumors in patients with poor life expectancy: conservative removal. Long-term results were gratifying in all benign lesions and low-grade malignancies even though two patients with chordomas needed reoperations. Sarcomas had relatively poor results.
A patient with contralateral monoparesis of the leg due to subarachnoid hemorrhage (SAH) from an aneurysm of the first posterior inferior cerebellar artery (PICA) segment is reported. The monoparesis may well be associated with the close anatomical relationships between the site of the aneurysm and the PICA blood supply of the corticospinal fibers to the contralateral leg.
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Hemangiopericytoma, a tumor whose origin is controversial, rarely affects the central nervous system and is even more rarely spinal. We report a case of the spinal variety and discuss its clinical and pathological features, diagnosis, and treatment in the light of relevant literature.
Three cases with large space-occupying cysts in the cerebellopontine angle are reported. CT and MRI findings were not typical for acoustic schwannomas but at operation, besides the large cysts, small acoustic schwannomas could be detected and removed. The clinical and neuroradiological features of this unusual variety and the CT and MRI differential diagnosis of cerebellopontine angle lesions are discussed.
We report the case of an adult with a posttraumatic intradiploic leptomeningeal cyst that caused a circumscribed osteolytic skull lesion. Local pain, the only symptom of the lesion, regressed after surgery. Intradiploic leptomeningeal cysts must be distinguished from intradiploic arachnoid cysts, which are of congenital origin.
We report a case of association of a brain tumor with multiple intestinal polyposis (Turcot's syndrome) and offer a critical analysis of the relevant literature with a view to revising the classification of the syndrome in relation to familial multiple polyposis and Gardner's syndrome. For this purpose, we considered only cases of intestinal polyposis associated with a primary neuroepithelial tumor (medulloblastoma, glioma, or glioblastoma) as originally described by Turcot. Differences emerged, depending on the central nervous system tumor type, which suggests that this neoplastic association may be classified as two distinct syndromes.
Cavernous angiomas represent 5 to 12% of spinal vascular malformations and usually are located at the vertebral body level with possible extension into the extradural space. The intradural intramedullary cavernoma occurs in about 3% of cases, whereas extramedullary localization is extremely rare. A new case of an intradural extramedullary cavernous angioma is reported, and the clinical, diagnostic, and therapeutic aspects of this rare malformation are analyzed.
Forty-one patients with brain lesions underwent brain biopsy using either a computerized tomography (CT)-guided stereotactic approach or an ultrasound-guided technique. The cases were selected according to location and size of the mass lesion. Lesions 15 mm or less in diameter and those in the posterior fossa were biopsied by a CT-guided stereotactic technique (18 patients). Supratentorial lesions with a diameter larger than 15 mm were approached using ultrasound guidance (23 patients). These criteria for procedure selection provided a diagnostic yield of 94% for the CT-guided procedures and 91% for those guided by ultrasound. Safety for the two procedures was similar. The ultrasound procedure was more rapid, simpler, and less costly to perform. It is concluded that, with the protocol described, CT-guided stereotactic procedures could be reserved for cases in which absolute accuracy is mandatory.
A column immunoadsorption method, based on the high affinity between the protein Avidin and the vitamin Biotin has been used to obtain positive CD34+ or S313+ marrow cells selection. Cell suspensions from marrow samples of six healthy subjects were incubated either the monoclonal antibody S313 or HPCA 1 (My 10-like) and a biotinilated goat anti-mouse Immunoglobulins antiserum, passed over a column containing an Avidin coated Polyacrylamide matrix, at the flow rate of 1 ml/min. The phenotype and the clonogenic efficiency of the recovered adherent cell fraction were studied by cytofluorimetric analysis and haemopoietic progenitors short term cultures. The results obtained show a mean CD34+ and S313+ cells recovery greater than 50% with a lower stem cells enrichment. Although these data could not considered optimal for clinical application in haematologic neoplasias, these preliminary studies demonstrate the possible use of the method for autologous bone marrow transplantation.
Stenosis of the spinal canal is a very frequent finding in achondroplastic patients. It is secondary to abnormalities of endochondrial ossification, which is responsible for formation of the vertebral bone structures, and, subsequently, to bone degeneration. Cervical stenosis (most frequently involving the first metameres and the craniospinal junction) is more common in children while thoraco-lumbar stenosis favors adults. The surgical indications for cervical stenosis depend mainly on the clinical evolution but also on neurophysiological data. In thoraco lumbar stenosis too, clinical data determine the surgical indications and the route of attack depends on the type of anatomical alteration affecting the spine.