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L Angelini

Publications and source records attributed to L Angelini.

At least 19 recordsLinked to original sources
PubMed1995

Neuronal ceroid-lipofuscinosis: a clinical and morphological study of 19 patients.

We report on clinical, electrophysiological, neuroradiological, and morphological data from 19 patients with different types (late infantile, juvenile, and adult) of neuronal ceroid-lipofuscinosis (NCL), observed in the last 10 years at the Neurological Institute of Milan. Late Infantile NCL (LINCL) (8 patients, 4m/4f). Age at onset: 2-4 1/2 years. Seizures (6 patients) or decline of mental capacities (2 patients) were the presenting symptoms, followed by myoclonus and ataxia; visual loss and optic atrophy occurred in 6 patients within 3 years. All but 2 children became bedridden within 3 1/2 years. CT and MRI demonstrated different degrees of cerebral and cerebellar atrophy within 3 years from onset of the disease. Ultrastructural studies showed fingerprint profiles (FP) and osmiophilic bodies (OB) in circulating lymphocytes; curvilinear bodies (CB) and FP were detected in eccrine secretory cells. Juvenile NCL (JNCL) (7 patients, 4m/3f). Age at onset: 6-9 years. Visual loss with retinal degeneration was the presenting symptoms, accompanied in all but 2 patients by slight mental impairment. Seizures occurred within 2-4 years. CT and MRI detected cerebral or cerebellar atrophy in those patients (5 patients) with a clinical follow-up longer than 4 years. Electron microscopy showed FP on circulating lymphocytes, and both FP and CB on skin biopsy specimens. Adult NCL (ANCL) (4 patients, 3 m/1f). Age at onset: 12-50 years. Progressive myoclonus epilepsy (1 patient) or dementia with motor disturbances (3 patients) were the clinical phenotypes of the disease. MRI demonstrated cerebral and cerebellar atrophy within 6 years from onset. Electron microscopy disclosed FP in cytoplasmic vacuoles inside eccrine secretory cells.

Adolescent↗
PubMed1995

Paroxysmal dystonia and paroxysmal tremor in a young patient with multiple sclerosis.

A 16-year-old patient with multiple sclerosis (MS) showed paroxysmal movement disorders during a recurrence of the disease. The paroxysms took the form ot brief unilateral dystonic posturings of the right body suggestive of paroxysmal dystonia (PD); they completely receded with acetazolamide. A single episode of a high amplitude, rythmic slow and coarse generalized tremor, present at rest and increasing with movement, particularly involving the head in a no-no movement, occurred soon after recovery from PD and lasted three hours. The present report provides evidence that MS has to be considered in the diagnostic approach to symptomatic childhood PD and underlines the efficacy of acetazolamide in the treatment of PD attacks. It also describes a rare paroxysmal movement disorder, defined as paroxysmal dystonic tremor, that can be considered as falling within the spectrum of PD.

Acetazolamide↗
PubMed1994

Primary antiphospholipid syndrome (PAPS) and isolated partial seizures in adolescence. A case report.

The case of a young male patient presenting isolated clustered partial seizures is reported. Despite the normality of the neurological features, as well as of ictal and interictal EEG, the MRI (performed three days after the symptoms) disclosed bilateral signal alterations in the parietal cortical region. These abnormalities disappeared at the MRI control examination performed one month later. The finding of positive anticardiolipine antibodies made possible the diagnosis of partial epileptic seizures symptomatic of a vascular disorder ascribed to a Primary antiphospholipid Syndrome (PAPS).

Adolescent↗
PubMed1994

Transanal endoscopic microsurgery in Italy.

The Italian experience with Transanal Endoscopic Microsurgery (TEM) started in 1991. Until April, 1994, 122 patients were operated on by such a technique in six centres. The surgical protocol in the 66 patients with benign lesions was similar to that described by Buess. In contrast to the German experience, the indications of TEM for cancer have been extended to more advanced tumours and in 22 out of 56 patients with rectal carcinoma adjuvant radiation- or radiation-chemotherapy have been applied according to various protocols. In 88% of TEM for rectal tumours the operation has been carried out according to a full-thickness technique, with or without perirectal fat excision. Postoperative morbidity of TEM for adenoma was 15.8% and that of TEM for carcinoma 29.6%. There was no postoperative mortality. Local recurrence rate after TEM for adenoma was 10.5%, while that after TEM for cancer was 9.25%. No local recurrence has been reported among patients treated with a combination of TEM and adjuvant radiation treatments. The median follow-up in the 6 centres ranged between 7 and 16 months. A randomised prospective clinical trial has been planned in order to evaluate the role of transanal endoscopic microsurgery in the treatment of locally advanced rectal cancer.

Adenocarcinoma↗
PubMed1994

High prevalence of antiphospholipid antibodies in children with idiopathic cerebral ischemia.

BACKGROUND: The presence of circulating antiphospholipid antibodies (aPLs) is frequently associated with thromboembolic phenomena. OBJECTIVE: To investigate the prevalence of aPLs, detected as lupus anticoagulant (LA) or anticardiolipin antibody (aCL), in a group of unselected children with idiopathic cerebral ischemia. DESIGN: Prospective, case series. SETTING: A pediatric neurology department. PATIENTS: Thirteen children with cerebral ischemia (eight with stroke, three with transient ischemic attacks, and two with ocular ischemia). Age-matched apparently healthy children served as controls. MEASUREMENTS: LA and aCL determination was performed within 3 days after the occurrence of the ischemic event and was repeated after 3 to 6 months. To be defined as aPL-positive, patients had to have either a positive LA test or positive IgG and/or IgM aCL at moderate/high level in both determinations. MAIN RESULTS: Ten (76%) of the 13 patients were positive for either LA or aCL. No differences were found between aPL-positive and aPL-negative patients with respect to clinical manifestations or radiological features. Six (46%) of the 13 patients had a history of multiple ischemic events. CONCLUSIONS: Our results show a very high prevalence of aPLs in children with idiopathic cerebral ischemia. Because the presence of these antibodies has relevant therapeutic implications, their determination in children with cerebral ischemia is recommended.

Adolescent↗
PubMed1994

[Is surgery duration really a complication factor?].

Of the numerous factors which may cause postoperative complications, some authors sustain that the durations of surgery plays a fundamentally important role. On the basis of a retrospective survey and analysis of cases operated, the authors observe that this factor, if considered alone, does not appear to cause a significant increase in septic complications, anastomotic dehiscence or mean postoperative hospital stay. Four homogeneous groups of patients were examined. Two groups included 100 who had undergone abdominal surgery which had not contaminated the peritoneal cavity. The other two groups included 150 patients who had undergone partially polluting colorectal surgery. The general preparation of patients was similar, with the exception of antibiotic prophylaxis. The mean time was calculated by analysing anesthesiological records. From an analysis of the most recent data, which are characterized by the absence of anastomotic dehiscence and operative mortality, it can be seen that the incidence of sepsis has fallen to 4% even if mean operating time was long. Mean postoperative hospital stay was also reduced from 17.3 days to 13 days. The prolongation of operating times does not depend on the complexity of surgery, intraoperative complications or the surgeon's inexperience, but may be the result of the meticulous implementation of procedures and steps taken to prevent complications which, given that they require extreme care and attention, necessarily contribute to increasing the duration of surgery. With regard to antibiotic prophylaxis and the duration of surgery, it was observed that if antibiotic prophylaxis preceded the operation by more than one hour, the incidence of sepsis was about 20%. If treatment coincided with the induction of anesthesia or the start of surgery, then the incidence of sepsis fell to 13%.(ABSTRACT TRUNCATED AT 250 WORDS)

Anti-Bacterial Agents↗
PubMed1994

Chemoembolization in liver malignant involvement. Experiences on 17 cases.

INTRODUCTION: Liver invasion is the major cause of organ failure in patients with primary liver cancer and metastatic large bowel cancer. Furthermore it causes high morbidity in many other carcinomas. The normal liver presents a double circulation: 75% from portal circulation and 25% from hepatic artery. In malignant primary and secondary lesions the blood support is given by hepatic artery. Antineoplastic drugs mixed to selectively injecting embolic particles, such as polyvinyl alcohol and gelatin powder (Gelfoam), can be injected to infarct tumors and to obtain a therapeutical advantage. Chemoembolization using an emulsion of Lipiodol ultra-fluid (LUF) and drugs is a recent tool in liver regional therapy. LUF has been shown to be taken up hepatocellular carcinoma and retained for a long period of time in the tumor bed. Chemoembolization causes massive shrinkage due to ischemia and increasing the local drug intensity and drug exposure. Our study reports the results of multi-agents chemoembolization (MACHEM) in 17 patients bearing massive liver involvement. MATERIAL AND METHODS: From January 1988 we treated 17 patients (5 HCCs, 7 large bowell carcinomas, 1 gastric cancer, 1 ocular melanoma, 1 pancreas, 1 soft tissue sarcoma, 1 carcinoid) using a transfemoral approach to cannulate the celiac axis and then the hepatic artery. The catheter was advanced into the vessel responsible for the majority of the tumor blood supply and a mixture of Gelfoam, radiopaque contrast media, followed by chemotherapy (mitomycin 10 mg/sqm, cisplatin 20 mg/sqm, epirubicin 20 mg/sqm) mixed to LUF was injected until vascular stasis occurred. After chemoembolization, analgesics and anti-pyretics were administered. Liver function tests were monitored daily. RESULTS: Objective tumor regression was observed in 11 out 15 full evaluable patients; the median duration of survival was 9.5 months. Within 8 weeks shrinkage, due to development of necrosis, appeared in the tumors. One patient with high levels of 5-HIAA due to carcinoid, demonstrated more than 75% decreasing in urinary excretion. In 6 patients out 8 with CEA elevation a clear reduction was documented as well in 2 HCCs out 5 with alfa-fetoprotein elevation. DISCUSSION: The palliation of HCC and metastatic liver disease have been extremely disappointing. Systemic chemotherapy produces in HCC a response rate of no more than 20% and does not increase the median survival. Venook obtained 24% of PRs and liquefaction in 35 out 50 HCC treated with chemoembolization. Some results have been also demonstrated in the treatment of metastatic liver tumors by Carrasco, Daniels and Modiano. Moertel stressed that chemoembolization could be incorporated in the initial management of carcinoid. Because of the difference in chemoembolization protocols it is difficult to compare the relative efficacy of this tool, although encouraging response rates have been reported in palliation of bulky tumors. In our study Gelfoam given before LUF and antineoplastic agents mixture produce a distal arteriolar occlusion and this would facilitate the migration of the polychemotherapy emulsion toward the tumor. Our MACHEM program has been shown to have significant activity even in heavily pretreated patients with an acceptable toxicity. We conclude that hepatic arterial chemoembolization will be improved by mean of better combination of chemotherapy with embolizing agents in well selected patients.

Adult↗
PubMed1994

Transitional papillary cell carcinoma of the ureter: a histological and ultrastructural study.

We report a case of transitional papillary carcinoma of the distal left ureter. The histological and ultrastructural features were seen and compared by SEM and TEM. Observations confirm the data of literature that the deep cells of neoplasm are a normal morphological finding while the superficial cells do not have the characteristics present in normal transitional cells. In addition, SEM shows small groups or single cells with "pleomorphic microvilli" on the cell surface. These, in ureter, confirm the studies of many authors who have observed these abnormalities in carcinomas of several organs like breast, colon, liver and mesothelium.

Aged↗
PubMed1994

Laparoscopic ultrasonography: limits and potential of present technologies.

During recent years, laparoscopic contact sonography has been introduced into clinical practice to remedy the limitations of minimal invasive surgery. The technology of laparoscopic ultrasound probes is described in this paper as well as the technique of US contact scanning via the laparoscopic approach. This method is a tool which allows the examination of tissues and non-palpable parenchymal organs in endoscopic surgery, thus permitting correct assessment of both anatomy and the spreading of neoplasms, with consequent impact upon surgical decision making.

Cholecystectomy, Laparoscopic↗
PubMed1993

Hemidystonia symptomatic of primary antiphospholipid syndrome in childhood.

We report three children with hemidystonia in whom anti-cardiolipin (aCL) antibodies were demonstrated. Systemic lupus erythematosus was excluded on the basis of both clinical and serological criteria, and the diagnosis of primary antiphospholipid syndrome (PAPS) was made. In two cases, aCL antibodies could be causally related to a presumed immune-mediated thrombotic event involving the basal ganglia as shown by magnetic resonance imaging (MRI). In the remaining patient the finding of white matter alteration on NMR might be due to cross-reactivity of anti-phospholipid (aPL) antibodies with cerebral phospholipids, resulting in demyelination. We suggest that PAPS must always be considered when isolated or recurrent focal cerebral ischaemia, and particularly hemidystonia, occur in childhood.

Antibodies, Anticardiolipin↗
PubMed1993

Chronic GM1 gangliosidosis presenting as dystonia: clinical and biochemical studies in a new case.

Clinical and biochemical findings in a patient affected by chronic GM1 gangliosidosis, presenting as progressive dystonia and mental deterioration, are reported. The patient, a 13-year-old male, showed, at the age of 3 years, an impairment of gait with frequent falls, dysarthria and stuttering. At the age of 6, writing dystonia appeared and subsequently mental deterioration and dystonic postures of arms and legs became evident. The clinical features presented by this patient are similar to those shown by the cases of adult/chronic GM1 gangliosidosis previously reported, except for the early onset. This observation emphasizes the occurrence of dystonia as prominent symptom in chronic GM1 gangliosidosis, underlining that this disease must be considered in the diagnostic approach to the progressive dystonias of the early infancy.

Child↗
PubMed1992

["Multiorgan failure" in surgery: its physiopathology, prevention and treatment. A review of the literature].

At present multiorgan failure (MOF) is the complication with the highest mortality after emergency or elective general surgery. The syndrome seems to find its pathogenesis out of the very complex interactions of endogenous and exogenous mediators; instead the physiopathology seems to depend on cellular oxygen deficiency. The authors refer the common criteria defining organ and/or system damage during MOF and analyse the available possibilities for their treatment. In this regard they stress the role of prevention, which relies on the opportune treatment of earlier stage of shock, constantly preceding the syndrome, and on the intensive organ/systems functions monitoring to obtain an early and rational correction of the alterations taken away.

Emergencies↗
PubMed1992

Progressive dystonia symptomatic of juvenile GM2 gangliosidosis.

A 9-year-old boy showed a progressive generalized dystonia, with onset at the age of 4 years, combined with mental deterioration and behavioral disturbances. The values of beta-hexosaminidase activities studied in plasma, leukocytes, and fibroblasts obtained using two different substrates (MUG-NAc and MUG-NAc-6-S) were significantly reduced but higher than in Tay-Sachs disease and similar to those found in the juvenile chronic form of GM2 gangliosidosis. With anticholinergic therapy, for 1.5 years, the dystonic symptoms did not progress and the boy can still care for himself and attend school. The description of another case of the disease, clinically expressed as dystonia, corroborates the existence of a dystonic phenotype of GM2 gangliosidosis.

Child↗