Oculomotor palsy and supraclinoid internal carotid artery aneurysms. Personal experience and review of the literature.

Oculomotor palsy related to the presence of an intracranial aneurysm arising from the supraclinoid internal carotid artery (ICA) is a well known and described clinical condition. Recent microanatomical and clinical evidences seem to demonstrate that the pathophysiology of the aneurysm-related III nerve palsy could be interpreted as that of any other cranial nerve's neurovascular compression syndrome. The authors review their personal experience with supraclinoid ICA aneurysms related to oculomotor palsy and the data of the literature, aiming to elucidate a better clinical, therapeutic and prognostic correlation to these factors.

Symptomatic subdural hygroma after petroclival meningioma surgery. Case report of a intractable complication.

Although subdural hygroma development after craniotomy or aneurysm surgery is a well-known complication and subdural peritoneal or V-P shunt are commonly successful procedures, there are situations that cannot be treated by available surgical options. We reported a case of a 28-year-old young man who developed a symptomatic subdural hygroma after removal of petroclival meningioma. This hygroma increased in size and became bilateral in spite of implantation of V-P with programmable valve at open pressure variable from 30 to 180 mmH2O, placement of subdural-peritoneal shunt with low-low valve or without valve and external diversion. The occurrence of this case showed that there other factors can play role in hygroma development as disturbance of normal CSF dynamic with shunt of CSF from basal arachnoidal to subdural spaces secondary to surgery and/or slow growth of petroclival meningioma as well as postoperative progressive cerebral atrophy. Actually there are not effective diagnostic tools to detect causes and therefore, there are restricted therapeutic possibilities. This potential and serious complication should be always considered when planning petroclival meningioma surgery because of the severe consequences on functional outcome.

[Hyperkeratotic Kaposi sarcoma with leg lymphoedema after prolonged corticosteroid therapy for SLE. Case report and review of the literature]. / Sarcoma di Kaposi i ipercheratosico con linfedema della gamba dopo prolungata terapia corticosteroidea per LES. Caso clinico e rassegna della letteratura.

Kaposi sarcoma (KS) is a malignant vascular neoplasia with a viral etiology, characterized by development of multiple hyperpigmentate lesions, primarily at cutaneous level with associated edema and ulcerations, but frequently involving also the mucous membranes and/or visceral organs. In this study, we describe (in the light of the relevant literature) the clinical case of an elderly (78 yrs-old) woman, who developed red-blues multiple hyperkeratotic nodules in the right leg and foot with marked lymphoedema, blushing and pain, after a long period of a low-dose corticosteroid therapy for LES (at least 10 years of continuous treatment). The diagnosis of KS was made on the basis of histologic findings. The patient HLA-typing showed the haplotypes HLA-A2-10, -B21-35, -Bw4-6, -Cw4 and HLA-DR11-13 (some of which are known to predispose to LES, but not to KS). The KS, first described by Moritz Kaposi in 1872, has been a very rare pathology until the 80s, afterwards its frequency has steadly increased, favored by immunosuppressive therapy for autoimmune diseases or tranplants and by immunodepression of AIDS. Concerning the pathogenesis, it is crucial the role of HHV-8 of the herpesvirus family (found in the lesions and in the circulating cells of all KS patients), for which a prevailing sexual transmission is postulated. General physicians and specialists of internal medicine and angiology should know this disease, which can be undiagnosed because of the low incidence in the general population and the consequent poor knowledge of this vascular neoplastic disease, which is now reported with increasing frequency.

Insulin sensitivity of blood glucose versus insulin sensitivity of blood free fatty acids in normal, obese, and obese-diabetic subjects.

We calculated insulin sensitivity indices (ISI) concerning the insulin effect on both glycemia and blood free fatty acids (FFA), named ISI(gly) and ISI(ffa), respectively, in 34 normal, 27 obese, and 11 obese-diabetic subjects by using the following formulas: ISI(gly)= 2/[(INSp x GLYp) +1], and ISI(ffa)= 2/[(INSp x FFAp)+1], in which INSp, GLYp, and FFAp = insulinemic, glycemic, and FFA areas during oral glucose tolerance test (OGTT) (75 g glucose, suggested sampling time: 0, 1, and 2 hours) of the person studied. A slight modification of these formulas allows the calculation of insulin resistance indices (IRI), ie, IRI(gly) and IRI(ffa). ISI and IRI are complementary, as their sum is always equal to 2, so that IRI can be deduced from ISI and vice versa. By using basal levels instead of areas, insulin sensitivity (or resistance) in the basal state can also be measured. Basal levels and areas are expressed by taking the mean normal value as 1, so that in normal subjects ISI(gly) and ISI(ffa), as well as IRI(gly) and IRI(ffa), are always around 1, with maximal variations comprised between 0 and 2. ISI(ffa) was markedly reduced in both the obese (mean, 0.47 +/- 0.04) and the obese-diabetic (mean, 0.41 +/- 0.06) subjects, whereas ISI(gly) was less reduced in the obese (mean, 0.57 +/- 0.04) than in the obese-diabetic (mean, 0.40 +/- 0.03) subjects. ISI(gly)-basal was less affected than ISI(ffa)-basal in both groups. Multiple regression showed that ISI(gly) and ISI(ffa) were significantly inversely correlated with age, body mass index (BMI), and diastolic (but not systolic) blood pressure. Meta-analysis of data from the literature showed that ISI(gly) was significantly correlated with the hyperinsulinemic-euglycemic clamp data. However, the "clamp" is performed under artificial, persistent hyperinsulinemia (which entails FFA suppression) as never occurs in the life of patients, whereas our indices are performed under physiologic conditions, and represent simple tools suitable for clinical or epidemiologic studies, allowing assessment of whole-body insulin sensitivity with regard to both glycemia and blood FFA.

[Bilateral sclerosing lipogranuloma of the gluteal region with calcification. Report of a clinical case and review of the literature]. / Lipogranuloma sclerosante bilaterale delle regioni glutee con calcificazioni. Descrizione di un caso clinico e revisione della letteratura.

A rare case of large bilateral sclerosing lipogranuloma with multiple calcifications of gluteal region is described in an old female patient affected by a cerebrovascular disease. The lesions appeared as firm, nontender, plaques, 9-10 cm in diameter, covered with hyperpigmented skin. This uncommon disorder is discussed on the basis of data obtained from an extensive literature review. The term "sclerosing lipogranuloma" was coined in 1950, and it defines a disease of the subcutaneous fat, which for a trauma or unknown reasons undergoes necrosis of fat cells with the release of fat droplets into intercellular spaces and a peculiar local sclerosing granulomatous reaction of fatty tissue. The cytosteatonecrosis and sclerosing lipogranuloma, post-traumatic or secondary to injection of exogenous oily substances, usually localized in the breast of women and in genitalia of men, are relatively well known. Sclerosing lipogranulomatosis of the orbita and eyelides, an infrequent but severe complication after endonasal surgery, has also been reported. Rarely, the lipogranuloma can be spontaneous or idiopathic or primitive. A particular form of genetic diffuse lipogranulomatosis is the Farber's syndrome, firstly described on 1947. In our patient, the absence of trauma seems to indicate a primitive lipogranuloma. The presence of an acute rheumatic syndrome responsive to corticosteroids, and the positivity of antimitochondrial autoantibodies are in accordance with the report of sclerosing multiple lipogranulomatosis associated with a lupus-like syndrome. Because of the long duration and the absence of acute local symptoms, this syndrome can be considered benign with favorable prognosis, but the physician should know it.

[Psoriasis complicated with severe mutilating psoriatic osteoarthropathy. Clinical case and review of the literature]. / Psoriasi complicata da grave osteoartropatia psoriasica mutilante. Caso clinico e rassegna della letteratura.

Aim of this paper is to discuss, on the basis of an extensive critical review of the recent literature, the case of a 56-yr-old male patient who suffered from cutaneous psoriasis and psoriatic arthritis mutilans (PA) (polyarticular, symmetric, destruent and erosive) with involvement of the hands, feet and spine, associated with android obesity and mild type 2 diabetes mellitus. HLA typing of the patient showed the HLA-A3-Ax, B14-B63 and Cw4-Cw6 haplotypes, some of which are associated or correlated with susceptibility to PA. Cutaneous psoriasis is a chronic inflammatory dermatitis, with onset at any age and affecting approximately 2% of the western populations. In 5-7% of patients, it is associated with articular manifestations or true arthritis. PA is a chronic, inflammatory, seronegative arthropathy which may develop in some psoriasis patients, may involve peripheral and axial (spondarthritis) joints and may lead to severe joint destruction. Genetic, immunologic and environmental (i.e., infectious agents or trauma) factors seem to play an important role in the onset and clinical appearance of PA. Although PA is a clinically monomorphic disease, it may show different heterogenous subgroups with differences in their etiopathogenesis. When PA is suspected, it is mandatory to analyze carefully the patient's familiar history, search attentively for the specific skin features, exclude a septic arthritis (especially if the involvement is monoarticular) and, in the cases of fulminant disease, consider always the possible coexistence of an acquired immunodeficiency syndrome. PA can occasionally be an aggressive, disfigurating and disabling disease and the treatment (incisive and precocious) should be similar to that for rheumatoid arthritis. At present, a definitive therapy does not yet exist, but the majority of PA patients can lead a fairly normal life and they do not show increased mortality rates (excluding the severe cases of erythrodermic or pustulosis psoriasis). However, as a result of the various problems of occupation and morbidity it causes, PA is a disease with great social involvement.

[A case of familial Bardet-Biedl syndrome (obesity, slight mental retardation, polydactyly, retinitis pigmentosum and renal failure) with insulin-resistant diabetes mellitus]. / Un caso di sindrome di Bardet-Biedl familiare (obesità, lieve ritardo mentale, polidattilia, retinite pigmentosa ed insufficienza renale) con diabete mellito insulino-resistente.

A case of familial Bardet-Biedl syndrome (BBS) in a 64-year-old woman is presented; it is characterized by abdominal obesity (BMI: 38.28; WHR: 0.98), slight mental retardation, polydactyly, pigmentary retinopathy and moderate renal failure, with insulin-resistant diabetes mellitus and severe inflammation of the left limb with necrosis of the last toe (the sixth) of the left foot. Four brothers and sisters of the patient presented the same syndrome. The patient had had healthy offsprings. The review of current literature indicates that BBS is a genetic autosomal recessive disease, formerly grouped with Laurence-Moon-Biedl syndrome but today considered as a separate entity. It is characterized by obesity, mental retardation, dysphormic extremities (syndactyly, brachydactyly or polydactyly), retinal dystrophy or pigmentary retinopathy, hypogonadism in males, and renal structural abnormalities or functional impairment. Extra- and intrafamilial variability of expressivity and severity of the various clinical manifestations was reported, among affected families and also in the same family. BBS is a rare but important syndrome, that should be known by the endocrinologist and the specialist in internal medicine, because it has an adverse prognosis, with early onset of blindness, insulin-resistant diabetes mellitus and severe renal impairment. Renal failure is a frequent cause of death early in life, even in the infant-juvenile years.