Rapidly progressive dementia due to neurosarcoidosis / Demência rapidamente progressiva causada por neurosarcoidose

Rapidly progressive dementia (RPD) is typically defined as a cognitive decline progressing to severe impairment in less than 1-2 years, typically within weeks or months. Accurate and prompt diagnosis is important because many conditions causing RPD are treatable. Neurosarcoidosis is often cited as an unusual reversible cause of RPD. METHODS: We report two cases of neurosarcoidosis presenting as RPD. RESULTS: Case 1: A 61-year-old woman developed a RPD associated with visual loss. In seven months she was dependent for self-care. Magnetic resonance imaging (MRI) revealed temporal and suprasellar brain lesions. Treatment with high-dose intravenous prednisolone was associated with partial improvement. Case 2: A 43-year-old woman who was being treated for diabetes insipidus developed a severe episodic amnesia one year after onset of cognitive symptoms. Previous MRI had shown a hypothalamic lesion and she had been treated with oral prednisone and cyclophosphamide. There was reduction of the MRI findings but no improvement in the cognitive deficits. Brain biopsy disclosed noncaseous granulomas and granulomatous angiitis; treatment was changed to high-dose intravenous methylprednisolone, with poor symptomatic response. CONCLUSION: The diagnosis of RPD due to neurosarcoidosis can be challenging when the disease is restricted to the nervous system. In these cases, clinical presentation of RPD associated with neuroendocrine and visual dysfunction, imaging findings showing hypothalamic lesions and, in some cases, brain biopsy, are the key to a correct diagnosis. It is possible that earlier diagnoses and treatment could have led to a better outcome in these patients.

Demência rapidamente progressiva (DRP) é tipicamente definida como um declínio cognitivo que progride para prejuízo funcional severo em menos de 1-2 anos, geralmente em semanas a meses. O diagnóstico rápido e acurado é fundamental, já que muitas condições que levam a DRP são reversíveis. MÉTODOS: Relatamos dois casos de neurosarcoidose que se apresentaram como DRP. RESULTADOS: Caso 1: Uma mulher de 61 anos desenvolveu uma DRP associada a perda de acuidade visual. Em sete meses evoluiu com dependência para auto-cuidado. A ressonância magnética (RM) revelou lesões encefálicas temporais e supraselares. Evoluiu com melhora parcial após tratamento com metilprednisolona intravenosa em altas doses. Caso 2: Uma mulher de 43 anos que estava em tratamento para diabetes insipidus desenvolveu uma amnésia episódica severa um ano após o início dos sintomas cognitivos. A RM anterior mostrava uma lesão hipotalâmica, e ela recebeu tratamento oral com prednisona e ciclofosfamida. Houve redução dos achados da RM, porem sem melhora dos déficits cognitivos. A biópsia cerebral mostrou granulomas não caseosos e angeíte granulomatosa; o tratamento foi modificado para metilprednisolona intravenosa em altas doses, com resposta sintomática pobre. CONCLUSÃO: O diagnóstico de DRP por neurosarcoidose pode ser desafiador quando a doença está restrita ao sistema nervoso central. Nestes casos, a apresentação clínica da DRP associada a disfunção neuroendócrina e visual, exames de imagem com lesões hipotalâmicas e, em alguns casos, a biópsia cerebral são fundamentais para um diagnóstico correto. é possível que o diagnóstico e tratamento precoces poderiam ter trazido melhores resultados nesses pacientes.

Rapidly progressive dementia due to neurosarcoidosis.

Rapidly progressive dementia (RPD) is typically defined as a cognitive decline progressing to severe impairment in less than 1-2 years, typically within weeks or months. Accurate and prompt diagnosis is important because many conditions causing RPD are treatable. Neurosarcoidosis is often cited as an unusual reversible cause of RPD. METHODS: We report two cases of neurosarcoidosis presenting as RPD. RESULTS: Case 1: A 61-year-old woman developed a RPD associated with visual loss. In seven months she was dependent for self-care. Magnetic resonance imaging (MRI) revealed temporal and suprasellar brain lesions. Treatment with high-dose intravenous prednisolone was associated with partial improvement. Case 2: A 43-year-old woman who was being treated for diabetes insipidus developed a severe episodic amnesia one year after onset of cognitive symptoms. Previous MRI had shown a hypothalamic lesion and she had been treated with oral prednisone and cyclophosphamide. There was reduction of the MRI findings but no improvement in the cognitive deficits. Brain biopsy disclosed noncaseous granulomas and granulomatous angiitis; treatment was changed to high-dose intravenous methylprednisolone, with poor symptomatic response. CONCLUSION: The diagnosis of RPD due to neurosarcoidosis can be challenging when the disease is restricted to the nervous system. In these cases, clinical presentation of RPD associated with neuroendocrine and visual dysfunction, imaging findings showing hypothalamic lesions and, in some cases, brain biopsy, are the key to a correct diagnosis. It is possible that earlier diagnoses and treatment could have led to a better outcome in these patients.

Demência rapidamente progressiva (DRP) é tipicamente definida como um declínio cognitivo que progride para prejuízo funcional severo em menos de 1-2 anos, geralmente em semanas a meses. O diagnóstico rápido e acurado é fundamental, já que muitas condições que levam a DRP são reversíveis. MÉTODOS: Relatamos dois casos de neurosarcoidose que se apresentaram como DRP. RESULTADOS: Caso 1: Uma mulher de 61 anos desenvolveu uma DRP associada a perda de acuidade visual. Em sete meses evoluiu com dependência para auto-cuidado. A ressonância magnética (RM) revelou lesões encefálicas temporais e supraselares. Evoluiu com melhora parcial após tratamento com metilprednisolona intravenosa em altas doses. Caso 2: Uma mulher de 43 anos que estava em tratamento para diabetes insipidus desenvolveu uma amnésia episódica severa um ano após o início dos sintomas cognitivos. A RM anterior mostrava uma lesão hipotalâmica, e ela recebeu tratamento oral com prednisona e ciclofosfamida. Houve redução dos achados da RM, porem sem melhora dos déficits cognitivos. A biópsia cerebral mostrou granulomas não caseosos e angeíte granulomatosa; o tratamento foi modificado para metilprednisolona intravenosa em altas doses, com resposta sintomática pobre. CONCLUSÃO: O diagnóstico de DRP por neurosarcoidose pode ser desafiador quando a doença está restrita ao sistema nervoso central. Nestes casos, a apresentação clínica da DRP associada a disfunção neuroendócrina e visual, exames de imagem com lesões hipotalâmicas e, em alguns casos, a biópsia cerebral são fundamentais para um diagnóstico correto. é possível que o diagnóstico e tratamento precoces poderiam ter trazido melhores resultados nesses pacientes.

Recurrent varicose veins of the legs. Analysis of a social problem.

The present study was aimed at assessing the experience of a single referral center with recurrent varicose veins of the legs (RVL) over the period 1993-2008. Among a total of 846 procedures for Leg Varices (LV), 74 procedures were for RVL (8.7%). The causes of recurrence were classified as classic: insufficient crossectomy (13); incompetent perforating veins (13); reticular phlebectasia (22); small saphenous vein insufficiency (9); accessory saphenous veins (4); and particular: post-hemodynamic treatment (5); incomplete stripping (1); Sapheno-Femoral Junction (SFJ) vascularization (5); post-thermal ablation (2). For the "classic" RVL the treatment consisted essentially of completing the previous treatment, both if the problem was linked to an insufficient earlier treatment and if it was due to a later onset. The most common cause in our series was reticular phlebectasia; when the simple sclerosing injections are not sufficient, this was treated by phlebectomy according to Mueller. The "particular" cases classified as 1, 2 and 4 were also treated by completing the traditional stripping procedure (+ crossectomy if this had not been done previously), considered to be the gold standard. In the presence of a SFJ neo-vascularization, with or without cavernoma, approximately 5 cm of femoral vein were explored, the afferent vessels ligated and, if cavernoma was present, it was removed. Although inguinal neo-angiogenesis is a possible mechanism, some doubt can be raised as to its importance as a primary factor in causing recurrent varicose veins, rather than their being due to a preexisting vein left in situ because it was ignored, regarded as insignificant, or poorly evident. In conclusion, we stress that LV is a progressive disease, so the treatment is unlikely to be confined to a single procedure. It is important to plan adequate monitoring during follow-up, and to be ready to reoperate when new problems present that, if left, could lead the patient to doubt the validity and efficacy of the original treatment.

[Penal liability from retained foreign body inside the surgical site]. / La responsabilità penale da derelizione di corpo estraneo in sito chirurgico.

The Authors focus on the liability of the surgery team members in the case they inadvertently forget behind in the patient's body a foreign object, which causes injuries and/or death. The Authors underline that, according to the current case law regarding medical malpractice, both the main surgeon and their assistant/subordinate are liable for engaging in a markedly imprudent and/or negligent conduct, such as not double-checking scrupulously the surgical site before its closure in order to highlight forgotten foreign bodies. As well, the Authors underline that either the circulator nurse or the theatre nurse can be considered punishable by law when that medical error occurs, even if they are responsible for the count of the instruments used in the course of the surgery. Conversely, the main surgeon and his or her assistant are always directly responsible, due to the fact that the nurses' count procedure represents merely an additional control measure, without substituting at all the check the surgeons must obligatory conduct on the surgical site. Finally, the Authors point out that, as the count procedure is performed by the members of a surgical team, where a hierarchy-based relationship rules, the main surgeon is the liable for any preventable and avoidable adverse event provoked by the nursing staff as a consequence of the objective responsibility due to culpa in eligendo and culpa in vigilando.

[Consideration on the treatment of chronic occlusive lesions of the iliac arteries]. / Considerazioni sul trattamento delle lesioni arteriose ostruttive croniche dell'asse iliaco.

This study addresses the problem of the best treatment for chronic unilateral obstructive lesions of the aorto-iliac arterial axis. After making a review of the literature, some considerations are made. In recent years this disease has increasingly been treated by endovascular methods with dilation (angioplasty) and stents. However, the classic indications (short stenotic obstruction), supported by national and international guidelines, are being continually widened by operators acquiring growing skills with this method. Nevertheless, no long term studies have demonstrated a clear superiority, in severe cases, of endovascular methods over traditional surgical methods (by-pass), as regards either long term results or costs. Among the traditional methods, many surgeons prefer the femoro-femoral by-pass to the orthopic aorto-femoral by-pass, particularly because of its lesser invasiveness. However, it should be noted that comparisons of the two methods have yielded results in favour of the orthotopic procedure, above all about long term results.

Ultrasound duplex as a sole exam for surgical purposes in lower limb arterial obstructive disease.

BACKGROUND: The aim of the research is to value the efficacy of ultrasound duplex as a sole preoperative exam in peripheral Arterial Obstructive Disease (PAOD). Angiography is undoubtedly known as the gold standard, though its risks and complications. In the last decades duplex scanning and its variant, has proven to be an irreplaceable tool in the diagnosis of these disease. METHODS: We evaluated five previous studies that compare angiography to duplex scanning. RESULTS: Out of 273 cases, 207 (73.8%) had a good correspondence between the two methods; besides if we consider each arterial tract duplex reliability is better in distal diseases (159 cases out of 200, 79.5%) than in proximal (48 out of 73.65%) contrasting with an other meta-analytic evaluation made by Koelemay et al. Moreover we wanted to verify our personal experience, by the intention to treat method, if the surgical operation selected for our patients would have been the same based only on pre-operative echo-duplex scanning. After the analysis of about 20 files the research was interrupted because there was a perfect correspondence between the two exam only in three cases, all of them re-operation for graft branches obstruction. The lacks of duplex has been: 1) non identification of important lesions on the aorto-iliac tract; 2) insufficient location of distal disease. CONCLUSIONS: The angiography results the fundamental choice in the treatment of PAOD particularly in the distal tracts. Debatable is the role of echography in aorto-iliac portion, where a number of lesions may be effectively treated by endovascular procedures.

A randomised, double masked, clinical trial of high dose vitamin A and vitamin E supplementation after photorefractive keratectomy.

AIM: To evaluate the effect of a high dose vitamin A and E supplementation on corneal re-epithelialisation time, visual acuity and haze following photorefractive keratectomy (PRK). METHODS: Two groups of 20 patients who underwent myopic PRK were supplemented with either 25 000 IU retinol palmitate and 230 mg alpha tocopheryl nicotinate or a placebo. Clinical outcomes were evaluated up to 360 days. RESULTS: In the vitamin treated group, re-epithelialisation time was significantly faster (p = 0.029) and haze incidence was reduced (p = 0.035), especially for high myopic corrections (p = 0.043). This group also reported a significantly better uncorrected visual acuity (p = 0.043). CONCLUSIONS: High dose vitamin A and E oral supplementation may accelerate re-epithelialisation time and may reduce corneal haze formation after PRK.

[Traumatic retroperitoneal lesions]. / Lesioni traumatiche retroperitoneali.

The presence of lesions on the retroperitoneum generally worsens the prognosis in traumatic pathology; it implies more attention and skills from both the medical and surgical aspect. All type of trauma, blunt or open, may involve retroperitoneal structures and organs; specifically there may be lesions on the great vessels, pancreas, duodenum, oesophagus and genitourinary apparatus. Mortality is high, compared to abdominal traumatic lesions confined within the peritoneal sac. Treatment of single or associated lesions requires a multidisciplinary approach, as the surgical repair implies a specific knowledge and experience on different organs, whose habitual pathology lies on the hands of more surgical specialists. Lesions of great vessels are immediately life-threatening; moreover the choice to "open" a patient for a retroperitoneal hematoma has to be taken upon a careful estimation. It could be better in more than a situation leave such hematoma in its place, specially in the iliac region, waiting for the spontaneous resolution of the hemorrhagic source and of the hematoma itself. The involvement of oesophagus, duodenum or pancreas determines instead a poorer prognosis at a distance. In conclusion retroperitoneal traumatic lesions are among the most challenging and serious emergencies, and necessitate a maximum of attention and expertise by the surgical team involved. Only with a careful judgement about the tactics and the procedures to carry on it is possible to obtain valid results, which often means to safe the patients life.

Surgery of abdominal aorta in octogenarians. Can indications be extended?

BACKGROUND: This paper describes the authors' experience with the management of the abdominal aorta in patients aged over 80 years. METHODS: Ten urgent procedures were performed on patients older than 80 years during a 2 year period. In 4 cases surgery was performed because of a ruptured aneurysm of the subrenal abdominal aorta, in 2 cases for active symptomatic aneurysms, in 3 cases for severe lower limb ischemia (occlusion of the iliac and femoral arteries) and in 1 case for a secondary aortoenteric fistula. RESULTS: The operative mortality rate was 20% (2 cases with a ruptured aneurysm). Five patients are still alive in good health conditions (one of them had been operated twice for two different diseases). Even if our findings refer to a small number of patients, although similar series on emergency operations found in the literature are not substantially larger, the results do not advise against operative treatment of the abdominal aorta in cases requiring a direct approach, even in patients over 80 years of age. CONCLUSIONS: If this treatment strategy is obviously adopted in emergency conditions, as with the patients we are reporting on, since the alternative to operation is usually death, it should also be carefully considered in elective circumstances, where alternative treatments such as endovascular stents did not to date obtain better results. In the elective scenario all the necessary biological and physical parameters as well as the patient's age should be taken into proper account in deciding whether to operate. This is specially true now that the average life spans of an individual is longer so that patients, who may incur serious problems if left untreated, may be offered a better quality of life.

Restoration of the pelvic circulation in patients with abdominal aortic aneurysms receiving aortobifemoral grafts.

When operating on abdominal aortic aneurysms associated with stenoses or occlusions of iliac vessels, surgeons may face the problem of reestablishing circulation to pelvic or gluteal territories. A new technique consists of anastomosing a posterior opening in the body or one of the branches of a bifurcated graft, distally sutured to the femoral artery, to the distal aortic stump, which contains all the patent vessels arising from the end of the aorta, such as inferior mesenteric and lumbar arteries. This technique, successfully performed in two cases, has the advantages of avoiding closure of the distal aortic stump and a possible backflow leak and of ensuring adequate pelvic circulation.

[Did the arrival of prostanoids modify therapeutic indications in peripheral obstructive arteriopathies? Considerations based on our experience]. / L'avvento dei prostanoidi ha modificato le indicazioni terapeutiche nell'arteriopatia periferica ostruttiva (PAOD)? Considerazioni sulla base della nostra esperienza.

Prostaglandins, particularly PGE1, are now widely used in PAOD as they act on the balance of microcirculation and endothelial function. The Authors report their experience in 21 patients (19 males, 2 females; median age 64) treated with PGE1, 80 micrograms per day i.v. and subcutaneous heparin 0.2 ml twice a day and followed from September 1993 to March 1995. Twenty patients were affected by PAOD; in this group 8 (5 diabetics) were suffering from claudicatio intermittens, II from critical ischaemia, while 1 patient had thromboangiitis obliterans. One of the 8 claudicating patients did not complete the protocol because he underwent a femoropopliteal bypass, while the other 7 experienced a significant and stable improvement (two of these walk quite freely). Only 7 of the 11 patients with C.L.I. received a medical treatment alone; 4 had healing of necrotic ulcers and in the other 3 the treatment was stopped because of the onset of severe hypertension or because they were non-responders. In 4 patients with C.L.I. PGE1 was associated with a surgical revascularization procedure, and its role has to be better defined. From the analysis of the results reported treatment with PGE1 may be an important step in Fontaine class IIb patients, before planning a surgical approach. Also in most cases of C.L.I. it proved its efficacy, however, the initial therapeutic option (either medical or surgical) should be evaluated in each single case.

Plasma lipids as a risk factor in peripheral vascular disease.

Plasma lipids were tested in 59 patients with symptomatic peripheral vascular disease (PVD) (confirmed by angiographic and, in many cases, operative examinations) and compared with the lipid balance in 47 nonarteriopathic subjects constituting the control group. Of all the elements considered, only the hypertriglyceridemia and the fall in the HDL cholesterol/total cholesterol ratio showed a statistically significant difference between the two groups. In particular, there was a significant difference between the two groups. In particular, there was a significant difference in the triglyceridemia present in the arteriopathic patients, as evidenced by the double check afforded by the frequency test (PVD: 25/59; control: 8/47; p less than 0.01) and the averages test (PVD: 201 +/- 131; control: 138 +/- 98; p less than 0.01).