Forniceal rupture secondary to ureterocele and increased urine flow

A case of a simple ureterocele presenting with spontaneous forniceal rupture is described. Rupture followed hypophysectomy for Cushing's disease and high urine outflow. This presentation has not been reported previously.     

Endovascular treatment of a superior mesenteric artery aneurysm secondary to Behcet's disease with Onyx (ethylene vinyl alcohol copolymer)

Behcet’s disease is a complex multisystemic chronic inflammatory disease that is characterized by oral and genital aphtous ulcers and vasculitis. Aneurysms of major arteries are the most important cause of mortality in Behcet’s disease. Four patients with superior mesenteric artery (SMA) aneurysms related to Behcet’s disease have been reported in the literature. We report here the first successful endovascular treatment of a giant, wide-necked SMA aneurysm secondary to Behcet’s disease. We performed a balloon-assisted embolization technique using ethylene vinyl alcohol copolymer (Onyx, ev3, Irvine, CA, USA). There were no signs of recurrence during 2-year follow-up.     

Adrenal incidentalomas showing unilateral concordant visualization by adrenocortical scintigraphy: Comparison with adenomas in Cushing’s syndrome

An adrenocortical adenoma causing Cushing's syndrome (Cushing's adenoma) produces a unilateral concordant visualization (UCV) imaging pattern in which the adenoma is only visualized on radioiodocholesterol adrenocortical scintigraphy. But because this imaging pattern is also noted in some patients with adrenal incidentalomas, we examined whether the UCV-incidentaloma was essentially identical with Cushing's adenoma and would develop Cushing's syndrome. The subjects were 9 patients with UCV-incidentalomas (mean size, 30 mm; range, 20-45 mm) and 6 patients with Cushing's adenomas (mean size, 28 mm; range, 25-35 mm). Endocrinological evaluations showed several abnormalities including blunted diurnal rhythm of plasma cortisol within the normal range, low plasma ACTH and/or high 24-hr urinary 17-OHCS levels in 8 of 9 patients with UCV-incidentalomas, but these abnormalities did not meet the diagnostic criteria of Cushing's syndrome. Adrenal uptake of the tracer in the patients with UCV-incidentalomas was not statistically different from that in the patients with Cushing's adenomas and had no relationship with hormonal values in either patient group. Tumor size on CT correlated with the levels of 24-hr urinary 17-OHCS (r = 0.75, p = 0.02) and plasma cortisol at 7:00 (r = 0.82, p = 0.007) in the patients with UCV-incidentalomas, but not in the patients with Cushing's adenomas. Although 3 UCV-incidentalomas increased slightly in size, none of 9 patients with UCV-incidentalomas has developed Cushing's syndrome for 4 to 52 months. These results suggest that the UCV-incidentaloma may be essentially different from the Cushing's adenoma and unlikely to develop Cushing's syndrome.     

Regional cerebral blood flow in Alzheimer's disease: comparison between short and long-term donepezil therapy

OBJECTIVE: Treatment with donepezil improves cognitive function of patients with Alzheimer's disease (AD) when compared to a placebo-controlled group. The purpose of this study was to investigate changes in regional cerebral blood flow (rCBF) of AD patients in short-term and long-term treatment with donepezil. METHODS: rCBF was measured by N-isopropyl-p-123I-iodoamphetamine (IMP) autoradiography method. CBF measurements were performed in 17 AD patients before treatment and after 3 months (short-term therapy) and 1 year (long-term therapy). Regions of interest were set at cerebral cortex and cerebellar hemisphere. We used absolute CBF and relative CBF expressed as ratio to cerebellar CBF. RESULTS: Significant increases in relative rCBF were noted in the frontal, parietal and temporal lobes at the end of short-term therapy. rCBF was decreased after the long-term therapy, whereas rCBF was still increased to a slight extent, as compared with the pre-treatment levels. Absolute rCBF showed minimal change and a tendency to decline. CONCLUSION: Relative rCBF significantly increased in the short-term donepezil therapy, while following the long-term therapy, rCBF decreased to the pre-treatment level.     

The Current Role of Venous Sampling in the Localization of Endocrine Disease

Endocrine venous sampling plays a specific role in the diagnosis of endocrine disorders. In this article, we cover inferior petrosal sinus sampling, selective parathyroid venous sampling, hepatic venous sampling with arterial stimulation, adrenal venous sampling, and ovarian venous sampling. We review their indications and the scientific evidence justifying these indications in the diagnosis and management of Cushing’s syndrome, hyperparathyroidism, pancreatic endocrine tumors, Conn’s syndrome, primary hyperaldosteronism, pheochromocytomas, and androgen-secreting ovarian tumors. For each sampling technique, we compare its diagnostic accuracy with that of other imaging techniques and, where possible, look at how it impacts patient management. Finally, we incorporate venous sampling into diagnostic algorithms used at our institution.     

Imaging in covert ectopic ACTH secretion: a CT pictorial review

The syndrome of ectopic adrenocorticotrophin secretion (EAS) is rare and is due to excess adrenocorticotrophin (ACTH) production from a nonpituitary tumour. These tumours can be covert, where the tumours are not readily apparent, and very small making them challenging to image. It is clinically and biochemically difficult to distinguish between covert EAS and Cushing’s disease. The first-line investigation in locating the source of ACTH production is computed tomography (CT). The aim of this pictorial review is to illustrate the likely covert sites and related imaging findings. We review the CT appearances of tumours resulting in covert EAS and the associated literature. The most common tumours were bronchial carcinoid tumours, which appear as small, well-defined, round or ovoid pulmonary lesions. Rarer causes included thymic carcinoids, gastrointestinal carcinoids and pancreatic neuroendocrine tumours. Awareness of the imaging characteristics will aid identification of the source of ACTH production and allow potentially curative surgical resection.     

Vascular rupture—a rare lethal complication of neurofibromatosis v. Recklinghausen

Neurofibromatosis type 1 (NF−1), a phacomatosis with an incidence of 1:3,000–4,000, is characterized by multiple benign nerve tumors, skin café-au-lait spots and a variety of other dysplastic abnormalities, e.g., of blood vessels. On rare occasions NF−1 is associated with arterial dysplasia (vascular neurofibrosis), which is poorly defined and can result, e.g., in stenosis, rupture, arterio-venous fistula, or the formation of aneurysms. Although a rare feature of neurofibromatosis, haemorrhage, e.g., after minimal trauma, is a potentially lethal complication of this disease. We report the case of a 40-year-old woman with a history of NF−1 who collapsed gasping for air and died subsequently. Autopsy revealed a massive cervicomediastinal haematoma with compression of the neck region due to a vessel rupture without an adequate trauma. This fatality demonstrates that, in rare cases of NF−1 patients, the possibility of internal haemorrhage due to vessel rupture being the cause of death should be taken into consideration in medicolegal investigations even as a spontaneous event in the absence of an adequate blunt trauma.     

Embolization of Hepatic and Adrenal Metastasis to Treat Cushing’s Syndrome Associated with Medullary Thyroid Carcinoma: A Case Report

We report functionally successful hepatic and left adrenal embolization with particles to treat Cushing’s syndrome associated with a medullary thyroid carcinoma.     

光动力疗法治疗脉络膜裂伤伴中心凹下新生血管的疗效观察

目的观察光动力疗法治疗脉络膜裂伤伴中心凹下新生血管的临床效果。方法经矫正视力、裂隙灯显微镜、眼底、荧光素眼底血管造影和吲哚菁绿血管造影检查确诊为黄斑区脉络膜裂伤伴脉络膜新生血管患者9例9只眼。静脉注射维替泊芬6 mg/m2,15 min后以波长689 nm、功率密度600 mW/cm2、能量密度50 J/cm2、时间83 s激光照射病灶,随访12个月。对比分析治疗前后患者的视力、病灶大小和脉络膜新生血管渗漏变化。结果 3只眼视力提高,6只眼视力稳定,无视力下降。荧光素眼底血管造影联合吲哚菁绿血管造影检查显示,3只眼渗漏消失或减轻,病灶范围缩小,6只眼渗漏无明显变化,所有患眼未见渗漏扩大。治疗后1个月脉络膜出血明显吸收,3个月出血完全吸收,病灶边缘色素沉着,12个月所有患者脉络膜新生血管渗漏未见进一步扩大,无再次出血和病灶纤维化。结论光动力疗法可作为治疗脉络膜裂伤伴中心凹下新生血管的选择。     

Wilson’s disease: cranial MRI observations and clinical correlation

Introduction Study of MRI changes may be useful in diagnosis, prognosis and better understanding of the pathophysiology of Wilson’s disease (WD). We aimed to describe and correlate the MRI abnormalities of the brain with clinical features in WD.Methods MRI evaluation was carried out in 100 patients (57 males, 43 females; mean age 19.3±8.9 years) using standard protocols. All but 18 patients were on de-coppering agents. Their history, clinical manifestations and scores for severity of disease were noted.Results The mean duration of illness and treatment were 8.3±10.8 years and 7.5±7.1 years respectively. MRI of the brain was abnormal in all the 93 symptomatic patients. The most conspicuous observations were atrophy of the cerebrum (70%), brainstem (66%) and cerebellum (52%). Signal abnormalities were also noted: putamen (72%), caudate (61%), thalami (58%), midbrain (49%), pons (20%), cerebral white matter (25%), cortex (9%), medulla (12%) and cerebellum (10%). The characteristic T2-W globus pallidal hypointensity (34%), “Face of giant panda” sign (12%), T1-W striatal hyperintensity (6%), central pontine myelinosis (7%), and bright claustral sign (4%) were also detected. MRI changes correlated with disease severity scores (P<0.001) but did not correlate with the duration of illness.Conclusion MRI changes were universal but diverse and involved almost all the structures of the brain in symptomatic patients. A fair correlation between MRI observations and various clinical features provides an explanation for the protean manifestations of the disease.     

The incidence of hyperthyroidism in Austria from 1987 to 1995 before and after an increase in salt iodization in 1990

Between 1963 and 1990, Austria had iodized salt prophylaxis of endemic goitre with 10 mg KI (7.5 mg I) per kg. This was obviously insufficient, as urinary iodine excretion ranged from 42 to 78 μg I per g of creatinine and goitre in adults remained in the endemic range of 15%–30%. Therefore salt iodization was doubled in 1990. The aim of this study was to assess the annual incidence of different types of hyperthyroidism (HT) before and after this increase in salt iodization. The incidence of HT was recorded in 14 nuclear medicine centres from 1987 to 1995. In five additional centres data were available from 1992 onwards. Data prior to 1992 were documented retrospectively, while those after 1992 were recorded prospectively. The 14 centres drew patients from an area with a population of approximately 4.23 million while all 19 institutes were estimated to cover an area with a population of 5.4 million (the total population of Austria is 7.86 million). A total of 414232 persons were examined for the first time in the participating centres. HT and the type of HT were defined by clinical examination, serum TSH, thyroid hormone levels in blood, ultrasonography, scintigraphy and serum autoantibody titres. HT was classified into immunogenic HT (Graves’ or Basedow’s disease, GD) and HT with intrinsic thyroid autonomy (uni-, multinodular or disseminated Plummers’ disease, PD). HT was also divided into overt (o) or subclinical (sc) disease. The following data were calculated: annual incidence per 100000 and the relative risk (RR) for HT with 95% confidence intervals (CI). In addition, linear trends were calculated for each type of HT by means of logistic regressions. In the 19 centres a total of 47834 patients with HT were registered from 1987 to 1995. PD accounted for 75% of all cases of HT and GD for 19%, while other types of HT were present in 6%. From 1987 to 1989 (time period T0), the annual incidence of oPD was 30.5 (95% CI 29.6-31.5) per 100000. The RR compared to the baseline period T0 was highest in 1992 (1.37; 1.3-1.45) and decreased to 1.17 (1.1-1.24) in 1995. The annual incidence of scPD in T0 was 27.4 (26.5–28.3) per 100000. The RR was highest in 1991 (1.64; 1.56-1.73) and was 1.60 (1.51–1.69) in 1995. In oPD and scPD a higher RR was observed in persons older than 50 years of age, particularly in men. The incidence of oGD in T0 was 10.4 (9.8–10.9) per 100000; the maximum RR increased to 2.19 (2.01-2.38) in 1993 and decreased to 1.95 (1.78–2.13) in 1995. The incidence of scGD was 1.9 (1.6-2.1) in T0. The maximum RR was observed in 1994 (2.47; 2.04-3.0) and it was still 2.26 (1.85–2.77) in 1995. The increased incidence of oGD and scGD was evenly distributed in all ages and both sexes. The time course of different types of HT following the increase in salt iodization could be divided into two phases: an increase in the incidences of HT with peaks after 1–4 years and a subsequent decrease, the only exception being scGD. The effect was more pronounced in GD than in PD. PD showed an age and gender dependency over time, while GD did not. Received 1 December and in revised form 15 December 1997     

Chondrosarcoma as a complicating factor in Paget’s disease of bone

 A 65-year-old male patient with an 8-year history of poliostotic Paget’s disease complained of shoulder pain that started 6 months prior to admission. An extensive lytic area was identified in the right proximal humerus along with Paget’s disease. There was cortical destruction and a soft tissue mass. Following an incisional biopsy, a diagnosis of grade 2 chondrosarcoma associated with Paget’s disease was made. The histologic identification of chondrosarcoma associated with Paget’s disease is rare. However, the presence of a calcified matrix in a destructive lesion associated with Paget’s disease should alert the radiologist and the pathologist to the possibility of a chondromatous differentiation taking place in the sarcoma associated with Paget’s disease. The histologic evaluation of the lesion will form the basis for the diagnosis.     

MR imaging of pseudosarcoma in Paget’s disease of bone: a report of two cases

Pseudosarcoma is a rare manifestation of Paget’s disease of bone. We report the MR imaging of two cases highlighting the difficulties in diagnosis. One of the cases is the first time this condition has been described outside the long bones of the lower limb. Received: 11 July 2000 Revision requested: 10 August 2000 Revision received: 8 November 2000 Accepted: 15 November 2000     

Renal arterial rupture secondary to percutaneous transluminal angioplasty treated without surgical intervention

A case of nonsurgical treatment of renal arterial rupture secondary to balloon angioplasty is reported.     

Decreased striatal D2 receptor density associated with severe behavioral abnormality in Alzheimer’s disease

Objectives: Since patients manifesting behavioral and psychological symptoms of dementia (BPSD) are a burden for their families and caregivers, the underlying neurobiological mechanism of this condition should be clarified. Using positron emission tomography (PET), we previously reported that wandering behavior in dementia was associated with a disturbed dopaminergic neuron system. We herein investigated the relationship between the severity of BPSD and the striatal D₂ receptor density in Alzheimer’s disease (AD).Methods: Ten patients with probable AD as per the National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) and the AD and Related Disorders Association (ADRDA) criteria and five normal subjects were examined with PET. The tracer used was [¹¹C]raclopride (D₂ antagonist). The uptake of [¹¹C]raclopride was calculated as the estimation of binding potential (BP) of the striatum to the cerebellum. The D patients were institutionalized in multiple nursing homes, and their BPSD were evaluated by the Behavioral Pathology in AD Frequency Weighted Severity Scale (BEHAVE-AD-FW) scale (Reisberg).Results: There was a significant inverse Spearman’s correlation between BEHAVE-AD-FW score and the BP, especially between the score of the behavioral domain and the BP values. The BP was found to be lower in severer BPSD patients.Conclusions: Patients with AD who manifest severe BPSD may have some dysfunction of striatal dopamine metabolism compared with those without BPSD.     

Synchronous Paget’s sarcoma of tibiae in which Paget’s disease was limited to these bones

A 51-year-old native of Rio de Janeiro presented with bilateral synchronous Paget’s sarcomas in the tibiae, which developed in the upper right tibia and in the distal third of the left tibia. There were no other areas of Paget’s disease. The largest tumor spread to the right inguinal nodes and also soft tissue. The tumor in the left tibia spread dramatically in the soft tissues up the leg and only involved the medullary cavity in its inferior portion. The patient died, but there was no autopsy. Comments are made about the prevalence of Paget’s disease in Rio de Janeiro. Received: 8 April 1999 Revision requested: 10 May 1999 Revision received: 23 June 1999 Accepted: 1 July 1999     

T1-weighted MR imaging for distinguishing large osteolysis of Paget's disease from sarcomatous degeneration

Objective. To report five symptomatic patients, four with unequivocal Paget’s disease and large areas of osteolysis and one patient with presumed osteolytic Paget’s disease, evaluated by MR imaging to confirm or exclude a sarcoma. Design and patients. Four men and one woman (median age 74 years) presented with new symptoms of pain. Four of these patients had unequivocal Paget’s disease with large areas of osteolysis; one patient presented with large focal osteolysis and no other finding. MR imaging was performed in each case to exclude malignancy in the area of osteolysis. Results. Two patients whose MR images showed a low signal abnormality on the T1-weighted sequence corresponding to osteolysis on the radiograph were found to have malignant degeneration. Three patients with osteolytic lesions on T1-weighted MR imaging showed preservation of fat signal in the areas of osteolysis, were not biopsied and have been free of malignant disease for from 12 months to 2¹/₂ years. One patient had one area of osteolysis in the iliac bone which showed malignancy and another area of osteolysis which showed preservation of fat signal on the T1-weighted sequence. Conclusions. The information obtained from T1-weighted MR imaging sequences performed on patients with Paget’s disease who have new symptoms and large areas of osteolysis could reliably be used in the clinical decision-making process between conservative follow-up and biopsy. Received: 26 December 2000 Revision requested: 16 January 2001 Revision received: 05 February 2001 Accepted: 07 February 2001     

Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a complex disease entity comprised of three distinct clinical syndromes that demonstrate indistinguishable histology. These syndromes are: eosinophilic granuloma, which is predominantly osseous or pulmonary; Hand-Schûller-Christian’s disease, which involves multiple organ systems and, most typically, the skull base; and Letterer-Siwe’s disease, the most severe disease manifestation, which typically involves the abdominal viscera. This article reviews our current understanding of Langerhans cell histiocytosis by discussing the history, histology, etiology, and treatment of the disease. It focuses on the radiographic findings and imaging modalities that are the most useful in disease diagnosis and management.     

Duplex ultrasound findings in men with peyronie’s disease

Peyronie’s disease is a fibrotic process involving the penis, which presents with painful erections, penile chordee, and, less frequently, impotence. Duplex Doppler ultrasound is particularly well suited to the evaluation of these patients, since it can provide information about Peyronie’s plaque and the penile vasculature noninvasively. Duplex ultrasound examinations were performed on 30 impotent and three potent men with Peyronie’s disease. Presenting symptoms included painful erections in 30% and abnormal curvature in 93%. A plaque was palpable in 31 (94%) of the patients. Ultrasound evaluation revealed evidence of a plaque in only 13 (39%) patients. The majority of these plaques were visualized as hyperechoic areas with or without shadowing. Some isoechoic plaques were identified due to significant thickening of the dorsal tissues. Doppler analysis of cavernosal artery blood flow revealed evidence of arterial disease in eight (27%) of the impotent patients. Although ultrasonography was not as sensitive as palpation in identifying Peyronie’s plaques, ultrasonography was better able to determine the depth of fibrosis extension into the corpora.     

Myonecrosis of Behcet’s disease

Behcet’s disease is an inflammatory disease of unknown cause characterized by intermittent episodes of acute inflammation manifested by oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. We report a rare case of myonecrosis associated with Behcet’s disease. Myonecrosis of Behcet’s disease can mimic soft tissue abscess and therefore awareness of this entity in the appropriate clinical setting is important for initiation of appropriate and timely treatment.