Felipe Welter Langer

Low health related quality of life associated with fractures in obese postmenopausal women in Santa Maria, Brazil

Although health-related quality of life is well studied in subjects with obesity or fractures, there are few studies approaching both diseases together. The aim of this study was to evaluate the health-related quality of life (HRQL) in obese postmenopausal women with fractures. A cross-sectional study was carried out at Santa Maria, Brazil. Postmenopausal women aged 55 years or older were recruited from March 1st to August 31st, 2013. Women with cognitive impairment were excluded. The Short-Form Health Survey (SF-36) were applied (QM0 16,471). Height and weight were measured according to the World Health Organization protocol. Bone fractures (excluding hand, feet, and head) that occur after age 45 years were considered as the outcome. Of the 1057 women allocated to study, 975 had their weight and height measured. Obese women with fractures had significantly lower SF-36 physical component scores when compared with non-obese subjects with fracture, obese subjects without fractures, and non-obese non-fracture subjects. Both obesity and fractures were independently associated with a lower SF-36 physical component score in the regression model. In conclusion, fractures appear to have an adverse effect on quality of life which is more pronounced in obese postmenopausal women.

Hemichorea-hemiballism: the role of imaging in diagnosing an unusual disorder in patients with nonketotic hyperglycemia.

An 81-year-old man presented to the emergency room with a 4-day history of progressive confusion followed by frontal headache and left-sided choreiform movements. His medical history was remarkable for smoking, dyslipidemia, and poorly-controlled hypertension, with no previous diagnosis of diabetes mellitus (DM). On laboratory investigation, his serum glucose was 460 mg/dL and his glycated hemoglobin was 17.4% (consistent with a prolonged period of undiagnosed DM). A computed tomography scan of the brain revealed hyperdensity of the right putamen without an associated mass effect (Figure 1), which suggested a diagnosis of hyperglycemic hemichorea-hemiballism (HCHB). The patient was started on insulin, and few hours following glucose correction there was great improvement in his mental status and a decrease in involuntary movements. On an unenhanced T1weighted spin-echo magnetic resonance imaging (MRI) sequence obtained two weeks after initial presentation, there were hyperintense lesions, consistent with hyperglycemic HCHB, located in right putamen. Diffusion-weighted imaging showed no corresponding signal alterations. T2*-weighted imaging demonstrated bilateral punctiform hypointensities in the globus pallidus, which were presumably physiological in nature and did not match the unilateral T1 abnormality (Figure 2). The patient completely recovered his previous cognitive and motor functions after glycemic control, being discharged without sequelae. Ballistic and choreic movements are characterized by hyperkinetic, random, involuntary movements in the proximal and distal extremities, respectively. Because they usually occur concomitantly, the term HCHB was created to unify these signs into a clinical syndrome when presented unilaterally. Although HCHB syndrome is secondary to lesions in the basal ganglia, the source of the neuronal damage is controversial, the putative mechanisms including disruption of the blood-brain barrier, decreased thalamic gamma-aminobutyric acid input secondary to anaerobic metabolism, small hemorrhages in the striatal region, hyperviscosity related to hyperglycemia, and Wallerian degeneration of putaminal white matter with protein desiccation. Vascular cerebral lesions constitute the most common cause of HCHB. Hyperglycemia is considered an important, albeit rare, risk factor for the development of HCHB, which is most commonly seen in elderly female patients with uncontrolled DM. The predominance of Asian patients in the published data suggests an ethnic predisposition. The clinical course tends to vary depending on the patient’s glycemic status—the hemiballism and hemichorea usually start together with the hyperglycemia, resolving after its correction. Computed tomography findings of hyperglycemic HCHB include unilateral hyperdensity in the basal ganglia contralateral to the affected site. On T1-weighted MRI scans, the most common finding is signal hyperintensity in the caudate nucleus and putamen, usually sparing the internal capsule. The apparent diffusion coefficient and diffusion-weighted MRI generally indicate

Posterior reversible encephalopathy syndrome as a complication of Henoch–Schönlein purpura in a seven-year-old girl

Introduction Henoch–Schönlein purpura is a multisystem small vessel vasculitis. Neurologic manifestations are uncommon. Posterior reversible encephalopathy syndrome is a rare complication of Henoch–Schönlein purpura with typical clinical and neuroimaging findings that occurs most commonly in the setting of severe hypertension and renal injury. Case presentation A seven-year-old girl was admitted to our institution presenting with clinical and laboratory findings suggestive of Henoch–Schönlein purpura. Glucocorticoid therapy was initiated, but five days following her admission, she developed altered consciousness, seizures, arterial hypertension, and cortical blindness. Brain MRI scan revealed areas of vasogenic oedema in parieto-occipital lobes, consistent with posterior reversible encephalopathy syndrome. She was immediately initiated on antihypertensives and antiepileptics, which successfully improved her neurologic symptoms. Further laboratory work-up disclosed a rapidly progressive glomerulonephritis secondary to Henoch–Schönlein purpura that was the likely cause of her sudden blood pressure elevation. Immunosuppressive therapy was undertaken, and at one-year follow-up, the patient exhibited complete renal and neurologic recovery. Conclusion Posterior reversible encephalopathy syndrome is a severe complication of Henoch–Schönlein purpura. If promptly diagnosed and treated, children with Henoch–Schönlein purpura presenting with posterior reversible encephalopathy syndrome usually have a good prognosis. Clinicians should be familiar with the characteristic presentation of posterior reversible encephalopathy syndrome and be aware that hypertension and renal injury may predispose Henoch–Schönlein purpura patients to developing this complication.

The role of imaging in diagnosing an unusual manifestation of neurofibromatosis type 1: calvarial dysplasia

A 25-year-old man was referred to our institution for investigation of a one-year history of gradually developing nodules covering his skin. His medical history was unremarkable. On physical examination, multiple cutaneous nodules were noted, as were café au lait spots, bilateral Lisch nodules (Figure 1A), and axillary freckles (Figure 1B). The results of neurologic and fundoscopic examinations were unremarkable. The father of the patient, who was also examined, presented with similar cutaneous nodules. Those findings met the criteria for neurofibromatosis type 1 (NF1), which had previously gone undiagnosed. To determine the extent of the newly diagnosed NF1, computed tomography (CT) of the head was performed. The CT scan revealed an unsuspected left-sided discontinuity in the occipital bone along the left lambdoid suture (Figures 1C and 1D), measuring 3.1 × 2.7 cm. There were no signs of brain herniation through the bone aperture, and we detected no neurofibromas over the bone defect on the clinical examination or on the CT scan. Given the presence of NF1 and the absence of a history of neurologic surgery that could account for such a finding, a diagnosis of occipital calvarial dysplasia was established. Because the patient had no neurological symptoms, we opted for periodic clinical and imaging follow-up over surgical intervention. After one year of follow-up, the lesion remained unchanged and no neurologic symptoms had arisen. NF1 is an autosomal dominant, multisystem disorder with extreme clinical heterogeneity. The incidence of bony defects is higher in patients with NF1 than in the general population, and studies in the recent radiology literature of Brazil have addressed the role of different imaging modalities in the evaluation of bone lesions. However, although sphenoid wing dysplasia, pseudarthrosis of the tibia, and vertebral defects are hallmarks of NF1 and compose its standard diagnostic criteria, calvarial involvement in NF1 is uncommon. Many patients with such defects are asymptomatic, although headache, visual symptoms, and concurrent bony skull defects can be present. Therefore, a thorough clinical and imaging evaluation is called for when calvarial defects are detected. Two hypotheses try to explain cranial vault defects in NF1. One theory suggests that the calvarial lesions stem from an intrinsic bone development abnormality related to mutations within the NF1 gene itself. However, some authors postulate that NF1-related bone defects derive from increased exogenous pressure exerted by underlying neurofibromas, thereby leading to bone erosion and patency of cranial sutures. Because calvarial lesions have been observed in the presence and absence of adjacent tumors, it remains unclear whether these defects represent

Incidentally detected massive scrotal cystocele

1. Cetrim – Centro de Treinamento em Imaginologia, João Pessoa, PB, Brazil. 2. Universidade Federal da Paraíba (UFPB), João Pessoa, PB, Brazil. 3. Escola Paulista de Medicina da Universidade Federal de São Paulo (EPM-Unifesp), São Paulo, SP, Brazil. Mailing address: Dr. Edward Araujo Júnior. Rua Belchior de Azevedo, 156, ap. 111, Torre Vitória, Vila Leopoldina. São Paulo, SP, Brazil, 05089030. E-mail: araujojred@terra.com.br. no associated complications were identified. Cesarean section was performed at 35 weeks of gestation, because of fetal distress. The newborn weighed 2183 g; the 1and 5-minute Apgar scores were 7 and 10, respectively. Postnatal echocardiography confirmed the LVD in the free wall of the left ventricle. On the 3rd day of life, the newborn underwent surgery to correct the defect, and there were no postoperative complications. The newborn remained in the neonatal intensive care unit for 9 days and was discharged from the hospital on the 18th day of life, with preserved cardiac function and no complications. An LVD is defined as a protrusion of the free wall of a ventricle. Although it is of unknown etiology, it is probably congenital. The weakness of the myocardial wall during embryogenesis can lead to a focal protrusion of the heart wall. An LVD has a narrow neck through which it communicates with the ventricular cavity; in contrast, a left ventricular aneurysm (LVA) has a wide base for connecting with the ventricular cavity. The wall of an LVA is akinetic, whereas an LVD contracts synchronously with the ventricle. An LVD can be accompanied by Figure 3. Spectral Doppler ultrasound showing triphasic flow and high pulsatility within the diverticulum. other congenital and cardiac anomalies such as the pentalogy of Cantrell. The prenatal diagnosis of LVD or LVA can be made by ultrasound, and these anomalies are frequently accompanied by pericardial effusion, which can cause fetal pulmonary hypoplasia and progressive hydrops. LVD is reported to have a more favorable long-term prognosis than does LVA. The prognosis is usually favorable when there is no change in the size of the diverticulum, which was the case in the patient described here. When a fibrous LVD has a thin wall, disruption can occur and is usually fatal, although such a development is rare. Prenatal monitoring, with serial examinations by fetal cardiology and cardiac surgery teams for proper programming of prenatal or postnatal interventions, will therefore be necessary.

Malignant peripheral nerve sheath tumor of the vagus nerve: an uncommon cause of progressive dyspnea

REFERENCES 1. Ferrari G, De Filippi G, Elia F, et al. Diaphragm ultrasound as a new index of discontinuation from mechanical ventilation. Crit Ultrasound J. 2014;6:8. 2. Matamis D, Soilemezi E, Tsagourias M, et al. Sonographic evaluation of the diaphragm in critically ill patients. Technique and clinical applications. Intensive Care Med. 2013;39:801–10. 3. Francis CA, Hoffer JA, Reynolds S. Ultrasonographic evaluation of diaphragm thickness during mechanical ventilation in intensive care patient. Am J Crit Care. 2016;25:e1–8. Rachel Zeitoune1, Ana Célia Baptista Koifman2, Marina Shu Fong1, Roberto Mogami1

Higher prevalence of clinical cardiovascular comorbidities in postmenopausal women with self-reported premenopausal hirsutism and/or oligo-amenorrhea

ABSTRACT Hirsutism is a common condition, being present in about 5–15% of women. It is characterized by the growth of terminal hair in a pattern typical for men, like as hair growth in upper lip, chin, cheek and lower and upper abdomen. Not infrequently, hirsutism is followed by other signs of hyerandrogenism such as alopecia, acne, and seborrhea. The current study evaluated the association between a self-reported history of hirsutism and oligo-amenorrhea during reproductive age and the presence of several comorbidities in women after menopause. A total of 1057 women were investigated in a cross-sectional study, and information on the age at menarche, menstrual history, complaints about excessive hair growth, and disease development was obtained. Participants from the study were postmenopausal women aged >55 y who attended ac primary care service at least once during the 24-month period. Exclusion criteria included the presence of cognitive impairment and/or communication difficulties. Main outcomes were the presence of comorbidities after menopause. The prevalence of comorbidities was significantly higher in women with a history of hirsutism and/or oligo-amenorrhea [OR = 1.6 (95% CI 1.1–2.4), p = 0.002] or isolated hirsutism [OR 2.0 (95% CI 1.3–3.2), p = 0.004]. The prevalence of stroke, angina or myocardial infarction, cardiac failure, chronic obstructive pulmonary disease, and osteoarthritis were significantly higher in postmenopausal women who had experienced hirsutism and/or oligomenorrhea (p < 0.03). Limitations of the study came from the absence of a clear differentiation between hirsutism and hypertrichosis. According our results, the presence of hirsutism and oligo-amenorrhea during the female reproductive period may indicate susceptibility to important diseases at old age.