Radiological findings of pulmonary tuberculosis in indigenous patients in Dourados, MS, Brazil.

OBJECTIVE: To describe the radiological findings of pulmonary tuberculosis in indigenous patients from the city of Dourados, MS, Brazil, according to age and sex. MATERIALS AND METHODS: Chest radiographic images of 81 patients with pulmonary tuberculosis, acquired in the period from 2007 to 2010, were retrospectively analyzed by two radiologists in consensus for the presence or absence of changes. The findings in abnormal radiographs were classified according to the changes observed and they were correlated to age and sex. The data were submitted to statistical analysis. RESULTS: The individuals' ages ranged from 1 to 97 years (mean: 36 years). Heterogeneous consolidations, nodules, pleural involvement and cavities were the most frequent imaging findings. Most patients (55/81 or 67.9%) were male, and upper lung and right lung were the most affected regions. Fibrosis, heterogeneous consolidations and involvement of the left lung apex were significantly more frequent in males (p < 0.05). Presence of a single type of finding at radiography was most frequent in children (p < 0.05). CONCLUSION: Based on the hypothesis that indigenous patients represent a population without genetically determined resistance to tuberculosis, the present study may enhance the knowledge about how the pulmonary form of this disease manifests in susceptible individuals.

OBJETIVO: Descrever os aspectos radiológicos da tuberculose pulmonar em pacientes indígenas da cidade de Dourados, MS, Brasil, de acordo com idade e sexo. MATERIAIS E MÉTODOS: Radiografias de tórax de 81 pacientes com tuberculose pulmonar, realizadas de 2007 a 2010, foram analisadas retrospectivamente por dois radiologistas, em consenso quanto à presença ou ausência de alterações. Os achados em radiografias anormais foram classificados de acordo com as alterações observadas e correlacionados com idade e sexo. Os dados foram submetidos a análise estatística. RESULTADOS: A idade dos indivíduos variou de 1 a 97 anos (idade média de 36 anos). Os achados mais frequentes foram consolidações heterogêneas, nódulos, acometimento pleural e escavações. As regiões pulmonares superiores e o pulmão direito foram mais afetados pela tuberculose e a maioria dos pacientes (55/81 ou 67,9%) era do sexo masculino. Fibrose, consolidações heterogêneas e envolvimento do ápice pulmonar esquerdo foram significativamente mais frequentes no sexo masculino (p < 0,05). Presença de apenas um tipo de achado radiográfico foi mais frequente em crianças (p < 0,05). CONCLUSÃO: Com base na hipótese de que pacientes indígenas representam pessoas ainda não geneticamente selecionadas para a resistência à tuberculose, esta pesquisa pode ampliar o conhecimento sobre como esta doença, em sua forma pulmonar, manifesta-se em indivíduos suscetíveis.

Radiological findings of pulmonary tuberculosis in indigenous patients in Dourados, MS, Brazil / Aspectos radiológicos da tuberculose pulmonar em indígenas de Dourados, MS, Brasil

AbstractObjective:To describe the radiological findings of pulmonary tuberculosis in indigenous patients from the city of Dourados, MS, Brazil, according to age and sex.Materials and Methods:Chest radiographic images of 81 patients with pulmonary tuberculosis, acquired in the period from 2007 to 2010, were retrospectively analyzed by two radiologists in consensus for the presence or absence of changes. The findings in abnormal radiographs were classified according to the changes observed and they were correlated to age and sex. The data were submitted to statistical analysis.Results:The individuals' ages ranged from 1 to 97 years (mean: 36 years). Heterogeneous consolidations, nodules, pleural involvement and cavities were the most frequent imaging findings. Most patients (55/81 or 67.9%) were male, and upper lung and right lung were the most affected regions. Fibrosis, heterogeneous consolidations and involvement of the left lung apex were significantly more frequent in males (p < 0.05). Presence of a single type of finding at radiography was most frequent in children (p < 0.05).Conclusion:Based on the hypothesis that indigenous patients represent a population without genetically determined resistance to tuberculosis, the present study may enhance the knowledge about how the pulmonary form of this disease manifests in susceptible individuals.

ResumoObjetivo:Descrever os aspectos radiológicos da tuberculose pulmonar em pacientes indígenas da cidade de Dourados, MS, Brasil, de acordo com idade e sexo.Materiais e Métodos:Radiografias de tórax de 81 pacientes com tuberculose pulmonar, realizadas de 2007 a 2010, foram analisadas retrospectivamente por dois radiologistas, em consenso quanto à presença ou ausência de alterações. Os achados em radiografias anormais foram classificados de acordo com as alterações observadas e correlacionados com idade e sexo. Os dados foram submetidos a análise estatística.Resultados:A idade dos indivíduos variou de 1 a 97 anos (idade média de 36 anos). Os achados mais frequentes foram consolidações heterogêneas, nódulos, acometimento pleural e escavações. As regiões pulmonares superiores e o pulmão direito foram mais afetados pela tuberculose e a maioria dos pacientes (55/81 ou 67,9%) era do sexo masculino. Fibrose, consolidações heterogêneas e envolvimento do ápice pulmonar esquerdo foram significativamente mais frequentes no sexo masculino (p < 0,05). Presença de apenas um tipo de achado radiográfico foi mais frequente em crianças (p < 0,05).Conclusão:Com base na hipótese de que pacientes indígenas representam pessoas ainda não geneticamente selecionadas para a resistência à tuberculose, esta pesquisa pode ampliar o conhecimento sobre como esta doença, em sua forma pulmonar, manifesta-se em indivíduos suscetíveis.

Horizontal gaze palsy with progressive scoliosis: CT and MR findings.

Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare congenital disorder characterized by absence of conjugate horizontal eye movements and progressive scoliosis developing in childhood and adolescence. We present a child with clinical and neuroimaging findings typical of HGPPS. CT and MRI of the brain demonstrated pons hypoplasia, absence of the facial colliculi, butterfly configuration of the medulla and a deep midline pontine cleft. We briefly discuss the imaging aspects of this rare entity in light of the current literature.

Sonographic evaluation of gallbladder contractility in patients with spinal cord injury.

PURPOSE: To determine gallbladder volume with sonography during fasting and in response to a fatty meal in patients with spinal cord injuries (SCIs) and compare the results with those obtained in healthy controls. METHOD: Forty-three patients with SCI and 40 healthy volunteers without clinical evidence of gallbladder disease underwent sonography before and 30 and 60 minutes after the ingestion of a standard fatty meal. The gallbladder fasting volume, resting volume, and gallbladder contractility were calculated, and the results were compared. Correlation between gallbladder contractility and level of lesion, time since injury, use of oxybutynin, and body mass index (BMI) was also assessed. RESULTS: The mean ejection fraction was significantly lower in the patients with SCIs (40%) compared with healthy controls (63%) (p < 0.001). Gallbladder mean residual volume 60 minutes after ingestion of the fatty meal was lower in the control group (p < 0.001). CONCLUSION: Gallbladder contractility is impaired in patients with SCI, which may predispose these patients to gallstone formation. There was no correlation between gallbladder contractility and level of the lesion, time since injury, use of oxybutynin, or BMI.

Leukoencephalopathy with brainstem and spinal cord involvement and high brain lactate: report of three Brazilian patients.

A novel leukoencephalopathy was recently identified based on magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy ((1)H-MRS) findings. Leukoencephalopathy with brainstem and spinal cord involvement and high lactate (LBSL) is an autosomal recessive disorder characterized by early onset of symptoms and slowly progressive cerebellar, pyramidal and spinal cord dorsal column dysfunction. MRI and (1)H-MRS typically show abnormalities within cerebral and cerebellar white matter, a characteristic involvement of brainstem and spinal cord tracts and elevated lactate in the abnormal white matter. We present three cases with characteristic clinical and neuroimaging findings of this disorder. Some additional unique findings of our patients are discussed, like distal motor neuropathy and elevated creatine kinase in the serum.

[Neuronal ceroid lipofuscinosis: clinical and neuroradiological findings]. / Lipofuscinose ceróide neuronal: achados clínicos e neurorradiológicos.

The neuronal ceroid lipofuscinoses (NCL) are a group of neurodegenerative disorders, characterized by abnormal storage of an autofluorescent substance of lipopigments, resembling ceroid and lipofuscin, within lysosomes of neurons and other types of cells. The main phenotypic subtypes have been established on the basis of age of onset, clinical course, and ultra structural morphology, and classified as infantile, late infantile, juvenile and adult forms. Six genes have been associated with human NCL and approximately 150 mutations have been described. The aim of this study is to report the clinical, neuroradiological, and morphological characteristics of seven patients evaluated at Sarah Network of Hospitals for Reabilitation-Fortaleza-Ceará-Brazil. Five cases were histopathologically confirmed with skin biopsy and two were siblings of confirmed patients. An early diagnosis of NCL, an autosomal recessive disease, is mandatory for genetic counseling and to avoid further cases in the family. Imaging findings can contribute to the differential diagnosis.

Patent foramen ovale and ischemic stroke in young people: statistical association or causal relation?

OBJECTIVES: To determine if there are evidences of a causal relation between patent foramen ovale (PFO) x cryptogenic ischemic stroke (IS) in the young population and to analyze this relation in terms of causal criteria. METHODS: A total of 168 young patients with IS was retrospectively evaluated and divided into two groups: cryptogenic and with a defined cause. As a routine procedure, the patients underwent investigation of the PFO by means of transesophageal echocardiogram and/or transcranial Doppler sonography, both of them associated with the bubble test. Multivariate analysis was performed after demonstration of univariate statistical association between PFO x IS. RESULTS: After multivariate analysis, the association between PFO x cryptogenic IS was still statistically significant with odds ratio (adjusted OR = 3.3; 95% CI: 1.5-7.4). The total number of cerebral lesions also presented a significant association with cryptogenic IS (adjusted OR = 0.4; 95% CI: 0.2-0.9). The association between PFO and cryptogenic IS met all the causality criteria. CONCLUSION: The causal relation between PFO and cryptogenic IS in the young population is highly probable. This fact should be considered in the therapeutic decision.

Lipofuscinose ceróide neuronal: achados clínicos e neurorradiológicos / Neuronal ceroid lipofuscinosis: clinical and neuroradiological findings

Lipofuscinose ceróide neuronal (LCN) constitui um grupo de doenças neurodegenerativas caracterizadas pelo depósito anormal de uma substância autofluorescente de lipopigmentos, que lembra ceróide e lipofuscina, dentro dos lisossomos dos neurônios e outros tipos de células. Os principais subtipos fenotípicos, baseando-se na idade de início, curso clínico e morfologia ultraestrutural, são classificados em formas infantil, infantil tardia, juvenil e adulta. Seis genes associados a lipofuscinose ceróide foram identificados e aproximadamente 150 mutações também são descritas. Relatamos sete pacientes com LCN baseados na história clínica, achados neurorradiológicos e patológicos avaliados na Rede Sarah de Hospitais de Reabilitação - Fortaleza - Ceará - Brasil. Cinco casos foram confirmados com biópsia de pele, sendo dois casos irmãos de pacientes confirmados. O diagnóstico precoce de LCN, uma doença com herança autossômica recessiva, é mandatório para aconselhamento genético e prevenção de outros casos na família. Os achados de imagem podem contribuir no diagnóstico diferencial.

The neuronal ceroid lipofuscinoses (NCL) are a group of neurodegenerative disorders, characterized by abnormal storage of an autofluorescent substance of lipopigments, resembling ceroid and lipofuscin, within lysosomes of neurons and other types of cells. The main phenotypic subtypes have been established on the basis of age of onset, clinical course, and ultra structural morphology, and classified as infantile, late infantile, juvenile and adult forms. Six genes have been associated with human NCL and approximately 150 mutations have been described. The aim of this study is to report the clinical, neuroradiological, and morphological characteristics of seven patients evaluated at Sarah Network of Hospitals for Reabilitation - Fortaleza - Ceará - Brazil. Five cases were histopathologically confirmed with skin biopsy and two were siblings of confirmed patients. An early diagnosis of NCL, an autosomal recessive disease, is mandatory for genetic counseling and to avoid further cases in the family. Imaging findings can contribute to the differential diagnosis.

Leukoencephalopathy with brainstem and spinal cord involvement and high brain lactate: report of three brazilian patients

A novel leukoencephalopathy was recently identified based on magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (¹H-MRS) findings. Leukoencephalopathy with brainstem and spinal cord involvement and high lactate (LBSL) is an autosomal recessive disorder characterized by early onset of symptoms and slowly progressive cerebellar, pyramidal and spinal cord dorsal column dysfunction. MRI and ¹H-MRS typically show abnormalities within cerebral and cerebellar white matter, a characteristic involvement of brainstem and spinal cord tracts and elevated lactate in the abnormal white matter. We present three cases with characteristic clinical and neuroimaging findings of this disorder. Some additional unique findings of our patients are discussed, like distal motor neuropathy and elevated creatine kinase in the serum.

Uma nova leucoencefalopatia foi recentemente descrita com base em achados característicos de ressonância magnética e espectroscopia de prótons por ressonância magnética. Leucoencefalopatia com envolvimento do tronco cerebral e da medula espinal e elevação do lactato cerebral é uma doença autossômica recessiva de aparecimento precoce e evolução lenta, caracterizada por disfunção cerebelar, piramidal e das colunas dorsais da medula. Ressonância magnética e espectroscopia de prótons tipicamente demonstram anormalidades na substância branca cerebral e cerebelar, com envolvimento característico de tratos no tronco encefálico e na medula espinhal e aumento de lactato na substância branca cerebral anormal. Relatamos três casos com achados clínicos e de neuroimagem característicos. Achados adicionais peculiares aos nossos pacientes são discutidos, como a elevação da creatina-quinase sérica e a presença de neuropatia motora distal.

Forame oval patente e acidente vascular cerebral isquêmico em jovens: associação causal ou estatística? / Patent foramen ovale and ischemic stroke in young people: statistical association or causal relation?

OBJETIVOS: Determinar se há evidências de uma relação causal entre forame oval patente (FOP) e acidente vascular cerebral isquêmico (AVCI) criptogênico em jovens. Analisar essa relação à luz dos critérios de causalidade. MÉTODOS: Avaliaram-se, retrospectivamente, 168 pacientes jovens com AVCI, divididos em dois grupos: criptogênico e de causa definida. Como parte da rotina, os pacientes foram submetidos a pesquisa de FOP por ecocardiograma transesofágico e/ou Doppler transcraniano, ambos associados ao teste de bolhas. Demonstrada a associação estatística univariada entre FOP e AVCI, procedeu-se a análise multivariada. RESULTADOS: Após análise multivariada, a associação FOP e AVCI criptogênico mostrou-se ainda estatisticamente significante, com razão de chance (RCajustada de 3,3 (IC95 por cento 1,5-7,4). O número total de lesões no encéfalo também apresentou associação significativa com o AVCI criptogênico (RCajustada= 0,4 IC95 por cento 0,2-0,9). A associação FOP e AVCI criptogênico satisfez todos os critérios de causalidade. CONCLUSÃO: A relação causal entre o FOP e o AVCI criptogênico em jovens é altamente provável. Esse fato deve ser considerado na decisão terapêutica.

OBJECTIVES: To determine if there are evidences of a causal relation between patent foramen ovale (PFO) x cryptogenic ischemic stroke (IS) in the young population and to analyze this relation in terms of causal criteria. METHODS: A total of 168 young patients with IS was retrospectively evaluated and divided into two groups: cryptogenic and with a defined cause. As a routine procedure, the patients underwent investigation of the PFO by means of transesophageal echocardiogram and/or transcranial Doppler sonography, both of them associated with the bubble test. Multivariate analysis was performed after demonstration of univariate statistical association between PFO x IS. RESULTS: After multivariate analysis, the association between PFO x cryptogenic IS was still statistically significant with odds ratio (adjusted OR = 3.3; 95 percent CI: 1.5-7.4). The total number of cerebral lesions also presented a significant association with cryptogenic IS (adjusted OR = 0.4; 95 percent CI: 0.2-0.9). The association between PFO and cryptogenic IS met all the causality criteria. CONCLUSION: The causal relation between PFO and cryptogenic IS in the young population is highly probable. This fact should be considered in the therapeutic decision.

MRI and 1H-MRS findings of three patients with Sjögren-Larsson syndrome.

Sjögren-Larsson syndrome (SLS) is a rare autosomal recessive neurocutaneous disorder caused by deficiency of the microsomal enzyme fatty aldehyde dehydrogenase. Patients present the classical triad of congenital ichthyosis, mental retardation and spastic di- or tetraplegia. Magnetic resonance imaging (MRI) of the brain usually shows hypomyelination involving the periventricular white matter. Cerebral proton MR spectroscopy ((1)H-MRS) reveals a characteristic abnormal lipid peak. We report three cases of SLS from different families with the typical clinical triad. The MRI and (1)H-MRS findings are discussed.

MRI and ¹H-MRS findings of three patients with Sjõgren-Larsson syndrome

A síndrome de Sjögren-Larsson (SJL) é distúrbio raro, autossômico recessivo, caracterizado pela tríade clássica de ictiose congênita, retardo mental e tetraplegia ou diplegia espástica. Trata-se de um erro inato do metabolismo dos lipídios, causado pela deficiência da enzima microssômica aldeído graxo desidrogenase. Os achados de imagem do encéfalo na SJL demonstram atrofia cerebral e alteração da substância branca. A espectroscopia de prótons, com poucos casos relatados, caracteriza-se pelo elevado pico de lipídios e redução de N-acetil-aspartato. Apresentamos três casos de SJL, com ênfase nos achados da ressonância magnética e da espectroscopia de prótons.