RESUMEN
OBJECTIVE: We aimed to assess circulating thrombin activatable fibrinolysis inhibitor (TAFI) levels and carotid intima-media thickness (CIMT) in PCOS patients and control subjects. In this study we aimed to evaluate the relation between the levels of TAFI and homocysteine, high sensitive CRP (hsCRP), fibrinogen and CIMT in PCOS patients carrying a potential risk for developing CVD and diabetes and compared with age- and body mass index-matched controls. RESEARCH DESIGN AND METHODS: We studied 68 PCOS patients and 26 healthy controls. We conducted an observational study examining noninvasive markers of early CV disease in women with PCOS including structural CIMT. Noninvasive markers of early CVD, CIMT were measured in PCOS patients and control subjects. Metabolic parameters included fasting insulin and glucose levels, lipid and androgen levels, TAFI levels, hsCRP. RESULTS: Fasting glucose levels, prolactin, TSH, Total-cholesterol, LDL-cholesterol, triglyceride, estradiol, DHEA-S and age were similar in the two groups, whereas serum insulin, fibrinogen, hs-CRP, 17-OHP, free-testosterone, total testosterone, HOMA-IR, HDL were significantly elevated in PCOS patients in comparison to control subjects (p<0.05). Plasma TAFI levels were similarly in PCOS patients compared with healthy controls. No difference was observed in the combined IMT among the studied groups. CONCLUSIONS: In our study, no significant difference in lipid parameters was determined between patients with PCOS and healthy controls. In our study, we did not observed any difference in CIMT measurements and TAFI levels between patients with PCOS and healthy controls that can be explained by their low ages and short duration of PCOS.
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Carboxipeptidasa B2/sangre , Enfermedades Cardiovasculares/epidemiología , Síndrome del Ovario Poliquístico/enzimología , Adulto , Glucemia/metabolismo , Índice de Masa Corporal , Femenino , Humanos , Síndrome del Ovario Poliquístico/sangre , Síndrome del Ovario Poliquístico/patología , Prolactina/sangre , Valores de Referencia , Factores de Riesgo , Testosterona/sangre , Túnica Íntima/anatomía & histología , Túnica Íntima/patología , Túnica Media/anatomía & histología , Túnica Media/patologíaRESUMEN
OBJECTIVE: Polycystic ovary syndrome is a syndrome of ovarian dysfunction. Oxidative stress, inflammation and endothelial cell activation are thought to play concomitant roles in the pathogenesis of the above diseases particularly in the development of atherosclerotic lesions. RESEARCH DESIGN AND METHODS: We studied 58 polycystic ovary syndrome patients and age-matched 25 healthy controls consisting of women that have regular, ovulatory cycles and normal androgen levels. Homeostasis Model Assessment-Insulin Resistance for this study was taken as 1.75 that is the upper level of confidence interval of %95 of the mean of the healthy group. PCOS patients were divided into two groups as for below the cut-off level (<1.75) and above the cut-off level (> or =1.75). hs-CRP, fibrinogen, malondialdehyde, nitric oxide and disulfide level results were compared both in PCOS and control groups. RESULTS: In this study, sensitive CRP was found to be statical significantly higher in polycystic ovary syndrome groups whose Homeostasis Model Assessment-Insulin Resistance were > or =1.75 and <1.75 when compared to the control group. But, no significantly correlation was determined between malondialdehyde, nitric oxide and disulfide levels and CRP elevation. CONCLUSIONS: In our study, because those participants were young and non- obese patients with PCOS, malondialdehyde, nitric oxide and disulfide levels and Carotid Artery Intima-Media Thickness measurements as a pre-indicator of cardiovascular disease were not found to be different from those of the controls.
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Biomarcadores/sangre , Proteína C-Reactiva/metabolismo , Resistencia a la Insulina/fisiología , Estrés Oxidativo/fisiología , Síndrome del Ovario Poliquístico/fisiopatología , Adolescente , Adulto , Disulfuros/sangre , Femenino , Fibrinógeno/metabolismo , Humanos , Malondialdehído/sangre , Óxido Nítrico/sangreRESUMEN
OBJECTIVE: Interleukin-6 (IL-6) is a key pro-inflammatory and immune-modulatory cytokine of relevance for cardiovascular (CD) diseases. Cardiovascular risk factors that have been reported include oxidative stress markers [nitric oxide (NO), malondialdehyde (MDA), disulphite (SH)]. We aimed to evaluate the relation between the IL-6 G/C gene polymorphism and oxidative stress markers in polycystic ovary syndrome (PCOS) patients. DESIGN AND PATIENTS: We studied 85 PCOS patients and 115 healthy controls. PCOS was defined by the Rotterdam PCOS consensus criteria. RESULTS: The genotype IL-6 distribution did differ between the control group (CC 9.6%, GC 63.4%, GG 27.0%) and the PCOS patients (CC 4.7%, GC 29.4%, GG 65.9%) (p<0.001). The frequency of the polymorphic G allele was also not similar for the group with PCOS as for the control group with 80.6% and 58.7%, respectively (p<0.001). No statistically significant difference was determined for MDA and NO levels in PCOS patients and control group (p>0.05). Only SH levels were found to be high in favor of patient group (p<0.05). No statistically significant difference was determined between IL-6 G/C gene polymorphism and oxidative stress markers in PCOS patients and in the control group. CONCLUSION: Gene polymorphism of IL-6 -174 G>C is a risk factor for PCOS in Turkish patients. IL-6 gene polymorphisms are not related to NO, MDA, and SH levels in PCOS. Our negative results in risks factors of CV disorders can probably be explained by the fact that metabolic parameters and endothelial systems of patients may not yet be affected in this short period of time.
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Biomarcadores/metabolismo , Interleucina-6/genética , Estrés Oxidativo , Síndrome del Ovario Poliquístico , Polimorfismo de Nucleótido Simple , Adulto , Femenino , Predisposición Genética a la Enfermedad , Humanos , Masculino , Malondialdehído/sangre , Óxido Nítrico/sangre , Síndrome del Ovario Poliquístico/genética , Síndrome del Ovario Poliquístico/inmunología , Sulfitos/sangre , Turquía , Adulto JovenRESUMEN
The process of aging presents itself with various alterations in physiological events. Among many theories, the free radical (FR) theory of aging which reflects the FR damage to cellular components is accepted as one of the most important theories. Recently, the increases in catecholamine metabolism in aging have also attracted attention, and monoamine oxidase (MAO), a key enzyme in this process has been extensively studied. The aim of this study was to assess the role of FR species via MAO, a possible source of FRs, in physiological aging by determining the lipid peroxidation products (LPP) (malondialdehyde, diene conjugates) and antioxidant enzyme levels (superoxide dismutase (SOD) and catalase (CAT) in young (3 months old, n=10) and aging (16-18 months old, n=10) rat brain tissues of Swiss male albino rats. In the second part of the study, the same parameters were determined after the acute administration of MAO inhibitors (deprenyl and pargyline, 25 mg/kg i.p.) to investigate whether these agents have any beneficial effects in reducing oxidant stress via inhibition of MAO. In old rat brains, MAO activities showed a significant increase (P=0.000) in addition to an insignificant increase in LPP, while SOD (P=0.007) and CAT activities showed a decrease with advancing age. After the acute administration of both deprenyl and pargyline, a significant decrease in the MAO activities of both young (P=0.0002 for each) and aging rats (P=0.0002 for deprenyl and P=0.0001 for pargyline) were observed. It was noted that deprenyl causes a significant increase in CAT activity (P<0.05) but a significant decrease in SOD activity (P<0.05) in young rats, while it causes only a significant increase in SOD activity in aging rats (P<0.05). Both deprenyl and pargyline cause a significant decrease in conjugated diene levels of aging rats (P<0.05). These results confirm the role of catecholamine oxidation and MAO activity as one of the causative factors in increased oxidant stress during aging. By reducing the oxidant stress observed in aging brain, MAO inhibitors, especially deprenyl, may contribute to the control of the aging process.
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Envejecimiento/fisiología , Química Encefálica/fisiología , Encéfalo/crecimiento & desarrollo , Inhibidores de la Monoaminooxidasa/farmacología , Estrés Oxidativo/fisiología , Animales , Encéfalo/efectos de los fármacos , Química Encefálica/efectos de los fármacos , Catalasa/metabolismo , Radicales Libres/metabolismo , Peróxido de Hidrógeno/metabolismo , Peroxidación de Lípido/efectos de los fármacos , Masculino , Malondialdehído/metabolismo , Ratones , Monoaminooxidasa/metabolismo , Estrés Oxidativo/efectos de los fármacos , Espectrometría de Fluorescencia , Superóxido Dismutasa/metabolismoRESUMEN
We describe a case of listerial rhombencephalitis in a previously healthy 40-year-old man. The diagnosis was based on the clinical findings, results of cerebrospinal fluid analysis, blood culture, and MR imaging findings. The treatment was started before culture results were available, and the patient had a full clinical recovery.
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Encefalitis/diagnóstico , Encefalitis/microbiología , Listeriosis , Imagen por Resonancia Magnética , Rombencéfalo , Adulto , Antiinfecciosos/uso terapéutico , Quimioterapia Combinada , Encefalitis/tratamiento farmacológico , Humanos , Listeriosis/tratamiento farmacológico , Masculino , Sulfametoxazol/uso terapéutico , Trimetoprim/uso terapéuticoRESUMEN
The aim of this study was to document the magnetic resonance imaging (MRI) findings of cases with a history of severe neonatal indirect hyperbilirubinemia. Ten cases (eight cases with neurologic findings, two normal cases) with a history of severe neonatal indirect hyperbilirubinemia were studied. Neurologic findings and MRI results were described and correlated. Seven of eight cases with neurologic findings demonstrated symmetric and uniform increased T2 signal changes limited to globus pallidi. MRI scans of two cases without neurologic findings showed no abnormality. Severe neonatal indirect hyperbilirubinemia should be considered in the differential diagnosis of bilateral symmetric hyperintense signal changes in the globus pallidus on MRI. However, high levels of unconjugated bilirubin concentrations in the neonatal period may not always cause such lesions of globus pallidus on MRI despite the presence of neurologic findings.
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Globo Pálido/patología , Ictericia Neonatal/diagnóstico , Kernicterus/diagnóstico , Imagen por Resonancia Magnética , Preescolar , Dominancia Cerebral/fisiología , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Examen Neurológico , Factores de RiesgoRESUMEN
Benign macrocephaly of infancy is a common problem in the child neurology practice. The radiologic features of this entity are not well defined. In most of the previous studies, macrocephalic patients were evaluated by computed tomography. To define the radiologic characteristics of this entity, 20 children with macrocephaly with normal neurologic examinations were enrolled in the study. All the patients were evaluated by magnetic resonance imaging studies. Sixty-five percent of patients had enlargement of the subarachnoid space and 35% of patients had megalencephaly. None of the patients had subdural collections. The mean age of patients with enlargement of the subarachnoid space was found to be younger than those with megalencephaly. The cases with parental histories of macrocephaly demonstrated both enlargement of the subarachnoid space and megalencephaly. Our results suggest that the radiologic finding of benign macrocephaly can be both enlargement of the subarachnoid space and megalencephaly.
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Anomalías Craneofaciales/diagnóstico , Imagen por Resonancia Magnética , Cefalometría , Ventrículos Cerebrales/patología , Niño , Preescolar , Anomalías Craneofaciales/etiología , Anomalías Craneofaciales/genética , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Examen Neurológico , Factores de Riesgo , Cráneo/patologíaRESUMEN
Behçet's disease is a chronic, relapsing multisystem disorder, and nervous system involvement is one of the serious manifestations. Neuro-Behçet is rarely reported in children and may present with a wide variety of symptoms because the entire neuraxis may be affected. A case of cerebral vein thrombosis secondary to Behçet's disease is presented. The patient has recovered without any visual loss and had no complaints at 1-year follow-up.
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Síndrome de Behçet/diagnóstico , Trombosis del Seno Lateral/patología , Adolescente , Síndrome de Behçet/complicaciones , Síndrome de Behçet/patología , Venas Cerebrales/patología , Diagnóstico Diferencial , Femenino , Cefalea/etiología , Humanos , Hipertensión Intracraneal/etiología , Trombosis del Seno Lateral/etiología , Imagen por Resonancia Magnética , Náusea/etiología , Úlceras Bucales/etiología , Flebografía/métodos , Vómitos/etiologíaRESUMEN
Reported are two patients presenting with both thrombocytopenia and sagittal sinus thrombosis. The first patient is a 42-month-old male with no identified thrombophilic risk factors who developed acute neurologic symptoms after an acute infection. The second patient is a 22-month-old female with no history of preceding infection but had a positive lupus anticoagulant test. She also developed deep venous thrombosis and was treated with intravenous heparin. Both patients are currently doing well without neurologic deficits. To the authors' knowledge the second patient is the youngest reported patient with cerebral vein thrombosis associated with thrombocytopenia and lupus anticoagulant. These observations call attention to the need for a thorough investigation of thrombophilic risk factors in pediatric patients with thrombotic complications.
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Trombosis de los Senos Intracraneales/etiología , Trombocitopenia/complicaciones , Encéfalo/patología , Hemorragia Cerebral/etiología , Preescolar , Femenino , Humanos , Lactante , Inhibidor de Coagulación del Lupus/sangre , Masculino , Factores de Riesgo , Trombosis de los Senos Intracraneales/sangre , Trombosis de los Senos Intracraneales/diagnóstico , Trombosis de la Vena/etiologíaRESUMEN
Castleman syndrome (giant lymph node hyperplasia) is a rare, heterogeneous lymphoproliferative disorder of unknown etiology and pathogenesis. Most cases occur as mediastinal masses, although extrathoracic involvement including nodal and extranodal locations have been reported. The localized variants (solitary lesions) respond well to surgical excision. We report a 10-year-old boy who presented with headache, intermittent fever, and progressive weakness of his legs. MRI imaging showed an enhancing epidural mass with impingement on the spinal cord at the C6-T2 level. Other laboratory abnormalities included anemia, hypergammaglobulinemia, increased erythrocyte sedimentation rate, and cerebrospinal fluid pleocytosis with slightly increased cerebrospinal protein. The mass was partially resected and the histopathology showed lymphoplasmocytic infiltration compatible with Castleman syndrome. There was no evidence of malignancy. Castleman syndrome is the most likely diagnosis in the presence of the associated systemic findings, although the epidural site for lymphoplasmocytic inflammation is atypical.
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Enfermedad de Castleman/diagnóstico , Espacio Epidural , Compresión de la Médula Espinal/etiología , Enfermedad de Castleman/complicaciones , Enfermedad de Castleman/patología , Enfermedad de Castleman/cirugía , Niño , Diagnóstico Diferencial , Cefalea/etiología , Humanos , Pierna/inervación , Subgrupos Linfocitarios/patología , Linfoma/diagnóstico , Imagen por Resonancia Magnética , Masculino , Debilidad Muscular/etiología , Plasmacitoma/diagnósticoRESUMEN
Mortality is high in patients with fulminant hepatic failure (FHF). Neurologic complications of encephalopathy and cerebral edema are major contributors to mortality. Orthotopic liver transplantation has improved survival in these patients. However, the complexity of medical and surgical problems in this patient population, coupled with a severe shortage of organs, requires careful patient selection. The aim of this study was to describe the neurologic outcome of children with FHF who developed radiologically apparent cerebral edema. The hospital and outpatient records and radiologic studies of 20 children with FHF admitted to Children's Hospital of Pittsburgh from 1981-1995 who developed encephalopathy and computed tomographic evidence of cerebral edema were reviewed. Fourteen patients died (70%), three were left with severe neurologic deficits (15%), and three were left with moderate deficits (15%). Survival was correlated with a lesser degree of coma. Histopathologic examination of eight brains demonstrated cerebral edema and widespread ischemic neuronal necrosis in all eight. The presence of radiographic cerebral edema in children with FHF is an objective measure that indicates a very poor prognosis. Termination of care is a reasonable option. Comprehensive monitoring of cerebral function and intracranial pressure is required in children with FHF. Orthotopic liver transplantation should be performed in children with severe and worsening encephalopathy before the development of radiographically apparent cerebral edema.
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Edema Encefálico/terapia , Encefalopatía Hepática/complicaciones , Adolescente , Daño Encefálico Crónico/epidemiología , Daño Encefálico Crónico/etiología , Edema Encefálico/diagnóstico por imagen , Edema Encefálico/etiología , Edema Encefálico/mortalidad , Edema Encefálico/patología , Isquemia Encefálica/etiología , Isquemia Encefálica/patología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Encefalopatía Hepática/mortalidad , Humanos , Lactante , Presión Intracraneal , Trasplante de Hígado , Masculino , Necrosis , Neuronas/patología , Pennsylvania/epidemiología , Valor Predictivo de las Pruebas , Índice de Severidad de la Enfermedad , Método Simple Ciego , Análisis de Supervivencia , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
Hydroxyapatite (HA) and Demineralized Bone Matrix (DBM) are being investigated as potential osteogenic agents with hopes that these substances can be used to induce bone formation in non-union fractures. This study was done to determine the relative effects of HA and DBM implanted as moldable phospholipid composites in bone defects that result in non-unions. We studied 22 ten-month-old Long-Evans male rats with 5.0 mm unilateral radial defects implanted with HA, DBM, and a combination of both substances. Control defects were left unfilled. Eight weeks after implantation, the histological sections demonstrated a decrease in bone formation with HA relative to controls. The HA crystals were encapsulated by connective tissue stroma made up of collagenous elements, fibroblasts, and blood vessels. There were no indications of bone formation within the fibrous stroma. 45Calcium, alkaline phosphatase, and bone gla protein (BGP) assays demonstrated a 16% increase in bone formation in rats implanted with DBM, an 80% decrease in groups implanted with HA (p = 0.01) and an 80% decrease with DBM plus HA (p = 0.01). Radiologic analysis corresponds well with histological and biochemical results. We conclude that osteogenesis in non-union defects is enhanced by the implantation of DBM, while HA interferes with bone formation in the rat model. In the presence of both substances, HA appears to impede new bone growth, negating any positive effects seen with DBM.
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Matriz Ósea/fisiología , Regeneración Ósea , Huesos/metabolismo , Calcificación Fisiológica , Hidroxiapatitas , Osteogénesis , Fosfatasa Alcalina/metabolismo , Animales , Huesos/anatomía & histología , Calcio/metabolismo , Durapatita , Masculino , Osteocalcina/metabolismo , RatasRESUMEN
A 4 year-old boy with mental retardation and seizures presented to the pediatric endocrinology clinic because of a history of hypoglycemia; a 16 month-old girl with developmental delay presented with bilateral breast tissue enlargement; in both, a diagnosis of Kabuki syndrome was made because of typical facial features, neurodevelopmental delay and other stigmata consistent with Kabuki syndrome. Kabuki syndrome is a mental retardation-malformation syndrome affecting multiple organ systems with a broad spectrum of abnormalities. The facial features of the syndrome are specific and independent of ethnic origin. In addition to presenting with endocrine problems, the patients reported here exhibit some novel findings such as congenital alopecia areata and hyperpigmented skin lesion. The diagnosis of Kabuki syndrome should be considered in patients with hypoglycemia or premature thelarche when associated with developmental delay and a peculiar facies.
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Discapacidades del Desarrollo/complicaciones , Enfermedades del Sistema Endocrino/etiología , Facies , Discapacidad Intelectual/complicaciones , Convulsiones/clasificación , Preescolar , Femenino , Humanos , Lactante , Masculino , SíndromeRESUMEN
This study examined the effectiveness of dialectical behavior therapy (DBT) in the treatment of people diagnosed with borderline personality disorder (BPD). The frequency of self-injurious behaviors of 15 women hospitalized on a DBT unit was traced over a 4-week period. Data on self-injurious behaviors and the use of the DBT approach were collected from a review of incident reports and patients' charts. The self-injurious behaviors decreased by almost 50%. In addition, four RNs, who were involved on the DBT unit both before and after the institution of the treatment approach, were interviewed. Semi-structured interviews were conducted to determine the nurses perceptions of the effectiveness of DBT. Their responses were uniformly positive. The most commonly occurring phrase was "it works."
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Terapia Conductista/métodos , Trastorno de Personalidad Limítrofe/terapia , Adaptación Psicológica , Adulto , Trastorno de Personalidad Limítrofe/complicaciones , Trastorno de Personalidad Limítrofe/rehabilitación , Femenino , Hospitalización , Humanos , Entrevista Psicológica , Persona de Mediana Edad , Conducta Autodestructiva/complicaciones , Conducta Autodestructiva/diagnóstico , Conducta Autodestructiva/terapiaRESUMEN
Inclusion complex formation of benzoic acid with α-, ß- and γ-cyclodextrins in water and in 0.2 M solutions of inorganic salts (KCl, KBr, KH2PO4 and K2SO4) has been studied by means of 1H NMR at 298.15 K. Binding constants have been determined and role of biologically active inorganic anions in the inclusion complex formation has been revealed. It has been shown that effects of the anions are determined not only by changing the ionic strength. More pronounced influence of Br- and H2PO4- compared with Cl- and SO4(2-) is caused by specific ion-molecular interactions, occurrence of which depends on the physical-chemical properties of the anions as well as on the binding mode of cyclodextrins with benzoic acid. Competing interactions of cyclodextrin-anion were observed in the presence of KBr, while the ternary complex formation was detected upon addition of KH2PO4.
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Ácido Benzoico/química , Ciclodextrinas/química , Sales (Química)/química , Aniones/química , Sitios de Unión , Espectroscopía de Resonancia Magnética , SolucionesRESUMEN
Solution of an anti-inflammatory drug ibuprofen ((RS)-2-(4-isobutylphenyl) propionic acid) in chloroform was studied by nuclear magnetic resonance spectroscopy. A set of 2D NOESY spectra was analyzed in order to obtain atom-atom distances. Since ibuprofen is known to exist as an ensemble of different conformations, these distances are averaged over the ensemble. To compare experimental and calculated distances, three models of averaging were concerned. Our data allowed to determine the dominant conformers of ibuprofen dissolved in chloroform. The population of conformers in the saturated solution leads to a certain crystal morphology formed within the nucleation process. Observed and calculated (13)C chemical shifts (at the DFT/B3LYP/6-311+G(2d,p) level) were in good agreement.
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Cloroformo/química , Ibuprofeno/química , Conformación Molecular , Soluciones/química , Análisis Espectral/métodosRESUMEN
INTRODUCTION: The incidence and epidemiological causes of maxillofacial (MF) trauma varies widely. The objective of this study is to point out maxillofacial trauma patients' epidemiological properties and trauma patterns with simultaneous injuries in different areas of the body that may help emergency physicians to deliver more accurate diagnosis and decisions. METHODS: In this study we analyze etiology and pattern of MF trauma and coexisting injuries if any, in patients whose maxillofacial CT scans was obtained in a three year period, retrospectively. RESULTS: 754 patients included in the study consisting of 73.7% male and 26.3% female, and the male-to-female ratio was 2.8:1. Mean age was 40.3 ± 17.2 years with a range of 18 to 97. 57.4% of the patients were between the ages of 18-39 years and predominantly male. Above 60 years of age, referrals were mostly woman. The most common cause of injuries were violence, accounting for 39.7% of the sample, followed by falls 27.9% and road traffic accidents 27.2%. The primary cause of injuries were violence between ages 20 and 49 and falls after 50. Bone fractures found in 56,0% of individuals. Of the total of 701 fractured bones in 422 patients the most frequent was maxillary bone 28,0% followed by nasal bone 25,3%, zygoma 20,2%, mandible 8,4%, frontal bone 8,1% and nasoethmoidoorbital bone 3,1%. Fractures to maxillary bone were uppermost in each age group.8, 9% of the patients had brain injury and only frontal fractures is significantly associated to TBI (p < 0.05) if coexisting facial bone fracture occurred. Male gender has statistically stronger association for suffering TBI than female (p < 0, 05). Most common cause of TBI in MF trauma patients was violence (47, 8%).158 of the 754 patients had consumed alcohol before trauma. No statistically significant data were revealed between alcohol consumption gender and presence of fracture. Violence is statistically significant (p < 0.05) in these patients. CONCLUSION: Studies subjected maxillofacial traumas yield various etiologic factors, demographic properties and fracture patterns probably due to social, cultural and governmental differences. Young males subjected to maxillofacial trauma more commonly as a result of interpersonal violence.
RESUMEN
Isolated brain stem lesions presenting with acute neurologic findings create a major diagnostic dilemma in children. Although the brain stem is frequently involved in ADEM, solitary brain stem lesions are unusual. We performed a retrospective review in 6 children who presented with an inflammatory lesion confined to the brain stem. Two children were diagnosed with connective tissue disorder, CNS lupus, and localized scleroderma. The etiology could not be determined in 1, and clinical features suggested monophasic demyelination in 3. In these 3 children, initial lesions demonstrated vasogenic edema; all showed dramatic response to high-dose corticosteroids and made a full clinical recovery. Follow-up MRI showed complete resolution of lesions, and none had relapses at >2 years of follow-up. In retrospect, these cases are best regarded as a localized form of ADEM. We conclude that though ADEM is typically a disseminated disease with multifocal lesions, it rarely presents with monofocal demyelination confined to the brain stem.