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1.
Neuropediatrics ; 54(1): 82-87, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36564024

RESUMO

Claude syndrome is a rare midbrain stroke syndrome characterized by ipsilateral third cranial nerve palsy and contralateral hemiataxia. So far, only a few cases have been reported in childhood. We present two children with Claude syndrome at 9 and 15 years of age. The typical clinical picture was consistent with brain magnetic resonance imaging findings. A thorough investigation regarding the underlying etiology revealed no definite diagnosis but clues suggestive of probable neuro-Behcet disease. Awareness of pediatric neurologists on arterial ischemic stroke has been increasing over the past decades, enabling timely diagnosis and appropriate management of rare childhood cases with midbrain stroke.


Assuntos
Síndrome de Behçet , Infartos do Tronco Encefálico , Doenças do Nervo Oculomotor , Acidente Vascular Cerebral , Criança , Humanos , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/diagnóstico por imagem , Doenças do Nervo Oculomotor/complicações , Imageamento por Ressonância Magnética , Acidente Vascular Cerebral/complicações , Infartos do Tronco Encefálico/complicações
2.
Hum Psychopharmacol ; 38(1): e2857, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36382404

RESUMO

OBJECTIVE: The aim of this study was to evaluate brain connectivity by diffusion tensor imaging (DTI) in schizophrenia patients with clozapine-induced obsessive compulsive symptoms (OCS). METHODS: Eighteen schizophrenia patients, nine of which had clozapine-induced OCS (Clz-OCS (+)), 9 without OCS (Clz-OCS (-)) and 9 healthy controls were included. Psychopathology was evaluated with Positive and Negative Syndrome Scale and Yale-Brown Obsession and Compulsion Scale in the patient groups. All groups were assesed with neurocognitive tests and DTI. RESULTS: Tract-Based Spatial Statistics based comparison of DTI revealed lower fractional anisotropy in the genu of corpus callosum (CC), right cingulum, left frontal white matter (WM) in the Clz-OCS (+) group, compared to controls. Fractional anisotropy was found to be lower in the bilateral occipital WM and higher in the bilateral medial temporal regions, anterior limb of internal capsule, cingulum, frontoparietal peripheral WM, right external capsule and genu of CC in Clz-OCS (+) patients compared to Clz-OCS (-). CONCLUSIONS: WM integrity in several pathways such as cortico-striato-thalamo-cortical circuitry and orbito-frontal tracts seems to be affected differently in patients with Clz-OCS (+). Different neuroplastic effects of clozapine leading to occurrence of OCS in a subgroup of patients is possible, and needs further evaluation by longitudinal follow-up studies.


Assuntos
Clozapina , Transtorno Obsessivo-Compulsivo , Esquizofrenia , Humanos , Imagem de Tensor de Difusão/métodos , Clozapina/efeitos adversos , Esquizofrenia/tratamento farmacológico , Encéfalo , Transtorno Obsessivo-Compulsivo/diagnóstico por imagem
3.
Int J Neurosci ; : 1-6, 2023 May 04.
Artigo em Inglês | MEDLINE | ID: mdl-37099669

RESUMO

OBJECTIVES: Beta-propeller protein-associated neurodegeneration (BPAN) is a rare X-linked dominant neurodegenerative disease, which is characterized by iron accumulation in the basal ganglia. BPAN is associated with pathogenic variation in WDR45, which has been reported almost exclusively in females most probably due to male lethality in the hemizygous state. METHODS: Whole exome sequencing (WES) and targeted deep sequencing were performed for a male with a clinical diagnosis of BPAN at the age of 37. RESULTS: The novel frameshift variant in WDR45 detected by WES was further analyzed with targeted resequencing to detect a mosaic variant with a level of 85.5% in the blood sample of the proband. DISCUSSION: Although the main role of WDR45 remains elusive, recent studies show that WDR45 may contribute to neurodegeneration through defects in autophagy, iron storage and ferritin metabolism, mitochondria organization, and endoplasmic reticulum homeostasis. The extend of spatiotemporal haploinsufficiency of WDR45 frameshifting variants caused by mosaicism in males may lead to variable clinical severity, which may be hard to elaborate clinically. Promising genetic analysis strategies using targeted deep sequencing may help determine the clinical outcome of somatic mosaicism in neurological disorders including BPAN. Additionally, we suggest that deep sequencing should be conducted in cerebrospinal fluid samples to provide more reliable results in terms of reflecting the mosaicism level in the brain for future studies.

4.
Neuroradiology ; 64(1): 99-107, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34611716

RESUMO

PURPOSE: Synthetic MRI (SyMRI) enables to quantify brain tissue and morphometry. We aimed to investigate the WM and myelin alterations in patients with unilateral hippocampal sclerosis (HS) with SyMRI. METHODS: Adult patients with isolated unilateral HS and age-matched control subjects (CSs) were included in this study. The SyMRI sequence QRAPMASTER in the coronal plane perpendicular to the hippocampi was obtained from the whole brain. Automatic segmentation of the whole brain was processed by SyMRI Diagnostic software (Version 11.2). Two neuroradiologists also performed quantitative analyses independently from symmetrical 14 ROIs placed in temporal and extratemporal WM, hippocampi, and amygdalae in both hemispheres. RESULTS: Sixteen patients (F/M = 6/10, mean age = 32.5 ± 11.3 years; right/left HS: 8/8) and 10 CSs (F/M = 5/5, mean age = 30.7 ± 7 years) were included. Left HS patients had significantly lower myelin and WM volumes than CSs (p < .05). Myelin was reduced significantly in the ipsilateral temporal lobe of patients than CSs, greater in left HS (p < .05). Histopathological examination including luxol fast blue stain also revealed myelin pallor in all of 6 patients who were operated. Ipsilateral temporal pole and sub-insular WM had significantly reduced myelin than the corresponding contralateral regions in patients (p < .05). No significant difference was found in WM values. GM values were significantly lower in hippocampi in patients than CSs (p < .05). CONCLUSION: SyMRI revealed myelin reduction in the ipsilateral temporal lobe and sub-insular WM of patients with HS. Whether this finding correlates with electrophysiological features and SyMRI could serve as lateralization of temporal lobe epilepsy need to be investigated.


Assuntos
Epilepsia do Lobo Temporal , Bainha de Mielina , Adulto , Epilepsia do Lobo Temporal/diagnóstico por imagem , Epilepsia do Lobo Temporal/patologia , Hipocampo/diagnóstico por imagem , Hipocampo/patologia , Humanos , Imageamento por Ressonância Magnética , Bainha de Mielina/patologia , Esclerose/diagnóstico por imagem , Esclerose/patologia , Adulto Jovem
5.
NMR Biomed ; 33(4): e4247, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31970849

RESUMO

Multi-contrast images are commonly acquired together to maximize complementary diagnostic information, albeit at the expense of longer scan times. A time-efficient strategy to acquire high-quality multi-contrast images is to accelerate individual sequences and then reconstruct undersampled data with joint regularization terms that leverage common information across contrasts. However, these terms can cause features that are unique to a subset of contrasts to leak into the other contrasts. Such leakage-of-features may appear as artificial tissues, thereby misleading diagnosis. The goal of this study is to develop a compressive sensing method for multi-channel multi-contrast magnetic resonance imaging (MRI) that optimally utilizes shared information while preventing feature leakage. Joint regularization terms group sparsity and colour total variation are used to exploit common features across images while individual sparsity and total variation are also used to prevent leakage of distinct features across contrasts. The multi-channel multi-contrast reconstruction problem is solved via a fast algorithm based on Alternating Direction Method of Multipliers. The proposed method is compared against using only individual and only joint regularization terms in reconstruction. Comparisons were performed on single-channel simulated and multi-channel in-vivo datasets in terms of reconstruction quality and neuroradiologist reader scores. The proposed method demonstrates rapid convergence and improved image quality for both simulated and in-vivo datasets. Furthermore, while reconstructions that solely use joint regularization terms are prone to leakage-of-features, the proposed method reliably avoids leakage via simultaneous use of joint and individual terms, thereby holding great promise for clinical use.


Assuntos
Imageamento por Ressonância Magnética , Mapeamento Encefálico , Simulação por Computador , Humanos , Imagens de Fantasmas , Razão Sinal-Ruído
6.
Pediatr Blood Cancer ; 67(12): e28722, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32970355

RESUMO

BACKGROUND: Brain atrophy, abnormal pituitary morphology, corpus callosum, and posterior fossa abnormalities have been described in patients with Fanconi anemia (FA). We aimed to provide an overview of cranial neuroimaging findings and to evaluate the clinical implications in FA patients. PROCEDURE: Cranial magnetic resonance imaging (MRI) studies of 34 patients with FA were retrospectively evaluated, and patients' clinical data were correlated with the imaging findings. RESULTS: The patients' median age was 17.6 (range, 3.9-28) years. At least one pathological brain imaging finding was demonstrated in 22 (65%) patients. These findings included corpus callosum abnormalities and other related supratentorial malformations in nine, pituitary abnormalities in eight, craniovertebral junction and posterior fossa abnormalities in eight, vascular lesions in six, and intracerebral calcifications in two patients. Among the 22 patients who had abnormal cranial MRI findings, six (27%) had mild to moderate intellectual disability (ID), three (14%) had epilepsy, one (5%) had mild hearing loss, and one patient (5%) had hemiplegia. Among these 34 patients, 14 (41%) were transfusion dependent. There was no significant difference between patients with congenital and acquired neuroimaging findings and patients with normal neuroimaging regarding transfusion dependency. CONCLUSIONS: Acquired abnormalities in brain tissue, such as white matter intensity changes, white matter T2 hyperintense discrete foci, or infarcts along with congenital abnormalities, were identified in this study. Variable abnormal brain imaging findings in FA patients, although some were not associated with clinical neurological manifestations, suggest that brain imaging could be part of screening in FA.


Assuntos
Doenças do Sistema Nervoso Central/diagnóstico , Doenças do Sistema Nervoso Central/etiologia , Anemia de Fanconi/complicações , Neuroimagem/métodos , Adolescente , Adulto , Doenças do Sistema Nervoso Central/diagnóstico por imagem , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Prognóstico , Estudos Retrospectivos , Adulto Jovem
7.
Neuroimage ; 190: 242-253, 2019 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-29626609

RESUMO

The occipital lobe contains a substantial part of the neural machinery involved in visual perception. Mutations in the LAMC3 gene have recently been shown to cause complex bilateral occipital cortical gyration abnormalities. However, to what extent these structural changes impact visual behavior is not known. We recorded responses for two screening test batteries targeting visual function (Leuven - Perceptual Organization Screening Test, Cortical Vision Screening Test) and measured eye fixation performance in a visual attention experiment from a patient with homozygous LAMC3 gene mutation. Using voxel-based morphometry (VBM) we quantitatively assessed the extent of structural changes brought on by the genetic mutation by comparing mean cortical curvature, cortical thickness, and gray matter volume in 34 cortical areas between patient and an age-, sex-, and education-matched control group. Anatomical connectivity between these cortical areas was investigated by a structural covariance analysis. Visual screening-, and behavioral results revealed that the patient's impairments were predominantly in visuo-spatial attention. Consistent with this, VBM and structural connectivity results revealed significant structural changes in cortical regions subserving attentional functions. We conclude that the LAMC3 gene mutation affects cortical areas beyond the occipital lobe and primarily those visual functions that involve heavily distributed networks - such as visuo-spatial attention.


Assuntos
Atenção/fisiologia , Córtex Cerebral/anormalidades , Movimentos Oculares/fisiologia , Laminina/genética , Rede Nervosa/anormalidades , Transtornos da Percepção/patologia , Transtornos da Percepção/fisiopatologia , Percepção Visual/fisiologia , Adulto , Córtex Cerebral/diagnóstico por imagem , Feminino , Humanos , Imageamento por Ressonância Magnética , Mutação , Rede Nervosa/diagnóstico por imagem , Neuroimagem , Lobo Occipital/anormalidades , Lobo Occipital/diagnóstico por imagem , Transtornos da Percepção/diagnóstico por imagem , Transtornos da Percepção/genética
8.
Neuropediatrics ; 49(1): 63-67, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-28938499

RESUMO

Herpes simplex encephalitis (HSE) has been increasingly reported after neurosurgical procedures, mostly after tumor resections in patients with a prior history of HSE. Early detection and appropriate treatment are essential to prevent high mortality of the disease; however, there are diagnostic difficulties due to nonspecific prodromal symptoms. In addition, anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been reported after HSE as an immunological relapse. Here, we report a case of postherpetic anti-NMDAR encephalitis following right hemispherotomy for intractable startle-induced seizures, to emphasize the importance of early diagnosis and appropriate treatment. To our knowledge, this is the first reported case of anti-NMDAR encephalitis after postoperative HSE, and the third reported case of hemispherotomy as a curative treatment for startle epilepsy.


Assuntos
Epilepsia/etiologia , Hemisferectomia/efeitos adversos , Complicações Pós-Operatórias/etiologia , Adolescente , Encefalite Antirreceptor de N-Metil-D-Aspartato/diagnóstico por imagem , Encefalite Antirreceptor de N-Metil-D-Aspartato/cirurgia , Epilepsia/diagnóstico por imagem , Feminino , Humanos , Imageamento por Ressonância Magnética
9.
Int J Neurosci ; 128(5): 421-428, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-28988523

RESUMO

PURPOSE: There is evidence that autoimmunity has a specific role in temporal lobe seizures of limbic encephalitis patients. Our aim in this study was to investigate any histopathological clues of autoimmune process in refractory temporal lobe epilepsy (TLE) patients with different pathologically proven hippocampal sclerosis (HS) types. METHODS: 22 patients who had undergone epilepsy surgery due to mesial TLE-HS were included. The sera of patients are tested for neuronal antibodies to N-methyl-D-aspartate receptors (NMDAR), leucine-rich, glioma inactivated 1 (LGI1), contactin-associated protein 2 (CASPR2), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma-aminobutyric acid B receptor (GABABR) and glutamic acid decarboxylase (GAD). Pathological and immunohistochemical investigations including neuronal nuclei (NeuN), NMDAR, GAD, glial fibrillary acidic protein (GFAP), CD8+-CD3+ lymphocytes and immunoglobulin G (IgG) were done. Patients were grouped according to type of HS. Clinical features and immunohistochemical changes were defined in these groups. RESULTS: Available sera of 15 patients did not have any neuronal antibodies. Thirteen of 22 patients had HS type 1, three had HS type 2 and two had HS type 3. According to immunohistochemical investigations CD3+ and CD8+ T cell infiltration was more prominent in the hippocampus of patients with classical HS (International League Against Epilepsy (ILAE) Type 1 HS) and there was a significant negative correlation between epilepsy duration and numbers of CD3+-CD8+ lymphocytes in temporal lobe parenchyma. CONCLUSION: The role of T cell-mediated immunopathology and immunopathological difference in a variety of drug resistant TLE-H2S patients was suggested. These findings can be helpful in understanding the epileptogenicity of HS.


Assuntos
Epilepsia do Lobo Temporal/sangue , Epilepsia do Lobo Temporal/imunologia , Hipocampo/patologia , Imunoglobulina G/sangue , Linfócitos/metabolismo , Adolescente , Adulto , Antígenos CD/imunologia , Epilepsia do Lobo Temporal/complicações , Feminino , Glutamato Descarboxilase/metabolismo , Hipocampo/metabolismo , Humanos , Masculino , Proteínas do Tecido Nervoso/imunologia , Receptores de Superfície Celular/imunologia , Estudos Retrospectivos , Esclerose/classificação , Esclerose/etiologia , Esclerose/patologia , Estatística como Assunto , Lobo Temporal/metabolismo , Lobo Temporal/patologia , Adulto Jovem
10.
Neuropediatrics ; 47(4): 259-62, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27104483

RESUMO

Stroke-like migraine attacks after radiation therapy (SMART) syndrome is a rare complication of cranial radiotherapy characterized by migraine-like headache and transient neurological deficits with typical gyriform enhancement on magnetic resonance imaging (MRI). Potential underlying mechanisms are endothelial damage or dysfunction, vascular instability, vasospasm and, neuronal dysfunction.We report an 11-year-old girl with a primary diagnosis of medulloblastoma presented with acute-onset severe headache and left-sided weakness, 20 months after completing cranial radiotherapy. MRI demonstrated unilateral cortical swelling and concomitant leptomeningeal, gyral contrast enhancement, and MR perfusion imaging showed increased cortical perfusion in the right temporo-parieto-occipital region. Her symptoms resolved spontaneously over several days.SMART syndrome appears to be a reversible, long-term complication of cranial radiotherapy. So far, a limited number of pediatric patients with SMART syndrome have been reported. Prompt recognition of clinical signs and radiological imaging of SMART syndrome may help prevent unnecessary interventions and initiate appropriate diagnostic workup and management.


Assuntos
Cérebro/diagnóstico por imagem , Irradiação Craniana/efeitos adversos , Transtornos de Enxaqueca/etiologia , Neoplasias Cerebelares/radioterapia , Cérebro/irrigação sanguínea , Criança , Feminino , Humanos , Angiografia por Ressonância Magnética , Imageamento por Ressonância Magnética , Meduloblastoma/radioterapia , Transtornos de Enxaqueca/diagnóstico por imagem , Síndrome
11.
Int J Neurosci ; 126(9): 805-811, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26268999

RESUMO

PURPOSE: Smoking has been associated with an increased risk of developing multiple sclerosis, disease progression and clinical disability. We detected the effects of smoking on regional brain volumes and lesion load in patients with clinically isolated syndrome using quantitative magnetic resonance imaging. MATERIALS AND METHODS: Smoker patients (n = 16), smoker healthy controls (n = 13), non-smoker patients (n = 17) and non-smoker healthy controls (n = 14) underwent magnetic resonance imaging and neocortical volumes were measured. Lesion load was calculated in terms of number and volume of white matter hyperintensities. RESULTS: Smoking was associated with increased gray matter volumes in several regions of the brain. A tendency towards greater lesion load in smoker patients was found. Smoking duration was significantly negatively correlated with intracranial volume and left hemisphere cortical gray matter volume. There was no relationship between regional brain volumes and clinical disability scores, lesion load duration of the disease and degree of smoking exposure. CONCLUSIONS: Clinically isolated syndrome related regional brain atrophy might vary in extent and severity with smoking. Despite increased lesion load, less cortical and deep gray matter damage with a possible neuroprotective effect occurs in smoking.


Assuntos
Doenças Desmielinizantes/diagnóstico por imagem , Substância Cinzenta/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Fumar/efeitos adversos , Substância Branca/diagnóstico por imagem , Adulto , Atrofia/diagnóstico por imagem , Estudos Transversais , Feminino , Humanos , Masculino , Adulto Jovem
12.
J Stroke Cerebrovasc Dis ; 25(2): 480-3, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26652209

RESUMO

BACKGROUND: Early elevations in the parenchymal signal intensity on T2-weighted images, including fluid-attenuated inversion recovery (FLAIR) sequences, within the ischemic territory are considered as a marker of successful recanalization following thrombolytic treatment. In this study, our aim was to assess whether quantitatively determined FLAIR hyperintensity increases could be predictive of improved functional outcome in patients with acute ischemic stroke. METHODS: Patients receiving intravenous thrombolysis for proximal anterior circulation strokes were included in the study. FLAIR hyperintensity ratio was determined on magnetic resonance imaging obtained within 72 hours of symptom onset. Univariate and multivariate analyses were performed to determine predictors of good functional outcome at 90 days. RESULTS: The study population was composed of 65 patients. The median (interquartile range) FLAIR hyperintensity ratio was significantly higher among patients with good functional outcome (modified Rankin Scale score ≤ 3 at day 90, 1.4 [1.2-1.7] versus 1.2 [1.1-1.4], P = .005). Patients with a FLAIR hyperintensity ratio of 1.3 or higher were 4.4 (95% confidence interval 1.6-12.7) times more likely to be independent functionally at the end of 3 months. Higher admission National Institutes of Health Stroke Scale score and age, together with lower FLAIR hyperintensity ratio (P = .006), were found to be significantly and independently related to unfavorable outcome at 90-day follow-up in multivariate analyses. CONCLUSIONS: Our findings suggest that a rise in FLAIR hyperintensity signal within the ischemic tissue is suggestive of favorable outcome in patients undergoing intravenous thrombolysis. This tissue marker of favorable outcome is irrespective of other parameters that are crucial in the prognosis of ischemic stroke, such as age and stroke severity.


Assuntos
Isquemia Encefálica/tratamento farmacológico , Encéfalo/patologia , Fibrinolíticos/uso terapêutico , Acidente Vascular Cerebral/tratamento farmacológico , Ativador de Plasminogênio Tecidual/uso terapêutico , Idoso , Idoso de 80 Anos ou mais , Isquemia Encefálica/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Prognóstico , Acidente Vascular Cerebral/patologia , Terapia Trombolítica , Resultado do Tratamento
13.
Stroke ; 46(3): 634-40, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25634003

RESUMO

BACKGROUND AND PURPOSE: The widespread use of ambulatory cardiac monitoring has not only increased the detection of high-risk arrhythmias like persistent and paroxysmal atrial fibrillation (AF), but also made it possible to identify other aberrations such as short-lasting (<30 seconds) irregular runs of supraventricular tachycardia. Ischemic stroke phenotype might be helpful in understanding whether these nonsustained episodes play a similar role in stroke pathophysiology like their persistent and paroxysmal counterparts. METHODS: In a consecutive series of patients with ischemic stroke, we retrospectively determined clinical and imaging features associated with nonsustained AF (n=126), defined as <30-second-lasting supraventricular tachyarrhythmias with irregular RR interval on 24-hour Holter monitoring, and compared them to patients with persistent/paroxysmal AF (n=239) and no AF (n=246). RESULTS: Patients with persistent/paroxysmal AF significantly differed from patients with nonsustained AF by a higher prevalence of female sex (odds ratio [95% confidence interval], 1.8 [1.1-2.9]), coronary artery disease (1.9 [1.1-3.0]), and embolic imaging features (2.7 [1.1-6.5]), and lower frequency of smoking (0.4 [0.2-0.8]) and hyperlipidemia (0.5 [0.3-0.8]). In contrast, patients with no AF were younger (0.5 [0.4-0.6] per decade) and more likely to be male (1.7 [1.0-2.8]) in comparison with nonsustained AF population. The prevalence of nonsustained AF was similar among cryptogenic and noncryptogenic stroke patients (32% versus 29%). Voxel-wise comparison of lesion probability maps revealed no significant difference between cryptogenic stroke patients with and without nonsustained AF. CONCLUSIONS: Clinical features of patients with nonsustained AF exhibited an intermediary phenotype in between patients with persistent/paroxysmal AF and no AF. Furthermore, imaging features did not entirely resemble patterns observed in patients with longer durations of AF.


Assuntos
Fibrilação Atrial/fisiopatologia , Eletrocardiografia Ambulatorial/métodos , Acidente Vascular Cerebral/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Fibrilação Atrial/diagnóstico , Isquemia Encefálica/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Probabilidade , Estudos Retrospectivos , Taquicardia Supraventricular/diagnóstico
14.
Childs Nerv Syst ; 31(6): 971-5, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25686891

RESUMO

INTRODUCTION: Rosai-Dorfman Disease (RDD) is a benign histiocytic disorder that commonly presents with massive lymphadenopathy. Central nervous system (CNS) involvement is only 5%; isolated CNS involvement is much rarer. The patient presented here has been diagnosed with isolated pontine RDD. CASE REPORT: A 5-year-old child was admitted to the hospital with the history of double vision and ptosis of the left eye. Magnetic resonance imaging revealed presence of a mass at the pontomesencephalic junction. Subtotal tumor excision was performed. Microscopic examination of the lesion showed histologic features consistent with RDD. The patient was followed up without medication, with a small residue. CONCLUSION: RDD is rare in childhood, and isolated CNS involvement is also quite rare. The tumor is located in pons in our case. The treatment of choice is gross total resection. Even total excision is not possible, follow up without medication may be an option because of the indolent and self-limited course of the disease.


Assuntos
Histiocitose Sinusal/patologia , Histiocitose Sinusal/cirurgia , Procedimentos Neurocirúrgicos/métodos , Ponte/patologia , Pré-Escolar , Histiócitos/patologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Ponte/cirurgia
15.
Epileptic Disord ; 16(4): 500-5, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25496967

RESUMO

Musicogenic seizure is classified as a rare form of complex reflex seizures. We present a patient with musicogenic seizures from whom invasive recordings were obtained using subdural arrays, as well as hippocampal depth electrodes. Interestingly, this patient had both spontaneous seizures and musicogenic seizures, and they originated from different hippocampi. Due to bilateral independent musicogenic seizures and spontaneous seizures, our patient was not eligible for surgery, but vagal nerve stimulation treatment was almost successful. [Published with video sequence].


Assuntos
Eletroencefalografia/métodos , Epilepsia Reflexa/diagnóstico , Epilepsia do Lobo Temporal/diagnóstico , Hipocampo/fisiopatologia , Lobo Temporal/fisiopatologia , Adulto , Eletrodos Implantados , Epilepsia Reflexa/fisiopatologia , Epilepsia do Lobo Temporal/fisiopatologia , Feminino , Humanos
16.
J Stroke Cerebrovasc Dis ; 23(1): 12-6, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-22748712

RESUMO

The use of narrower window width settings on computed tomography (CT) improves sensitivity for detection of early ischemic changes in acute ischemic stroke. This study analyzed the effect of optimization of window settings on the accuracy of Alberta Stroke Program Early Computed Tomography Score (ASPECTS) performed on noncontrast CT (NCCT) and CT angiography source images (CTA-SI). ASPECTS was calculated on NCCT and CTA-SI with standard and optimized window width/center settings in a consecutive series of patients with acute ishcemic stroke. The difference between CT-based ASPECTS and ASPECTS performed on follow-up magnetic resonance imaging (MRI) were calculated to determine the disparity between initial estimates of the extent of ischemia on CT and follow-up lesion imaging by MRI. Forty-four patients were included into the study. The mean difference with respect to follow-up MRI-ASPECTS was 4.1 ± 2.2 for standard NCCT-ASPECTS, 3.7 ± 2.3 for optimized NCCT-ASPECTS, 3.0 ± 2.2 for standard CTA-SI-ASPECTS, and 2.7 ± 2.1 for optimized CTA-SI-ASPECTS. The improvement introduced by the optimization of window settings and use of CTA-SI was statistically significant (P < .01). Our data indicate that the accuracy of ASPECTS is improved with optimized window display settings. This improvement is irrespective of experience or specialty of the rater performing the assessment.


Assuntos
Isquemia Encefálica/diagnóstico , Angiografia Cerebral/métodos , Angiografia Cerebral/normas , Acidente Vascular Cerebral/diagnóstico , Tomografia Computadorizada por Raios X/métodos , Tomografia Computadorizada por Raios X/normas , Adulto , Idoso , Alberta , Encéfalo/diagnóstico por imagem , Isquemia Encefálica/diagnóstico por imagem , Isquemia Encefálica/patologia , Progressão da Doença , Feminino , Seguimentos , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Reprodutibilidade dos Testes , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/patologia , Terapia Trombolítica
17.
J Stroke Cerebrovasc Dis ; 23(3): 421-6, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23583014

RESUMO

BACKGROUND: Aging is associated with decreased penumbral salvage in patients with ischemic stroke. Another critical factor that determines the fate of penumbra tissue is the degree of collateral circulation, which decreases significantly with aging in experimental models of stroke. In this study, we sought to identify whether these observations could be translated to humans and, therefore, analyzed the effect of patient age on extent of leptomeningeal collaterals in patients with ischemic stroke. METHODS: Computed tomography angiography (CTA) source images were used to assess the degree of collateral circulation in a retrospective series of patients with proximal middle cerebral artery (MCA) occlusion. Bivariate and multivariate analyses were used to explore the relationship between patient age and degree of collateral circulation. RESULTS: A total of 70 patients were included into the study. Older age (P = .005), history of hypertension (P = .036), higher admission National Institutes of Health Stroke Scale (NIHSS) scores (P = .013), and increased time to CTA (P = .013) were associated with inadequate collaterals in bivariate analyses. In multivariate analysis, older age (P = .008) and higher NIHSS scores (P = .032) remained as the only significant independent variables that were associated with inadequate collaterals. A 10-year increment in patient age increased the odds of inadequate collateral circulation by 1.87 (95% confidence interval: 1.18-2.97). CONCLUSION: Our findings show that there is a significant interplay between patient age and adequacy of leptomeningeal collateral circulation in patients with proximal MCA occlusion. The relationship could contribute to adverse tissue outcome and thereby to unfavorable clinical outcome observed in elderly patients with ischemic stroke.


Assuntos
Envelhecimento , Circulação Cerebrovascular , Circulação Colateral , Infarto da Artéria Cerebral Média/fisiopatologia , Meninges/irrigação sanguínea , Fatores Etários , Idoso , Angiografia Cerebral/métodos , Distribuição de Qui-Quadrado , Avaliação da Deficiência , Feminino , Humanos , Infarto da Artéria Cerebral Média/diagnóstico por imagem , Infarto da Artéria Cerebral Média/etiologia , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Razão de Chances , Prognóstico , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X
18.
Med Image Anal ; 94: 103121, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38402791

RESUMO

Curation of large, diverse MRI datasets via multi-institutional collaborations can help improve learning of generalizable synthesis models that reliably translate source- onto target-contrast images. To facilitate collaborations, federated learning (FL) adopts decentralized model training while mitigating privacy concerns by avoiding sharing of imaging data. However, conventional FL methods can be impaired by the inherent heterogeneity in the data distribution, with domain shifts evident within and across imaging sites. Here we introduce the first personalized FL method for MRI Synthesis (pFLSynth) that improves reliability against data heterogeneity via model specialization to individual sites and synthesis tasks (i.e., source-target contrasts). To do this, pFLSynth leverages an adversarial model equipped with novel personalization blocks that control the statistics of generated feature maps across the spatial/channel dimensions, given latent variables specific to sites and tasks. To further promote communication efficiency and site specialization, partial network aggregation is employed over later generator stages while earlier generator stages and the discriminator are trained locally. As such, pFLSynth enables multi-task training of multi-site synthesis models with high generalization performance across sites and tasks. Comprehensive experiments demonstrate the superior performance and reliability of pFLSynth in MRI synthesis against prior federated methods.


Assuntos
Aprendizagem , Imageamento por Ressonância Magnética , Humanos , Reprodutibilidade dos Testes
19.
J Stroke Cerebrovasc Dis ; 22(3): 250-4, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-21963218

RESUMO

Concomitant acute ischemic lesions are detected in a subset of patients with intracerebral hemorrhage (ICH). In this study, our aim was to analyze the pattern of acute ischemic lesions detected by diffusion-weighted imaging (DWI) in patients with ICH, and to use this information, in combination with clinical characteristics of patients, to understand the underlying mechanisms of these lesions. We retrospectively analyzed patients with a diagnosis of ICH who underwent DWI within 14 days of symptom onset. We compared demographic, clinical, and imaging characteristics in patients with and without acute ischemic lesions. We also assessed the number, location, and topographic distribution of DWI bright lesions. Acute ischemic lesions were detected in 15 of 86 patients (17.4%); the lesions had a small, dot-like appearance in 13 patients (87%) and were located in an arterial territory separate from the incident ICH in 12 patients (80%). Patients with acute ischemic lesions had higher admission systolic, diastolic, and mean arterial blood pressure levels; greater periventricular leukoaraiosis burden; more microbleeds, and lower admission Glasgow Coma Scale score. In multivariate analyses, admission mean arterial blood pressure (P < .01) and Glasgow Coma Scale score (P =.03) remained as the only significant variables associated with DWI lesion positivity. Our findings highlight the role of elevated admission blood pressure in the development of concomitant acute ischemic lesions in patients with ICH. The pattern of DWI bright lesions, together with a trend toward an increased burden of leukoaraiosis and microbleeds in patients with acute ischemic lesions, suggest an underlying dysfunctional cerebral microvasculature in the etiology of these lesions.


Assuntos
Pressão Sanguínea , Isquemia Encefálica/complicações , Encéfalo/patologia , Hemorragia Cerebral/complicações , Hipertensão/complicações , Admissão do Paciente , Doença Aguda , Idoso , Isquemia Encefálica/diagnóstico , Isquemia Encefálica/fisiopatologia , Hemorragia Cerebral/diagnóstico , Hemorragia Cerebral/fisiopatologia , Distribuição de Qui-Quadrado , Imagem de Difusão por Ressonância Magnética , Feminino , Escala de Coma de Glasgow , Humanos , Hipertensão/diagnóstico , Hipertensão/fisiopatologia , Hemorragia Intracraniana Hipertensiva/complicações , Hemorragia Intracraniana Hipertensiva/fisiopatologia , Leucoaraiose , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Razão de Chances , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores de Risco
20.
Ann Otol Rhinol Laryngol ; 132(7): 825-827, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35904196

RESUMO

OBJECTIVE: Our aim is to describe an unusual pattern of craniocervical pneumatization resulting in a spontaneous fracture in a patient, who presented with tinnitus. METHODS: Temporal CT was ordered in a patient with tinnitus lasting over a year. His audiogram was normal. No history of previous trauma, recent flight, diving, or weight lifting was present. Further questioning revealed his habitual Valsalva maneuvers to relieve symptoms of nasal congestion. RESULTS: Temporal CT showed abnormal pneumatization of the occipital bone and C1 vertebra. A definite cortical defect resulted with the presence of air around the neighboring soft tissues. The emphysema was most prominent in the suboccipital fossa but it was also seen in the epidural space of the spinal canal. CONCLUSION: Abnormal pneumatization of the skull base and cervical vertebra can be symptomatic due to its tendency to fracture. The presumed cause of pneumatization in this patient is habitual Valsalva maneuvers.


Assuntos
Zumbido , Humanos , Tomografia Computadorizada por Raios X , Base do Crânio/diagnóstico por imagem , Vértebras Cervicais , Osso Occipital
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