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Weng Wen-Chin - - 2010
Acute necrotizing encephalopathy of childhood is a rare acute encephalopathy occurring after febrile illness with subsequent rapid deterioration of consciousness and convulsions. Typical brain magnetic resonance imaging (MRI) findings are symmetric involvement of the bilateral thalami, brainstem, and cerebral periventricular white matter or the cerebellum. Spinal cord involvement has never ...
Shah Reena S RS Departments of Neurology, University of Maryland, Baltimore, MD 21201, - - 2010
Granulocytic sarcomas, or chloromas, are extramedullary collections of immature granulocytes. Central nervous system involvement is rare and of those cases described, most are complications of acute myelogenous leukemia. A 40-year-old man with chronic myelogenous leukemia presented with seizure and encephalopathy. Magnetic resonance imaging of the brain revealed temporal T2 hyperintensities ...
Tian Hui-jun - - 2010
MRI has had an important role in the diagnosis of Creutzfeldt-Jakob disease (CJD). The aim of our study was to compare the efficacy of different MRI sequences among six biopsy-proven patients with sporadic CJD (sCJD) and seven patients with probable sCJD. These 13 patients with CJD aged from 36 years ...
Koehl Bérengère - - 2010
We report the case of a 4-year-old boy, diagnosed with atypical hemolytic uremic syndrome (HUS) due to a hybrid factor H. He progressed to end-stage renal failure despite plasmatherapy and underwent bilateral nephrectomy because of uncontrolled hypertension. Three days after, he had partial complex seizures with normal blood pressure, normal ...
Kim Tae Kyun - - 2010
We report the case of a 46-year-old woman presenting with postoperative bilateral cerebral visual loss that was initially misinterpreted as an irreversible ischemic event. Magnetic resonance imaging of the brain showed high signal intensity on T2-weighted and fluid-attenuated inversion recovery images and normal signal intensity on diffusion-weighted images of the ...
Tatsumoto N - - 2010
A 59-year-old male presented at our hospital with disturbance of consciousness. He had severe neurological disturbances associated with uremia caused by severe renal insufficiency. Cranial computed tomography (CT) was normal on admission. FLAIR-weighted MRI showed increased signal intensities bilaterally in the cortical and subcortical areas of the occipital lobe. Repeated ...
Palus Viktor - - 2010
Thiamine (vitamin B(1)) is an essential component of a number of metabolic pathways and thiamine deficiency results in a progressive encephalopathy in both humans and animals. Confirming thiamine deficiency is problematic and relies on demonstrating reduced red blood cells transketolase activity, or indirect methods including urinary organic acid analysis and ...
Salvatore Elena - - 2010
Benign hereditary chorea is an autosomal dominant disorder characterized by early onset nonprogressive chorea, caused by mutations of the thyroid transcription factor-1 (TITF-1) gene. Clinical heterogeneity has been reported and thyroid and respiratory abnormalities may be present. We describe 3 patients of an Italian family carrying the S145X mutation in ...
Baek Shin-Hye - - 2010
This is the first case of virus-associated encephalitis/encephalopathy in which the pathogen was Hantaan virus. A 53-yr-old man presented fever, renal failure and a hemorrhagic tendency and he was diagnosed with hemorrhagic fever with renal failure syndrome (HFRS). In the course of his illness, mild neurologic symptoms such as dizziness ...
Zuccoli G - - 2011
SUMMARY: We present the neuroimaging and clinical findings in 2 nonalcoholic adult patients with WE as assessed by MR imaging. The first patient presented with gait ataxia and changes in consciousness. MR imaging disclosed bilateral lesions in the dorsal striatum and cerebellum. None of the regions typically affected in WE ...
Dehkharghani S - - 2010
Stenosis of a DVA may result in chronic venous ischemia. We present 6 patients (3 men, 3 women; age range, 30-79 years; mean age, 53 years) with unilateral calcification of the caudate and putamen on noncontrast CT. This calcification typically spared the anterior limb of the internal capsule. No patient ...
Vang Fredrik Johannes - - 2010
Magnetic resonance imaging was used to compare subcortical volumes of seven suicide attempters with those of six healthy controls. Suicide attempters had 10% smaller right caudate nucleus and 19% bilaterally smaller globus pallidus. In suicide attempters, volumes of the globus pallidus correlated negatively with previously reported measures of solidity (non-impulsive ...
Song Ji-Eun - - 2010
We report a 59-year-old woman who presented to our facility with conduction aphasia as an initial symptom which, within 3months, was followed by generalized myoclonus and global aphasia. She had difficulty repeating words during the Korean-Western Aphasia Battery test. Diffusion-weighted MRI demonstrated ribbon-like hyperintensities in the bilateral temporal, parietal and ...
Kumar Gyanendra - - 2010
Bilateral basal ganglia lesions are neither diagnostic nor pathognomonic of uremic encephalopathy (UE). Nonetheless, bilateral basal ganglia T2/FLAIR hyperintensities have been widely reported to be associated with UE. The aim of this study was to describe a unique neuroradiological sign seen on the MRI brain in UE, present a retrospective ...
Burrus Tamika M - - 2010
Little is known of the nature of the neurologic manifestations in thrombotic thrombocytopenic purpura (TTP). We have recently reported posterior reversible encephalopathy syndrome (PRES) as the predominant brain abnormality in patients with TTP. Posterior reversible encephalopathy syndrome has been associated with a variety of medications and pathologic states including increased ...
Thomas Katherine P - - 2010
Pseudohypoparathyroidism is a rare cause of paroxysmal dyskinesias. We describe an otherwise well 10-year-old girl who was diagnosed with pseudohypoparathyroidism type Ib after presenting with involuntary movements of the hands and feet that occurred while running or walking. Magnetic resonance imaging of the brain indicated T(1) hyperintensities of the bilateral ...
Gutierrez Leonardo Guilhermino - - 2010
Non-neonatal hypoxic-ischemic encephalopathy is a clinical condition often related to cardiopulmonary arrest that demands critical management and treatment decisions. Management depends mainly on the degree of neurological impairment and prognostic considerations. Computed tomography (CT) is often used to exclude associated or mimicking pathology. If any, only nonspecific signs such as ...
Rufa Alessandra - - 2010
The supranuclear paresis of the abducens system, also known as posterior internuclear ophthalmoplegia of abduction, is a very rare disorder clinically characterized by unilateral or bilateral abduction paresis sometimes associated with nystagmus of the contralateral adducting eye, slowing of abduction saccades, and intact horizontal vestibulo-ocular reflex. Here, we report a ...
Zhang Xia-ping - - 2010
To report a case of Wernicke encephalopathy in the early stage after surgery. A nonalcoholic female patient with hepatitis B-related cirrhosis and hypersplenism underwent splenectomy in a local hospital. No surgical complications occurred and the patient recovered well. However, on the eighth postoperative day she developed psychiatric and neurological disturbance ...
Naqi Rohana - - 2010
Posterior Reversible Encephalopathy Syndrome (PRES) refers to a clinicoradiologic entity with characteristic features on neuro-imaging and non-specific symptoms comprising headache, confusion, visual disturbances and seizures. The lesions in PRES are thought to be due to vasogenic oedema, predominantly in the posterior cerebral hemispheres, and are reversible with appropriate management. We ...
Fugate Jennifer E JE Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, - - 2010
To identify and define clinical associations and radiologic findings of posterior reversible encephalopathy syndrome (PRES). Patients prospectively diagnosed as having PRES from October 1, 2005, through April 30, 2009, were pooled with retrospectively identified patients admitted from August 1, 1999, through September 30, 2005. We performed a detailed review of ...
Wang Zhen J - - 2010
OBJECTIVE: To assess the imaging features of celiac ganglia on multi-detector row computed tomography (MDCT). METHODS: We retrospectively reviewed 103 consecutive MDCT studies and recorded the frequency of visualization, location, morphologic feature, size, and enhancement pattern of the celiac ganglia. RESULTS: The left celiac ganglion was visualized more often than ...
Prashanth L K - - 2010
Magnetic resonance imaging (MRI) is frequently used in the evaluation of various extrapyramidal disorders. Among the plethora of MRI features in Wilson's disease (WD), only "face of the giant panda" sign has been recognized to distinguish WD from other early onset extrapyramidal disorders (EOEPD). To ascertain the value of various ...
Tzoulis Charalampos - - 2010
Mutations in the catalytic subunit of the mitochondrial DNA-polymerase gamma cause a wide spectrum of clinical disease ranging from infantile hepato-encephalopathy to juvenile/adult-onset spinocerebellar ataxia and late onset progressive external ophthalmoplegia. Several of these syndromes are associated with an encephalopathy that characteristically shows episodes of rapid neurological deterioration and the ...
Dicuonzo Franca - - 2010
Acute movement disorders associated with bilateral lesions in the basal ganglia are increasingly described in patients affected by diabetes and uremia. Pathophysiology has not been utterly understood yet, but it is likely to be multifactorial, with both ischemic/microvascular and metabolic/toxic factors determining the lesions and symptoms. We have studied a ...
Azzopardi Denis D Institute of Clinical Sciences, Imperial College London and MRC Clinical Sciences Centre, Hammersmith Hospital, London, UK. - - 2010
Evaluation of infants with hypoxic ischemic encephalopathy by magnetic resonance spectroscopy and imaging is useful to direct clinical care, and may assist the evaluation of candidate neuroprotective therapies. Cerebral metabolites measured by magnetic resonance spectroscopy, and visual analysis of magnetic resonance images during the first 30 days after birth accurately ...
Santos Andrade C - - 2010
Wernicke's encephalopathy (WE) is a serious neurological disorder secondary to thiamine deficiency. Improved recognition by radiologists and allied health providers of the different clinical settings and imaging findings associated with this emergency can optimise the management of this condition and help prevent its severe consequences. The aim of this study ...
Ormitti F - - 2010
The recently emerged novel influenza A(H1N1) virus continues to spread globally. The clinical disease generally appears mild, but unfavorable outcomes have been reported. We describe a case of a 3-year-old Italian girl infected with influenza A(H1N1) virus presenting with neurologic deterioration. CT findings were negative, but MR imaging findings were ...
Noguchi Tomoyuki - - 2010
OBJECTIVE: It is important to differentiate human herpesvirus 6 (HHV-6)-associated encephalopathy from herpes simplex encephalitis (HSE). Although these conditions are similar with regard to involvement of the mesial temporal lobe, HSE is sensitive to acyclovir but HHV-6 encephalopathy is not. We compared the imaging findings of the two conditions. MATERIALS ...
Allmendinger Andrew M - - 2010
Susac syndrome is a rare neurologic disorder first described by Susac et al. in 1979. Clinically, Susac syndrome consists of a triad including encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Microinfarction is believed to be the basic histologic feature and MR is considered the best imaging modality. This ...
Massaro A N - - 2010
To evaluate whether quantitative measures from magnetic resonance imaging (MRI) performed in hypothermia-treated encephalopathic newborns can differentiate patients with unfavorable neurological outcome. Retrospective analysis of clinical data and MRI studies was performed in 47 full-term infants treated with whole-body hypothermia for neonatal encephalopathy. Apparent diffusion coefficients (ADCs) and T1 and ...
Tesseki Kin - - 2010
Brainstem encephalitis is not a classic paraneoplastic syndrome and usually involves monophasic neurological deterioration and has negative magnetic resonance imaging (MRI) findings. We describe a patient with brainstem encephalitis who had elevated anti-Ri antibody levels and double-step neurological deterioration associated with different abnormal lesions on MRI. Immunosuppression with steroids and ...
Pinto R B - - 2010
BACKGROUND: Few studies have evaluated abnormalities on brain magnetic resonance imaging (MRI) in children and adolescents with chronic liver disease. AIMS: The aim of this study was to investigate the presence of T1 hyperintensity in the basal ganglia of pediatric patients with portal hypertension and its association with blood manganese ...
Vuolo Luisa - - 2010
Beh?et's disease is a multisystem disorder first described in 1937 as a triad of oral and genital ulcerations and uveitis. The etiology is unknown. Involvement of the central nervous system (CNS) occurs in 10-25% of patients. Neuro-Beh?et's disease (NBD) can be classified as: (1) parenchymal, with a predilection for brainstem, ...
Suzuki Kei - - 2010
A 30-year-old Brazilian man hospitalized with AIDS developed a high-grade fever. Neither culture studies nor radiological examinations revealed the cause; small yet highly intense signals in the basal ganglia were detected upon gadolinium (Gd)-enhanced T1-weighted magnetic resonance imaging (MRI) of the head. This finding was equivocal at that time but ...
Jurgens Caroline K - - 2010
Increased iron levels have been demonstrated in the basal ganglia of manifest Huntington's disease (HD). An excess in iron accumulation correlates with MRI T2-weighted hypointensity. Determination of the amount of hypointensities in the basal ganglia in the premanifest phase of HD may give more insight in the role of iron ...
Niwa Fumitoshi - - 2010
We describe a 72-year-old man who developed subacute onset parkinsonism caused by neuro-Sweet disease (NSD). Magnetic resonance imaging of this patient's brain suggested inflammation of the bilateral basal ganglia and amygdalae. Clinical symptoms and MRI findings gradually improved without medication. However, his parkinsonism recurred one month after discharge from the ...
Lyytinen Jukka - - 2010
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder in which accumulation of a pathogenic isoform of prion protein (PrP(Sc)) induces neuronal damage with distinct pathologic features. The prognosis of sCJD is devastating: rapid clinical decline is followed by death generally within months after onset of symptoms. The classic clinical ...
Asbury Arthur K - - 2010
On the occasion of the 125th anniversary of the Philadelphia Neurologic Society, 3 short talks were given that highlighted accomplishments by Philadelphia's neurologists over the past 30 years. The theme for the celebration was "Contributions to Neurology by Philadelphia Neurologists, 1980-2008." Each of the 3 speakers was chosen because of ...
Gimovsky Martin L - - 2010
BACKGROUND: Late postpartum eclampsia is more frequently recognized than past reports indicate. This report describes the association of a reversible encephalopathy in a woman with late postpartum eclampsia. CASE: A woman with lupus nephritis presented 7 days postpartum with eclampsia. Postseizure findings included dramatic short-term memory loss. Although a computed ...
Wu Hung-Ming - - 2010
To ascertain the characteristics of patients with sporadic Creutzfeldt-Jakob disease (CJD) and to determine the findings of electroencephalography (EEG) and brain magnetic resonance imaging (MRI). We pooled patients at a hospital from 2000 to 2008, and classified them according to WHO diagnostic criteria as having probable or possible CJD. We ...
Nagata Riya - - 2010
Gliomatosis cerebri (GC) is a specific entity defined as diffuse infiltration of neoplastic glial cells into at least three cerebral lobes and preservation of the surrounding neuronal architecture. We report a patient with secondary GC that mimicked clinicoradiological features of limbic encephalitis (LE). A 72-year-old man had developed headache and ...
Sung Pi-Shan - - 2010
We report a patient suffering from delayed encephalopathy 21 days after an acute CO intoxication. The initial magnetic resonance (MR) images in the acute stage show a recent infarct corresponding to a right middle cerebral artery (MCA) stenosis. MR images on the 24th day post-intoxication show typical changes of delayed ...
Chang K-H - - 2010
BACKGROUND: Non-ketotic hyperglycemic chorea-ballism (NKHCB) had special reversible hyperintense on T1-weighted imaging (T1WI) lesion in comparsion to gray matter. However, the mechanism accounts for these lesions is still unclear. METHODS: Patients diagnosed with NKHCB were recruited from 2002 to 2004. The demographic, clinical, magnetic resonance imaging (MRI), and spectroscopy (MRS) ...
Sikk K - - 2010
OBJECTIVE: To identify biomarkers supporting the clinical diagnosis of manganism in patients several years after exposure to manganese (Mn). METHODS: Neurophysiological examinations, magnetic resonance imaging (MRI), single-photon emission computed tomography and fluorodeoxyglycose (FDG) positron emission tomography were performed in four former ephedrone addicts with extrapyramidal symptoms. RESULTS: Peripheral nervous system ...
Kimura Ryo - - 2010
OBJECTIVE: To highlight the association between posterior reversible encephalopathy syndrome (PRES) and chronic alcoholism. METHODS: We present a case report, a review of the literature and a discussion. RESULTS: We report on the case of a 51-year-old man with chronic alcoholism, who suddenly developed visual disturbance and confusion. Magnetic resonance ...
Geibprasert Sasikhan - - 2010
This review provides an overview of structural magnetic resonance imaging and computed tomography findings of direct and indirect alcohol-related toxic effects on the brain. In addition to ethanol-related changes to the brain, this article will also describe imaging findings in the acute setting of methanol and ethylene glycol poisoning. Alcohol ...
Kremer Stéphane - - 2010
The clinical and radiological presentations of Epstein-Barr virus (EBV) encephalitis are pleomorphic, but a common and characteristic finding is an increased T2-weighted signal in the bilateral thalami and basal ganglia. We report here a case of post-transplant acute limbic encephalitis (PALE) syndrome that was possibly related to EBV infection. Six ...
Dung N M NM Centre for Tropical Diseases, Cho Quan Hospital, Ho Chi Minh City, - - 2009
Japanese encephalitis virus (JEV) is estimated to cause 30–50,000 cases of encephalitis every year. The disease occurs mainly in rural Asia and is transmitted to humans from birds and pigs by mosquitoes of the genus Culex. JE is diagnosed with antibody testing of the serum and CSF, but this is ...
Del Gaizo Andrew - - 2009
Glycogen storage disease type II (GSDII), also referred to as Pompe disease or acid maltase deficiency, is a rare inherited condition caused by a deficiency in acid alpha-glucosidase (GAA) enzyme activity (Tinkle andLeslie. GeneReviews, 2008. http://www.genetests.org). The condition is often classified by age of presentation,with infantile and late onset variants ...
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