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Suiqiang Zhu - One of the best experts on this subject based on the ideXlab platform.
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Isolated Cerebellar Involvement in posterior reversible encephalopathy syndrome.
Journal of the neurological sciences, 2015Co-Authors: Lifei Lian, Suiqiang ZhuAbstract:Posterior reversible encephalopathy syndrome (PRES) is a serious and increasingly recognized disorder in humans. However, isolated Cerebellar Involvement in PRES is extremely uncommon. In this study, we sought to investigate its clinical and radiological features by describing a cohort of cases with PRES and isolated Cerebellar Involvement. We report 2 patients with PRES with only Cerebellar Involvement and identified additional 9 cases using the PubMed database with the MeSH terms "posterior reversible encephalopathy syndrome", "hypertensive encephalopathy", "hypertension", "cerebellum", "encephalopathy", and "magnetic resonance imaging". We then collectively analyzed the clinical and imaging characteristics of these 11 cases. The average age was 28years, with 8 male and 3 female patients. All cases had severe acute hypertension and T2 hyperintensity on MRI exclusively centered within the cerebellum. Of 11 patients, 7 had hypertensive retinopathy, a favorable clinical course with only antihypertensive treatment, and resolution of the Cerebellar lesions on follow-up imaging. A total of 5 of the 11 patients received external ventricular drainage due to obstructive hydrocephalus and only 2 of the 11 had a seizure. Isolated Cerebellar Involvement in PRES may be a unique variant that affects younger, male cases with severe acute hypertension and hypertensive retinopathy, but not necessarily seizure. Most patients have full recovery after fast control of blood pressure. Awareness of atypical neuroimaging features in PRES is critical for appropriate treatment. Copyright © 2015 Elsevier B.V. All rights reserved.
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Isolated Cerebellar Involvement in posterior reversible encephalopathy syndrome.
Journal of the Neurological Sciences, 2015Co-Authors: Lifei Lian, Suiqiang ZhuAbstract:Abstract Background Posterior reversible encephalopathy syndrome (PRES) is a serious and increasingly recognized disorder in humans. However, isolated Cerebellar Involvement in PRES is extremely uncommon. In this study, we sought to investigate its clinical and radiological features by describing a cohort of cases with PRES and isolated Cerebellar Involvement. Methods We report 2 patients with PRES with only Cerebellar Involvement and identified additional 9 cases using the PubMed database with the MeSH terms “posterior reversible encephalopathy syndrome”, “hypertensive encephalopathy”, “hypertension”, “cerebellum”, “encephalopathy”, and “magnetic resonance imaging”. We then collectively analyzed the clinical and imaging characteristics of these 11 cases. Results The average age was 28 years, with 8 male and 3 female patients. All cases had severe acute hypertension and T2 hyperintensity on MRI exclusively centered within the cerebellum. Of 11 patients, 7 had hypertensive retinopathy, a favorable clinical course with only antihypertensive treatment, and resolution of the Cerebellar lesions on follow-up imaging. A total of 5 of the 11 patients received external ventricular drainage due to obstructive hydrocephalus and only 2 of the 11 had a seizure. Conclusions Isolated Cerebellar Involvement in PRES may be a unique variant that affects younger, male cases with severe acute hypertension and hypertensive retinopathy, but not necessarily seizure. Most patients have full recovery after fast control of blood pressure. Awareness of atypical neuroimaging features in PRES is critical for appropriate treatment.
Lifei Lian - One of the best experts on this subject based on the ideXlab platform.
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Isolated Cerebellar Involvement in posterior reversible encephalopathy syndrome.
Journal of the neurological sciences, 2015Co-Authors: Lifei Lian, Suiqiang ZhuAbstract:Posterior reversible encephalopathy syndrome (PRES) is a serious and increasingly recognized disorder in humans. However, isolated Cerebellar Involvement in PRES is extremely uncommon. In this study, we sought to investigate its clinical and radiological features by describing a cohort of cases with PRES and isolated Cerebellar Involvement. We report 2 patients with PRES with only Cerebellar Involvement and identified additional 9 cases using the PubMed database with the MeSH terms "posterior reversible encephalopathy syndrome", "hypertensive encephalopathy", "hypertension", "cerebellum", "encephalopathy", and "magnetic resonance imaging". We then collectively analyzed the clinical and imaging characteristics of these 11 cases. The average age was 28years, with 8 male and 3 female patients. All cases had severe acute hypertension and T2 hyperintensity on MRI exclusively centered within the cerebellum. Of 11 patients, 7 had hypertensive retinopathy, a favorable clinical course with only antihypertensive treatment, and resolution of the Cerebellar lesions on follow-up imaging. A total of 5 of the 11 patients received external ventricular drainage due to obstructive hydrocephalus and only 2 of the 11 had a seizure. Isolated Cerebellar Involvement in PRES may be a unique variant that affects younger, male cases with severe acute hypertension and hypertensive retinopathy, but not necessarily seizure. Most patients have full recovery after fast control of blood pressure. Awareness of atypical neuroimaging features in PRES is critical for appropriate treatment. Copyright © 2015 Elsevier B.V. All rights reserved.
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Isolated Cerebellar Involvement in posterior reversible encephalopathy syndrome.
Journal of the Neurological Sciences, 2015Co-Authors: Lifei Lian, Suiqiang ZhuAbstract:Abstract Background Posterior reversible encephalopathy syndrome (PRES) is a serious and increasingly recognized disorder in humans. However, isolated Cerebellar Involvement in PRES is extremely uncommon. In this study, we sought to investigate its clinical and radiological features by describing a cohort of cases with PRES and isolated Cerebellar Involvement. Methods We report 2 patients with PRES with only Cerebellar Involvement and identified additional 9 cases using the PubMed database with the MeSH terms “posterior reversible encephalopathy syndrome”, “hypertensive encephalopathy”, “hypertension”, “cerebellum”, “encephalopathy”, and “magnetic resonance imaging”. We then collectively analyzed the clinical and imaging characteristics of these 11 cases. Results The average age was 28 years, with 8 male and 3 female patients. All cases had severe acute hypertension and T2 hyperintensity on MRI exclusively centered within the cerebellum. Of 11 patients, 7 had hypertensive retinopathy, a favorable clinical course with only antihypertensive treatment, and resolution of the Cerebellar lesions on follow-up imaging. A total of 5 of the 11 patients received external ventricular drainage due to obstructive hydrocephalus and only 2 of the 11 had a seizure. Conclusions Isolated Cerebellar Involvement in PRES may be a unique variant that affects younger, male cases with severe acute hypertension and hypertensive retinopathy, but not necessarily seizure. Most patients have full recovery after fast control of blood pressure. Awareness of atypical neuroimaging features in PRES is critical for appropriate treatment.
Eugen Boltshauser - One of the best experts on this subject based on the ideXlab platform.
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Clinical and neuroimaging features as diagnostic guides in neonatal neurology diseases with Cerebellar Involvement
Cerebellum & Ataxias, 2016Co-Authors: Jessica L. Klein, Eugen Boltshauser, Thierry A.g.m. Huisman, Monica E. Lemmon, Frances J. Northington, Andrea PorettiAbstract:Cerebellar abnormalities are encountered in a high number of neurological diseases that present in the neonatal period. These disorders can be categorized broadly as inherited (e.g. malformations, inborn errors of metabolism) or acquired (e.g. hemorrhages, infections, stroke). In some disorders such as Dandy-Walker malformation or Joubert syndrome, the main abnormalities are located within the cerebellum and brainstem. In other disorders such as Krabbe disease or sulfite oxidase deficiency, the main abnormalities are found within the supratentorial brain, but the Cerebellar Involvement may be helpful for diagnostic purposes. In In this article, we review neurological disorders with onset in the neonatal period and Cerebellar Involvement with a focus on how characterization of Cerebellar Involvement can facilitate accurate diagnosis and improved accuracy of neuro-functional prognosis.
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Sturge-Weber syndrome with Cerebellar Involvement.
Journal of neuroradiology = Journal de neuroradiologie, 2008Co-Authors: M. Smith Pearl, Eugen Boltshauser, W.m.a. Abdalla, Doris D.m. Lin, Anne M. Comi, Philippe Gailloud, T.a.g.m. HuismanAbstract:Sturge-Weber syndrome is a rare neurocutaneous disorder that typically presents with angiomas involving the face, ocular choroid and ipsilateral supratentorial leptomeninges. Posterior fossa Involvement is extremely rare. We present two patients with simultaneous supra- and infratentorial Involvement. Magnetic resonance imaging (MRI) and digital subtracted angiography (DSA) findings are discussed.
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disruption of Cerebellar development potential complication of extreme prematurity
American Journal of Neuroradiology, 2005Co-Authors: Agnes Messerschmidt, Eugen Boltshauser, Peter Brugger, Gerlinde Zoder, Walter Sterniste, Robert Birnbacher, Daniela PrayerAbstract:BACKGROUND AND PURPOSE: Infants with very low birth weight are at high risk for cerebral lesions. Although supratentorial brain damage is a common radiologic finding, posterior fossa pathologies are rare. We studied the morphology of Cerebellar Involvement in a large series of 28 premature infants born before the 30th week of gestation to define typical patterns and identify possible risk factors for this pathology. METHODS: Cranial sonograms were obtained in the early neonatal period. MR imaging was performed between the 2nd month and the 6th year of life. Morphologic patterns of Cerebellar Involvement were evaluated. RESULTS: Three morphologic patterns of Cerebellar Involvement were recognized: (1) symmetric volume reduction of the Cerebellar hemispheres, which were floating immediately beneath the tentorium, and a small vermis with preserved shape; (2) symmetrical reduction in hemispheric volume with an enlarged, balloon-shaped fourth ventricle and a small, deformed vermis; and (3) normal overall Cerebellar shape with extensive reduction of its dimensions. A small brain stem with flattened anterior curvature of the pons and loss of supratentorial white matter was present in all patients. CONCLUSION: Symmetric Cerebellar volume reduction was found as a consequence of extreme prematurity. Selective vulnerability of the developing cerebellum in the window of 24–30 weeks of gestation, combined with several additive perinatal risk factors (eg, hemosiderin deposits) seems to lead to destruction of immature structures and developmental arrest. Therefore, the resulting condition is the consequence of disrupted Cerebellar development.
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Progressive white-matter disease with primary Cerebellar Involvement: a separate entity?
Neuroradiology, 2002Co-Authors: Cengiz Yalcinkaya, İlknur Arslanoğlu, Civan Islak, Ahmet Aydin, Eugen BoltshauserAbstract:Although its metabolic basis has not yet been clarified, we report a progressive white-matter disease in a Turkish girl, starting in the cerebellum and spreading to supratentorial white matter. The onset was at the age of 2.5 years with diabetes insipidus, followed by ataxia and pyramidal signs resulting in loss of walking. Aqueduct stenosis was first recognised at the age of 8 years. To our knowledge, this MRI and clinical pattern does not correspond to a recognised, well-defined white-matter disease and may indicate a separate entity.
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Cerebellar Involvement in metabolic disorders: a pattern-recognition approach.
Neuroradiology, 1998Co-Authors: Maja Steinlin, Susan Blaser, Eugen BoltshauserAbstract:Inborn errors of metabolism can affect the cerebellum during development, maturation and later during life. We have established criteria for pattern recognition of Cerebellar abnormalities in metabolic disorders. The abnormalities can be divided into four major groups: Cerebellar hypoplasia (CH), hyperplasia, Cerebellar atrophy (CA), Cerebellar white matter abnormalities (WMA) or swelling, and Involvement of the dentate nuclei (DN) or Cerebellar cortex. CH can be an isolated typical finding, as in adenylsuccinase deficiency, but is also occasionally seen in many other disorders. Differentiation from CH and CA is often difficult, as in carbohydrate deficient glycoprotein syndrome or 2-L-hydroxyglutaric acidaemia. In cases of atrophy the relationship of Cerebellar to cerebral atrophy is important. WMA may be diffuse or patchy, frequently predominantly around the DN. Severe swelling of white matter is present during metabolic crisis in maple syrup urine disease. The DN can be affected by metabolite deposition, necrosis, calcification or demyelination. Involvement of Cerebellar cortex is seen in infantile neuroaxonal dystrophy. Changes in DN and Cerebellar cortex are rather typical and therefore most helpful; additional features should be sought as they are useful in narrowing down the differential diagnosis.
Brian G. Weinshenker - One of the best experts on this subject based on the ideXlab platform.
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brainstem and Cerebellar Involvement in mog igg associated disorder versus aquaporin 4 igg and ms
Journal of Neurology Neurosurgery and Psychiatry, 2021Co-Authors: Samantha A. Banks, Padraig P. Morris, John J. Chen, Sean J. Pittock, Elia Sechi, Amy Kunchok, Jan-mendelt Tillema, James P. Fryer, Brian G. WeinshenkerAbstract:Objective To determine the frequency and characteristics of brainstem or Cerebellar Involvement in myelin-oligodendrocyte-glycoprotein-antibody-associated-disorder (MOGAD) versus aquaporin-4-IgG-seropositive-neuromyelitis optica spectrum disorder (AQP4-IgG-NMOSD) and multiple sclerosis (MS). Methods In this observational study, we retrospectively identified 185 Mayo Clinic MOGAD patients with: (1) characteristic MOGAD phenotype, (2) MOG-IgG seropositivity by live cell-based assay and (3) MRI lesion(s) of brainstem, cerebellum or both. We compared the symptomatic attacks to AQP4-IgG-NMOSD (n=30) and MS (n=30). Results Brainstem or Cerebellar Involvement occurred in 62/185 (34%) MOGAD patients of which 39/62 (63%) were symptomatic. Ataxia (45%) and diplopia (26%) were common manifestations. The median age in years (range) in MOGAD of 24 (2–65) was younger than MS at 36 (16–65; p=0.046) and AQP4-IgG-NMOSD at 45 (6–72; p=0.006). Isolated attacks involving the brainstem, cerebellum or both were less frequent in MOGAD (9/39 (23%)) than MS (22/30 (73%); p Conclusion Involvement of the brainstem, cerebellum or both is common in MOGAD but usually occurs as a component of a multifocal central nervous system attack rather than in isolation. We identified clinical, CSF and MRI attributes that can help discriminate MOGAD from AQP4-IgG-NMOSD and MS.
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Brainstem and Cerebellar Involvement in MOG-IgG-associated disorder versus aquaporin-4-IgG and MS
Journal of neurology neurosurgery and psychiatry, 2020Co-Authors: Samantha A. Banks, Padraig P. Morris, John J. Chen, Sean J. Pittock, Elia Sechi, Amy Kunchok, Jan-mendelt Tillema, James P. Fryer, Brian G. Weinshenker, Karl N. KreckeAbstract:OBJECTIVE To determine the frequency and characteristics of brainstem or Cerebellar Involvement in myelin-oligodendrocyte-glycoprotein-antibody-associated-disorder (MOGAD) versus aquaporin-4-IgG-seropositive-neuromyelitis optica spectrum disorder (AQP4-IgG-NMOSD) and multiple sclerosis (MS). METHODS In this observational study, we retrospectively identified 185 Mayo Clinic MOGAD patients with: (1) characteristic MOGAD phenotype, (2) MOG-IgG seropositivity by live cell-based assay and (3) MRI lesion(s) of brainstem, cerebellum or both. We compared the symptomatic attacks to AQP4-IgG-NMOSD (n=30) and MS (n=30). RESULTS Brainstem or Cerebellar Involvement occurred in 62/185 (34%) MOGAD patients of which 39/62 (63%) were symptomatic. Ataxia (45%) and diplopia (26%) were common manifestations. The median age in years (range) in MOGAD of 24 (2-65) was younger than MS at 36 (16-65; p=0.046) and AQP4-IgG-NMOSD at 45 (6-72; p=0.006). Isolated attacks involving the brainstem, cerebellum or both were less frequent in MOGAD (9/39 (23%)) than MS (22/30 (73%); p
Phil Hyu Lee - One of the best experts on this subject based on the ideXlab platform.
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Volumetric analysis of the cerebellum in patients with progressive supranuclear palsy
European journal of neurology, 2016Co-Authors: Yoonju Lee, D. K. Lee, Jong-min Lee, Su Jin Chung, J. J. Lee, Young H. Sohn, Phil Hyu LeeAbstract:Background and purpose Although early Cerebellar symptoms are one of the exclusive criteria in the diagnosis of progressive supranuclear palsy (PSP), Cerebellar Involvement in PSP is evident both clinically and pathologically. However, structural analysis focusing on the cerebellum has not been previously studied in patients with PSP. We aimed to evaluate Cerebellar Involvement in PSP using a magnetic resonance imaging-based segmental volumetric analysis. Methods We retrospectively enrolled 48 patients with PSP composed of 25 patients with PSP–Richardson's syndrome (RS) and 23 patients with pure akinesia with gait freezing, 39 patients with Parkinson's disease (PD) and 34 healthy controls. Data on both the whole and segmented Cerebellar volumes were analyzed using a fully automated procedure. Results A general linear model showed that whole Cerebellar volume in patients with PSP was significantly smaller compared with that of patients with PD or controls after controlling for age, sex and intracranial volume (P = 0.34). In addition, patients with PSP exhibited decreased regional volume in the crus I, lobule VIIIa and lobule VIIIb, which play roles as secondary representations of motor tasks, compared with patients with PD or controls. In subgroup analysis of PSP, volume loss in the whole and segmental cerebellum was more pronounced in patients with PSP-RS than in those with pure akinesia with gait freezing, PD or control subjects. Conclusion These data demonstrate that Cerebellar atrophy is evident in patients with PSP and is especially prominent in the PSP-RS group. These findings increase understanding of the clinicopathological basis of Cerebellar Involvement in PSP.