Clinicopathological Analysis of Sturge-Weber Syndrome with Focal Cortical Dysplasia FCD IIIc. 2023

Juan Cao, and Guocheng Yang, and Shoujun Xu, and Pengyue Tang, and Yue Wang, and Yingying Shan, and Yongxian Chen, and Peng He
Department of Pathology, Shenzhen Children's Hospital, Shenzhen, Guangdong Province, China.

Objective: To investigate the clinicopathological features of children with Sturge-Weber syndrome and to analyze the correlation between the distribution area of leptomeningeal angiomatosis, the degree of cerebral cortical calcification, and the degree of cerebral atrophy associated with epileptic seizures. Methods: 10 children were diagnosed with SWS with FCD IIIc by histopathology and immunohistochemistry. Spearman correlation analysis was used to calculate the association of SWS with FCD IIIc and seizures in children. Results: The leptomeningeal angiomatosis area was markedly positively correlated with the degree of brain atrophy in 10 children with SWS (r = 0.783, p = 0.007). The distribution of leptomeningeal hemangiomatosis, the degree of cortical calcification, and brain atrophy were not significantly correlated with epilepsy. Conclusion: SWS may be accompanied by FCD IIIc. The more extensive the cerebral lobes of leptomeningeal angiomatosis in SWS, the more pronounced the brain atrophy.

UI MeSH Term Description Entries
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D004827 Epilepsy A disorder characterized by recurrent episodes of paroxysmal brain dysfunction due to a sudden, disorderly, and excessive neuronal discharge. Epilepsy classification systems are generally based upon: (1) clinical features of the seizure episodes (e.g., motor seizure), (2) etiology (e.g., post-traumatic), (3) anatomic site of seizure origin (e.g., frontal lobe seizure), (4) tendency to spread to other structures in the brain, and (5) temporal patterns (e.g., nocturnal epilepsy). (From Adams et al., Principles of Neurology, 6th ed, p313) Aura,Awakening Epilepsy,Seizure Disorder,Epilepsy, Cryptogenic,Auras,Cryptogenic Epilepsies,Cryptogenic Epilepsy,Epilepsies,Epilepsies, Cryptogenic,Epilepsy, Awakening,Seizure Disorders
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000092222 Focal Cortical Dysplasia Abnormal, non-neoplastic cell proliferation of the CEREBRAL CORTEX confined to an area in any lobe. Focal cortical dysplasia in the temporal lobe is associated with TEMPORAL LOBE EPILEPSY. Taylor Dysplasia,Taylor Focal Cortical Dysplasia,Taylor's FCD,Cortical Dysplasia, Focal,Dysplasia, Focal Cortical,Dysplasia, Taylor,FCD, Taylor's,Focal Cortical Dysplasias,Taylor FCD,Taylors FCD
D000798 Angiomatosis A condition with multiple tumor-like lesions caused either by congenital or developmental malformations of BLOOD VESSELS, or reactive vascular proliferations, such as in bacillary angiomatosis. Angiomatosis is considered non-neoplastic. Angiomatoses
D001284 Atrophy Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes. Atrophies
D012640 Seizures Clinical or subclinical disturbances of cortical function due to a sudden, abnormal, excessive, and disorganized discharge of brain cells. Clinical manifestations include abnormal motor, sensory and psychic phenomena. Recurrent seizures are usually referred to as EPILEPSY or "seizure disorder." Absence Seizure,Absence Seizures,Atonic Absence Seizure,Atonic Seizure,Clonic Seizure,Complex Partial Seizure,Convulsion,Convulsions,Convulsive Seizure,Convulsive Seizures,Epileptic Seizure,Epileptic Seizures,Generalized Absence Seizure,Generalized Tonic-Clonic Seizures,Jacksonian Seizure,Myoclonic Seizure,Non-Epileptic Seizure,Nonepileptic Seizure,Partial Seizure,Seizure,Seizures, Convulsive,Seizures, Focal,Seizures, Generalized,Seizures, Motor,Seizures, Sensory,Tonic Clonic Seizure,Tonic Seizure,Tonic-Clonic Seizure,Atonic Absence Seizures,Atonic Seizures,Clonic Seizures,Complex Partial Seizures,Convulsion, Non-Epileptic,Generalized Absence Seizures,Myoclonic Seizures,Non-Epileptic Seizures,Nonepileptic Seizures,Partial Seizures,Petit Mal Convulsion,Seizures, Auditory,Seizures, Clonic,Seizures, Epileptic,Seizures, Gustatory,Seizures, Olfactory,Seizures, Somatosensory,Seizures, Tonic,Seizures, Tonic-Clonic,Seizures, Vertiginous,Seizures, Vestibular,Seizures, Visual,Single Seizure,Tonic Seizures,Tonic-Clonic Seizures,Absence Seizure, Atonic,Absence Seizure, Generalized,Absence Seizures, Atonic,Absence Seizures, Generalized,Auditory Seizure,Auditory Seizures,Clonic Seizure, Tonic,Clonic Seizures, Tonic,Convulsion, Non Epileptic,Convulsion, Petit Mal,Convulsions, Non-Epileptic,Focal Seizure,Focal Seizures,Generalized Seizure,Generalized Seizures,Generalized Tonic Clonic Seizures,Generalized Tonic-Clonic Seizure,Gustatory Seizure,Gustatory Seizures,Motor Seizure,Motor Seizures,Non Epileptic Seizure,Non Epileptic Seizures,Non-Epileptic Convulsion,Non-Epileptic Convulsions,Olfactory Seizure,Olfactory Seizures,Partial Seizure, Complex,Partial Seizures, Complex,Seizure, Absence,Seizure, Atonic,Seizure, Atonic Absence,Seizure, Auditory,Seizure, Clonic,Seizure, Complex Partial,Seizure, Convulsive,Seizure, Epileptic,Seizure, Focal,Seizure, Generalized,Seizure, Generalized Absence,Seizure, Generalized Tonic-Clonic,Seizure, Gustatory,Seizure, Jacksonian,Seizure, Motor,Seizure, Myoclonic,Seizure, Non-Epileptic,Seizure, Nonepileptic,Seizure, Olfactory,Seizure, Partial,Seizure, Sensory,Seizure, Single,Seizure, Somatosensory,Seizure, Tonic,Seizure, Tonic Clonic,Seizure, Tonic-Clonic,Seizure, Vertiginous,Seizure, Vestibular,Seizure, Visual,Seizures, Generalized Tonic-Clonic,Seizures, Nonepileptic,Sensory Seizure,Sensory Seizures,Single Seizures,Somatosensory Seizure,Somatosensory Seizures,Tonic Clonic Seizures,Tonic-Clonic Seizure, Generalized,Tonic-Clonic Seizures, Generalized,Vertiginous Seizure,Vertiginous Seizures,Vestibular Seizure,Vestibular Seizures,Visual Seizure,Visual Seizures
D013341 Sturge-Weber Syndrome A non-inherited congenital condition with vascular and neurological abnormalities. It is characterized by facial vascular nevi (PORT-WINE STAIN), and capillary angiomatosis of intracranial membranes (MENINGES; CHOROID). Neurological features include EPILEPSY; cognitive deficits; GLAUCOMA; and visual defects. Neuroretinoangiomatosis,Phakomatosis, Sturge-Weber,Angiomatosis Oculoorbital-Thalamic Syndrome,Encephalofacial Hemangiomatosis Syndrome,Meningo-Oculo-Facial Angiomatosis,Meningofacial Angiomatosis-Cerebral Calcification Syndrome,Parkes Weber Syndrome,Parkes-Weber Syndrome,Sturge Disease,Sturge Syndrome,Sturge's Syndrome,Sturge-Kalischer-Weber Syndrome,Sturge-Weber-Dimitri Syndrome,Sturge-Weber-Krabbe Syndrome,Angiomatosis, Meningo-Oculo-Facial,Hemangiomatosis Syndrome, Encephalofacial,Meningo Oculo Facial Angiomatosis,Phakomatosis, Sturge Weber,Sturge Kalischer Weber Syndrome,Sturge Weber Dimitri Syndrome,Sturge Weber Krabbe Syndrome,Sturge Weber Syndrome,Sturge-Weber Phakomatosis,Syndrome, Encephalofacial Hemangiomatosis,Syndrome, Parkes Weber,Syndrome, Parkes-Weber,Syndrome, Sturge,Syndrome, Sturge's,Syndrome, Sturge-Kalischer-Weber,Syndrome, Sturge-Weber,Syndrome, Sturge-Weber-Dimitri,Syndrome, Sturge-Weber-Krabbe

Related Publications

Juan Cao, and Guocheng Yang, and Shoujun Xu, and Pengyue Tang, and Yue Wang, and Yingying Shan, and Yongxian Chen, and Peng He
January 2024, Fetal and pediatric pathology,
Juan Cao, and Guocheng Yang, and Shoujun Xu, and Pengyue Tang, and Yue Wang, and Yingying Shan, and Yongxian Chen, and Peng He
October 2009, Computerized medical imaging and graphics : the official journal of the Computerized Medical Imaging Society,
Juan Cao, and Guocheng Yang, and Shoujun Xu, and Pengyue Tang, and Yue Wang, and Yingying Shan, and Yongxian Chen, and Peng He
November 2012, Epilepsia,
Juan Cao, and Guocheng Yang, and Shoujun Xu, and Pengyue Tang, and Yue Wang, and Yingying Shan, and Yongxian Chen, and Peng He
December 2014, Epilepsy research,
Juan Cao, and Guocheng Yang, and Shoujun Xu, and Pengyue Tang, and Yue Wang, and Yingying Shan, and Yongxian Chen, and Peng He
March 1996, Clinical nuclear medicine,
Juan Cao, and Guocheng Yang, and Shoujun Xu, and Pengyue Tang, and Yue Wang, and Yingying Shan, and Yongxian Chen, and Peng He
May 2015, Brain pathology (Zurich, Switzerland),
Juan Cao, and Guocheng Yang, and Shoujun Xu, and Pengyue Tang, and Yue Wang, and Yingying Shan, and Yongxian Chen, and Peng He
December 2014, Epilepsy research,
Juan Cao, and Guocheng Yang, and Shoujun Xu, and Pengyue Tang, and Yue Wang, and Yingying Shan, and Yongxian Chen, and Peng He
January 2022, Indian journal of pathology & microbiology,
Juan Cao, and Guocheng Yang, and Shoujun Xu, and Pengyue Tang, and Yue Wang, and Yingying Shan, and Yongxian Chen, and Peng He
May 2019, Journal of Korean Neurosurgical Society,
Juan Cao, and Guocheng Yang, and Shoujun Xu, and Pengyue Tang, and Yue Wang, and Yingying Shan, and Yongxian Chen, and Peng He
February 2013, Revista de neurologia,
Copied contents to your clipboard!