BIOMAT Database Logo BIOMAT Database Logo

[Congenital bilateral perisylvian syndrome: a case report]. 2008

Hong Zhou Duan, and Jia Yong Zhang, and Sheng De Bao
Department of Neurosurgery, Peking University First Hospital, Beijing, China.

Congenital bilateral perisylvian syndrome (CBPS) is rare in literature, especially in China. In this article, we report the clinical and treatment of a patient with CBPS and discuss its mechanism, clinical features and therapy. This patient was a 28-year-old man. His main clinical features were pseudobulbar palsy, cognitive deficits and intractable epilepsy. MRI shows bilateral thickening of the cortex around the sylvian fissures which were deeper than normal and polymicrogyria. The electroencephalogram demonstrated slow spike in right temporal lobe and left frontal lobe. Rhythmal 4 Hz theta waves exist in left frontal and parietal lobe. As the epilepsy was poorly controlled by antiepileptic, section of the corpus callosum was carried out. After callosotomy, there was pronounced seizure reduction and intelligence development improvement. CBPS is characterized by pseudobulbar palsy, cognitive deficits, and bilateral perisylvian abnormalities in imaging studies. If intractable epilepsy is combined, callosotomy may be effective.

UI MeSH Term Description Entries
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297 Male Males
D008607 Intellectual Disability Subnormal intellectual functioning which originates during the developmental period. This has multiple potential etiologies, including genetic defects and perinatal insults. Intelligence quotient (IQ) scores are commonly used to determine whether an individual has an intellectual disability. IQ scores between 70 and 79 are in the borderline range. Scores below 67 are in the disabled range. (from Joynt, Clinical Neurology, 1992, Ch55, p28) Disability, Intellectual,Idiocy,Mental Retardation,Retardation, Mental,Deficiency, Mental,Intellectual Development Disorder,Mental Deficiency,Mental Retardation, Psychosocial,Deficiencies, Mental,Development Disorder, Intellectual,Development Disorders, Intellectual,Disabilities, Intellectual,Disorder, Intellectual Development,Disorders, Intellectual Development,Intellectual Development Disorders,Intellectual Disabilities,Mental Deficiencies,Mental Retardations, Psychosocial,Psychosocial Mental Retardation,Psychosocial Mental Retardations,Retardation, Psychosocial Mental,Retardations, Psychosocial Mental
D010291 Paresis A general term referring to a mild to moderate degree of muscular weakness, occasionally used as a synonym for PARALYSIS (severe or complete loss of motor function). In the older literature, paresis often referred specifically to paretic neurosyphilis (see NEUROSYPHILIS). "General paresis" and "general paralysis" may still carry that connotation. Bilateral lower extremity paresis is referred to as PARAPARESIS. Hemiparesis,Muscle Paresis,Brachial Paresis,Crural Paresis,Lower Extremity Paresis,Monoparesis,Muscular Paresis,Upper Extremity Paresis,Brachial Pareses,Crural Pareses,Extremity Pareses, Lower,Extremity Pareses, Upper,Extremity Paresis, Lower,Extremity Paresis, Upper,Hemipareses,Lower Extremity Pareses,Monopareses,Muscle Pareses,Muscular Pareses,Pareses,Pareses, Brachial,Pareses, Crural,Pareses, Lower Extremity,Pareses, Muscle,Pareses, Muscular,Pareses, Upper Extremity,Paresis, Brachial,Paresis, Crural,Paresis, Lower Extremity,Paresis, Muscle,Paresis, Muscular,Paresis, Upper Extremity,Upper Extremity Pareses
D004569 Electroencephalography Recording of electric currents developed in the brain by means of electrodes applied to the scalp, to the surface of the brain, or placed within the substance of the brain. EEG,Electroencephalogram,Electroencephalograms
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
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes
D020828 Pseudobulbar Palsy A syndrome characterized by DYSARTHRIA, dysphagia, dysphonia, impairment of voluntary movements of tongue and facial muscles, and emotional lability. This condition is caused by diseases that affect the motor fibers that travel from the cerebral cortex to the lower BRAIN STEM (i.e., corticobulbar tracts); including MULTIPLE SCLEROSIS; MOTOR NEURON DISEASE; and CEREBROVASCULAR DISORDERS. (From Adams et al., Principles of Neurology, 6th ed, p489) Dysarthria, Pseudobulbar,Paralysis, Pseudobulbar,Spastic Bulbar Palsy,Pseudobulbar Mutism,Pseudobulbar Paralysis,Pseudobulbar Paresis,Pseudobulbar Syndrome,Bulbar Palsies, Spastic,Bulbar Palsy, Spastic,Dysarthrias, Pseudobulbar,Mutism, Pseudobulbar,Mutisms, Pseudobulbar,Palsies, Pseudobulbar,Palsies, Spastic Bulbar,Palsy, Pseudobulbar,Palsy, Spastic Bulbar,Paralyses, Pseudobulbar,Pareses, Pseudobulbar,Paresis, Pseudobulbar,Pseudobulbar Dysarthria,Pseudobulbar Dysarthrias,Pseudobulbar Mutisms,Pseudobulbar Palsies,Pseudobulbar Paralyses,Pseudobulbar Pareses,Pseudobulbar Syndromes,Spastic Bulbar Palsies,Syndrome, Pseudobulbar,Syndromes, Pseudobulbar

Related Publications

Hong Zhou Duan, and Jia Yong Zhang, and Sheng De Bao
Congenital bilateral perisylvian syndrome: a case study.
February 2009, The Clinical neuropsychologist,
Hong Zhou Duan, and Jia Yong Zhang, and Sheng De Bao
Congenital bilateral perisylvian syndrome.
January 2018, Neurology India,
Hong Zhou Duan, and Jia Yong Zhang, and Sheng De Bao
[Congenital bilateral perisylvian syndrome].
July 1998, Ugeskrift for laeger,
Hong Zhou Duan, and Jia Yong Zhang, and Sheng De Bao
[Congenital bilateral perisylvian syndrome].
January 2002, Ryoikibetsu shokogun shirizu,
Hong Zhou Duan, and Jia Yong Zhang, and Sheng De Bao
A case of congenital bilateral perisylvian syndrome due to bilateral schizencephaly.
June 2007, Epileptic disorders : international epilepsy journal with videotape,
Hong Zhou Duan, and Jia Yong Zhang, and Sheng De Bao
Congenital bilateral perisylvian syndrome: first report in a Japanese patient.
March 1996, The Japanese journal of human genetics,
Hong Zhou Duan, and Jia Yong Zhang, and Sheng De Bao
Congenital bilateral perisylvian syndrome: MR findings.
January 2006, JBR-BTR : organe de la Societe royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie (KBVR),
Hong Zhou Duan, and Jia Yong Zhang, and Sheng De Bao
[The congenital bilateral perisylvian syndrome: a report of 2 new cases].
June 1996, Anales espanoles de pediatria,
Hong Zhou Duan, and Jia Yong Zhang, and Sheng De Bao
[Bilateral perisylvian polymicrogyria/bilateral perisylvian syndrome].
January 2000, Ryoikibetsu shokogun shirizu,
Hong Zhou Duan, and Jia Yong Zhang, and Sheng De Bao
Congenital bilateral perisylvian syndrome with partial epilepsy. Case report with long-term follow-up.
January 2005, Brain & development,
Copied contents to your clipboard!