EEG in Holoprosencephaly (Arhinencephaly)

DeMyer, William; White, Philip T.

doi:10.1001/archneur.1964.00460230057005

Cited by 59 publications

(95 citation statements)

References 8 publications

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“…In accordance with the literature it can show the following EEG patterns: isoelectric activity 28 , depression areas or periodic discharges of paroxysmic nature and subclinical critical activity 8 . Our case of holoprosencephaly showe d the expected isoelectric activity pattern.…”

Section: Commentssupporting

confidence: 88%

Electroencephalography in congenital malformations of the central nervous system

Campos

Cruz²,

Lizarraga³

et al. 1994

Arq. Neuro-Psiquiatr.

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SUMMARY -We studied clinical and EEG features of 36 cases with congenital malformations of the CNS. Patients were followed at the outpatient clinic of Hospital Cayetano Heredia and of Hogar Clinica San Juan de Dios in Lima-Peru, from January 1984 to June 1992. Eighty percent of the patients had convulsive syndromes and mental retardation. The most frequent malformation was agenesis of corpus callosum, and it was not possible to find a "typical" EEG pattern. The second were porencephalic cysts, with a good clinical-EEG correlation. There were two typical cases of schizencephaly, one of hemimegalencephaly with good prognosis, and one of holoprosencephaly. The results are compared to those obtained for a series we previously reported. Data discussed take into account reports on the subject registered in the literature. It is concluded that EEG is an useful method to evaluate possible CNS malformations in developing countries.KEY WORDS: central nervous system, congenital malformations, corpus callosum agnesis, porencephaly, hemimegalencephaly, hydrocephaly, EEG. Electroencefalografia e n las malformaciones congénitas del sistema nervioso centralRESUMEN -Estudiamos aspectos clínicos e del EEG de 36 casos de malformaciones congénitas del sistema nervioso central. Los pacientes fueron seguidos en los consultorios externos del Hospital Cayetano Heredia y del Hogar Clínica San Juan de Dios en Lima-Peru, desde enero 1984 hasta junio 1992. Ochenta por ciento de los pacientes presentaron sindrome convulsivo y retardo mental. La anormalidad mas frecuente correspondió a agenesia de cuerpo calloso y no fue posible identificar un patron EEG "típico". El segundo lugar correspondió a quistes porencefálicos, con buena correlación clínico-EEG. Ademas, hubieron dos casos clínicamente típicos de esquizencefalia, una hemimegalencefalia con buen prognóstico y un caso de holoprosencefalia. Se comparan los resultados con aquellos de casos previamente revisados. Se discuten los dados frente a la literatura acerca de los patrones EEG mas frecuentemente relatados. Se concluye en la utilidad del EEG en países en desarrollo para hacer posible un alto grado de sospecha de una malformación del SNC aun en ausencia de CAT-scan. PALABRAS-CLAVE: sistema nervioso central, malformaciones, agenesia de cuerpo calloso, porencefalia, hemimegalencefalia, holoprosencefalia, EEG.The congenital anormalies of the central nervous system (CNS) represent a frequent cause of fetal an d perinatal death . Together with congenital error s of th e metabolism, chromosomopathie s and vascular abnormalities, they play a major role in the pathology of the cerebral lesions responsible for mental retardatio n and/or cerebral paralysis, associate d o r not with convulsive syndromes 17 * 30 .

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Section: Commentssupporting

confidence: 88%

Electroencephalography in congenital malformations of the central nervous system

Campos

Cruz²,

Lizarraga³

et al. 1994

Arq. Neuro-Psiquiatr.

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“…Holoprosencephaly (HP) results from failure of the prosencephalon to differentiate into the cerebral hemispheres and lateral ventricles between the fourth and eighth week of gestation [1][2][3]. A spectrum of defects of malformations of the brain and face exists, which range from complete to partial failure of cleavage of the prosencephalon.…”

Section: Discussionmentioning

confidence: 99%

“…Holoprosencephaly (HPE) results from failure of the prosencephalon to differentiate into the cerebral hemispheres and lateral ventricles between the fourth and eighth week of gestation [1,2]. HPE has an incidence rate of 1:250 in utero.…”

Section: Introductionmentioning

confidence: 99%

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Lobar Holoprosencephaly: Cranial Sonography and MRI Imaging Features

Bagul¹,

Tripathi²,

Mishra³

2016

JCR

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Abstract:Introduction: Holoprosencephaly (HPE) is a rare congenital anomaly due to incomplete or absent division of the prosencephalon or forebrain into distinct cerebral hemispheres. Case Report: A near term baby boy, born at 37 weeks of gestation to a 30 year old mother, in peripheral hospital without antenatal investigation like ultrasonography was admitted to our hospital with provisional diagnosis of anticipated sepsis. Cranial sonography of the patient revealed absence of septum pellucidum with dysplastic corpus callosum, partially formed frontal horns of lateral ventricles, colpocephaly and azygous anterior cerebral artery diagnosed as lobar holoprosencephaly. MRI brain confirmed all the features of cranial sonography with additional depiction of fused cingulated gyrus. Conclusion: Holoprosencephaly is a rare congenital structural anomaly of the prosencephalon that results in incomplete development of the brain. In its severe form it is incompatible with life. It is prudent to diagnose holoprosencephaly prenatally and determine the type to classify severity, complications and survival rate.

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“…Types of holoprosencephaly were classified into four grades of severity, according to the classification of Demyer [14] and the findings of neuroimaging studies [3,15]: alobar, semilobar, lobar, and middle interhemispheric variant. In alobar holoprosencephaly, the most severe form of this condition, only a single ventricle is evident, and the cerebral hemispheres are not separated.…”

Section: Study Populationmentioning

confidence: 99%

Clinical Features and Outcomes of Holoprosencephaly in Korea

Kim

2010

Pediatric Neurology

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EEG in Holoprosencephaly (Arhinencephaly)

Cited by 59 publications

References 8 publications

Electroencephalography in congenital malformations of the central nervous system

Electroencephalography in congenital malformations of the central nervous system

Lobar Holoprosencephaly: Cranial Sonography and MRI Imaging Features

Clinical Features and Outcomes of Holoprosencephaly in Korea

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