The differences in epileptic characteristics in patients with porencephaly and schizencephaly

Shimizu, Miki; Maeda, Tomoki; Izumi, Tatsuro

doi:10.1016/j.braindev.2011.10.001

Cited by 8 publications

(5 citation statements)

References 21 publications

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“…Extensive porencephaly communicates through the subarachnoid space to the ventricles, which shows similar morphological findings to those of open-lip schizencephaly. Patients with these two types of defective lesions present with severe developmental delays and intractable epilepsy [3]. Cavitation in schizencephaly is lined by dysplastic cortex, usually associated with polymicrogyria [4].…”

Section: Introductionmentioning

confidence: 99%

Porencephaly and cortical dysplasia as cause of seizures in a dog

et al. 2012

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BackgroundSeizures are a common problem in small animal neurology and it may be related to underlying diseases. Porencephaly is an extremely rare disorder, and in Veterinary Medicine it affects more often ruminants, with only few reports in dogs.Case presentationA one-year-old intact male Shih-Tzu dog was referred to Veterinary University Hospital with history of abnormal gait and generalized tonic-clonic seizures. Signs included hypermetria, abnormal nystagmus and increased myotatic reflexes. At necropsy, during the brain analysis, a cleft was observed in the left parietal and occipital lobes, creating a communication between the subarachnoid space and the left lateral ventricle, consistent with porencephaly; and also a focal atrophy of the caudal paravermal and vermal portions of the cerebellum. Furthermore, the histological examination showed cortical and cerebellar neuronal dysplasia.ConclusionsReports of seizures due to porencephaly are rare in dogs. In this case, the dog presented a group of brain abnormalities which per se or in assemblage could result in seizure manifestation.

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Section: Introductionmentioning

confidence: 99%

Porencephaly and cortical dysplasia as cause of seizures in a dog

et al. 2012

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“…En otros reportes, sólo la mitad de pacientes tenían actividad epileptiforme focal, la mayoría presentaban actividad epileptiforme multifocal sobre la localización de los quistes y en otros se observaron descargas epileptiformes difusas o incluso contralaterales (8). Las anormalidades epileptiformes generalizadas del EEG son comunes en la epilepsia relacionada con la porencefalia (9). Las descargas de complejos puntaonda lenta bilateralmente sincrónicas, cuando no están acompañadas de descargas epilépticas independientes del hemisferio "sano", pueden representar sincronía bilateral secundaria procedente del hemisferio porencefálico (7).…”

Section: Discussionunclassified

“…Recibido: 09/05/2017 Aceptado: 15/10/2017 Según la literatura, la zona epileptogénica puede encontrarse sobre la lesión quística o localizarse en un sitio distante sobre el mismo hemisferio, o en el hemisferio contralateral (8,9). Por ejemplo, en series de casos de epilepsia asociada a porencefalia y más aún en aquellos que evaluaron epilepsia refractaria, se demostró que la esclerosis temporal mesial a menudo coexistía con la porencefalia y era el foco probable de las crisis según datos electro-clínicos concordantes (10,11).…”

Section: Rev Neuropsiquiatr 80 (4) 2017unclassified

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Epilepsia por porencefalia familiar: hallazgos clínicos, electroencefalográficos y de resonancia magnética.

Ramírez

F²

2017

Rev Neuropsiquiatr

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RESUMENLa porencefalia familiar es un trastorno genético raro que produce quistes porencefálico, los cuales son secundarios a un infarto vascular prenatal o perinatal. La hemiparesia congénita, el retardo mental y la epilepsia de grado variable son las manifestaciones más frecuentes. Se describe el caso de dos hermanos, uno varón y la otra mujer, quienes presentan hallazgos en imágenes de resonancia magnética cerebral de quistes porencefálicos extensos que comprometen las regiones fronto-parieto-temporales de un hemisferio distinto en cada uno. Ambos presentaban hemiparesia congénita, retardo mental y epilepsia medicamente tratable con hallazgos electro-clínicos, de imágenes y neuropsicológicos que permitieron localizar la zona epileptogénica sobre la región dorsolateral del lóbulo frontal yacente al quiste porencefálico. En pacientes con hemiparesia congénita, retardo mental y epilepsia con quiste porencefálico se debe considerar la posibilidad de porencefalia familiar e indagar antecedentes familiares de este trastorno.PALABRAS CLAVE: Quiste porencefálico, porencefalia familiar, epilepsia. SUMMARYFamilial porencephaly is a rare genetic disorder resulting in porencephalic cysts, which are secondary to prenatal or perinatal vascular infarction. Congenital hemiparesis, mental retardation, and epilepsy in variable degrees are the most frequent manifestations. We describe the case of two siblings, one male and the other female, who present findings in brain magnetic resonance imaging of extensive porencephalic cysts that compromise the fronto-parietaltemporal regions of a different hemisphere in each. Both presented congenital hemiparesis, mental retardation and medically treatable epilepsy with electro-clinical, imaging and neuropsychological findings allowed to locate the epileptogenic zone on the dorsolateral region of the frontal lobe lying to the porencephalic cyst. In patients with congenital hemiparesis, mental retardation and epilepsy with porencephalic cyst, the possibility of familial porencephaly should be considered and a family history of this disorder should be investigated.

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“…A characteristic feature on EEG of patients with porencephalic cyst is the presence of focal, rhythmic epileptiform discharges that spatially correlate with the cyst and its margins [4]. Patients with extensive porencephaly may have bilateral synchronous paroxysms [5]. The diagnosis porencephalic cyst is confirmed on imaging; CT head reveals hypodense intracranial cyst with a well-defined border, while MRI brain shows a cyst lined by white matter, containing CSF with low signal intensity on T1/FLAIR, high signal intensity on T2, and no restricted diffusion on MRI [6].…”

Section: Casementioning

confidence: 99%

Porencephalic cyst: a rare cause of new-onset seizure in an adult

Qureshi

Jehangir

York

2018

Journal of Community Hospital Internal Medicine Perspectives

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We present a case of a 56-year-old male with a history of perinatal intracerebral hemorrhage who presented to the emergency department after a witnessed new-onset generalized tonic–clonic seizure. Computerized tomography and magnetic resonance imaging of the head revealed a large frontal lobe porencephalic cyst, with encephalomalacia in the right parietal lobe and temporal lobe (the patient did not have any prior cranial imaging). The patient has subsequently remained seizure-free on levetiracetam. Porencephalic cyst is a rare condition of cerebrospinal fluid accumulation in the brain parenchyma that is usually related to perinatal vascular events. These cysts can have a wide array of clinical presentations. This can include partial or generalized seizures, which are usually managed by antiepileptics.

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The differences in epileptic characteristics in patients with porencephaly and schizencephaly

Cited by 8 publications

References 21 publications

Porencephaly and cortical dysplasia as cause of seizures in a dog

Porencephaly and cortical dysplasia as cause of seizures in a dog

Epilepsia por porencefalia familiar: hallazgos clínicos, electroencefalográficos y de resonancia magnética.

Porencephalic cyst: a rare cause of new-onset seizure in an adult

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