Hydranencephaly in an infant with vascular malformations

Stevenson, David A.; Hart, Blaine L.; Clericuzio, Carol L.

doi:10.1002/ajmg.10051

Cited by 22 publications

(20 citation statements)

References 19 publications

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“…The most widely accepted etiology is a compromised fetal vascular system, mainly the occlusion of the internal carotid artery [4]. Clinical diagnostic confirmation of hydranencephaly is generally determined by magnetic resonance imaging (MRI) [5 Á7].…”

Section: Introductionmentioning

confidence: 99%

Brainstem mediation of the stapedius muscle reflex in hydranencephaly

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2007

Acta Oto-Laryngologica

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Hydranencephaly is a rare condition in which the cerebral hemispheres are absent at birth and are replaced by membranous sacs in a cerebrospinal fluid-filled cranium. Surviving hydranencephalic patients have a functional brainstem and possible remnants of the cerebral cortex. This case report examines hearing function and the integrity of the brainstem mediated stapedius muscle reflex (SMR) in an adult with hydranencephaly, using middle ear impedance change measures. The brainstem mediated ipsilateral and cross-brainstem contralateral SMRs were elicited in the right ear at normal threshold levels for noise bands of 0.25-1.0, 1.0-4.0, and 0.25-4.0 kHz (broadband), and at the sinusoidal frequencies of 0.5, 1.0, and 2.0 kHz. The ipsilateral and contralateral SMR decay rates were normal. The growth in the SMR amplitude in response to noise and pure tone stimuli from threshold to saturation over a 15-20 dB range was normal and showed essentially sigmoidal curves. The normal ipsilateral and crossed brainstem contralateral electrophysiological SMR in this hydranencephalic patient demonstrated the preservation of peripheral hearing reception and functional brainstem auditory afferent and efferent tracts and nuclei in the absence of corticofugal influence.

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Section: Introductionmentioning

confidence: 99%

Brainstem mediation of the stapedius muscle reflex in hydranencephaly

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2007

Acta Oto-Laryngologica

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“…Hydranencephaly develops as a result of the same encephaloclastic processes that produce porencephaly; however, the lesion is much more severe. In cases of hydranencephaly there is complete loss of brain parenchyma leading to a large fluid filled cavity within the brain that is covered by a pial-glial membrane (Barkovich and Norman, 1989;Stevenson et al, 2001). Hydranencephaly is classified as a type of porencephaly (Barkovich and Norman, 1989).…”

Section: Discussionmentioning

confidence: 99%

Congenital porencephaly with cerebellar hypoplasia in a Holstein calf: a case report

Lee¹,

Furuoka²,

Sasaki³

et al. 2011

Vet. Med. - Czech

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ABSTRACT:We describe the case of a nine-day-old female Holstein calf which had cheiloschisis, a moderate dome-shaped head, ataxia and opisthotonus since birth. No significant findings except the dome-shaped head were observed on survey radiography of the skull. Computed tomography (CT) images showed bilateral lateral ventriculomegaly, cerebellar hypoplasia and a cyst-like lesion communicating with the right lateral ventricle. Post-mortem examination revealed a cerebral defect in the frontoparietal lobe, which communicated with the right lateral ventricle, and cerebellar hypoplasia. CT provided a characteristic finding of porencephaly and was helpful for diagnosing the accompanying anomalies. We suggest that porencephaly should be included as a specific anomaly in the differential diagnosis of congenital brain malformation.

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“…Falx cerebri is partially missing or absent and brainstem is preserved. The pused and continuous color Doppler US show abnormal pathway and occlusion of the anterior cerebral artery crawling under the skull (Bernard et al, 2002;Stevenson et al, 2001). …”

Section: Hydranencephalymentioning

confidence: 99%