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At least 19 recordsLinked to original sources
PubMed1989

Hydranencephaly and maximal hydrocephalus: usefulness of electrophysiological studies for their differentiation.

The EEG was evaluated in three cases of hydranencephaly and two cases of maximal hydrocephalus, and the visual evoked potential was studied in one case each of hydranencephaly and maximal hydrocephalus. The EEG tracing of hydranencephaly typically showed a flat pattern in most bipolar derivations because of the differential amplification of the same activity from remote generators. However, the EEG of maximal hydrocephalus did not demonstrate a flat pattern in bipolar as well as referential derivation, and the activities were different from various electrodes in referential derivation. The visual evoked potential of maximal hydrocephalus showed a normal pattern, while that of hydranencephaly showed no response. Electrophysiological examinations (such as EEG and visual evoked potential) are useful for differentiation of hydranencephaly and maximal hydrocephalus in cases whose computed tomographic scans do not provide clear differentiation.

Anencephaly↗
PubMed1990

The use of two-dimensional Doppler sonography (color Doppler) in the diagnosis of hydranencephaly.

Hydranencephaly, first described by Cruveilhier, may be defined as a rare condition in which the cerebral hemispheres are replaced by membranous sacs filled with cerebrospinal fluid. Cerebral angiography is especially useful in differentiating hydranencephaly and severe hydrocephalus, massive subdural hygromas and alobar holoprosencephaly, but it is no better than other neuroradiological diagnosis methods in the neonate. This report outlines the diagnostic use of two-dimensional Doppler (color Doppler) in a child with hydranencephaly. Color Doppler was performed using an SSA-270A (Toshiba) with a 3.75 MHz phase-array sector scanning probe and no sedation. In this study, color Doppler demonstrated the internal carotid artery in the proximal portion, but the secondary and tertiary branches were not observed. In summary, color Doppler permitted early, easy diagnosis of hydranencephaly. In addition, the use of color Doppler was beneficial in diagnosing other anomalies of the central nervous system in neonates.

Blood Flow Velocity↗
PubMed1995

The familial syndrome of proliferative vasculopathy and hydranencephaly-hydrocephaly: immunocytochemical and ultrastructural evidence for endothelial proliferation.

This is the fourth report of Fowler-type hydranencephaly, or proliferative vasculopathy and hydranencephaly-hydrocephaly (PVHH), and is both the first case in Europe and the first case reported in an Asian family. A 17-week fetus showed severe arthrogryposis, pterygia and muscular hypoplasia. Massive cystic dilatation of the cerebral ventricles with thin disorganized pallium was associated with calcifications and characteristic glomeruloid vasculopathy throughout the CNS. Hydranencephaly in a previous pregnancy was demonstrated ultrasonographically at 13 weeks gestation. The glomeruloid vasculopathy, unique to this disorder, has ill-defined vascular channels, prominent reticulin network and inclusion-bearing cells which our immunocytological and ultrastructural studies suggest are endothelial cells. Aetiopathogenesis remains uncertain; previous hypothesis include congenital infection or primary neuro-ectodermal failure. Our present clinical and morphological findings suggest a primary role for the glomeruloid vasculopathy at the time of vascular invasion of the cerebral mantle during the first trimester. Previous and present case data support autosomal recessive inheritance, in contradistinction to sporadic, encephaloclastic, hydranencephaly from which PVHH can be readily differentiated by microscopic examination.

Adult↗
PubMed1993

Primary congenital rhabdoid tumor of the brain with neoplastic hydranencephaly.

A child with shunted hydranencephaly and presumed ventriculitis was found to have a primary congenital rhabdoid tumor (RT) of the brain. The child died and a complete autopsy was carried out. The cerebral hemispheres were replaced by a single thin-walled cavity studed with tumor nodules and filled with thick, viscous fluid. The posterior fossa and visceral organs were free of tumor. This case is unique because the rhabdoid tumor was primary to the brain, it was congenital, and it massively replaced the cerebral hemispheres, causing hydranencephaly. Only three other cases of primary RT of the brain with complete autopsy examination have been reported. Cases of congenital rhabdoid tumors are not known in the literature. Hydranencephaly with a highly proteinaceous fluid should alert the physician to the possibility of a neoplasm. When the fluid in presumed ventriculitis is sterile, cerebral biopsy should be considered.

Biomarkers, Tumor↗
PubMed1977

[Hydranencephaly versus spelencephaly--a report of two cases (author's transl)].

Two cases are presented, one of hydranencephaly and one of spelencephaly. Spelencephaly represents an intermediate form between multicystic encephalopathy and hydranencephaly and is characterized by preservation of the ventricular walls. There is no clinical distinction between spelencephaly and hydranencephaly. Diagnosis can be made by pneumencephalography, as described in this paper, if the gas is applied via both lumbar and ventricular puncture. Autopsy findings of both cases are presented and the possible vascular etiology of the condition is discussed.

Anencephaly↗
PubMed1990

An outbreak of congenital hydranencephaly and cerebellar hypoplasia among calves in South Kyushu, Japan: a pathological study.

An outbreak of congenital hydranencephaly and cerebellar hypoplasia occurred between November 1985 and May 1986 in Miyazaki, South Kyushu, Japan. Seventy-three calves had nervous signs of varying severity such as inability to stand, locomotor difficulties, defective vision and difficulty in sucking. At necropsy, 62 calves had macroscopic lesions in the central nervous system: hydranencephaly accompanied by cerebellar hypoplasia in 47; hydranencephaly alone in eight; and dilatation of the lateral ventricle in seven; none had arthrogryposis. Microscopically, all 62 cases involved various degrees of hypoplasia of neural components, such as total or partial thinning of the cerebral or cerebellar laminae. Heterotopia, such as abnormal islands of granule cells or Purkinje cells was also observed. Fourteen of these animals had other lesions such as non-purulent encephalitis, focal gliosis, neuronal degeneration, calcification or pseudocalcification, and cholesterol deposits, activation of vascular endothelial cells and haemorrhage. From the findings, these cases were considered to represent mainly hypoplasia of nerve tissue due to infection with a virus different from Akabane virus.

Animals↗
PubMed1993

Suspected maternal infanticide in a case of hydranencephaly.

A medico-legal autopsy case of hydranencephaly in a male infant which was first suspected of maternal infanticide is reported. The infant was 48 cm in height, weighed 2.86 kg and the circumference of the head, the chest and the abdomen was 32.2 cm, 31.0 cm and 30.4 cm, respectively, with no deformities of the head or body. Autopsy examination, however, revealed a severe defect in the central nervous system. The cranial cavity was filled with a cloudy dark red fluid (ca. 310 ml) instead of the cerebral hemispheres. The residual central nervous tissues were mostly subtentorial structures from the midbrain to the spinal cord namely, corpus mamillare, corpora quadrigemina, corpus pineale, crus cerebri, pons, cerebellum, medulla oblongata and spinal cord. The basal ganglia, thalamus, hypothalamus and chiasma opticum could not be found, although atrophic hypophysis, eyeballs and optic nerves were present. The usual distribution of cerebral blood vessels, especially the branches of the anterior and middle cerebral arteries and Willis' ring, was absent despite the presence of the internal and external carotid arteries. Other organs were, in general, congestive. The marked cortical atrophy of the adrenal glands (left 0.5 g, right 0.6 g), especially the zona fasciculata, was characteristic. The hydrostatic lung test gave partially positive results, but this was considered to be due to artificial respiration by an ambulance man because amniotic fluid components were microscopically noted and fully expanded alveoli were not found. In conclusion, the cause of the infant's death was diagnosed as stillbirth due to aspiration of amniotic fluid caused by the severe defect of vegetative hypothalamic function through hydranencephaly.

Brain↗
PubMed1980

Hydranencephaly in twins.

The sixth case of hydranencephaly in a twin is reported. The patient is an 11-year-old girl, and her twin was stillborn and macerated. Intrauterine disseminated intravascular coagulation may be the cause of hydranencephaly in this patient.

Anencephaly↗
PubMed1980

Diagnostic image evaluation of hydranencephaly and pictorially similar entities, with emphasis on computed tomography.

The radiographic evaluation of a spectrum of vascular cerebral damage is presented, ranging from simple porencephaly to the most severe form, classic hydranencephaly. With a combination of clinical materal and computed tomography, it is possible in most cases to arrive at a specific diagnosis of hydranencephaly or severe bilateral porencephaly. Pictorially similar entities, such as massive congenital subdural hematomas or hygromas, alobar holoprosencephaly, massive hydrocephalus, and massive postanoxic or infective encephalopathies, are discussed.

Anencephaly↗
PubMed1985

Lung growth and development in anencephaly and hydranencephaly.

Significant qualitative and quantitative differences were observed in the lungs of 4 of 6 infants with anencephaly and hydranencephaly. In 3 of 4 of them, the findings were explicable on the basis of the presence of associated congenital anomalies, and in the fourth there was polyhydramnios. The infants with normal lungs did not have associated congenital abnormalities. The absence of the pituitary did not correlate with the degree of development of the lungs. Studies of lung growth in anencephaly and hydranencephaly must clearly note the presence of associated anomalies because anencephaly/hydranencephaly per se may not cause pulmonary hypoplasia in the majority of cases. The most satisfactory assessment in evaluation of lung growth and development was obtained by combining morphologic findings with more than one morphometric parameter. Radial count estimation alone was found to be a poor predictor of lung maturity (morphologic age) in hypoplastic lungs. Radial count and fixed lung volume together provide a better assessment of lung growth and development than does the ratio of lung weight to body weight, which is of use only if severely depressed. The terms immaturity and hypoplasia of the lungs are not necessarily synonymous.

Anencephaly↗
PubMed1983

Bluetongue virus-induced hydranencephaly in cattle.

Direct inoculation of bluetongue virus into 125-day bovine fetuses resulted in development of hydranencephaly. The earliest lesions after virus inoculation were a severe necrotizing encephalitis, which was most prominent in the cerebrum, and an associated nonsuppurative meningitis. At birth, the brains of infected fetuses had thin-walled cerebral hemispheres, dilated lateral ventricles, and cerebral cysts. No gross lesions were observed in the brain stem or cerebellum. Two morphologically different lesions were present in the brain of a fetus sacrificed 20 days after virus inoculation. There were discrete foci of hemorrhagic cerebral necrosis that resembled infarcts and widespread microcavitations of the intermediate and subventricular zones. Changes consistent with vascular damage were present in the brains of fetuses sacrificed 12 and 20 days after virus inoculation. Calves with bluetongue virus-induced hydranencephaly would have poor viability, but they would not be expected to have any significance as virus reservoirs.

Anencephaly↗
PubMed1982

[Hydranencephaly: clinicopathological study of 6 cases].

The study of six patients with hydranencephaly is reported. Clinical and necroscopic findings have been performed in correlation to routine laboratory examination. The Authors have concluded that transilumination is an important procedure for early diagnostic of these cases, especially when the patients have a milder or incomplete form of the syndrome. The necroscopic findings suggest that hydranencephaly may be the result of encephaloclastic lesions (inflammatory, mechanical or vascular) that, either before or after birth, lead to complete destruction of the brain, with sparing of sub-tentorial structures.

Anencephaly↗
PubMed1976

Bluetongue virus as a cause of hydranencephaly in cattle.

Hydranencephaly was produced in a foetus and a calf by intra-uterine infection with an attenuated Type 10 bluetongue virus. Laparotomy was performed on the respective dams and the foetuses, respectively 126 days and 138 days old, were inoculated intramuscularly through the uterine wall with 1 ml of a virus suspension containing 5 x 103 tissue culture infective doese. The younger feotus was aborted on Day 262, while the other one was born alive on Day 273. Both foetuses showed marked hydranencephaly.

Abortion, Veterinary↗
PubMed1977

Hydrops amnii in sheep associated with hydranencephaly and arthrogryposis with wesselsbron disease and rift valley fever viruses as aetiological agents.

During the 1974/75 lambing season numerous reports were received from various parts of the Republic of South Africa and South West Africa of severe abdominal distension in ewes after vaccination with the attenuated Rift Valley fever and/or attenuated Wesselsbron disease vaccine. The ewes were vaccinated at different stages of gestation in spite of recommendations to the contrary, the syndrome being especially obvious in ewes immunized with one or both of these vaccines during the first trimester of pregnancy. In some of the flocks hydrops amnii was recorded in as many as 15% of the ewes. Many of the ewes so affected showed a prolonged gestation of up to 6-7 months and, towards the end of gestation, were unable to rise or walk. They eventually died of ketosis, hypostatic pneumonia and complications due to dystocia. The foetuses examined were malformed and larger than normal with a mass of 3,6-6,7 kg. They usually showed arthrogryposis, brachygnathy inferior, hydranencephaly, hypoplasia or segmental aplasia of the spinal cord and neurogenic muscular atrophy. The amnion contained 8,0-18,0 1 of amniotic fluid, the endometrium was oedematous, and cystic tube-like dilatations, 1-10 mm in diameter, filled with a clear fluid, were scattered in the endometrium. No definite conclusions as to the aetiology of the syndrome could be drawn from serological tests performed on the ewes, lambs or foetuses. Preliminary experimental work confirmed previous observations that the attenuated Wesselsbron disease vaccine virus is responsible for this syndrome and that the wild-type virus is also implicated. In addition, the attenuated Rift Valley fever vaccine virus was found to the responsible for arthrogryposis and hydranencephaly without hydrops amnii and for micrencephaly and arthrogryposis associated with hydrops amnii in the ewe.

Anencephaly↗
PubMed1979

Hydranencephaly in a twin gestation.

Hydranencephaly in a twin gestation was demonstrated prenatally by ultrasonography and confirmed following delivery by cranial computerized axial tomography (CAT). Prenatal diagnosis of intracranial anomalies is possible with the use of ultrasonography. This is the third report of the occurrence of hydranencephaly in a twin gestation.

Adult↗
PubMedDate not supplied

[Diagnostic and etiologic problems of hydranencephaly (author's transl)].

A report of hydranencephaly is made, featuring normal cephalic perimeter and where diagnosis was a pathological finding. There were no clinical suspiscions and fetal distress along with post-partum anoxia were thought to be the chief causes of the disease. A revision of clinical, diagnostic and etiological factors of this particular entity is made. It is of concern to aim this report in the direction of considering phosphorous-organic products to have a teratogenic action and therefore to be an interesting etiological aspect in the production of hydranencephaly.

Anencephaly↗
PubMed1983

[Pathology of hydranencephaly].

Data on pathological manifestations of hydranencephaly (3 cases) consisting of complete or nearly complete absence of cerebral hemispheres with the intact stem, cerebellum, spinal cord, meninges and skull bones are presented. A number of structural disorders were found in the remaining structures of the brain. A hypothesis on the origin of this central nervous system developmental defect is proposed: hydranencephaly is associated with disorders in the development of leptomeninges at the early stages of embryogenesis.

Adult↗