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Brain functions of an infant with hydranencephaly revealed by auditory evoked potentials.

Hydranencephaly is a severe condition of brain characterized by the almost complete absence of the cerebral hemispheres with the thalamus, cerebellum and brainstem being preserved. This paper reports hydranencephaly in a 3-month-old boy. Cranial magnetic resonance imaging (MRI) revealed the condition and an auditory evoked potential study which was performed in order to investigate residual brain function. They showed normal auditory brainstem function but no appearance of other auditory evoked potentials originating from the higher brain.

Brain↗

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↗

Hydranencephaly and cerebellar hypoplasia in two kittens attributed to intrauterine parvovirus infection.

Six weeks after vaccination with modified live feline parvovirus vaccine, a cat gave birth to five kittens, three of which died soon afterwards. The remaining two kittens (A and B) survived, but at 8 weeks of age were unable to walk and showed abnormal behaviour, with lack of menace and oculovestibular responses, and severe dysmetria. These signs suggested multifocal disease associated with the cerebrum and cerebellum. Magnetic resonance imaging demonstrated severe bilateral (kitten A) or unilateral (kitten B) hydrocephalus or hydranencephaly, combined with cerebellar agenesis (kitten A) or severe hypoplasia (kitten B). Hydranencephaly was confirmed histopathologically in both kittens. Parvovirus was isolated from the kidney of one kitten. Parvoviral DNA was amplified by the polymerase chain reaction (PCR) from paraffin wax-embedded brain of both kittens. The severe malformations observed in these kittens presumably resulted from an in-utero parvovirus infection, possibly due to vaccination, that occurred late in the first, or early in the second, trimester of pregnancy.

Animals↗

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↗

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↗

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↗

Hydranencephaly, cerebellar hypoplasia, and myopathy in chick embryos infected with aino virus.

Pathogenesis of Aino virus (AIV), a suspected causative agent of congenital abnormalities of calves, has not yet been established by experimental infection of dams. To investigate the pathogenesis, 10(3) median tissue culture infective doses per 0.2 ml of AIV strain JaNAr 28 was inoculated into the yolk sac of 8-day-old chick embryos. At 4, 7, 10, and 13 days post-inoculation (PI) 20 eggs were opened and macro- and microscopic studies combined with virus recovery and immunohistochemical detection of the virus antigen were performed. At 7 to 13 days PI chick embryos manifested marked hydranencephaly, cerebellar hypoplasia, arthrogryposis, and scoliosis, with the highest incidences of 86.7%, 73.3%, 80.0%, and 20.0%, respectively. At 4 days PI the viral antigen was found in nerve cells, gitter cells in mild necrotic foci of the central nervous system (CNS), degenerative myotubules, and macrophages in the interstitium, which was associated with the early phase of AIV-induced encephalitis and polymyositis, with occasional accompanying hemorrhage and clumping of myotubular fragments. From 7 to 10 days PI, AIV antigen increased markedly in the liquefactive necrosis and in both degenerative and normal-looking myotubules in conjunction with developing hydranencephaly and arthrogryposis. The encephalitis and myositis had a tendency to mitigate by 10 days PI, coincident with a slight decrease in amount of AIV antigen. At 13 days PI there was almost no detectable AIV antigen in CNS and skeletal muscles, probably due to depletion of cells having affinity to AIV.

Animals↗

Hydranencephaly owing to twin-twin transfusion: serial fetal ultrasonography and magnetic resonance imaging findings.

We report a newborn girl with hydranencephaly. In the setting of a monochorionic twin pregnancy, one twin's demise was detected by ultrasonography at 18 weeks of gestation, apparently the result of a twin-twin transfusion. In the surviving twin, the evolution of ventriculomegaly, first noted at 18 weeks, to hydranencephaly at 27 weeks is documented by serial sonograms. These findings were confirmed with fetal and postnatal magnetic resonance imaging.

Adult↗

[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↗

Intrauterine fetal death of one of twins, coexisting with hydranencephaly in the surviving co-twin: a case report.

Hydranencephaly, a relatively rare malformation of the brain, is characterised by absence of the cerebral hemispheres and their replacement by sacs filled with cerebrospinal fluid. It is one of the recognised forms of intracranial malformations associated with intrauterine fetal demise of one of twins in monochorionic twin gestation. This report illustrates the development of hydranencephaly in a surviving twin sequel to intrauterine fetal demise of the co-twin.

Diseases in Twins↗

[Hydranencephaly. (Severe cerebral malformations in three newborn infants)].

Three neonatal cases of hydranencephaly are reported. Clinical and radiological characteristics of this disease are also described. Factors which can have any role in the development of hydranencephaly are reviewed. The importance of intrauterine ultrasonography and the quick, early diagnosis is emphasized. Therapeutic approaches are also discussed.

Apgar Score↗

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↗

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↗

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↗

[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↗

[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↗

Hydranencephaly versus maximal hydrocephalus: an important clinical distinction.

To define better the syndromes of hydranencephaly and maximal hydrocephalus, we followed 10 neonates demonstrating (by computerized tomography) virtual absence of the cerebral substance with serial computerized tomographic (CT) scans, electroencephalograms (EEGs), and developmental evaluations. The follow-up period ranged from 4 to 23 months. Two well-defined syndromes emerged. Five patients showed absence of cortical activity on EEG and a CT picture of minimal occipital brain parenchyma connected by a thin bridge of tissue to intact basal ganglia. These patients were shunted and showed no improvement with time, either neurologically or radiologically. Five patients showed minimal frontal cerebral mantle on CT and the presence of electrical activity on EEG. These patients required bilateral shunts to control head size and showed remarkable progress with time. Serial CT scans demonstrated the appearance of considerable brain substance, and neurological development was either normal or slightly delayed. We believe that the former pattern of only minimal occipital brain and absent EEG activity defines true hydranencephaly, whereas the presence of small amounts of frontal brain tissue associated with EEG activity seems to represent maximal hydrocephalus. The differences in CT pattern, EEG, and clinical progress all suggest a different pathophysiology for these two conditions. (Neurosurgery, 6: 35--38, 1980).

Anencephaly↗