Congenital hydrocephalus in three sheep: Clinical, electroencephalographic and pathological features (original) (raw)
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Spontaneous Congenital Hydrocephalus in Sprague Dawley Rat
2014
Hydrocephalus is a neurological disorder that results from the accumulation of excess cerebrospinal fluid in the ventricles of the brain culminating in an enlarged cranium. This sporadic disorder may occur as a congenital malformation in many mammalian species including inbred rodent colonies at an early juvenile stage of life. Under conventional husbandry practice of breeding, 5 pups showing some clinical signs of neurological dysfunction at the age of 20 days were examined thoroughly. Detailed macroscopic examination demonstrated dome shaped head, thinned and deformed parietal bone, open/closed suture depending upon the severity of ventricular dilatation. Microscopic examination revealed dilated lateral ventricles, compressed and attenuated cortical mantle, spongy appearance of the sub-ventricular zone, stretched ventricular ependyma, flattened ependymal cell lining and infiltration of mononuclear cells in the ventricular lining.
Electroencephalographic aspects in dogs with congenital hydrocephalus
Bulletin of University of Agricultural Sciences and Veterinary Medicine Cluj-Napoca. Veterinary Medicine, 2014
Congenital hydrocephalus is a common neurolo gic disorder in dogs de ined as an increa sed cerebrospinal luid volume within the cranial cavity, which appears secondary to an excessive production or to an absorption de icit of cerebrospinal luid (Shihab, 2011; Wünschmann, 2001). Congenital hydrocephalus is apparent at birth (even if in many cases, clinical signs are irst noticed at several months of age) and is caused by stenosis of the mesencephalic aqueduct associated with fusion of the rostral colliculi, genetic factors and in utero exposure to infectious agents and tera togenic chemicals (Thomas, 2010). Clinical signs of congenital hydrocephalus may include an enlarged, dome-shaped head with persis tent fontanelles, ventral or ventro-lateral strabismus due to malformation of the orbit and neurologic de icits including abnormal behavior, cognitive dysfunctions, ataxia, circling, blindness, seizures and vestibular dysfunction. Neurologic de icits may progress over time, remain static, or even improve after 1 to 2 years of age. Affected patients are often fragile and can worsen later in life coincident with other diseases or minor head trauma.
Hydrocephalus and Its Diagnosis - A Review
Journal of Bahria University Medical and Dental College, 2019
Hydrocephalus is enlargement of the ventricular system of the brain due to increased cerebrospinal fluid (CSF) volume and pressure. Congenital hydrocephalus is further classified as communicating and non-communicating depending on whether there is an obstruction to the flow of CSF or not. Multiple causes have been identified in literature which has been summarized as an imbalance in the production and absorption of CSF. It can lead to cognitive impairment, cerebral palsy and visual field defects. It is crucial to identify this condition prenatally as it can leave a debilitating impact on the fetus. Several modalities like ultrasound, computed tomography scans (CT) and magnetic resonance imaging (MRI) have been used to diagnose hydrocephalus. These can help reduce the disease burden and provide means for timely decisions.
Autosomal recessive hydrocephalus with third ventricle obstruction
American Journal of Medical Genetics, 1990
Here we report a brother and sister who presented in the neonatal period with hydrocephalus. Ultrasonography showed marked dilatation of the lateral ventricles but not the third ventricle. One child with postnatal onset was shunted and had normal development at 3 years. The other child had severe hydrocephalus at birth and was not treated. Neuropathologic studies demonstrated dilatation of the lateral ventricles and marked narrowing of the posterior part of the third ventricle but no other malformations other than those that result directly from hydrocephalus. The potential for a good prognosis is emphasized.
Pteridines, 2014
The aim of this study was to draw the attention of specialists faced with fetal hydrocephalus in the postnatal period to the possibilities of prenatal diagnosis and further monitoring by studying isolated and syndrome cases in fetuses. One hundred and nine fetuses from a total of 2238 autopsies were the subject of observation in this study. In 64 (58.7%) of the studied fetuses, isolated hydrocephalus was found, while the other 45 cases were associated with the following malformations: Arnold-Chiari type II, Dandy-Walker, stenosis of the aqueductus sylvii, agenesis of corpus callosum (partial and total) and numerical chromosomal aberrations such as trisomy 13, 15 and 18. In cases of isolated hydrocephalus and a stable condition of the fetus, it is possible to wait until the term, or to induce labor without danger to the child, followed by a shunting intervention.
Intrauterine hydrocephalus and ventriculomegaly: associated anomalies and fetal outcome
Canadian Journal of Neurological Sciences, 1985
Advances in fetal diagnostic techniques have opened many areas to prenatal anatomical scrutiny. Intrauterine hydrocephalus and ventriculomegaly are conditions which are readily diagnosed. Fetal intervention has been undertaken in humans in order to minimize the craniofacial disfigurement and to maximize the growth potential of the brain. To justify such an approach, the significance of all anomalies should be recognized prior to treatment. The authors have reviewed 41 cases of hydrocephalus diagnosed in utero in order to define associated anomalies and patient outcome. 75% of our personal series and 72% of the reviewed literature cases had other anomalies of the central nervous system. Other system malformations, some of which proved fatal, were seen commonly. Prenatal diagnostic techniques did not always reveal these additional problems. The outcome of these pregnancies is not good. Approximately one third of these fetuses have survived to be treated postnatally and to be followed ...
Congenital hydrocephalus: gestational and neonatal outcomes
Archives of Gynecology and Obstetrics, 2010
Purpose To evaluate gestational and neonatal outcomes in pregnancies complicated by fetal hydrocephalus. Methods Retrospective analysis of 287 cases of fetal hydrocephalus followed at the Fetal Medicine Unit of the University of Campinas in the period of 1996 to 2006. Results Mean maternal age was 25 years, mean gestational age at diagnosis was 27 weeks. There were 50 cases of isolated ventriculomegaly, 95 cases of Chiari II malformation and 142 cases of ventriculomegaly associated with other malformations. Preterm delivery and vaginal delivery were more frequent in the group of ventriculomegaly associated with other malformations. Cardiac, skeletal and renal malformations were the most common associated malformations. Cesarean section was common (95%) in the Chiari II group. Fetal and neonatal death occurred more frequently (29 and 68%, respectively) in the group of ventriculomegaly associated with other malformations. Chromosomal anomalies were present in 15% of 165 investigated cases.
Hereditary lissencephaly and cerebellar hypoplasia in Churra lambs
BMC Veterinary Research, 2013
Background: Lissencephaly is a rare developmental brain disorder in veterinary and human medicine associated with defects in neuronal migration leading to a characteristic marked reduction or absence of the convolutional pattern of the cerebral hemispheres. In many human cases the disease has a genetic basis. In sheep, brain malformations, mainly cerebellar hypoplasia and forms of hydrocephalus, are frequently due to in utero viral infections. Although breed-related malformations of the brain have been described in sheep, breed-related lissencephaly has not been previously recorded in a peer reviewed publication. Results: Here we report neuropathological findings in 42 newborn lambs from a pure Churra breed flock, with clinical signs of weakness, inability to walk, difficulty in sucking and muscular rigidity observed immediately after birth. All the lambs showed near-total agyria with only a rudimentary formation of few sulci and gyri, and a severe cerebellar hypoplasia. On coronal section, the cerebral grey matter was markedly thicker than that of age-matched unaffected lambs and the ventricular system was moderately dilated. Histologically, the normal layers of the cerebral cortex were disorganized and, using an immunohistochemical technique against neurofilaments, three layers were identified instead of the six present in normal brains. The hippocampus was also markedly disorganised and the number and size of lobules were reduced in the cerebellum. Heterotopic neurons were present in different areas of the white matter. The remainder of the brain structures appeared normal. The pathological features reported are consistent with the type LCH-b (lissencephaly with cerebellar hypoplasia group b) defined in human medicine. No involvement of pestivirus or bluetongue virus was detected by immunohistochemistry. An analysis of pedigree data was consistent with a monogenic autosomal recessive pattern inheritance. Conclusions: The study describes the clinical and pathological findings of lissencephaly with cerebellar hypoplasia in Churra lambs for which an autosomal recessive inheritance was the most likely cause. Histopathological features observed in the cerebral cortex and hippocampus are consistent with a possible failure in neuronal migration during brain development. This report suggests that lissencephaly should be considered in the differential diagnosis of congenital neurological disease in newborn lambs showing weakness, inability to walk and difficulty sucking.
Developmental brain anomaly due to fetal hydrocephalus
Journal of Pediatric Neuroradiology, 2015
In this article, the authors intend to illustrate a striking brain malformation due to early onset severe fetal hydrocephalus that superficially suggests the presence of semilobar holoprosencephaly.