Biomaterial Database

Congenital hydranencephaly and arthrogryposis of Corriedale sheep. 1988

R J Whittington, and J R Glastonbury, and J W Plant, and M R Barry

New South Wales Department of Agriculture, Regional Veterinary Laboratory.

A syndrome of congenital malformations in Corriedale sheep characterised by brachygnathia inferior, campylognathia, tetraemlic arthrogryposis, kyphoscoliosis, hydranencephaly and hypoplasia of the brain stem, cerebellum and spinal cord occurring in various combinations is described. Histologically there was generalised hypomyelinogenesis and hypoplasia of the central nervous system with neurogenic atrophy of skeletal muscle. The syndrome resembled that caused by congenital infection with Akabane virus, however, serological, pathological and epidemiological data indicated that Akabane virus was not involved. The results of a breeding trial suggested that the disorder may be transmitted with an autosomal recessive mode of inheritance.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D005260	Female		Females
D006832	Hydranencephaly	A congenital condition where the greater portions of the cerebral hemispheres and CORPUS STRIATUM are replaced by CSF and glial tissue. The meninges and the skull are well formed, which is consistent with earlier normal embryogenesis of the telencephalon. Bilateral occlusions of the internal carotid arteries in utero is a potential mechanism. Clinical features include intact brainstem reflexes without evidence of higher cortical activity. (Menkes, Textbook of Child Neurology, 5th ed, p307)	Cerebral Hemispheres, Absence, Congenital,Hemihydranencephaly,Absence of Cerebral Hemispheres, Congenital,Congenital Absence of Cerebral Hemispheres,Hydranencephaly with Proliferative Vasculopathy,Hemihydranencephalies,Hydranencephalies
D000757	Anencephaly	A malformation of the nervous system caused by failure of the anterior neuropore to close. Infants are born with intact spinal cords, cerebellums, and brainstems, but lack formation of neural structures above this level. The skull is only partially formed but the eyes are usually normal. This condition may be associated with folate deficiency. Affected infants are only capable of primitive (brain stem) reflexes and usually do not survive for more than two weeks. (From Menkes, Textbook of Child Neurology, 5th ed, p247)	Aprosencephaly,Absence of Brain, Congenital,Anencephalia,Anencephalus,Congenital Absence of Brain,Hemicranial Anencephaly,Incomplete Anencephaly,Partial Anencephaly,Anencephalias,Anencephalies, Partial,Anencephaly, Hemicranial,Anencephaly, Incomplete,Anencephaly, Partial,Aprosencephalies,Brain Congenital Absence,Partial Anencephalies
D000818	Animals	Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA.	Animal,Metazoa,Animalia
D001176	Arthrogryposis	Persistent flexure or contracture of a joint.	Amyoplasia Congenita,Arthromyodysplasia, Congenital,Guerin-Stern Syndrome,Myodystrophia Fetalis Deformans,Arthrogryposis Multiplex Congenita,Arthrogryposis Multiplex Congenita (AMC),Congenital Arthromyodysplasia,Congenital Multiple Arthrogryposis,Fibrous Ankylosis of Multiple Joints,Guérin-Stern Syndrome,Otto Syndrome,Rocher-Sheldon Syndrome,Rossi Syndrome,Arthrogryposes,Arthrogryposes, Congenital Multiple,Arthrogryposis Multiplex Congenitas,Arthrogryposis Multiplex Congenitas (AMC),Arthrogryposis, Congenital Multiple,Arthromyodysplasias, Congenital,Congenita, Arthrogryposis Multiplex,Congenita, Arthrogryposis Multiplex (AMC),Congenital Arthromyodysplasias,Congenital Multiple Arthrogryposes,Congenitas, Arthrogryposis Multiplex,Congenitas, Arthrogryposis Multiplex (AMC),Guerin Stern Syndrome,Guérin Stern Syndrome,Multiple Arthrogryposes, Congenital,Multiple Arthrogryposis, Congenital,Multiplex Congenita, Arthrogryposis,Multiplex Congenita, Arthrogryposis (AMC),Multiplex Congenitas, Arthrogryposis,Multiplex Congenitas, Arthrogryposis (AMC),Rocher Sheldon Syndrome,Syndrome, Guerin-Stern,Syndrome, Guérin-Stern,Syndrome, Otto,Syndrome, Rocher-Sheldon,Syndrome, Rossi
D012756	Sheep	Any of the ruminant mammals with curved horns in the genus Ovis, family Bovidae. They possess lachrymal grooves and interdigital glands, which are absent in GOATS.	Ovis,Sheep, Dall,Dall Sheep,Ovis dalli
D012757	Sheep Diseases	Diseases of domestic and mountain sheep of the genus Ovis.	Ovine Diseases,Disease, Ovine,Disease, Sheep,Diseases, Ovine,Diseases, Sheep,Ovine Disease,Sheep Disease
D013577	Syndrome	A characteristic symptom complex.	Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes

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