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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)
Also Known As:
Absence of Cerebral Hemispheres, Congenital; Congenital Absence of Cerebral Hemispheres; Hydranencephaly with Proliferative Vasculopathy; Hemihydranencephalies; Hydranencephalies; Cerebral Hemispheres, Absence, Congenital; Hemihydranencephaly
Networked: 53 relevant articles (1 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Hydrocephalus (Hydrocephaly)
2. Arthrogryposis
3. Lissencephaly
4. Porencephaly
5. Neurogenic Diabetes Insipidus (Central Diabetes Insipidus)

Experts

1. Albright, A Leland: 2 articles (05/2020 - 07/2014)
2. Malheiros, José Augusto: 2 articles (09/2017 - 03/2010)
3. Oliveira, Marcelo Magaldi: 2 articles (09/2017 - 03/2010)
4. Challamel, Marie-Josèphe: 1 article (01/2021)
5. Debilly, Gabriel: 1 article (01/2021)
6. Franco, Patricia: 1 article (01/2021)
7. Hartley, Sarah: 1 article (01/2021)
8. Lahlou, Saadi: 1 article (01/2021)
9. Ferson, Susan S: 1 article (05/2020)
10. Thiong'o, Grace M: 1 article (05/2020)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hydranencephaly:
1. cytidylyl-(3'-5')-cytidine (CpC)IBA
2. Amino AcidsFDA Link
3. Neutralizing AntibodiesIBA
4. VaccinesIBA
5. AntibodiesIBA
6. polyalanineIBA
7. Retinaldehyde (Retinal)IBA
8. Estrogens (Estrogen)FDA Link
9. Cocaine (Cocaine HCl)IBA
10. AntigensIBA

Therapies and Procedures

1. Ventriculostomy
2. Ventriculoperitoneal Shunt
3. Hemispherectomy
4. Cautery
5. Thyroidectomy