Congenital Absence of Cerebral Hemispheres is a medical condition where a newborn is born without fully developed cerebral hemispheres. The word "congenital" refers to a condition present at birth, while "absence" means that the hemispheres are missing completely. The word "cerebral" refers to the brain, and "hemispheres" refers to the two halves of the brain. The IPA phonetic transcription for this condition is kənˈdʒɛnətəl æbsəns əv ˈsɪrəbrəl ˈhɛmɪsfɪrz.
Congenital absence of cerebral hemispheres, also known as cerebrum agenesis or holoprosencephaly, is a rare developmental disorder characterized by the incomplete or complete absence of the cerebral hemispheres - the two large parts of the brain that control various functions such as thought, memory, emotions, and movement.
In a normally developing brain, the cerebral hemispheres are responsible for performing intricate tasks and coordinating complex behaviors. However, in individuals with congenital absence of cerebral hemispheres, this crucial part of the brain fails to form properly during fetal development, leading to serious neurological deficits.
The condition is typically apparent at birth, and affected infants may exhibit a range of cognitive, physical, and neurological impairments. Common symptoms include severe intellectual disability, feeding difficulties, seizures, visual impairments, muscle weakness or paralysis, and hormonal imbalances. The severity of the symptoms can vary widely among individuals, ranging from mild to severe.
The underlying causes of congenital absence of cerebral hemispheres can be genetic or environmental factors, including chromosomal abnormalities, maternal infections or exposure to certain medications or toxins during pregnancy. Although there is no cure for this condition, treatment focuses on managing symptoms and improving the individual's quality of life. This may involve a multimodal approach, including medication to control seizures, physical therapy to improve muscle strength and coordination, and supportive care to address feeding difficulties and other medical complications.
Overall, congenital absence of cerebral hemispheres is a rare and complex disorder that significantly impacts brain function and development, requiring lifelong care and support.