What does Pontocerebellar hypoplasia do?
What does Pontocerebellar hypoplasia do?
Pontocerebellar hypoplasia also causes impaired growth of other parts of the brain, leading to an unusually small head size (microcephaly ). This microcephaly is usually not apparent at birth but becomes noticeable as brain growth continues to be slow in infancy and early childhood.
What is brainstem hypoplasia?
Pontocerebellar hypoplasia (PCH) refers to a heterogeneous group of disorders characterized by an abnormally small cerebellum and brainstem. Clinical features vary, but usually include severe developmental delay, dysmorphic features, seizures, and early death (summary by Durmaz et al., 2009).
How do you fix hypoplasia?
In the case of sensitivity, cavities, or tooth structure showing wear, treatment options include:
- Resin-bonded sealant. This can improve tooth sensitivity.
- Resin-based composite fillings.
- Dental amalgam fillings.
- Gold fillings.
- Crowns.
- Enamel microabrasion.
- Professional dental whitening.
What causes hypoplasia?
Enamel hypoplasia caused by environmental factors carries the same symptoms as hereditary enamel hypoplasia, but can be caused by a variety of factors, such as premature birth, malnutrition, bacterial and viral infections, or trauma to newly developing teeth and mouth.
What are the treatment options for pontocerebellar hypoplasia type 1?
Treatment for pontocerebellar hypoplasia type 1 (PCH1) is aimed at treating the signs and symptoms present in each individual. Management options may include physical therapy or braces on the limbs to help with joint contractures, ventilation machines for breathing assistance, anti- seizure medication,…
What kind of brain damage does pontocerebellar hypoplasia cause?
Pontocerebellar hypoplasias (PCH) are a group of rare heterogeneous conditions characterized by prenatal development of an abnormally small cerebellum and brain stem, which is usually associated with profound psychomotor retardation.
Can a child with pontocerebellar hypoplasia live to adulthood?
The brain abnormalities are usually present at birth, and in some cases they can be detected before birth. Many children with pontocerebellar hypoplasia live only into infancy or childhood, although some affected individuals have lived into adulthood.
Is there a cure or cure for PCH?
Despite the discovery of new pathways involved in PCH, treatment is still symptomatic. However, correct diagnosis of PCH is important to provide suitable care and counseling regarding prognosis, and offer appropriate (prenatal) genetic testing to families.