Snowdrop

What is Absence of the Septum Pellucidum?

Absence of the septum pellucidum (ASP) is a rare disorder, (occuring in an estimated 2 to 3 individuals per 1 00,000 people in the general population). It is characterised by abnormal development of a thin membrane located at the midline of the brain. It runs down from the corpus callosum, the structure which connects the two cerebral hemispheres of the brain and effectively acts as a separator for the two hemispheres. The disorder usually occurs with other neurological abnormalities such as agenesis / dysgenesis of the corpus callosum.

Individuals with ASP may experience vision impairment or blindness. They may also have coordination problems and hormone deficiencies that result in short stature. Intelligence is usually affected and learning disabilities are common. The disorder usually manifests early in life, often as a consequence of discovering the other neurological abnormalities, such as corpus callosum abnormalities or septo – optic dysplasia. Symptoms include involuntary eye movements, a wasting of a part or parts of the body, and short stature. Seizures and inappropriate behaviour, such as displays of 'sham rage' may also occur. The cause of ASP is currently unknown.

What is the prognosis?

The prognosis of ASP varies depending on the severity of co-occurring abnormalities. Many cranial abnormalities are life threatening, but alone ASP is not a life-threatening disorder.

Can ASP be treated?

When a part of the brain is actually missing, - having not developed at all, then obviously no amount of treatment is going to be able to restore that missing neurology. What we can strive to do is to enable the neurology which is present to function at maximum efficiency and therefore give the child the opportunity to achieve his / her maximum potential. We believe that that at Snowdrop, we teach parents how to provide an appropriately stimulating developmental environment for this to happen