Hydcrocephalus

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Excess CSF in the intracranial cavity is termed hydrocephalus and most cases are caused by obstruction of the flow of CSF.

The term 'hydrocephalus' is used to describe conditions in which there is increase in the CSF volume within the brain, with expansion of the cerebral ventricles.
The most common type, which is termed non-communicating hydrocephalus or obstructive hydrocephalus, is caused by blockage of the CSF pathway from the ventricles to the sub-arachnoid space. A less common type is communicating hydrocephalus, in which there is impairment of resorption of CSF at the arachnoid villi along the dural venous sinuses, usually precipitated by previous infection or bleeding into the sub-arachnoid space.

Among the main causes of obstructive hydrocephalus is congenital hydrocephalus, seen in about 1:1000 births. Some cases have stenosis of the aqueduct of Sylvius, some have associated Arnold-Chiari malformation, and some are inherited as an X-linked trait.

Tumours may obstruct the ventricular system, particularly those located in the brain stem, cerebellum, or pineal region which block the cerebral aqueduct or IVth ventricle.

Scarring and blockage of the CSF exit foramina at the base of the brain are common complications of meningitis or sub-arachnoid haemorrhage.

Haemorrhage in the brain or sub-arachnoid space may obstruct CSF drainage pathways.

Macroscopically, there is dilatation of the ventricular cavities of the brain proximal to the site of obstruction. The effects of hydrocephalus depend on the speed of development of disease and age of the patient. 

In acute hydrocephalus, swelling of the brain may be rapid and may cause death due to cerebral herniation. 

In chronic hydrocephalus, signs and symptoms develop slowly and there are clinical features of raised intracranial pressure. When hydrocephalus develops in children, before fusion of the skull bones, there is progressive enlargement of the head circumference. In adults, in whom the skull is rigidly fused, prolonged disease causes thinning of the skull vault. In the absence of treatment, long-standing disease causes axonal damage and gliosis in the white matter. In children this leads to mental subnormality and in adults can lead to the development of a dementia syndrome, with gait disturbance and incontinence as prominent features.
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