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Are there different types of hydrocephalus?

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Why this site is of interest

The information given on this website is aimed at those who experience difficulties in their everyday lives because of hydrocephalus. Most difficulties are associated with how the brain is affected as it is developing and growing. Children and young people with congenital or acquired hydrocephalus (early in life), are more likely to experience and ‘grow into’ difficulties than those that acquire hydrocephalus later in life.

For this reason, topics in the Living with hydrocephalus section are discussed with reference to children and young people. However, many of the examples illustrate hints and tips that could be adapted for any age. In addition, the Hydrocephalus and the brain section looks at how the brain’s processing is affected by hydrocephalus and may be relevant to readers of any age.

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Congenital hydrocephalus

‘Congenital’ means that the hydrocephalus is present at birth.

It can be associated with:

  • Conditions that occur while a foetus is growing (eg spina bifida)
  • Infections transmitted from the mother (eg mumps or rubella).
  • Malformation of the brain (eg Dandy Walker syndrome)
  • Traumatic injury to the brain.

In some cases the hydrocephalus will be detected prior to birth. If a baby is thought to have an enlarged head additional scans may be carried out. This might include ultrasound, computerised tomography (CT) or magnetic resonance imaging (MRI).

After birth, signs of hydrocephalus might include:

An enlarged head; possibly ‘bulging’ fontanelle (soft spot) on the top of the head; a thinning of the skin on the scalp.

  • An enlarged head; possibly ‘bulging’ fontanelle (soft spot) on the top of the head; a thinning of the skin on the scalp
  • A downward gaze.
  • Stiffening of the legs and possible muscle spasms.
  • Being sleepy or irritable.
  • Vomiting or not feeding well.

Hydrocephalus that is diagnosed at birth will be treated within days of birth. See How is hydrocephalus treated? for more details.

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Acquired hydrocephalus

Acquired hydrocephalus happens after birth and is the result or consequence of some other illness or condition. It can occur in children or adults (see What causes hydrocephalus? for more details).

Some of the signs of hydrocephalus in babies or very young children may be similar to those described above.

Older children and adults may show symptoms such as:

  • Headaches and/or neck pain
  • Feeling and/or being sick
  • Being drowsy (which could progress to a coma if left untreated)
  • Confusion, irritability
  • Visual disturbances
  • Difficulty in walking
  • An inability to control bladder and sometimes bowel movements

Headaches and nausea may be noticed more in the mornings as the flow of CSF is affected more when lying down.

If hydrocephalus is suspected, the person is likely to have computerised tomography (CT) or magnetic resonance imaging (MRI) of the brain. Testing for pressure is likely to include a lumbar puncture.

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Normal Pressure Hydrocephalus

Normal pressure hydrocephalus (NPH) is relatively rare and tends to affect people over the age of 50. While it sometimes follows a head injury, stroke or an illness such as meningitis, the cause is not always known. NPH occurs in the same way as other forms of hydrocephalus (a blockage in the flow of CSF) but there is little or no increase in pressure around the brain, hence the name.

There are three symptoms common to everyone diagnosed with NPH. These are:

  1. Gait (walking) disturbance: This is usually the first symptom that people notice. Although there is no "classical" pattern, some people develop a wide based walk whilst others take small shuffling steps. They may have poor balance and have frequent falls.
  2. Urinary incontinence: Usually starts as frequency and/or urgency. But, incontinence may be due to walking difficulties (unable to reach the toilet in time) or normal consequence of age eg prostate problems.
  3. Dementia: Dementia is usually mild, progressing to moderate and will include short term memory loss, forgetfulness and difficulty in dealing with everyday tasks.

Because these symptoms are of gradual onset and are commonly associated with increasing age, many people think that this is the norm and assume that they must learn to live with their problems. Even GPs and other medical professionals may not initially consider a diagnosis of NPH when presented with a patient with these symptoms.

Diagnosis of NPH will be made in the same way as for acquired hydrocephalus however the lumbar puncture will show normal or near normal pressure. NPH is likely to be treated in the same way as other forms of hydrocephalus including the surgical insertion of a shunt. See How is hydrocephalus treated? and What is a shunt?/Shunt alert cards for more details.

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Intracranial Hypertension

Intracranial Hypertension (IH) is essentially raised pressure inside the skull (intracranial). It can be used as a term to describe symptoms of hydrocephalus but it is also used when someone has another condition that affects intracranial pressure (eg a tumour or an infection; the result of a stroke).

Sometimes the ‘cause’ of the increased pressure is unknown. This is known as Idiopathic Intracranial Hypertension (IIH). This is diagnosed when neurological examinations and brain scans are normal, but a lumbar puncture shows increased pressure. In addition there will be swelling of the optic discs of the eyes known as papilledema. The most common symptoms of IIH include:

  • Severe headaches.
  • Temporary loss of vision
  • Double vision
  • A "whooshing noise" in the ears
  • Pain behind the eye

Other symptoms may be very similar to those associated with hydrocephalus such as: vomiting; dizziness; dislike of bright lights; confusion and irritability; visual-spatial problems.

IIH can happen in all age groups and is far more common in females. In the teenage group it has an equal prevalence in males and females. The person tends to be grossly overweight.

For more information about IIH see the IIHUK website.

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