Normal pressure hydrocephalus

NPH (normal pressure hydrocephalus) is an excessive build-up of fluid in the head.

What are the symptoms?

The three main symptoms are mobility problems, some degree of forgetfulness / confusion (dementia) and sometimes urinary incontinence.

Dementia will include short-term memory loss, forgetfulness and difficulty in dealing with everyday tasks. You may find it harder to remember previously learnt skills such as finding your way around, or reading a map. However with practice these skills should return to some extent.

Mobility problems may include a shuffling or ‘wide-based’ gait which could result in frequent falls. The main problem seems to be lack of confidence in walking – particularly if you have had previous falls.

Urinary incontinence is usually the last symptom to appear, often starting when someone has difficulty getting to the toilet in time.

The slow onset means that people with NPH do not have the symptoms of raised intracranial pressure, such as headache, vomiting, nausea, sight disturbances or seizures, that children and young people with hydrocephalus have. As symptoms appear gradually and may be associated with old age, many people think they are normal or assume they must learn to live with them.

You may find that you become much more tired than you would expect. Some people experience depression when coping with a long term condition, so talk to your GP if you are experiencing a low mood which doesn’t seem to shift.

What causes NPH?

For most patients the cause is unknown, though it can develop after injury or stroke.

Under normal conditions cerebrospinal fluid (CSF) circulates through the brain, its ventricles and around the spinal cord, acting as a protective cushion and provider of nutrients. About a pint of CSF is produced daily in the adult brain. NPH results when the flow of CSF is blocked in some way.

NPH is so called because the three people first described with the condition (by Hakim and Adams in 1965) had normal pressure CSF, even though their brain ventricles had expanded.

Does NPH affect people of a certain age or gender?

The likelihood of developing normal pressure hydrocephalus increases slightly with age, and it occurs most often in people aged over 60. It seems to affect males and females equally. NPH is not hereditary.

How is it diagnosed?

Diagnosis can be difficult as some of the symptoms are similar to other disorders such as Alzheimer's or Parkinson's disease. Many cases go unrecognised and are never treated.

A GP should consider referring patients with the three main symptoms to a neurologist or geriatrician.

The most common diagnostic tools are neuro-imaging devices – such as computerised tomography (CT) or magnetic resonance imaging (MRI) – and a careful clinical assessment, which usually involves a lumbar puncture test, a walking assessment by a physiotherapist and neuropsychological testing.

Can it be treated?

A programmable shunt is the Gold Standard treatment for NPH. The shunt – a fine tube and valve – is inserted by an operation into one of the spaces of the brain. This enables excess fluid to drain away to another part of the body (usually the abdomen), where it is reabsorbed into the bloodstream.

Having a shunt fitted will usually halt the memory loss, and control your NPH. It will not, however, prevent conditions that are part of normal ageing.

The level and rate of improvement will vary considerably between people, but generally, the earlier the diagnosis the more successful the outcome.

Will I need my shunt replaced after a certain period of time?

If your shunt malfunctions then it will need replacing. If everything goes as expected, your shunt should last you for life.

How often should I have a check-up with the neurosurgeon?

Most neurosurgeons see their patients with NPH once or twice after the shunt is fitted, after which you will probably be discharged. Contact your GP if you then experience any problems – they might refer you back to the neurosurgeon.

What should I do if my condition seems to be deteriorating?

If you have increasing difficulty walking, it can be a sign that the shunt needs adjusting. Go to your GP, who should refer you back to your neurosurgeon. However, your GP may be more familiar with the common signs of shunt problems seen in people with other forms of hydrocephalus (headache, vomiting, nausea, sight disturbance, seizures), so may not be aware that a gradual decline in walking is the key sign that a shunt needs adjusting with NPH.

Are there things I can do to improve my walking?

Just keep on walking! Remember the old saying ‘use it or lose it’. Some people find going to a gym useful – a programme of gentle walking and some leg exercises will help. If you’re having problems getting up and about, ask your GP to refer you to a physiotherapist. Some people with NPH experience a rolling gait, often when tired or stressed. If you experience this all the time, go to your GP, as your shunt may need adjusting. However, some people with NPH, especially if they already had poor mobility, do find that a rolling gait is the best they can achieve.

Are there any things I can do to help improve my memory?

Think of your memory as another part of you that needs exercising, so doing things you enjoy such as crosswords, playing card games, reading books and newspapers can help. Joanna Iddon, a psychologist interested in hydrocephalus, has co-authored a book ‘Memory Booster Workout’ with lots of good ideas. Keep a notebook with you and write down everything that you need to remember. Use post-it notes as aides memoires.

Can I go through the scanner at airports?

Modern programmable shunts are designed to withstand MRI scanners so should, in theory, be unaffected by airport scanners. But there have been many reports of shunts being accidentally reprogrammed so it is best to be cautious and to avoid airport scanners. You should show your Shunt Alert Card (available from Shine on 01733 555988) and follow the guidelines for people with heart pacemakers.

Can I dye my hair?

Yes you can dye or perm your hair as usual, once the shunt scar has healed. And you can sit under the old-fashioned driers with no ill effects.

At what stage after the onset of NPH should I notify the DVLA and, if so, will they take my driving licence away?

You need to let the DVLA know as soon as you are diagnosed and have a date for surgery. You will not be allowed to drive until at least six months after your shunt is fitted. Contact the DVLA again six months after your surgery, as they will need to ask your neurosurgeon to confirm that you are fit to drive (i.e. have had no seizures or blackouts). If the top end of your shunt (the part in your brain) needs to be revised, you will need to inform the DVLA. You do not need to tell them if you only have your shunt pressure adjusted.

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