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The term ‘hydrocephalus’ is from two Greek words meaning ‘water in the head’. In fact, the ‘water’ is cerebrospinal fluid (CSF), a clear liquid which is produced all the time in the cavities or ventricles inside the brain. It passes from one ventricle to the next (four in all) through narrow pathways, then circulates around the surface of the brain – a little also goes down the spinal cord – and is absorbed back into the bloodstream. The absorption takes place through specialised veins inside the skull which have a sieve-like surface. Though much slower than the circulation of the blood, the CSF is constantly being produced, circulated and reabsorbed.
Hydrocephalus can result when either too much CSF is produced (very rare), or when it is prevented from circulating or being reabsorbed – the two most common causes. As in these circumstances CSF is constantly produced but cannot get out, it accumulates and causes raised pressure inside the brain. The ventricles swell and the brain tissue is stretched and squashed. The skull bones in babies and young children are not fixed together as they are in later life, and the pressure causes the head to increase in size. However it is important to realise that hydrocephalus can also arise in older children and in adults, when the skull bones are fixed and the head cannot increase in size.
The causes of Hydrocephalus
A number of conditions can give rise to hydrocephalus. Uncommonly it can be due to a genetic disorder, in which case some other members of the family are usually affected. Non-genetic causes are more common. In babies who are born with hydrocephalus the condition is said to be congenital, and it must be realised that this means simply that it is present at birth, and not that it is hereditary. In congenital hydrocephalus the actual cause is usually impossible to determine but it is assumed to be due to events during the baby’s development before birth, such as damage to local blood supply or infection. A known cause of congenital hydrocephalus, uncommon in this country, is toxoplasmosis, which is caught from animals or from eating undercooked meat. Most of us have had this infection and are now immune but a woman who becomes pregnant and who is not immune might catch the infection. The baby is then at risk of catching the disease in the womb and developing hydrocephalus and eye problems.
About 25 years ago the commonest form of hydrocephalus was that associated with spina bifida. At least 80-90% of people with spina bifida have some degree of hydrocephalus, although it is not always in need of treatment. Here the interference with CSF flow is due to abnormalities of the brain structure at the back of the head which develop at an early stage of the pregnancy. This is often called the Arnold-Chiari malformation.
In recent years far fewer babies have been born with spina bifida, and the largest number of cases of hydrocephalus in infancy occur in babies born prematurely, due sometimes to maternal ill-health or smoking. Even very small babies can now survive, but their vital functions, normally taken care of during pregnancy by the mother, have to be controlled artificially. Unavoidable rapid changes in blood pressure can cause bleeding in the brain, and the blood from this haemorrhage blocks the sieve-like CSF absorption system leading to post-haemorrhagic hydrocephalus. Brain haemorrhage in adults (stroke) is similar and can also lead to hydrocephalus in survivors. Head injury sometimes has the same effect.
Another way in which the CSF pathways can be blocked is by debris and pus in acute infections of the membranes surrounding the brain (meningitis). This can arise in previously healthy babies if they are unlucky enough to develop a serious bloodstream infection soon after birth, but it can happen to anyone in any age group. Fortunately, meningitis is not common and one major cause, haemophilus, has been greatly reduced by the introduction of the HIB vaccine. Vaccine is available for another type of meningitis, meningococcus C (but not yet for type B). College and university students are now offered type C vaccine. Hydrocephalus is not a reason to refuse vaccination, but if in doubt consult your doctor.
Other types of hydrocephalus can be due to obstruction by cysts in the ventricles. Dandy-Walker cysts, which block the fourth ventricle at the base of the brain are an example of these swellings. Brain tumours can obstruct the CSF flow, either by their bulk or by causing brain swelling. In adults, blockage of one of the connecting pathways between the ventricles (aqueduct stenosis) can cause the onset of hydrocephalus.
Two other conditions, benign intracranial hypertension (not very benign for those who have it) in which the CSF pressure is raised, and normal pressure hydrocephalus (NPH) in which, as the name suggests, the CSF pressure is not raised, are also seen in adults. NPH usually affects those in the third part of life, and can give rise to memory loss, walking difficulties and urinary incontinence.
Finally, hydrocephalus is often classified as either communicating or non-communicating. In the first type, the problem is usually failure to absorb the CSF at the end of the system, whereas in the second there is blockage of the CSF pathways within the ventricular system.
The effects of Hydrocephalus
Hydrocephalus involves accumulation of cerebrospinal fluid (CSF) in the ventricles of the brain, with an increase in the pressure inside the head.
There are two sources of this pressure. One is that of the CSF itself, but a much higher pressure is produced by the heart in order to pump blood to the brain. If the CSF pressure rises, it eventually interferes with the blood supply to the brain, depriving it of oxygen and glucose which it needs in constant amounts to continue to function. Initially this causes tiredness, irritability and drowsiness, but if it progresses then loss of consciousness will result as the brain begins to shut down.
The immediate effects of this interference with the blood supply disappear if the CSF pressure is returned to normal, such as by ventricular tap or insertion of a shunt. However, in most cases the process has been continuing for some time before diagnosis of hydrocephalus is made. During this time the interference with the blood supply leads first to a ‘dying back’ of the very fine blood vessels in the brain. Even this process is largely reversible if prompt action is taken, but at this time there is often insufficient clinical evidence to suspect hydrocephalus. The next stages involve progressive damage to the actual nerve cells in the brain and to their eventual destruction, and this cannot be reversed.
Because of the areas of the brain most affected, functions associated with thought and learning, as well as with co-ordinated skilled movement, begin to deteriorate. The precise effects differ between individuals and are further complicated by other abnormalities, as well as by the pre-existing degrees of ability and personality of each person affected. It is not surprising therefore that while, for instance, learning disorders are common amongst those with hydrocephalus, their exact effects vary considerably.
Much is said and written about intelligence, and particularly about IQ (intelligence quotient) in people with hydrocephalus. In fact this is far more complicated, and a good deal less informative, than many believe. The IQ is made up of several components which can be thought of as verbal and non-verbal, or performance-related tests. People with hydrocephalus generally score better on verbal IQ than on performance IQ and this is thought to reflect the distribution of nerve damage in the brain as described above. Certainly, during periods of rising CSF pressure, such as in untreated cases or when a shunt is blocked, the effect on performance IQ is more marked. Generally speaking, people who have had hydrocephalus since birth or childhood have, as a group, a lower average IQ than a comparable group without hydrocephalus, but it is important to realise that there is a wide range in each group, and some people with hydrocephalus have very high scores.
The practical implications of these features of hydrocephalus are that there may be subtle problems of co-ordination of hand movements with what the person sees, as well as a degree of clumsiness, which make it difficult to perform certain tasks or do certain jobs. With regard to learning in the home or to education in school, there may be real problems with concentration and reasoning which require a sympathetic but skilled approach. For instance, it will often be necessary to teach simple everyday tasks like getting out of bed, washing one’s face, dressing and going downstairs as separate short items rather than all at once, and to keep them consistent and repetitive. This does not indicate ‘stupidity’, but is caused by damage to the nerves in the brain which normally allow us to learn very quickly how to do a complex series of things. Much can be done to help, and the following chapters of this book give parents and teachers practical advice on how to help children overcome many of these difficulties. Professional advice should be sought where needed.
Psychological development in children and adolescents with hydrocephalus may proceed normally, but sometimes the changes associated with puberty (breast development, body hair growth etc) appear much earlier than expected, and the intrusion of psychological aspects of sexual development into a mind which is emotionally still very immature can cause distressing problems (see ‘Precocious puberty’, Again, specialist advice should be sought if necessary. Other effects of hydrocephalus may also be seen, and some of these are difficult to explain. For instance, some people are very seriously distressed by every-day noises such as vacuum cleaners or washing machines.
One effect of raised CSF pressure may be seen in the eyes, and this is why your doctor sometimes looks for ‘papilloedema’. This is caused by pressure on the blood supply to the back of the eye. It is important to realise that it may not always be present, even when the pressure is high. If CSF pressure remains high for too long, damage to the optic nerves can become permanent resulting in blindness, though fortunately nowadays this is uncommon. Another appearance, particularly in babies, is the so-called ‘sunset’ eye sign, where the eyes are fixed in a downward position. This is due to CSF pressure affecting important nerves running from the brain which control eye movement.
If untreated the rise in CSF pressure can cause other serious problems in the brain, unrelated to blood supply. Many of our vital functions, such as heartbeat, breathing etc, are controlled from the brain stem, a structure joining the spinal cord to the brain. Very high CSF pressure can compress this sufficiently to cause the heart and breathing to stop. Once again, this is uncommon as signs of raised pressure are usually recognised before this. A similar problem might sometimes arise, particularly in those with spina bifida, due to compression of the cerebellum, a part of the brain lying at the back of the head. This can also give rise to breathing, speaking and swallowing difficulties.
About 1:3 children with hydrocephalus will develop epilepsy which will be treated by a neurologist.
Reading a catalogue of the effects of hydrocephalus, such as I have produced here, can be very alarming. However, it should be realised that some people with hydrocephalus may have very few of these problems, and hopefully none of the more serious ones. Also, many of those which I have described are found either in untreated hydrocephalus or when the treatment fails, and when successful treatment has been promptly introduced they often improve or sometimes disappear. On the other hand, the more subtle learning and reasoning problems are usually present in some degree and are very important where a child’s development and education are concerned.
There can be learning difficulties associated with hydrocephalus such as problems with learning difficulties, behaviour, motivation and visual problems.
Treatment of Hydrocephalus
Hydrocephalus, irrespective of its cause, is due to accumulation of cerebrospinal fluid (CSF) within the ventricles of the brain, resulting in raised pressure inside the head. In principle the solution is simple: that is, to insert a tube into the swollen ventricles and drain off the excess fluid, thereby returning the pressure inside the head to normal again. Historically, many attempts to do this have been recorded but the results were usually disappointing, due to rejection of the tubing by the body’s defences, to infection, or to blockage of the tubing. However, this began to change in the 1950s.
Charles ‘Casey’ Holter, the son of an American engineer, was found to have spina bifida and hydrocephalus. The surgeon explained that there was no satisfactory treatment. John Holter, the child’s father, questioned the surgeon and gained an idea of the sort of treatment required, if only it existed. He experimented at home and soon developed such a device, made from silicone rubber, which had never been implanted into people before. The surgeon decided to try this, with great success. The Holter valve soon became the treatment of choice for hydrocephalus, and it also revolutionised the treatment of spina bifida, removing the principal cause of death and encouraging orthopaedic and urological surgeons to develop procedures to improve mobility and continence. Other similar devices were developed soon afterwards, all made from the same material and having similar valve mechanisms.
In the 1960s investigations were proceeding into how CSF was produced, and drugs were tried experimentally to reduce the rate of CSF production. Some of these showed initially promising results, but they had unpleasant side effects and their use was abandoned. However, new investigations are now being undertaken on drugs which might reduce CSF production in the hope of avoiding a shunt, but even if this approach is successful it could be many years before it is used.
A relatively new approach, based on an old method, is to pass a tube (endoscope) into the ventricles and to use this to remove part of the CSF-producing mechanism (choroid plexus). A similar approach can also be used to make an opening in the bottom of the third ventricle to allow CSF to escape into the rest of the CSF circulation system (third ventriculostomy). Such techniques, if successful, avoid the use of a shunt but they do not always work, and some patients will have a type of hydrocephalus for which the treatment is not suitable. Third ventriculostomy has been used to treat many patients without the need for a shunt, although as with a shunt, the hydrocephalus is not “cured”, but controlled. Today there are numerous types of shunt but while they all look different they work in a very similar way. None can be said to be significantly better or worse than another, and the shunt is usually chosen by the surgeon on the grounds of experience, cost and personal preference.
Due to revisions of part of the shunt, sometimes in several different hospitals, parts of different shunt systems can be found in the same patient.
Originally shunts were inserted so that a tube drained CSF from the ventricles in the brain, through the valve and through another tube into a vein in the neck and then into the heart (ventriculo-atrial or VA shunt). While these are still used, most nowadays drain the CSF into the abdomen (ventriculo-peritoneal or VP shunt) and the bottom tube can be felt over the ribs. Despite all these developments, shunting can have complications. These can be divided into under-drainage, over-drainage and infection.
Under-drainage, in which the fluid is not removed quickly enough and the symptoms of hydrocephalus return, is one of the commonest problems. It is usually due to blockage of the upper or lower tubes of the shunt by tissue, though it can be due to the shunt breaking or its parts becoming disconnected from each other. It is rarely due to the valve itself, which usually continues to function in the same way for years. Pressure may sometimes build up rapidly, resulting in loss of consciousness, and treatment is required as an emergency. However, in most cases the onset is more gradual, and can follow a minor illness such as a cold. Headaches increase in frequency and severity, often worse on waking in the morning. Vomiting and dizziness also occur, and sometimes other symptoms which vary from patient to patient. In these cases the parents or carers will be able to recognise the symptoms from previous episodes. Specialist hospital staff are now fully aware of the various presentations of ‘blocked shunt’, but non-specialists and family doctors may not be.
Shunt blockage can also have much more subtle consequences and the headaches may be infrequent, the main problem being behavioural deterioration. In older children this might take the form of increased irritability, ‘laziness’, poor or disruptive school performance or even more antisocial activity. This may be very difficult to distinguish from the usual teenage angst but, if there is any reason to suspect that the deterioration in behaviour is not ‘normal’, assessment must be carried out by an experienced educational psychologist with a knowledge of hydrocephalus.
The basis for the effects of high CSF pressure have been explained previously. If the shunt is to blame, a dramatic improvement can result from appropriate treatment, though this form of shunt problem is particularly difficult to diagnose. It may be necessary to monitor CSF pressure, often over 24 hours. This can be done using a pressure monitor in the scalp connected to a recorder. In this way pressure can be recorded during sleep and changes in posture. Scans to show the size of the ventricles are particularly useful if they can be compared to previous scans, though in someone with clear symptoms of either high or low CSF pressure they may also serve to support the diagnosis.
In the case of over-drainage, the shunt allows CSF to drain from the ventricles more quickly than it is produced. If this happens suddenly, usually soon after the shunt is inserted, then the ventricles in the brain collapse, tearing delicate blood vessels on the outside of the brain and causing a haemorrhage (‘subdural haematoma’). This can be trivial or it can cause symptoms similar to those of a stroke. The blood may have to be removed, and in some cases if this is not done it may be a cause of epilepsy later. If the overdrainage is more gradual, the ventricles collapse gradually to become slit-like (‘slit ventricles’). This often interferes with shunt function causing the opposite problem, high CSF pressure, to reappear, but unfortunately the slit ventricles do not always increase in size again, producing the situation where there is very high CSF pressure with headache, vomiting etc but very small ventricles on scan.
The symptoms of over-drainage can be very similar to those of under-drainage though there are important differences. Headaches, dizziness and fainting occur and are often worse after getting up from lying down, whereas the headaches caused by high CSF pressure are often worse on waking, before rising in the morning. However the best way to diagnose the problem, having recognised that one exists, is to measure the CSF pressure over 24 hours.
Under-drainage can be caused by blockage of the shunt tubing either at the top end, by tissue plugging the entry holes or by the position of the tube changing; or at the bottom end, by tissue in the abdomen sealing off the drainage tube. In VA shunts blockage can occur at the bottom end as the child grows, and a revision operation is sometimes necessary to lengthen the shunt at the age of 12-18 months.
Over-drainage is a more difficult problem. There is no clear relationship between the type of valve (high or low pressure) or the brand, and over-drainage. A change of valve to a higher pressure cannot be relied upon to cure it, though it appears to do so in some cases. Studies have shown that the use of an ‘antisyphon device’, a small button inserted into the shunt tubing, will often solve the problem, but this does not always work. Some shunts have these built-in, but neurosurgical opinion varies as to whether they should be used. To change a valve pressure it is necessary to remove the valve and insert another. A ‘programmable’ or adjustable shunt is intended to allow adjustment of the working pressure of the valve without operation. The valve contains magnets, which allow the setting to be changed by laying a second magnetic device on the scalp. This is undoubtedly useful where the need for a valve of a different pressure arises, but the adjustable valve is no less prone to over-drainage than any other and it cannot be used to treat this condition.
It was long believed that a raised protein level in the CSF would block the shunt, and in babies with hydrocephalus shunting has been delayed until the protein level has fallen. Recent research has shown that a raised CSF protein level has no ill-effect on shunt function, nor does it increase the risk of infection, and there is now no reason to delay unless blood is also present.
The third complication of CSF shunting is infection. This is almost always due to bacteria from the skin getting into the CSF or shunt at operation, and is remarkably difficult to prevent. Antibiotics have not been shown to be of benefit for this purpose, and other measures often have only a temporary effect, though obviously the care and expertise of the surgical team is one of the most important factors in reducing the rate of infection to a minimum. However, even in the best of hands infection still occurs. As a result of research originally sponsored by ASBAH, we at Nottingham University have developed a shunt which is resistant to bacterial infection. Thousands of these shunts have now been used worldwide with considerable success, and a recent trial in South Africa showed a dramatic fall in infection rate. Further clinical trials are being carried out and we hope that it will be capable of reducing shunt infection by more than 80%.
In VP shunts, infection will usually show itself within a few weeks or months of operation as a shunt blockage with return of the features of hydrocephalus, although there may also be occasional fever and abdominal pain. Redness and swelling may be seen over the lower shunt tubing. It is important to distinguish between blockage of the VP shunt due to infection and that from other causes as the treatment is different. In VA shunts blockage due to infection is rare, and many months or years can go by before the infection becomes apparent. During this time there will be tiredness, irritability, poor appetite, various aches and pains, skin rashes and other signs but all of these can be due to common disorders. A blood test will usually reveal anaemia and this is an important though, on its own, not a specific indication of infection. Blood cultures and even CSF cultures can be negative. Later, blood may appear in the urine due to secondary kidney damage.
While shunt infections can sometimes be very easy to diagnose, they are often difficult and any delay increases the chance of further damage. Special blood tests have been developed which allow a reliable diagnosis to be made rapidly in people with VA shunts, but these are not widely available. VA and VP shunt infections are both treated in the same way. Until recently this involved operations, long periods in hospital and disappointing relapses before a new ‘clean’ shunt could eventually be inserted. We have developed an approach which shortens the time taken to treat most shunt infections to 7-10 days, with a very low relapse rate, and this is now incorporated into UK national guidelines and is being adopted by others. Unfortunately the infected shunt still has to be removed, though in future it may be possible to treat these infections successfully without taking out the shunt, and research into this aspect is continuing.
Though complications of shunting remain an important problem in the treatment of hydrocephalus, I hope I have managed to explain some of their mysteries, and to show that solutions are being found.
Shunt Malfunctions
Shunts remain the mainstay of treatment for hydrocephalus and, in the majority of cases, this remains an effective treatment.
However, they can never perfectly mimic normal physiology and, like any mechanical device, they are prone to malfunctions of various sorts.
Shunt blockage, along with shunt infection, remains the commonest cause of shunt malfunction. In the vast majority of cases of shunt blockage prompt investigation and revision of the shunt is associated with full recovery and discharge from hospital within a few days. In rare situations shunt blockage can be fatal, particularly when the diagnosis is delayed.
There hasn’t been a significant improvement in the level of blockages in recent years. The rate of shunt blockages is highest in the first year after insertion, when it can be in the order of 20-30% – decreasing to approximately 5% per year thereafter. Over half of patients who have a shunt fitted will need at least one shunt revision in the following 10 year period.
Obstruction can occur in any part of the shunt. If the shunt is not working properly, either all or part of it will need to be replaced. It is the ventricular catheter (the part of the shunt that passes into the ventricles of the brain) where the shunt most commonly blocks. The holes in the shunt tubing may become obstructed by the choroid plexus (this is the membrane which manufactures the CSF) or by a build up of cellular debris.
The clinical presentation is usually dominated by signs of raised pressure as the brain fluid (CSF) builds up. This increase in pressure results in symptoms of, most typically, headache, vomiting and drowsiness. Sometimes the symptoms come on quickly over hour or days, but occasionally they may develop over many weeks with intermittent headache, and tiredness, change in behaviour or deterioration in schoolwork. In babies an enlarging head circumference, bulging fontanelle (the soft part at the top of the head), CSF tracking along the course of the shunt and, rarely, seizures, are additional indicators of underlying shunt malfunction.
Less common symptoms include seizures, abdominal swelling (due to malabsorption of CSF by the peritoneum or encysting of CSF in the peritoneal cavity), cranial nerve palsies (particularly in the spina bifida child).
If a shunted child becomes unwell, it’s important that the possibility of a shunt blockage be considered (and hopefully excluded) as soon as possible.
Two prospective studies of 104 admissions to the paediatric neurosurgical unit at Great Ormond Street Hospital NHS Trust showed that drowsiness was by far the best, but not definite, clinical predictor of shunt blockage. Headache, vomiting and irritability were less predictive as to whether the child’s shunt was actually blocked, and nor was the duration of the symptoms. The presence of a raised temperature suggests an alternative diagnosis for the symptoms or may indicate that the shunt is malfunctioning because it is infected.
It is important that children who have had treatment for hydrocephalus (this includes those who have had endoscopic third ventriculostomy) and their families are aware of the symptoms and signs of shunt blockage and who to contact if they are concerned. Most paediatric neurosurgical units provide 24hr telephone advice and many offer an open access policy for children who have shunts.
Diagnosing shunt blockage is not always straightforward. Commonly there will be an alternative explanation for the symptoms for example ear infection, common colds etc. In fact, parents can be as successful at diagnosing shunt blockage as GPs and paediatricians.
Whilst additional investigations such as CT scan, plain X-rays and a shunt tap may be decisive, a definitive diagnosis is sometimes only possible through surgery.
Families should always be advised about shunt blockages, why and how they happen, at the time a shunt is first fitted. All children who have a shunt in situ should be registered with a designated neurosurgical unit to which they can be referred when the shunt goes wrong. If a child moves to another part of the country with their parents they must be reassigned to a new neurosurgical facility as soon as possible.
Families should have open access to their neurosurgical unit for emergency advice. Deteriorating conscious level visual failure/obscurations, neck pain/stiffness or slow pulse rate all imply dangerously elevated intracranial pressure and constitute a neurosurgical emergency. Urgent medical advice must be sought in this situation; this may mean that the child will be seen at their local hospital initially where doctors can stabilise the child’s condition and liaise with the neurosurgical team regarding emergency transfer.
It is important to repeat that most children who develop a shunt blockage will make a full recovery once the problem is treated. Children with shunts are as prone to all the usual childhood ailments as children without and new symptoms or signs will commonly have an alternative explanation. It is always better to seek advice sooner rather than later – when in doubt shout!
ETV – An Alternative Treatment
Endoscopic third ventriculostomy (ETV) is a procedure used to treat hydrocephalus, as an alternative to a shunt.
Although the procedure, which is a natural way of draining the CSF fluid from the brain, is only suitable for a small percentage of hydrocephalus patients, it has many advantages over shunting.
Link talked to Conor Mallucci, Consultant Paediatric Neurosurgeon at the Royal Liverpool Children’s Hospital and The Walton Centre for Neurology and Neurosurgery, to learn more about ETV.
“Endoscopic third ventriculostomy was attempted years ago, before shunts were invented. A man called Dandy performed it as an open operation in the early twentieth century. But basic endoscopic attempts with primitive endoscopes even preceded this. It was always a logical way to try and treat hydrocephalus.
Modern equipment to carry out ETV didn’t exist until about twenty years ago, so it is only now that we are able to review the procedure, and look at success rates and possible complications.
Hydrocephalus is a group of conditions with different causes, and the treatment should naturally select itself depending on the cause.
Traditionally we have used shunts to drain the cerebral spinal fluid (CSF) from the ventricles in the brain to another part of the body, and this is still the main method of treatment.
Third ventriculostomy, on the other hand, does the opposite to a shunt. Instead of draining the CSF away, it keeps it within the brain and spinal cord.
We make a hole in the thin membrane at the base of the third ventricle, which allows the fluid into the area that lines the brain and the spinal cord called the sub arachnoid space. Once the fluid has drained into this space it can be absorbed.
The ETV technique is performed via a hole similar to that of a shunt, usually at the front of the head, just behind the hairline, using an endoscope of between 2 and 5mm in diameter.
This is a beautiful technique, which drains the CSF more naturally, but unfortunately this is only a solution for less than a quarter of all patients.
The patients for whom ETV is going to work well are people who have pure obstructions within the brain such as aqueduct stenosis, or patients who have tumours.
A second set of people who respond well are those who have had shunts for many years and have developed an obstructive form of hydrocephalus. An MRI scan will show if the patient has a blockage of the CSF pathway in their brain.
The success of ETV procedures depends on the experience of the surgeon and most importantly, the patient who is selected.
If patients are carefully chosen, the success rate for pure obstructive hydrocephalus in new patients is around 70 per cent. The failure rate is highest in the first 2 to 3 months, and if it doesn’t work then, we can go to shunt.
If the ETV has worked beyond those initial few months, then it is more likely to stay working. But it is important that patients are aware that the procedure isn’t a cure for hydrocephalus, it is an alternative treatment.
There is currently an international study looking at the success rates of third ventriculostomy in the under ones, because it is felt that in these children, despite the anatomy being suitable, the success rate isn’t as high as later on.
This is possibly because the child’s brain and skull is not fully formed, and the pressures generated within the brain aren’t high enough to keep the third ventriculostomy open.
I tend to offer third ventriculostomy in any child who has purely obstructive hydrocephalus, but I make the parents aware that success rates are probably lower in newborn children than they are later on in life.
If patients are suitable for a third ventriculostomy, then there are several advantages to the procedure over shunts. Firstly the infection rate, at two per cent, is very low. The other main advantage is that we do not see the over-drainage problems, which can lead to headaches, because it is a natural way of draining the CSF.
So while shunts still remain the mainstay of hydrocephalus treatment, third ventriculostomy is a complementary procedure suitable for a group of patients, and remains a useful adjunct for patients who have shunt problems or blockages later in life.”
What is NPH?
Normal pressure hydrocephalus (NPH) is an excessive build-up of fluid in the head.
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. It occurs most often in people aged over 60.
The three main symptoms are mobility problems, forgetfulness/confusion (dementia) and urinary incontinence.
However, diagnosis is still difficult due to some of the symptoms being similar to other disorders, e.g. Alzheimer’s, Parkinson’s disease or simply increasing age. Many cases go completely unrecognised and are never treated.
Dementia will include short -term memory loss, forgetfulness and difficulty in dealing with everyday tasks. Because these symptoms appear gradually and are commonly associated with old age, many people think they are normal and assume they must learn to live with their problems.
Mobility problems may include a shuffling or wide based gait which could result in frequent falls.
Urinary incontinence is usually the last symptom to appear, often starting when someone has difficulty in getting to the toilet in time.
For most patients the cause is unknown.
In some cases an imbalance in the production or absorption of CSF causes the hydrocephalus.
Normal pressure hydrocephalus is not that common; however UK research suggests that up to 2% of patients in the UK aged 60 and over who are thought to have dementia may in fact have NPH. This 2% could represent up to 10,000 people who may be suitable for assessment for treatment.
A GP should consider referring patients with the three main symptoms to a neurologist or geriatrician.
While it can be difficult to diagnose, the most common diagnostic tools are neuro-imaging devices – such as computerised tomography (CT) or magnetic resonance imaging (MRI) – and a careful clinical assessment; this usually involves a lumbar puncture, when a thin needle is passed into the spinal fluid space30.05.06ne to see if the symptoms are temporarily relieved.
Generally, the earlier the diagnosis the better chance of successful treatment.
Assessment of suitability for a shunt varies between hospitals but investigations may include an MR scan; measuring the pressure inside the head either by lumbar puncture or by an intracranial pressure measuring device inserted under anaesthetic through the skull under the scalp and by drainage of CSF by lumbar puncture for a few days to see if a patient improves.
Treatment is by operation when a fine tube and valve (called a shunt) is inserted into one of the spaces of the brain to drain away excess fluid. The fluid is diverted to another part of the body (usually the abdomen), where it is reabsorbed into the bloodstream.
People will respond to the operation in different ways and while in most cases an improvement results (the success rate for shunting can be as high as 80%), the level of this improvement will vary. The rate of improvement will also vary considerably between patients.
The above information,was optained from http://www.asbah.org/
Posted in Intracranial Hypertension - IIH | Tagged Hydrocephalus, Intracranial Hypertension - IIH | 1 Comment »
Simply speaking, Pseudotumor Cerebri is a condition in which the body either produces too much cerebrospinal fluid pressure or doesn’t absorb it well, resulting in increased cerebrospinal fluid pressure.
Pseudotumor cerebri literally means “false brain tumor” because it’s symptoms mimic those of brain tumors. It is also (more accurately) called Idiopathic Intracranial Hypertension (IIH). The term idiopathic is applied because it’s cause is not truly known. It is likely due to high pressure caused by the buildup or poor absorption of cerebrospinal fluid in the subarachnoid space surrounding the brain, but the reason for the buildup or poor absorption are also unknown. The disorder is most common in women between the ages of 20 and 50. Being overweight seems to be a a possible contributing factor, but PTC should not be ruled out based only on body weight, age, or gender.
Symptoms of pseudotumor cerebri:
Diagnosis of PTC:
The only truly definitive diagnostic test for PTC is a lumbar puncture (LP) (spinal tap) with the cerebrospinal fluid (CSF) pressure measured when the needle is inserted. In some patients, swelling of the optic nerve (papilledema) can be observed in a thorough eye exam. However, it is important to note that the absence of papilledema does not rule out PTC. Not all patients with PTC exhibit papilledema. When the LP is performed, it is also essential that the protein level and cell count of the fluid be tested. The presence of protein or elevated white blood cell count indicate can indicate that inflammation or infection could be causing the elevated CSF pressure.
Secondary Intracranial Hypertension:
While PTC and IIH are idiopathic in origin, Secondary Intracranial Hypertension always has a cause. Diagnosis of Secondary IH is the same as IIH, but Secondary IH can be traced back to causes such as other conditions or medications:
Treatment:
Prognosis:
Close, repeated ophthalmologic exams are required to monitor any changes in vision. Surgery may be needed to remove pressure on the optic nerve. The disorder may cause progressive, permanent visual loss in some patients.
How does PTC relate to headaches and Migraine and their treatment?
PTC can cause headaches and/or trigger Migraines. It can also keep headache and Migraine preventives from working properly.
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Click here to download information in PDF format
Feelings and Disability
Many people with long-term physical difficulties don’t like using the word ‘disabled’ about themselves but it is the word that the government uses to talk about people who have a long-term health problem. If you need to claim Benefits then it may be a ‘Disability’ Benefit or if an employer or a college treat you unfairly then it will ‘Disability’ law that is being broken. So we will carry on using this word here.Everybody, disabled or not, goes through times when they feel ‘down’ or low in mood, sometimes for days or weeks at a time. Sometimes we know why we feel this way – losing somebody close, or having other things go on in our lives which we don’t want, for instance.Because we can’t control everything that happens in our lives, bad things are bound to happen from time to time. What this means is that feeling down is ‘normal’. If someone close to you dies it can take many months to get over it but during this time your mind is working hard at getting used to the loss. At first, you may be sad all the time; gradually you are sad for less time and eventually you may be able to remember the person with fondness and even smile at good memories.We call this feeling ‘grief’ and the process ‘grieving’. But other changes in our lives can make us feel the same way. Breaking up with a close friend, losing a job we like, and so on. While we are getting used to the changes, we will feel low.Sometimes we don’t know why we are feeling this way but there is always a reason. Sometimes we are so used to trying to deal with what is making us feel low that we stop noticing it. When the feelings go on for a long time this can be puzzling for us, and for those around us who can see that there is something wrong.
Living with any type of disability is difficult. It can often seem that other people around you get what they want more easily. It may seem to you that there are things you would like to do that you will never be able to. Other people are not always kind if they know you are disabled. But because disabled people have to cope with these things every day, they sometimes stop realising how hard it is, begin to feel very down and sometimes don’t know why. If you get down sometimes, try to think how someone who is not disabled would feel if they suddenly became disabled and had to cope with all the problems you have to cope with all the time. You are probably doing very well to cope with all your problems. Actually, a lot of people who become disabled, as a result of accidents for instance, do find it very hard to cope and get very down before they learn how to cope.Even when we know why we are feeling low it is often helpful to talk about it to somebody else. Sharing problems with somebody you trust is usually a good idea, especially if they have experienced the same difficulties themselves. If that isn’t possible then you can sometimes find people who are trained to help.So, if you feel down for a week or so, try:To talk to someone else you trust about how you feel
To remember that what you are having to cope with is difficult
Not to get angry with yourself –remember that you are coping with something that some people couldn’t cope with at all
To treat yourself as sympathetically as you would want to treat somebody else who was going through the same thing.
To keep on meeting friends and doing the things you know you enjoy- even if you don’t really feel like it you will probably feel better afterwards.Disability and DepressionIf you feel low for more than a few weeks and you don’t know why; if you can’t tell yourself to ‘snap out of it’ or if the things that usually interest you don’t cheer you up then you may be experiencing what is often called ‘depression’ and you may need special help to start feeling better.Most disabled people are not depressed and disabled people who become depressed do not stay depressed. This is another way of saying that it may not be the disability itself which causes depression but how people cope with it. If you become depressed it may be that you are having to cope with change or something new.
The difference between feeling ‘down’ and feeling depressed is that when you are depressed, the feelings are deeper and last for longer. If you are down, you can often tell yourself to do things to make you feel better; that doesn’t work when people are depressed because they lose interest totally.
This is list of what might happen if you or someone you know gets depressed.
Feeling sad nearly all the time
Feeling hopeless about your future
Feeling like a failure or feeling worthless
Not enjoying things you used to enjoy, losing interest in things you used to be interested in, losing interest in sex
Feeling that you are to blame for a lot of what goes wrong
Losing confidence in yourself
Wanting to harm or kill yourself
Crying or feeling like crying although you didn’t cry easily before
Being restless a lot of the time
Lack of energy and feeling tired a lot of the time
Sleeping much more than usual or having trouble getting off to sleep
Feeling bad tempered a lot of the time
Wanting to eat a lot more or a lot less than usual
Not being able to concentrate as well as before, finding it very difficult to make decisions
Smoking or drinking too much. Relying on social drugs.
If you have had a number of these problems for more than a couple of weeks, or if you know someone who seems to have these problems, then getting help from someone else is important.
Remember, becoming depressed is not being “silly” or a sign of weakness. There are always real reasons for becoming depressed and help is available.
You may be a part of a support group or be part of an organisation (such as ASBAH) which can help, so try this first.
Most people who become depressed go to their own doctor, who can make a further referral if specialist treatment is required.
Dealing with Depression
There are two main ways of dealing with depression:
Talking about problems to a trained person
Taking drugs called ‘anti-depressants’ which are prescribed by a doctor.
Your doctor should be able to refer you to a counsellor or another suitable therapist who will be able to find out why you are depressed and help you to deal with it. When this is available, this is first thing to try.
You will probably need to see your counsellor every one or two weeks to begin with and you should expect to be seeing them for at least 6 months – feelings of depression tend to get better quite slowly but you can expect to get back to normal eventually.Anti-DepressantsIf counselling is not available or if you are really depressed, your doctor might suggest you take anti-depressants.
It is true that taking pills will not solve problems but sometimes people are so depressed that they lack the mental energy to sort things out. Anti-depressants will sometimes help by making you feel more like tackling problems. Sometimes you can take anti-depressants and have counselling at the same time but the type of anti-depressant needs to be carefully chosen by your doctor for this to happen.
Most anti-depressants are not addictive and you can come off them quite easily when the time comes. You need to be taking them for 3 to 4 weeks before they start to work and you need to take them for at least 3 months before you can tell whether they are working for you. Doctors prefer depressed patients to keep taking them for 6 months.
Anti-depressants can make you feel peculiar or a bit poorly when you first start taking them but most people manage to cope. If you get severe problems you should tell your doctor. Do not increase or reduce the amount you take without discussing it with your doctor. Drinking alcohol or taking other drugs while you are taking anti-depressants can be dangerous – again, discuss this with your doctor.
When you feel the time has come to stop taking anti-depressants, discuss this with your doctor. You will usually need to reduce the amount you take gradually.
How to help other people who become depressed
People who become badly depressed can lose interest in helping themselves and they may have started to shut themselves away from other people. It may seem to you that they are not interested in being with you.
Underneath the depression though, there is usually a strong need to be close to others.
If they don’t seem interested in seeing you, try to show that you still want to see them. Listen to them if they want to talk about problems.
Being with a depressed person is often no fun – be prepared for their gloomy thoughts and lack of interest.
Don’t tell them to “pull themselves together”. Being badly depressed means that they can’t do this.
Most important, accept that you may not be able bring them out of depression yourself and try not to feel responsible if you seem to be failing.
If you are worried, suggest that they go to see their doctor if they haven’t already been. If they won’t, don’t be afraid to tell someone else who can help, even if the depressed person seems not to want you to. It is important that they get to see someone who can help without becoming too emotionally involved. The person’s doctor, a teacher or a parent are people you could tell.
A depressed person may not want you to do this at the time, but may well thank you later when they can understand that you needed to help.
With thanks to Dr James Meikle, Consultant Clinical Psychologist
For more help and information about depression contact:
MIND www.mind.org.uk Mindinfo Line: 0845 766 0163
The Depression Alliance www.depressionalliance.org 0845 123 23 20
Defeat Depression www.depression.org.uk
The Royal College of Psychiatrists www.rcpsych.ac.uk
Posted in Intracranial Hypertension - IIH | Tagged DEPRESSION, DISABILITY | Leave a Comment »
You may hear this condition referred to as Intracranial Hypertension (IH), Pseudotumor Cerebri (PTC), or Benign Intracranial Hypertension (BIH). These terms all refer to the same condition (IH will be used most often within this blog). Please keep in mind throughout these blogs, IIH affects every person differently. Do not let the experiences you read frighten you. Those who seek out support on-line may be the most difficult cases
What is Intracranial Hypertension?
Generally IIH is difficult to diagnose because it is a disease of absence. There is no brain tumor, signs found on a brain scan, no abnormalities in the CSF (other than opening pressure), and no localized findings on a neurological evaluation. This very lack of clues is what often distinguishes IIH early on. This makes diagnosis difficult (and sometimes long). It can be more frustrating if the individual does not exhibit all the telltale symptoms such as tinnitus (ringing or swooshing sound in the ears) or papilledema.
*What are some signs and symptoms of IH?
IIH literally means that the pressure of the CSF that surrounds the brain is too high. Raised intracranial pressure is transmitted along the paths of the Optic Nerves, causing the nerves to swell (Papilledema), which can lead to visual disturbances, loss of vision, and even blindness. A patient may experience transient altered vision (visual disturbances such as double vision, blurry vision, and a “blacking out” of vision) as signs of papilledema; and an early indication oh IIH.
The most common symptom is an unbearably painful headache that is not relieved by any medication. This can last days, months, and even years at a time. Some patients end up in the emergency room believing they’re suffering from the worst migraine headache imaginable. An LP may be done to temporarily ease the headache and CSF pressure on the Optic Nerve. This is not always effective, as CSF replaces itself 3-4 times a day.
Intracranial noise is a common trait of one with IIH, which many describe as a “swooshing” sound or heartbeat in the ears. This tends to get worse with exertion. Other symptoms can include stiff neck and neck pain, vertigo (dizziness), back pain, memory loss and confusion, pain behind the eyes, vomiting, fatigue, and exercise intolerance.
Additional symptoms reported include photo-phobia, problems with balance and spatial awareness, disorientation, pins and needles and loss of sensation in the hands; to name a few. In some cases, CSF leaks down the nose, or from the ears. It is important to exclude Cerebral Tumor as a cause of these symptoms.
People with raised ICP may find it difficult to cope with previously learned everyday tasks, e.g. handling money or using the telephone. They may be unable to find their way around a previously familiar town: traffic is confusing, they can be unaware of curb height, sometimes may be afraid to even step off the curb in case they step incorrectly, thus crossing the street can become a nightmare. Simple conversation can become a challenge for some.
IH takes a huge physical toll – a formerly active and productive person may become too sick to take part in everyday tasks. Unfortunately many times the patient appears outwardly well and is frustrated in attempting to convey his or her painful circumstances. As there is little awareness of IIH, even the most supportive of people often do not understand what the patient is truly going through. Physicians can be equally frustrated as they have so little to use to combat this disease.
*Is there a cure?
Unfortunately there is currently no cure for IIH. One group actively researching IIH is the IHR Foundation. Please visit their website at the IHR Foundation. Dr. Glueck of Ohio is also researching causative agents of IIH. He believes IIH is related to blood clotting disorders and clotting problems. For more information or to participate in his research study, please see our page on Dr. Glueck and his study (Dr.Glueck Info).
*How is IH Treated?
The most common initial treatment is with high doses of Carbonic Anhydrase Inhibitors. Diamox falls under these. Please advise your doctor of any prior allergic reaction to sulfa drugs. These drugs work to block the Carbonic Anhydrase Enzyme System. With these drugs the CSF production process is disrupted, which allows some degree of control of pressure. Unfortunately these drugs also block similar enzyme systems elsewhere, such as the eyes and kidneys. And in some people, the drugs simply do not work. There are potential drug complications including the infrequent risk of kidney stones, and a very rare risk of a serious blood disorder. Carbonic Anhydrase Inhibitors are very difficult to tolerate. A patient may also be prescribed diuretics (such as Lasix). These drugs work to clear your body of excess fluids. There are many, many other drugs an IIH patient may be given for treatment, including topamax.
Lumbar Punctures (LP’s) have often been used as treatment to relieve the excess CSF fluid. Some people may benefit from having repeated LP’s done while others may find the relief lasts a very short period of time. Some doctors are hesitant in performing repeat LP’s as this can create a whole new set of problems such as infection, brain herniation, chronic neck and back pain, and nerve damage. Spinal headaches may also result, leaving one incapacitated for a short period of time. Cerebral Spinal Fluid replaces itself anywhere from 3-6 times a day, often leaving this form of treatment, short term. Many Doctors have recently moved away from using LP’s as treatment for IIH.
Two types of surgery are performed when medical therapy fails or the patients vision is in serious jeopardy: Optic Nerve Sheath Fenestration (ONSF) and Shunting. You can read more about both of these procedures in the menu on your left. ONSF was first performed in 1872 to treat Papilledema, Neuro-Surgical Shunts were first used in 1892. In ONSF a small opening is made in the sheath around the Optic Nerve to relieve swelling. Shunts are surgically implanted tubes that are used to drain the CSF to another part of the body, such as the abdominal cavity. These can be placed in either the head (VP or VA), or the back (LP).
Surgical treatments often require repeat operations and can produce a new set of complications such as blindness, infection, and other life-threatening conditions. Because surgery is such a complex subject with a large diversity of opinion, it is best to thoroughly research the medical literature. Patients should always discuss the risks and benefits of any treatments with their physicians.
Posted in Intracranial Hypertension - IIH | Tagged What Is Intracranial Hypertension?BIH IIH PTC | Leave a Comment »
HI
THIS IS ME SAMANTHA, I’M 35, AND I LIVE IN NORTHUMBERLAND, IN THE UK.
I AM DOING THESE BLOGS, USING LOTS OF INFORMATION, FROM RESEARCH, IN THE HOPE, MY INFORMATION, WILL HELP NOT ONLY NEW DIAGNOSED PEOPLE, BUT ALL THE sufferers OUT THERE.
AS TIME PASSES, MORE AND MORE TREATMENTS ARE BECOMING READILY AVAILABLE.
MORE PAIN TREATMENTS AND MANAGEMENT, IS BECOMING MORE ACCESSIBLE.
AND THE CONDITION IS MORE RECOGNISED NOW THAN IT WAS 12 YEARS AGO, WHEN I WAS 1ST DIAGNOSED.
I AM HOPING THAT THROUGH TIME, I WILL HAVE BUILT UP, ENOUGH INFO ALL IN ONE PLACE. THAT WILL GO ON TO HELP HUNDREDS.
I ALSO BLOG ON MYSPACE, AND NOT ONLY COVER BIH, BUT ALSO,BIPOLA,AND OCD’S, WHICH ARE OBSESIVE COMPULSIVE DISSORDERS, WHICH OF LATE, HAVE STARTED TO SHOW ASSOSIATION, WITH BIH, ALONG WITH SEVERE MOOD CHANGES, AND SOME PERSONALITY DISSORDERS.
SO STAY TUNED,I AM NOT A DOCTOR, AND MUST STRESS, IF YOU HAVE A MEDICAL COMPLAINT, ITS ALWAYS BEST TO GO STRAIGHT TO YOUR GP. OR YOUR A N E DEPT IF ITS A EMERGANCY.
I AM PURELY BRINGING ALL INFORMATION, TO ONE PLACE, MY BLOG, TO MAKE IT ACCESSABLE TO YOU, THE READER.
THANK YOU
SAMANTHA
I HOPE TO BLOG SOME OF THIS, TO HELP PEOPLE.
Posted in Intracranial Hypertension - IIH | Tagged BIH.IIH, intracranial hypertension, PTC | 4 Comments »
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