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Normal pressure hydrocephalus, usually seen in older adults, is a completely treatable condition with good result. Unfortunately a good number of these individuals are misdiagnosed as some incurable condition like Alzheimer’s disease and left untreated.
The brain and spinal cord are surrounded by a clear fluid called cerebrospinal fluid (CSF). This fluid circulates around the brain and spinal cord. An excess of CSF can occur when the natural system for draining and absorbing the extra CSF does not work properly. The ventricles (CSF filled spaces in the centre of the brain) start to enlarge to accommodate the extra fluid and then press on different parts of the brain, causing a number of different symptoms. This condition, called hydrocephalus, causes very high pressure in the brain.
Normal pressure hydrocephalus (NPH) is a type of hydrocephalus that occurs in adults, usually older adults (>60 yrs). It develops slowly over time as the drainage of CSF is blocked gradually, and the excess fluid builds up slowly. This means that the fluid pressure in the brain may not be as high as in other types of hydrocephalus. However, the enlarged ventricles still press on the brain and can cause symptoms.
NPH can occur after head injury, bleeding around the brain (called subarachnoid haemorrhage), stroke, meningitis (infection of the protective membrane around the brain), brain tumours or after surgery on the brain.
At first, the symptoms in normal pressure hydrocephalus are usually very subtle and worsen very gradually. The symptoms are of three types: dementia (reduced intelligence), difficulty in walking and urinary symptoms.
The dementia symptoms include:
pathy (indifference) and withdrawal
Changes in behavior or mood
Difficulties with reasoning, paying attention, or judgment.
The walking problems include:
Lower limb weakness causing shuffling steps and sudden falls.
Difficulty in taking the first step, as if feet were stuck to the floor (magnetic gait).
"Getting stuck" or "freezing" while walking.
The urinary symptoms include:
Inability to hold urine
Urgency to urinate.
These symptoms can be confused with normal aging and the patient is left untreated but could be due to NPH which is a treatable condition. Any of these problems, or changes in mood or behaviour, warrants a visit to your health care provider. The symptoms of NPH can occur in Alzheimer disease and Parkinson disease. However, the combination of dementia-like symptoms, walking problems, and urinary problems should alert your health care provider to the possibility of NPH. Making the distinction is very important because the treatments for these conditions are quite different. Tests are available that can confirm the diagnosis. At any point in this process, your health care provider may refer you to a specialist in brain disorders (neurologist or neurosurgeon) to complete the evaluation and begin treatment.
The evaluation for NPH includes a detailed clinical assessment with detailed questions on the symptom followed by detailed physical examination to document your condition. This is necessary to rule out other problems that might cause similar symptoms. The examination will probably include tests of your mental status, such as answering questions and following simple directions Called mini mental status examination). Neuropsychological testing may be performed to document your dementia symptoms. Neuropsychological testing is the most accurate method of pinpointing and documenting a person's cognitive problems and strengths and helps to give a more accurate diagnosis of the problems thus helping in treatment planning. It assesses cognitive abilities such as memory, attention, orientation to time and place, use of language, and abilities to carry out various tasks and follow instructions. Reasoning, abstract thinking, and problem solving also are tested.
There is no lab test that confirms the diagnosis of NPH. Any lab tests that are done are probably being done to rule out conditions that might cause similar symptoms.
CT scan of the head.
MRI of the head.
Lumbar puncture involves removal of CSF from the area around the spinal cord in the lower back. The CSF pressure is measured, and the fluid that is removed is analyzed for abnormalities that might give a clue as to the problem. Usually, more fluid is removed than is necessary for these tests. The idea behind this is that removal of a large volume of CSF helps relieve symptoms. (This is checked by comparing examination results before and after). This effect usually is only temporary. However, improvement of symptoms with lumbar puncture is commonly interpreted as meaning that a surgical shunt would be helpful in that person.
Normal pressure hydrocephalus generally cannot be cured. It is a long-term condition. However, many people with the condition obtain substantial relief through surgical treatment. For those who are not candidates for surgery, treatment consists of measures to relieve mood and behavioral problems, cope with physical problems such as incontinence and walking difficulties, and maximize physical, mental, and social functioning.
Medical Treatment: No drug or other medical treatment is known to work in NPH.
Surgery: Occasionally the cause of the hydrocephalus can be treated directly through surgery. For example, a brain tumour blocking drainage of the CSF can be removed. In most cases, however, the underlying problem is not known or cannot be treated. The treatment in these cases is a shunt operation. A shunt is a thin tube that is implanted and drains the excess CSF away from the brain and spinal cord. The tube is routed under the skin. The shunt can be inserted either from the ventricles of the brain or the subarachnoid space of the lower spine to the peritoneum from where it is absorbed. A shunt operation is not a cure. It does not treat the underlying cause of NPH. It can, however, relieve the symptoms. The shunt remains in place indefinitely. If properly implanted, the shunt often is not obvious to other people. Most people who undergo a shunt operation have substantial symptom relief. Many surgeons perform a spinal tap before surgery to test whether the symptoms get better with removal of fluid. In some cases, the person is hospitalized for a few days while fluid is drained slowly through a small tube. This is another way of checking whether removing extra fluid will help symptoms.
The earlier the NPH is diagnosed, the better the chances that the surgery will help. In general, people with milder symptoms have better outcomes with this surgery. Like any surgery, the shunt operation can cause complications. Such complications include infection of the shunt and bleeding around the brain. Another operation is sometimes used instead of shunt placement. In endoscopic third ventriculostomy, an endoscope (thin tube with a lighted camera on the end) is used to create a small hole in the floor of the ventricles. The hole provides another way for CSF to drain from the brain.