Normal Pressure Hydrocephalus

  -What is Normal Pressure Hydrocephalus?

 Also termed NPH or Symptomatic Hydrocephalus, a type of Hydrocephalus (water on the brain). Normal pressure hydrocephalus is a neurological disorder in which  cerebrospinal fluid  (CSF) surrounding the brain is blocked or otherwise unable to circulate or drain in a normal manner.

-Normally the fluid (CSF) circulates around the brain, from ventricle to ventricle (4 total), cushioning, protecting and supplying the brain with necessary nutrients. But when this fluid builds up, it enlarges the fluid-filled chambers (ventricles) inside the brain.

 As the ventricles continue to expand, the increased  intracranial pressure  (ICP) can then compress brain tissue and cause damage.

Dr. Salomon Hakim first identified "normal" pressure hydrocephalus and the classic triad of symptoms in 1957, (gait, impaired bladder, cognition) with his paper describing cases soon to follow in 1964.
It is now known that many patients do, in fact, have fluctuating pressures, but during this time, methods of measuring CSF pressures were not available.
-The disorders name, however, remains its common name. It usually affects adults over the age of 60.

 * Be aware that  NPH symptoms , treatment and diagnosis are different than those for children with hydrocephalus. Also be careful when reading about general hydrocephalus, as it usually refers to children.

Normal Pressure Hydrocephalus Symptoms

 In the beginning, the symptoms of Normal Pressure Hydrocephalus are generally very subtle and get worse gradually.

- Difficulty walking : Usually the first and most dominant of NPH symptoms. Difficulty may be mild (slow, cautious gait) in the early stages and can progress to a marked gait problem (unsteady, obvious difficulty) and finally in very late stages may have complete inability to walk.

 In the early-mild stage complaints of tiredness and weakness in the legs and problems with stairs and obstacles regarding balance and stability.

-The problems seen at the marked stage show increased difficulty in raising the feet above obstacles (curbs, stairs) where, much like those with Parkinson's shuffling gait, the feet may appear to be stuck to the ground. (often referred to as a  magnetic  gait)

     Urinary NPH Symptoms:

  • Frequent need to urinate
  • Inability to keep urine, urgency
  • Involuntary urination appears late in the disorder.
  • Some cases never have bladder control problems

 - Dementia : Or mental decline in processing and reacting to information, though usually the responses are appropriate and correct, but slower. Dementia NPH symptoms present as:

  • Inattention and forgetfulness
  • Apathy (indifferent) as well as dull slowness.
  • Mood and behavioral changes
  • Difficulties in reasoning and judgment

 Memory problems can be the dominant symptom in some cases, leading to the misdiagnosis of Alzheimer's disease. With NPH symptoms however, there may be a vast and obvious difference between often-severe memory loss and much less impaired recognition, not typical in Alzheimer's.

*Symptoms may vary in severity, may not occur at the same time and also may not all be present. (may be only one or two symptoms)

  Normal Pressure Hydrocephalus Causes

 Most often there is no obvious cause of NPH, and doctors don't know the reason for CSF buildup. In this case it is known as Idiopathic NPH.

     Some known causes of NPH:

  • Brain tumor
  • Head trauma
  • Infections, such as Meningitis
  • Subarachnoid Hemorrhage

 People with High Blood Pressure, Low HDL levels, Diabetes and Ischemic Heart disease are all at a higher risk for NPH symptoms.

  Diagnosing Normal Pressure Hydrocephalus

 Although the symptoms of Normal Pressure Hydrocephalus can present in other disorders, the combination of the triad of NPH symptoms make it unique (when all present) and tests can be done to confirm the diagnosis.

 A Complete Physical and Psychological Exam

 A complete medical history and physical examination is necessary to rule out any other condition that could cause the symptoms.

-A psychological exam to discover any cognitive problems, consisting of a series of questions and verbal tests to address mental abilities such as memory, attention, following instructions, reasoning abilities, etc.

 A Lumbar Puncture is usually the first test taken in diagnosing NPH. Measurements of spinal fluid pressure are taken prior to and after removal of 30 ml or more of this fluid, and the degree of improvement after this test can be a good indicator for possible future shunt treatment.

-Conversely, even if only slight improvement is seen after fluid removal, many patients have still seen improvement with shunt treatment.

 MRI brain imaging can sometimes aid in diagnosing Normal Pressure Hydrocephalus, but in many cases other dementia disorders (such as Alzheimer's disease for example), can cause brain shrinkage and thereby also make the ventricles in the brain appear larger.

-A CT Scan -May sometimes show enlarged ventricles. 

  Treatment of NPH Symptoms

 Many people have heard through the media (esp. TV) that NPH is often misdiagnosed as Parkinson's, Alzheimer's or other form of dementia, and could in fact be a successfully treatable condition. (NPH) However, it is believed that NPH only accounts for about 2% of all cases of dementia. (Estimates vary from 2%, to as high as 10%, depending on the source)

-Although certainly still a possibility, most experts in the field agree and do not believe that to be the case. Unfortunately it is unlikely that a substantial number of people are so misdiagnosed. (have NPH) It is still a good idea to be screened if you suspect NPH, you may be one of the 2%!

 Normal Pressure Hydrocephalus is not a common disorder, and to a doctor who is experienced in diagnosing Alzheimer's or Parkinson's disease, it does appear different than these brain disorders.

-On occasion NPH can be surgically treated. A shunt, or long thin tube is inserted to drain the fluid in the brain, into the abdomen, where it is absorbed. But even when this type of surgery is successful, the mental decline is generally not the symptom most benefited, bladder control and walking abilities are the more likely to be improved.

  NPH Prognosis

 Normal pressure hydrocephalus is a long-term condition and it cannot be cured. Some people however, may find significant relief with surgery and can have a high degree of recovery and quality of life. (see video)

-All people are not candidates for surgery, depending on medical history.

 Further treatment for urinary, walking or dementia symptoms will likely be needed, will be ongoing, and of course vary on the individual.

-As with most any disease or medical condition, early diagnosis and subsequent treatment or surgery will improve chances of recovery.

  Hope for NPH?



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