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Is it Alzheimer’s, Parkinson’s or NPH?

NPH, Normal Pressure Hydrocephalus, is a brain disease that is often misdiagnosed because it mimics other brain conditions. This can result in patients waiting years to receive a treatment that could give them significant improvement… or never receiving it at all.

It is estimated that 15,000 Canadians and 700,000 Americans have NPH but less than 20% are diagnosed correctly. Instead, they are diagnosed with Alzheimer’s or Parkinson’s Disease, or just old age.

“Hydrocephalus” means increased water or fluid in the brain. This condition can also occur in children, but they usually have increased pressure in the brain while adults with the condition have pressure that fluctuates from normal to elevated. It is treated by surgically inserting a shunt to drain fluid from the brain and spinal cord (called cerebrospinal fluid) into another part of the body.

Adults produce 500 ml (2 cups) of cerebrospinal fluid each day. This fluid supports and cushions the brain, removes waste and distributes important substances. When its circulation is blocked, fluid accumulates, causing the cavities within the brain (called ventricles) to enlarge, pressing on parts of the brain and causing malfunction. The type of malfunction would depend on which area was receiving pressure.

Symptoms

The adult version of the disease, Normal Pressure Hydrocephalus, was named before newer forms of brain monitoring showed that pressure could be high as well as normal, but the name stuck even though it isn’t quite accurate. NPH most commonly occurs in adults over age 60. Symptoms include:

  • Difficulty standing and walking, a shuffling gait

  • Impaired bladder control

  • Memory problems and lack of concentration

It is not necessary to have all 3 symptoms to be diagnosed with NPH. In many cases, physical symptoms appear first, followed by mental symptoms like forgetfulness.

Cause

Most cases of NPH are “idiopathic”, which means the cause is unknown. This is also referred to as “primary NPH”. There is also “secondary NPH” that is caused by another condition, such as head injury, brain surgery, subarachnoid hemorrhage (bleeding in the brain), tumors, cysts (closed, sac-like abnormal structures that contain fluid, air or a semisolid substance), meningitis or other brain infections.

Symptoms progress with time. The longer and more severe the symptoms, the less likely treatment will be successful, but some patients with symptoms for years can improve with treatment. One study found that 87% of those treated had experienced improvement.

How is it diagnosed?

Often the affected person or a family member brings the symptoms to the attention of the family doctor, leading to a diagnosis. Sometimes enlarged ventricles (fluid-filled spaces) in the brain are found when the brain is scanned for another reason. It is recommended to see a neurologist or neurosurgeon if NPH is suspected for an evaluation and interpretation of test results.

3 types of testing can be used:

  1. An interview, physical exam (to rule out other causes) and a neurologic exam to assess brain function

    1. Observation of walking and turning

    2. Questions about bladder function

    3. Pencil and paper tests to examine attention, reaction time, memory, reasoning, language and emotional state).

  2. Brain imaging to look for enlarged ventricles in the brain

    1. CT scan

    2. MRI (can detect impaired fluid flow as well as enlarged ventricles)

  3. Tests to predict whether surgery will help

    1. Lumbar puncture/spinal tap (A thin needle is inserted into the fluid around the base of the spine in the lower back. Pressure can be tested and up to 50 ml of fluid is drained off to see if lowering fluid pressure will improve symptoms)

Because of the high rate of misdiagnosis, the Hydrocephalus Association is conducting a campaign to inform doctors and the public about NPH. Check out their website — it’s a good place to start, if you are interested in more information.

References:

CTV News

The Hydrocephalus Association

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Health

Could diabetes cause Alzheimer's?

Diabetes increases your risk of heart disease, stroke, and damage to blood vessels and nerves, but did you know that studies suggest it may also increase your risk of eventually developing Alzheimer’s disease and other forms of dementia?

And the damage to your brain may even start before you have diabetes…

Type 2 diabetes begins with spikes in levels of sugar in the blood, which trigger your body to produce higher amounts of insulin to process this sugar. At first the higher amounts of insulin can reduce blood sugar to normal (this stage is referred to as pre-diabetes) but eventually, as your body becomes increasingly resistant to the effect of insulin, blood sugar can no longer be controlled at normal levels, and you are diagnosed with diabetes.

Research is suggesting that these increased levels of insulin begin the process of damaging the inside of blood vessels and, in the brain, also stimulate formation of toxic proteins that damage brain cells. Researchers have discovered that the same protein that is formed in Alzheimer’s patients’ brains, is found in the brains of people with type 2 diabetes (T2D). T2D patients often experience a sharp decline in cognitive function and almost 70% of them eventually develop signs of Alzheimer’s or another dementia, according to http://www.alzheimers.net.

Melissa Schilling, professor at NYU, suggests that all patients with dementia or who are at risk of developing dementia should be tested for hyperinsulinemia (high insulin in the blood). She suggests that high insulin could be responsible for almost half of all cases of dementia. However, she recommends that further research needs to be done to verify her arguments and implications for treating Alzheimer’s. Her article: Unraveling Alzheimer’s: Making Sense of the Relationship between Diabetes and Alzheimer’s Disease was published in the Journal of Alzheimer’s disease in Jan 2016. Click here for the full text.

Research points to the protein, amylin, that is co-secreted with insulin and, along with the beta-amyloid protein, it is thought to be important in the formation of amyloid plaques found in the brains of Alzheimer’s patients.

Another possibly important factor, may be levels of insulin-degrading enzyme (IDE), the enzyme that breaks down insulin, reducing levels once it’s finished its job. The presence of insulin stimulates increased activity of IDE, the enzyme that breaks down insulin. Lower insulin leads to less activity of this enzyme. IDE also breaks down amyloid proteins, but it favours breakdown of insulin.

It may be an imbalance between IDE, insulin and amyloid-beta production that ultimately is shown to be a major cause of dementia. Here are 4 suggested scenarios:

  • Severe lack of insulin (as in type 1, insulin dependant diabetes), could lead to less IDE activity, and subsequent decrease in breakdown of amyloid proteins that create plaques in the brains of dementia patients.

  • Lack of production of IDE itself would lead to increased levels of both insulin and beta-amyloid

  • Excessively high levels of insulin (and amylin, secreted along with it) in early stages of type 2 diabetes could lead to competition for insulin-degrading enzyme between insulin and amyloid. Since the enzyme favours insulin, this would result in less breakdown of amyloid and increased amounts available for formation of plaque in the brain

  • An individual could produce higher than normal amounts of amyloid proteins that outpace the ability of IDE to break it down.

You can see how both a lack of insulin and too much insulin could be a cause of dementias. Too little insulin-degrading enzyme or increased production of amyloid proteins could cause problems in people with normal production of insulin. These confusing scenarios could explain why researchers have not yet determined the causal relationship between diabetes and Alzheimer’s, despite reporting on the connection as far back as 2003!

This type of research is especially important, as the drugs currently available to treat Alzheimer’s offer only small improvements in symptoms and do not stop the progression of the disease, in spite of their high cost.

So, ask your doctor to screen for early signs of diabetes and aggressively treat it when discovered to keep your insulin levels in the normal range. Eat a healthy diet and exercise regularly, as these are also known to lower the risk of developing diabetes. It seems likely that these strategies will lower your chances of developing dementia years down the road.

References: MayoClinic.org; Alzheimers.net