Normal pressure hydrocephalus in adults is associated with a variety of symptoms, which can mean its diagnosis is often delayed. A delayed diagnosis can affect the progression of the disease, and consequently, the patient’s quality of life. This is why it so important to be aware of the primary symptoms of normal pressure hydrocephalus.
Normal pressure hydrocephalus is a type of hydrocephalus in which there is an increase in cerebrospinal fluid, but intracranial pressure remains normal.
It mainly affects older adults, 60 years of age and older, and is becoming more prevalent due to the increase in life expectancy of the general population.
In fact, the highest number of cases is recorded in the 70 to 80 year-old age group, although it is possible the disease is under-diagnosed in people over 80.
Normal pressure hydrocephalus is the most common cause of reversible dementia, making it very important to be alert to the possible symptoms.
It is also important to know that, with the right treatment, significant improvement is possible within a few months.
Symptoms of Normal Pressure Hydrocephalus
The symptoms of normal pressure hydrocephalus can vary from person to person. However, the following are the key symptoms to watch for:
Abnormal gait
Difficulty walking, or a change in the manner of walking, is one of the most characteristic symptoms of normal pressure hydrocephalus.
Usually, the patient finds it hard to begin walking or to turn (gait apraxia). In addition, the patient’s gait tends to be slow and unsteady, and they may feel as though their feet are glued to the ground (magnetic gait).
The patient may also suffer a loss of balance or general instability, and a tendency to fall.
Cognitive impairment
The most common neurological or cognitive symptoms of normal pressure hydrocephalus are difficulty speaking, slowed thought processes, and memory loss (amnesia).
Dementia can also appear, although it sometimes appears in a late stage of the disease.
Urinary incontinence
Difficulty controlling urination is common in adult patients with normal pressure hydrocephalus. Although less frequent, in some cases there may also be difficulty controlling bowel movements (fecal incontinence).
Psychiatric disorders
Normal pressure hydrocephalus is frequently associated with psychiatric disorders, mainly of an affective and psychotic nature.
For example, it is sometimes associated with the onset of delirium or delusions.
Mood disorders
Fluid buildup in the brain can also leave the patient feeling extra tired, unmotivated, apathetic, and feeling sleepy during the day.
The Hakim-Adams triad, or Hakim triad, is a concept used in the diagnosis of normal pressure hydrocephalus. This refers to the presence of three key symptoms: altered gait, dementia, and urinary incontinence.
However, hydrocephalus in adults does not always exhibit these three clinical symptoms.
And it should be pointed out, that these symptoms can be caused by other diseases besides hydrocephalus.
In some cases, hydrocephalus may even be asymptomatic. That is, in the absence of drastic changes, the symptoms may go unnoticed for years, until it ends up causing a serious disability.
Diagnosis of Normal Pressure Hydrocephalus
Diagnosing hydrocephalus in adults can be a serious challenge.
First, because hydrocephalus can be caused by different things. And, second, because of the wide range of symptoms that associated with it.
It is common for the symptoms of normal pressure hydrocephalus to be mistaken for other diseases.
Among these are age-related memory loss, mild cognitive impairment, Alzheimer's disease, Parkinson's disease, and cervical myelopathy. Also, hypothyroidism and depression.
All of these conditions are much more common than hydrocephalus. In the case of hydrocephalus, it is estimated that there are only 0.2-5.5 new cases per 100,000 people per year, so it is considered a rare disease.
Because it is so rare, when symptoms such as cognitive impairment, psychological alterations, gait disorders, and/or urinary incontinence appear, it is first necessary to rule out other, more common, conditions that could be the cause.
The fact that these symptoms of normal pressure hydrocephalus might be associated with age or other conditions can be one of the main reasons for delay in getting the right diagnosis and treatment, because people let some time pass before consulting a specialist.
This is unfortunate, because early diagnosis is essential. A delay in either diagnosis or surgical treatment has been associated with subsequent progressive deterioration.
There is no single, specific test to diagnose hydrocephalus. For this reason, at Instituto Clavel, we combine neuroimaging techniques, neuropsychological tests, and the measurement of intracranial pressure by lumbar or intracerebral puncture.
Treatment of Adult Normal Pressure Hydrocephalus
The most common treatment for normal pressure hydrocephalus is a ventriculoperitoneal shunt accompanied by rehabilitation and neurological physiotherapy.
Surgical treatment
Ventriculoperitoneal shunt is a type of surgery to drain excess cerebrospinal fluid (CSF) from the central nervous system.
This procedure involves placing a catheter in the brain cavities called ventricles. The shunt is connected in series to a system with a valve to regulate the flow, which allows the excess fluid to drain off.
This surgery improves symptoms in up to 40%-70% of cases, with best results obtained in cases manifesting only minor symptoms.
The patient’s ability to walk normally is the symptom that is most likely to improve after this surgery.
Neurological Physical Therapy
Neurological physical therapy takes advantage of the neuroplasticity of the brain to stimulate and/or recover lost functions.
The fundamental objective of this treatment is to help the patient walk more normally and to gain sphincter control, but it also serves to the stimulate memory and other cognitive functions.
With this treatment, adults with hydrocephalus, and their family members, usually see significant improvement in walking over a period of 1 to 3 months.
The improvement of sphincter control and cognitive impairment will depend to a large extent on the pre-existing neurological condition of the patient.
Hydrocephalus in adults and life expectancy
Life expectancy with this type of chronic adult hydrocephalus depends on many factors.
Among them, whether the patient also suffers other neurological diseases, and how far the hydrocephalus has progressed by the time it is diagnosed and treated.
Ventriculoperitoneal (VP) shunt surgery for hydrocephalus can lead to improvement, to a greater or lesser degree, over a period of approximately three months. Before surgery, various tests are used to determine if such improvement is likely. Among them, the tests known as a cerebrospinal fluid tap test (TT), or an LP drain, which can show if draining the cerebrospinal fluid yields any clinical improvement.
However, this initial benefit may diminish over time. That is why it is so important to be able to accompany the surgery with rehabilitation and physical therapy.
In conclusion, hydrocephalus itself does not have to reduce life expectancy. In addition, it should be remembered that there are treatments that can help reduce symptoms and improve the patient’s health and well-being.
Instituto Clavel, specialists in adult normal pressure hydrocephalus
The team of neurosurgeons at Instituto Clavel are experts in the diagnosis and treatment of normal pressure hydrocephalus in adults.
We also have a team of physical therapists specialized in helping patients with recovery and rehabilitation after ventriculoperitoneal shunt surgery.
It is important to insist that you should always consult a neurosurgeon if you have the symptoms associated with normal pressure hydrocephalus described in this article, and other more frequent conditions with similar symptoms have been ruled out.
Whether the symptoms are due to hydrocephalus or any other neurological disease, early diagnosis and treatment are the keys to controlling the progression of the disease and improving the patient's quality of life.
Please do not hesitate to contact us if you have any questions or would like to make an appointment with one of our specialist doctors.
Dr. Matías Mondragón
Neurosurgeon
Specialized in spinal surgery
Categories: Brain treatments, Brain pathologies