Normal Pressure Hydrocephalus: From Diagnosis to Treatment
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Normal Pressure Hydrocephalus - Adam S. Mednick
illustrations.
Introduction
In late 2004, I was asked to consult on a case involving an elderly man who, over the past five years, had developed gradual onset of unsteadiness, incontinence, and, more recently, dementia. Being familiar with this set of symptoms, I was able to diagnose the man with normal pressure hydrocephalus (NPH).
Fortuitously, this gentleman knew Harold O. Conn, M.D.—they belonged to the same health club. Dr. Conn was a world-renowned liver specialist who was a professor of gastroenterology for over fifty years at the Yale School of Medicine. Dr. Conn contacted me to inquire how I was able to diagnose his friend so readily; it was his experience that many physicians did not understand NPH.
Dr. Conn himself had been diagnosed with NPH ten years earlier. He had received an initial diagnosis of Parkinson’s disease, but his symptoms did not improve with treatment. Finally, at age seventy-eight, he was given the correct diagnosis by a neurologist familiar with NPH. Dr. Conn underwent surgery in 2002 to correct his hydrocephalus. Following the surgery, he experienced dramatic improvements in his walking and in his clarity of thought. It was then that he vowed to help educate both the public and medical professionals about NPH. He wrote articles on the topic and appeared on various radio and television shows.
I first met Dr. Conn in 2004. Since then, he and I have had many hours of conversations about how to better educate the public about NPH. It was through these conversations that the idea for this book was born. Dr. Conn died in October 2011 at age 85. This book is a testament to his dedication as an educator and physician. I am pleased to say he was also a friend.
It is my hope that this book will help you understand NPH—this little-known, but treatable, brain disorder. If you believe that you or a loved one may have NPH, it is my hope that you get the proper diagnosis in a timely manner. To paraphrase Abraham Lincoln, may you add life to your years.
1
Normal Pressure Hydrocephalus: An Overview
If you’re like most people, you’ve probably never heard of the brain disorder called normal pressure hydrocephalus (NPH). This disease is more common than most people realize, however. It is rarely mentioned by the media, and few, if any, famous people have brought attention to NPH like they have with other diseases such as Parkinson’s disease and breast cancer. Consequently, NPH is little known, and, because its symptoms mimic those of other diseases, it is also often misdiagnosed and can be mistaken for Parkinson’s disease, Alzheimer’s disease, or other forms of dementia. Because the disease usually affects older individuals, symptoms are sometimes associated with just getting older.
Perhaps you’ve become interested in NPH because you or a loved one has been diagnosed with it. If this is the case, you may find some relief in learning about the disease—its symptoms, causes, and treatment. In many cases, the symptoms can be reversed with surgery.
What Is Normal Pressure Hydrocephalus?
NPH is a slowly developing condition in which too much cerebrospinal fluid builds up in the brain. This clear liquid circulates throughout the brain and the spine. The term hydrocephalus
is derived from the Greek word hydro,
which means water,
and the word cephalus,
which means head.
The term literally translates to water head.
In common terms it is sometimes referred to as water on the brain.
NPH is considered a neurodegenerative disorder, which means that the symptoms affecting the brain gradually worsen, usually over the course of many months to years.
Anatomy of the Brain
How NPH Develops
To better understand how NPH develops, let’s first take a brief look at the processes that occur in the brain that can lead to NPH. The human brain is bathed in a clear fluid called cerebrospinal fluid (CSF). This fluid provides nutrients to the brain and helps to cushion the brain against movement inside the skull. Cerebrospinal fluid circulates through four specialized chambers within the brain called ventricles. A group of specialized cells within the lining of these ventricles produces about one pint of this fluid per day.
The four ventricles of the brain are a network of cavities that carry cerebrospinal fluid. The illustration on the right indicates NPH—the ventricles are enlarged due to the build-up of cerebrospinal fluid.
After traveling through the brain, the cerebrospinal fluid circulates down through the center of the spinal cord and circulates back up toward the brain where it is reabsorbed back into the bloodstream. If the fluid is not reabsorbed properly, however, it slowly builds up within all of the ventricles, causing them to enlarge. This enlargement distorts the surrounding brain tissue and can cause permanent brain damage. It is this lack of reabsorption of fluid that causes normal pressure hydrocephalus.
The dark areas toward the center of the MRI scan show the ventricles. Notice the ventricles in the NPH brain are enlarged.
It may seem confusing that the word normal
is part of the name of a serious brain disorder. What does normal
mean in this context? In this case, normal
refers to the level of pressure the cerebrospinal fluid is putting on the brain. If doctors were to perform medical tests on a person with NPH to measure the pressure of the cerebrospinal fluid inside the brain, the pressure would appear normal or only slightly elevated. This is because NPH develops so slowly, over a period of months to years, that the brain is able to gradually increase its capacity to store the fluid within the ventricles. As a result, the pressure appears normal. The building pressure on the brain, however, eventually leads to the development of symptoms.
Types of Hydrocephalus
Congenital hydrocephalus is present at birth and may be caused by environmental influences during fetal development or by genetic factors.
Primary NPH can occur at any age, but is more common in the elderly. If the NPH is primary, it means the cause is unknown. The symptoms of primary NPH usually develop slowly over the course of months to years.
Secondary NPH means the disease resulted from previous injury such as hemorrhage, head trauma, brain infection, tumor, or complications of radiation therapy treatment for brain tumors. Symptoms in secondary NPH may develop quickly.
Hydrocephalus ex vacuo sometimes occurs in older people, who have had considerable shrinkage of their brains as part of the aging process. As