By Marvin Sussman, Ph.D.
The April 2014 issue of the AARP Bulletin featured an article entitled “Am I Losing My Mind? – Conditions That Mimic Dementia” by Margery D. Rosen. The article presents eight forms of dementia which mimic Alzheimer’s Disease (AD) but may be treatable. Dementia is a chronic or persistent disorder of the mental processes caused by a variety of brain diseases or injuries marked by memory disorders, personality changes, and impaired reasoning. The first cause discussed in the article is Normal Pressure Hydrocephalus or NPH. NPH is an accumulation of cerebrospinal fluid that causes the ventricles in the brain to gradually enlarge, sometimes with little or no increase in intracranial pressure. A distinct medical syndrome most commonly seen in older adults, it is accompanied by some or all of a triad (3) of symptoms that include gait disturbances, dementia, and impaired bladder control.
The article recounts the experience of Dr. Milton Newman, a dentist from Peekskill, NY, and an active member of the Hydrocephalus Association. After 33 years of practice, he was forced to retire. For a period of 15 years, Dr. Newman’s “…memory became fuzzy and his ability to do simple things around the house deteriorated to the point that his wife, Phyllis, was afraid to leave him home alone.” His experience is familiar to many with NPH. He went from doctor to doctor seeking answers, and underwent a battery of tests. He was incorrectly diagnosed as having the beginning symptoms of Alzheimer’s Disease. After the Newman’s moved to Arizona, a new doctor made the correct diagnosis – NPH. A permanent shunt was implanted and his symptoms were reversed.
“It was really a miracle,” Mrs. Newman said in the article. “I knew I had him back.”
Misdiagnosis, sometimes after years of searching for an answer, is an all too common occurrence for individuals living with NPH. In a memorial tribute to Dr. Harold O. Conn, a liver specialist of world renown, published in the journal Hepatology (Vol. 55, No. 2, 2012), a similar story is told:
“. . . he had contracted a disease unknown to him (normal pressure hydrocephalus; NPH). . . His NPH was erroneously diagnosed for 10 years as Parkinson’s disease and greatly affected his ability to walk or think clearly until the correct diagnosis was made. A miraculous remission followed brain surgery, and at age 78, he became an expert about, and a spokesperson for, NPH awareness. In the decade that followed, he wrote a dozen meaningful articles about NPH, its prevalence, and heredity and appeared on national radio and TV programs. In addition, he made himself available to advise patients and the families of friends as a good Samaritan about the diagnosis and treatment of NPH.”
A newly released study in Neurology entitled, “The Incidence of Idiopathic Normal Pressure Hydrocephalus (iNPH),” (Jaraj et al., Neurology, 2014) provides estimates on the extent of the problem. They estimate that approximately 2 million persons in Europe and 700,000 persons in the United States have iNPH. Considering the number of shunts implanted annually for all forms of hydrocephalus, the majority of iNPH patients remain untreated either because they are undiagnosed or misdiagnosed as having dementia associated with some other neurological disorder. It has been estimated that as many as 5%-10% of all patients identified as having dementia may actually have NPH. Since symptoms of NPH mimic untreatable dementias and/or may be confused with normal aging, many physicians look no further after diagnosing dementia or after attributing symptoms to “old age.” Left untreated, NPH causes progressive decline in function despite the fact that it is one of the few treatable forms of dementia. When properly identified and treated, adults with NPH can experience significant improvements in their symptoms and quality of life, such as their ability to care for themselves and to work, allowing them to remain in a home environment, a significant cost savings for the family and society. The longer treatment is delayed the less optimistic the prognosis for reversal of symptoms.
The National NPH Awareness and Education Campaign is a three year crusade undertaken by the Hydrocephalus Association to increase the awareness and knowledge about normal pressure hydrocephalus (NPH) – a “treatable dementia” – among patients, providers, researchers, policy makers and the general public. The campaign will focus on NPH, its causes, symptoms, diagnostic and treatment protocols. The ultimate goal is to significantly increase the number of older adults who are accurately diagnosed and successfully treated, and to foster research to eventually find a cure. The campaign will build upon HA’s existing NPH program, which was initiated in 2001, to educate the public through a series of media interviews, patient and provider symposia and national training programs. HA seeks to raise $2.6 million by the end of 2016 to fully fund the first phase of the project. Through the NPH Education and Awareness Campaign, the Hydrocephalus Association will bring the message of NPH, the treatable dementia, to more patients and clinicians with the end result that patients are diagnosed and treated, so that they may be able to have healthier and fuller lives. To support the NPH Education and Awareness Campaign, click here.