Adult Hydrocephalus and the Development of a Research Network

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Adult HydrocephalusHydrocephalus affects a wide range of people, from infants and children to young, middle-aged and older adults as well as those children transitioning to adulthood. The symptoms and challenges faced by adults are vastly different from hydrocephalus diagnosed in infancy and early childhood.  When hydrocephalus is diagnosed in adults aged 60 and older, it is typically referred to as Normal Pressure Hydrocephalus or NPH. When it’s diagnosed in young and middle aged adults it is called Syndrome of Hydrocephalus in Young and Middle Aged adults or SHYMA.

Normal Pressure Hydrocephalus is a chronic neurological disorder which often goes unrecognized or is misdiagnosed as Alzheimer’s disease, Parkinson’s disease or other neurodegenerative disorders. NPH is an abnormal accumulation of cerebrospinal fluid (CSF) that causes the ventricles in the brain to become enlarged, sometimes with little or no increase in intracranial pressure (ICP). Generally, there are three characteristic symptoms which help define NPH. These three symptoms are gait disturbances, mild dementia and impaired bladder control.  If left untreated, NPH can lead to severe dementia and permanent brain damage, but if treated at an early stage the progression can be stopped and symptoms can improve.  According the National Institutes of Health, about 375,000 older Americans have NPH and it is one of the few causes of dementia that can be controlled or reversed with treatment.

Hydrocephalus in young and middle aged adults is often referred to as SHYMA (Syndrome of Hydrocephalus in Young and Middle-Aged Adults); however other terms are also used to describe the condition. Some of the terms used are: late-onset idiopathic aqueductal stenosis, long-standing over ventriculomegaly of the adult (LOVA), and late-onset aqueductal stenosis.

SHYMA is similar in some ways to NPH but the symptoms are often much more subtle.  The most common symptoms of SHYMA are chronic headaches, gait disturbances, cognitive complaints and bladder control.  These symptoms can be debilitating and disruptive, having a profound effect on individuals’ lives, rendering them unable to work or to have difficulty functioning in day-to-day life.

Both NPH and SHYMA are treated surgically with an implantation of a shunt.  In some cases individuals with SHYMA may be good candidates for an endoscopic third ventriculostomy (ETV), an alternative procedure to shunting which involves making a hole in the floor of the third ventricle to allow free flow of spinal fluid.   Although these procedures save lives and provide many with better quality of life for years, they can become non-functioning at any time; which means the return of symptoms prior to treatment, and even the need for further surgical interventions. It is critical to further our understanding of hydrocephalus not only in young, middle aged and older adults but also for the many individuals who have been diagnosed in childhood and now have transitioned to adulthood.

Research for improving outcomes

It is important to note that clinical research in adult hydrocephalus is difficult to accomplish because, although centers of expertise individually attract many patients compared to general hospitals, they rarely have sufficient numbers of patients to accomplish important clinical research rapidly. Slowed or delayed recruitment ultimately results in delayed research results, which prolong the time to take an idea from concept, to research, to best practices.

The lack of sufficient number of patients in single centers and the frustration of parents of pediatric hydrocephalus patients was the inspiration for the founding of the Hydrocephalus Clinical Research Network (HCRN).  HCRN, which in 2012 has partnered with the Hydrocephalus Association, is a network of nine children hospitals that conduct innovative clinical research on pediatric hydrocephalus.  Since its conception, HCRN has published numerous scientific articles that are providing the scientific-based evidence needed to improve clinical outcomes in pediatric hydrocephalus.

The knowledge of the need for improved clinical research in adult hydrocephalus and seeing the success of HCRN provided the catalyst for the establishment of an adult network similar to HCRN.  In 2012, the Hydrocephalus Association began working with distinguished adult hydrocephalus researchers, including neurosurgeons, neurologists and neuropsychologists, to establish such a network.  This group wants to improve the lives’ of adults with hydrocephalus, raise awareness of the condition and cover issues related to transitional patients, congenital adults and other adult-onset cases. Five sites were selected and progress was made throughout 2012 and 2013 developing essential data elements and protocols for the network. The five participating sites and researchers in the network include:

Mark Hamilton, M.D., of the University of Calgary

Heather Katzen, Ph.D, of The University of Miami

Petra Klinge, M.D., of Brown University

Mark Luciano, M.D., Ph.D., of  Cleveland Clinic

Norman Relkin, M.D., Ph.D., of Weill Cornell Medical College

Michael Williams, M.D., of The Sandra and Malcolm Berman Brain & Spine Institute

Recently, Dr. John Kestle, co-founder of HCRN and advisor to this group, joined the group effort to study adult hydrocephalus, bringing the benefits of collaboration and his rich history in hydrocephalus research.

In February 2013, the adult group participated in an in-person meeting at the HA offices in Bethesda, MD, to discuss the data elements and to engage the data coordinating center (DCC) from the University of Utah. The researchers and the DCC will again come together this Friday and Saturday (November 1st and 2nd) to further work on finalizing data forms and protocols in the goal of starting the network in 2014.

Creation of an adult network is exciting because research is essential in improving the lives of patients.  Hydrocephalus is not just a pediatric condition.  It can strike at any age, and those children with hydrocephalus will be adults one day.  It is important to address the whole age spectrum of hydrocephalus and, for this reason, HA believes that the development of an adult research network is essential.

For more information, the Hydrocephalus Association provides a number of educational materials that can be found on our website.

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1 Comments for : Adult Hydrocephalus and the Development of a Research Network
    • Marguerite DeLeon
    • November 10, 2016

    I was diagnosed with NPH bt Christopher Iannotti in Arizona. CT was done at kaiser atlanta n Nph was noted on it but no diagnosis, treatment was mentioned this was n 2013 n I first saw Dr Iannotti in August 2016. He is my angel. Never made me feel like a nonresponder. Lumbar drain end of Sept and shunt Oct 27. He gave me my independence back and a reason for living! Hes my angel and his surgical team are poetry in motion. I will be eternally grateful to him and his surgical team where he is captain of his ship!!!

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