Syndrome of Hydrocephalus in Young and Middle-aged Adults

Hydrocephalus that is diagnosed in young and middle-aged adults is different from hydrocephalus diagnosed in infancy and early childhood, or adult-onset normal pressure hydrocephalus (NPH) found in older adults (typically age 60 and older). Doctors are just beginning to identify and describe this distinct form of hydrocephalus.

As yet, there is no universally agreed-upon term to describe this form of hydrocephalus. We have chosen to use the term coined by Dr. Michael Williams: the syndrome of hydrocephalus in young and middle-aged adults (SHYMA). (Other terms used to describe this and similar forms of hydrocephalus are late-onset idiopathic aqueductal stenosis, long-standing overt ventriculomegaly of the adult, and late-onset acqueductal stenosis.)

Symptoms

Symptoms of SHYMA may include headache, subtle gait disturbance, urinary frequency, visual disturbances and some level of impaired cognitive skills that can noticeably affect job performance and personal relationships. Correct diagnosis is oftentimes delayed, as the signs, symptoms and risk factors may not be recognized.

The degree of symptoms and their resultant effect varies widely among patients. If symptoms have been present for years, the patient may be more seriously disabled. Early diagnosis can be a factor in successful resolution of symptoms.

The cause of the hydrocephalus may be congenital (present at birth with few or no symptoms); acquired, from such things as head injury or trauma, meningitis, or a brain tumor; or idiopathic (no known cause). Additionally, some people who were shunted for hydrocephalus as infants but are no longer under the care of a pediatric specialist may exhibit gradual signs of unrecognized shunt failure due to uncompensated hydrocephalus.

Diagnosis

SHYMA is diagnosed using a combination of brain scans, intracranial pressure monitoring and clinical evaluation of symptoms.

Once symptoms of gait disturbance, mild dementia or bladder control have been identified, a physician who suspects hydrocephalus may recommend one or more additional tests. At this point in the diagnostic process, it is important that a neurologist and a neurosurgeon become part of your medical team, along with your primary care physician. Their involvement from the diagnostic stage onward is helpful not only in interpreting test results and selecting likely candidates for shunting but also in discussing the actual surgery and follow-up care, as well as expectations of surgery. The decision to order a given test may depend on the specific clinical situation, as well as the preference and experience of your medical team.

These tests may include computerized tomography (CT), magnetic resonance imaging (MRI), lumbar puncture, continuous lumbar CSF drainage, intracranial pressure (ICP) monitoring, measurement of cerebrospinal fluid outflow resistance or isotopic cisternography. Neuropsychological evaluation may also be recommended.

Treatment

In many cases, prompt treatment can reverse many of the symptoms of hydrocephalus, restoring much cognitive and physical functioning. If left untreated, however, symptoms can become quite disabling, leading to severe cognitive and physical decline.

The most common treatment for SHYMA, as with all forms of hydrocephalus, is shunting.

Hydrocephalus is a chronic condition. However, with early detection, effective treatment and appropriate interventional services, the future for individuals with hydrocephalus is promising.