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.
"Reprinted with permission of the Hydrocephalus Association. All rights
reserved."
