Sunday 6 May 2012

Hydrocephalus

 Hydrocephalus in Geriatric Population:

Hydrocephalus is a condition in which there is an abnormal increase in cerebrospinal fluid inside the head (Hydro means water; cephalus means head).

Normal Pressure Hydrocephalus
:
Hydrocephalus that occurs in geriatric population with average age being 60 years or above causes Normal Pressure Hydrocephalus (NPH) (www.emedicinehealth.com)

Normal pressure hydrocephalus is a type of hydrocephalus characterized by increased cerebrospinal fluid and a widening of the ventricles of the brain, usually without causing increased pressure inside the head.
The pressure is usually normal because the ventricles widen to make room for the extra fluid. Although normal pressure hydrocephalus usually presents without an increase of pressure inside in the head, increased pressure can happen if the condition goes untreated. The widening of ventricles and the increased fluid presses against certain brain structures, damaging or destroying them (www.medfriendly.com).
Normal pressure hydrocephalus is also known as occult hydrocephalus and Hakim's disease (www.medfriendly.com).
Signs and Symptoms:
There are three main signs of normal pressure hydrocephalus that usually occur gradually rather than suddenly. These signs are difficulty walking, difficulty holding in urine, and difficulty thinking.

The parts of the brain most often affected in Normal Pressure Hyedrocephalus are those that affect the legs, the bladder, and the "cognitive" mental processes such as memory, reasoning, problem solving, thinking and speaking. This decline in mental processes, if it is severe enough to interfere with everyday activities, is known as dementia.

Other symptoms include abnormal gait (difficulty walking), inability to hold urine (urinary incontinence), and, occasionally, inability to control the bowels (www.medfriendly.com).

Difficulty in Walking:
The difficulty walking usually presents as a slowed, shuffling walk, in which the feet appear stuck to the ground. The person may appear unsteady when walking, may have difficulty starting to walk, may have difficulty making turns, and may also complain of weakness in the legs. The difficulty walking may lead to sudden falls without a loss of consciousness. Difficulty walking in normal pressure hydrocephalus seems to be associated with increased tension in the extensor muscles of the legs. The extensor muscles are muscles that extend (the opposite of flex) body parts). It should be noted that some patients with normal pressure hydrocephalus only present with a difficulty walking.

(www.medfriendly.com).
Difficulty in holding urine and Stool/feces:

In addition to difficulty holding in urine, the person may also have difficulty holding in feces. This only occurs in rare circumstances, however. In earlier stages, patients may not lose control of their ability to hold in urine or feces, but they may have increased urges to urinate or have bowel movements, and may actually perform these bodily functions more often.
(www.medfriendly.com).



Difficulty in Thinking and Cognition:


The thinking difficulties in normal pressure hydrocephalus are usually characterized by confusion, poor attention, slowed thinking, and memory difficulties. The thinking difficulties can eventually progress into a dementia. Dementia is a mental disorder characterized by a significant loss of intellectual and cognitive abilities without impairment of perception or consciousness. It is estimated that about 5% of all dementias are due to normal pressure hydrocephalus. If treated early enough, dementia from normal pressure hydrocephalus can be reversible. The dementia will initially present as mild and can progress to severe.
(www.medfriendly.com).

Uncontrollable Movements and Tremors:

Patients with normal pressure hydrocephalus may have rigid muscles and tremors (uncontrollable shaking movements).
(www.medfriendly.com).
Speech:
Patients with normal pressure hydrocephalus may have an abnormally weak voice due to in-coordination of the muscles that control speech. They may not be very spontaneous in terms of movements or conversations, requiring other people to act first before they speak or move. Expressive speech may be impaired in people with normal pressure hydrocephalus. (www.medfriendly.com).
Mood:
A person with normal pressure hydrocephalus may have no observable mood. That is, they do not appear happy or sad, but rather indifferent and withdrawn. Their facial expressions may appear to be very restricted. (www.medfriendly.com).


Causes of Normal Pressure Hydrocephalus:


Many times, the cause of normal pressure hydrocephalus is unknown. However, the condition is often caused by a failure of the arachnoid granulations to absorb enough of the cerebrospinal fluid. As a result, the cerebrospinal fluid level increases, even though it is being produced in normal amounts

In other cases, something is blocking the flow of cerebrospinal fluid, causing normal pressure hydrocephalus. Any condition that causes a blockage of cerebrospinal fluid can lead to normal pressure hydrocephalus. Some causes of blocked cerebrospinal fluid include a closed head injury, brain surgery, brain tumors and cysts, and infections in the brain.

This results in the  widening of ventricles which distorts the central portion of corona radiate. Corona radiate is a fan- shaped structure that connects with the fibers of corpus callosum which functions to communicate between the two sides of the brain.

 Normal pressure hydrocephalus general occurs weeks after brain infections such as meningitis. Meningitis is an inflammation of the meninges (the three outer coverings of the brain and spinal cord). Meningitis can cause scarring to the arachnoid granulations, which help absorb cerebrospinal fluid. If the arachnoid granulations are scarred, they will not absorb the cerebrospinal fluid adequately.
A subarachnoid hemorrhage can also cause normal pressure hydrocephalus. A subarachnoid hemorrhage is when bleeding occurs in the space between two of the layers that cover the brain - the arachnoid mater and the pia mater. In the case of a subarachnoid hemorrhage, the blood is what blocks the flow of cerebrospinal fluid. Generally, this blockage occurs in the outlets of the fourth ventricle or in the cerebral aqueduct. The cerebral aqueduct is a narrow opening in the brain that connects the third ventricle with the fourth ventricle, allowing cerebrospinal fluid to flow between these two areas. It can be viewed in the picture above.

In a study the characteristics of chronic hydrocephalus following aneurysmal subarachnoid hemorrhage (SAH) in elderly patients were clarified. Of the 576 surgically treated patients, 289 were aged 59 years or younger, 169 were 60 to 69, and 118 were 70 years or older. The relationship between chronic hydrocephalus and the causative factors was analyzed for each age group. Of the 576 patients, chronic hydrocephalus was observed in 215 (37%), with the incidence increasing significantly with age (p < 0.001) and being the highest in the oldest age group. In elderly patients, the incidence of chronic hydrocephalus was relatively high, even after mild SAH. The study concluded that incidence of chronic hydrocephalus was high regardless of age in patients with severe SAH, such as in those with H&H grades III–IV, SAH grades III–IV, acute hydrocephalus, symptomatic vasospasm, and intraventricular hemorrhage, and in those with vertebro-basilar artery aneurysms. In the elderly, the incidence of chronic hydrocephalus following SAH was significantly higher than in younger patients, even after mild SAH. In elderly patients, careful observation and individualized treatment are necessary even if SAH is mild. (Yoshioka et. al. 2000)

About 10-20% of patients with a subarachnoid hemorrhage will develop normal pressure hydrocephalus that causes noticeable signs. Normal pressure hydrocephalus may take up to several months to develop after a subarachnoid hemorrhage has occurred. Many head injuries cause subarachnoid hemorrhages (www.medfriendly.com).

Although very rare, it does appear that in some cases normal pressure hydrocephalus can be inherited (passed on through the genes of one's parents). This is still debatable, however. Genes are tiny structures that contain coded instructions for how proteins should be constructed and how certain bodily characteristics should develop. For example, genes control the natural color of people's eyes and hair, and whether they will be male or female. Genes are passed on from parents to their children.

The nerve fibers in the corona radiata send nerve impulses to the legs and the urinary bladder, which is why difficulty walking and difficulty controlling the urge to urinate occurs.

Dementia results from distortion of the limbic system near the ventricles which is responsible for proper memory functioning and for producing emotions (www.medfriendly.com; Skoog et al. 1993).
Subcortical arteriosclerotic encephalopathy (SAE) may cause enlargement of ventricles thereby leading to hydrocephalus (Gotto , Ishii, Fukasawa 1981)
Diagnoses:

Unfortunately, normal pressure hydrocephalus is difficult to diagnose.
Computerized Tomography:
CT (computerized tomography) scanning is an advanced imaging technique that uses x-rays and computer technology to produce more clear and detailed pictures than a traditional x-ray for seeing the pictures inside the brain to diagnose hydrocephalus.

Magnetic Resonance Imaging:

MRI (Magnetic Resonance Imaging) scans produce extremely detailed pictures of the inside of the body by using very powerful magnets and computer technology. It is also used for the diagnoses of hydrocephalus.

Cisternogram:
A test known as a cisternogram may show changes of the flow of cerebrospinal fluid in the brain. Specifically, the cisternogram scan may show that the fluid flowed back into the ventricles (openings in the brain that produce cerebrospinal fluid). A cisternogram is a test in which the flow of cerebrospinal fluid can be monitored. This is done by injecting a substance into the cerebrospinal fluid by inserting a needle in the spinal canal. The substance flows throughout the cerebrospinal fluid and emits a signal. A scanner detects the signal and provides information about the flow of cerebrospinal fluid. It should be noted that although cisternograms were used in the past, they are usually not used now days

Although the results of CT scans, MRIs, and cisternograms may be consistent with normal pressure hydrocephalus, they may also be consistent with other conditions. Thus, these tests are not sufficient for a diagnosis of normal pressure hydrocephalus. CT and MRI scans will usually show widening of the ventricles.

Prevalence:
•    A Norwegian study of a population of 220,000 inhabitants found a prevalence of probable idiopathic NPH of 21.9 per 100,000 population and an incidence of 5.5 per 100,000 population; the investigators suggested that those numbers be regarded as minimum estimates (Brean and Eide 2008).
•    A Japanese study found radiological and clinical features consistent with idiopathic NPH in 2.9% of community-dwelling elderly subjects (Hiraoka, Meguro , Mori , 2008).
•    In another Japanese study, elderly individuals (age >65 y) underwent MRI and the prevalence of NPH was 1.4% (Tanaka et. al. 2009)
•    The prevalence of NPH may be as high as 14% in extended care facility patients (Marmarou ,Young, Aygok, 2007).


Treatment:
If a cause for normal hydrocephalus can be identified, the condition can often be treated and the effects can often be reversed. The most common type of treatment is called a shunt. A shunt is a flexible tube that is inserted into the ventricle, which drains the extra cerebrospinal fluid.
The most commonly used shunt (Dalvi and Prem Kumar, 2010) is called a ventriculperitoneal (VP) shunt because it drains cerebrospinal fluid from the ventricles to the peritoneum. The peritoneum is a layer of tissue that lines the belly and covers most of the organs in the belly. Another type of shunt is a ventriculoatrial shunt, so named because it drains cerebrospinal fluid from the ventricles to the atrium of the heart. Each chamber on the top of the heart is called an atrium
A shunt may be placed even if the physician is not certain that the cause of the patient's problems is normal pressure hydrocephalus. The decision on whether to place a shunt will be based on the results of a clinical evaluation by the doctor and the results of a CT scan. If the condition improves after placement of a shunt, health providers are generally confident that the cause of the patient's problems was normal pressure hydrocephalus. It is worth noting that shunting can help decrease the size of the ventricles in 3 to 4 days even if the hydrocephalus has been present for a year, and sometimes more.
Temporary relief of symptoms may be achieved with a spinal tap or treatment with Acetazolamide (Diamox). Acetazolamide is a diuretic medication that can decrease the production of CSF. A diuretic is a medication that helps to release fluids from the body in the form of urine. There is no definitive evidence yet, however, that medication can effectively treat normal pressure hydrocephalus (www.medfriendly.com).

A study retrospectively reviewed the clinical records of 27 patients older than 70 years who underwent endovascular treatment for aneurysmal SAH between January 1996 and July 2002. Thirteen patients with sub-arachnoid Hemorrhage (SAH) and a poor Hunt and Hess grade at initial presentation had been treated by endovascular means. Their outcomes were assessed by the using the Glasgow Outcome Scale (GOS). Two patients (15%) had a good outcome according to the GOS. Three patients (23%) were moderately disabled, two (15%) were severely disabled at the time of discharge from the hospital, and six (47%) died. Five patients (38%) developed clinical vasospasm and underwent balloon angioplasty. Three procedure-related deaths occurred (23%). Endovascular treatment has modified the management of poor-grade SAH in elderly patients, most of whom are high-risk surgical candidates. Endovascular treatment can be administered early after the initial ictus, reducing the risk of rebleeding and providing an option to pursue aggressive triple-H therapy. Symptomatic vasospasm can also be treated by endovascular means in the initial setting (Jain et al, 2004).

References:
1. http://www.medfriendly.com/normalpressurehydrocephalus.html
2. http://www.emedicinehealth.com/normal_pressure_hydrocephalus/article_em.htm
3. Yoshioka H, Inagawa T, Tokuda Y, Inokuchi F .Chronic hydrocephalus in elderly patients following subarachnoid hemorrhage. Surgical Neurosurgery 2000; 53 (2):119-125.
4. Goto K, Ishii N, Fukasawa H . Diffuse white-matter disease in the geriatric population. A clinical, neuropathological, and CT study. Radiology: Radiology 1981; 141: 687-695.
5. Skoog I, Nilsson L, Palmertz B, Andreasson L-A, Svanborg A. A Population-Based Study of Dementia in 85-Year-Olds. New England Journal Medicine 1993; 328:153-158.
6. Brean A, Eide PK. Prevalence of probable idiopathic normal pressure hydrocephalus in a Norwegian population. Acta Neurol Scand.  2008;118(1):48-53. [Medline].
7. Hiraoka K, Meguro K, Mori E. Prevalence of idiopathic normal-pressure hydrocephalus in the elderly population of a Japanese rural community. Neurol Med Chir (Tokyo).  2008;48(5):197-99; discussion 199-200. [Medline]
8. Tanaka N, Yamaguchi S, Ishikawa H, Ishii H, Meguro K. Prevalence of possible idiopathic normal-pressure hydrocephalus in Japan: the Osaki-Tajiri project. Neuroepidemiology. 2009;32(3):171-5. [Medline].
9. Marmarou A, Young HF, Aygok GA. Estimated incidence of normal pressure hydrocephalus and shunt outcome in patients residing in assisted-living and extended-care facilities. Neurosurg Focus.  2007;22(4):E1. [Medline]
10. Dalvi AI, Prem Kumar A.P. Normal Pressure Hydrocephalus: Treatment & Medication.e medicine 2010.http://emedicine.medscape.com/article/1150924-overview.
11. Rajan Jain, John Deveikis and Byron Gregory Thompson. Endovascular Management of Poor-Grade Aneurysmal Subarachnoid Hemorrhage in the Geriatric Population. American Journal of Neuroradiology 2004 ; 25:596-600.

- Dr Smita Pandey