Normal Pressure Hydrocephalus

Synonyms: chronic hydrocephalus, extraventricular obstructive hydrocephalus Normal pressure hydrocephalus (NPH) describes the condition of ventricular dilatation in the absence of raised CSF pressure on lumbar puncture, characterised by a triad of gait abnormality, urinary (usually) incontinence and dementia. The importance of this diagnosis lies in the fact that it is a potentially reversible cause of dementia, accounting for up to 6% of dementias.[1] It was first described by Hakim and Adams in 1965.[2] The three patients identified in the original series had dilation of the cerebral ventricles without an increase in CSF pressure on lumbar puncture. Further research has revealed that intracranial pressure (ICP) does indeed rise in the initial phase and that subsequent ventricular enlargement results in the ICP returning to normal. CSF pressure, as measured during an isolated lumbar puncture procedure, is a poor assessment of CSF dynamics in these patients, many of whom do indeed exhibit a rise in pressure, albeit on an intermittent basis.[3] Current studies suggest that CSF pressure is controlled by alterations in spinal compliance or decrease in CSF absorption at the sagittal sinus. Four subtypes of NPH have thus been identified depending on whether one or other or both of these mechanisms are operating. This may have important implications in terms of management.[4]

Aetiology
The cause of normal pressure hydrocephalus (NPH) is not always obvious. In 50% of cases, there is no known preceding cause (idiopathic NPH). In the remainder, it may be secondary to:

Subarachnoid haemorrhage. Meningitis. Head injury. Central nervous system (CNS) tumour.

All of these conditions can cause hydrocephalus but the pathological process that leads to NPH is not clear. These patients must be differentiated from patients whose ventricular enlargement is a result of shrinkage of surrounding tissue, as in Alzheimer's disease for example. See separate article Hydrocephalus for more detail.

Epidemiology
The condition occurs mainly in elderly patients. Prevalence ranges from 3.3 per 100,000 for people 50 to 59 years of age, to 49.3 per 100,000 for people 60 to 69 years of age, to 181.7 per 100,000 for people 70 to 79 years of age.[5]

Some researchers maintain that similar CSF hydrodynamic changes are seen in some hydrocephalic children.[3][6] There is no predominance of gender.

Presentation
Symptoms

The (gradually progressive) classic triad of symptoms is:[5]

Gait disturbance - this is due to distortion of the corona radiata by the dilated ventricles. This area contains the sacral motor fibres than innervate the legs. Movements are slow, broad-based and shuffling. The clinical impression is thus one of Parkinson's disease, except that rigidity and tremor are less marked and there is no response to carbidopa/levodopa. Freezing episodes can also occur. True ataxia and weakness is absent and the gait disturbance is referred to as gait apraxia. Sphincter disturbance - this is also due to involvement of the sacral nerve supply. Urinary incontinence is predominant although bowel incontinence can also occur. Dementia - this is due to distortion of the periventricular limbic system. The prominent features are memory loss, inattention, inertia and bradyphrenia (slowness of thought). The dementia progresses less rapidly than that seen with Alzheimer's disease.

Signs

Pyramidal tract signs may be present. Reflexes may be brisk. Papilloedema is absent (but there has been found to be an association with glaucoma, so glaucomatous optic disc changes may be noticed).[7]

Investigations[6]

Neuroimaging - MRI or CT scanning may show ventricular enlargement out of proportion to sulcal atrophy and periventricular lucency. Isotope cisternography may also be useful to demonstrate CSF dynamics, particularly when attempting to predict which patients will benefit from surgery.[8] Large-volume lumbar puncture (spinal or CSF tap test)[9] - CSF pressure will be normal, or intermittently raised. Furthermore, the effect of the lumbar puncture is assessed looking at improvement of the patient's symptoms which can last for a period of days to weeks. The value of this test is limited in diagnosing normal pressure hydrocephalus (NPH) but may be useful in narrowing the differential diagnosis. If it is positive and symptoms do improve, it can be used as a predictor of positive operative outcome (see below). Intraventricular monitoring - in NPH, this may show a particular pattern characterised by beta waves - see 'Management', below.[10] Lumbar infusion test (intrathecal infusion test)[9] - the CSF absorptive capacity is tested with a fluid challenge. An abnormal, sustained rise in CSF suggests NPH.

Although the invasive tests carry theoretical risks (infection, post-procedure headache, bleeding, localised pain and nerve root damage), evidence suggests that these are actually very safe procedures.[9] In these patients, it is also useful to check the serum sodium, as hyponatraemia has been reported.[11]

Diagnosis
This is based on the clinical triad described above, in the absence of papilloedema, backed by neuroimaging (specific criteria are defined) without evidence of raised intracranial pressure (ICP) and with evidence of symptom improvement on lumbar puncture.[9] Accurate diagnosis is the key to treatment success.[1]

Differential diagnosis[6]

Alzheimer's disease. Aphasia. Apraxia and related syndromes. Confusional states and acute memory disorders. Cortical basal ganglionic degeneration. Dementia with Lewy bodies. Dementia in motor neurone disease. EEG in dementia and encephalopathy. Frontal lobe syndromes. Frontal and temporal lobe dementia. Hydrocephalus.

Management
The identification and treatment of normal pressure hydrocephalus (NPH) is worthwhile, providing patients are carefully selected, as it remains one of the truly reversible causes of dementia.
Medical

Medical treatment of NPH includes acetazolamide and repeated lumbar puncture. These methods are rarely successful long-term and are usually used as temporary deferment measures or in patients who are too ill for surgery.
Surgical[3]

The mainstay of treatment is surgical insertion of a CSF shunt.[5][9] This could be to the peritoneum, the right atrium or, more recently, via external lumbar drainage. Selection of patients for surgery is important, as exposing patients to shunt-related complications such as mechanical failure or infection is unwarranted, unless a good clinical outcome is expected. Various parameters are used to predict which patients will benefit from surgery, but there is insufficient evidence for their efficacy. Features suggesting a good outcome include:

Presence of a clearly identified aetiology. Predominant gait difficulties with mild cognitive impairment. Normal-sized or occluded Sylvian fissures and cortical sulci on CT or MRI. Absent or moderate white matter lesions on MRI.

Insertion of a ventriculoperitoneal shunt is the first-line procedure, with ventriculo-atrial shunting being used as an alternative.

Prognosis
Prognosis is variable, even after shunt surgery. The combined rate of permanent neurological deficit or death has been reported as 6%, with the need for additional surgery occurring in 22%.[1][5] Over a period of 10 years, shunt revision was required in approximately 1 in 2-3 cases. There is good long-term survival of 39% after 5 years. Complications of shunt surgery are found in up to 10% and include:

Shunt occlusion Catheter breakage CSF hypotensive headaches Cerebral infarct Haemorrhage Infection Seizures

Lumbar puncture (spinal tap): This procedure involves removal of CSF from the area around the spinal cord in the lower back.

The CSF pressure is measured, and the fluid that is removed is analyzed for abnormalities that might give a clue as to the problem. Usually, more fluid is removed than is necessary for these tests. The idea behind this is that removal of a large volume of CSF helps relieve symptoms (this is checked by comparing exam results before and after). This effect usually is only temporary. However, improvement of symptoms with lumbar puncture is commonly interpreted as meaning that a surgical shunt would be helpful in that person.

What Is the Treatment for Normal Pressure Hydrocephalus?

Normal pressure hydrocephalus generally cannot be cured. It is a long-term condition. However, many people with the condition obtain substantial relief through surgical treatment. For those who are not candidates for surgery, treatment consists of measures to relieve mood and behavioral problems, cope with physical problems such as incontinence and walking difficulties, and maximize physical, mental, and social functioning. It's important to know that no drug or other medical treatment is known to work in NPH.
Surgery for Normal Pressure Hydrocephalus

Normal pressure hydrocephalus is not caused by any structural abnormality, such as a brain tumor. In most cases, the underlying problem is not known or cannot be treated. The treatment in these cases is a shunt operation.

Surgical treatment method

If drainage of CSF by a large volume spinal tap or insertion of a lumbar catheter improves a patients symptoms, a diagnosis of hydrocephalus is confirmed, and your doctor will suggest long-term solutions. Your neurosurgeon will propose placing a shunt to drain the excess CSF from the brain into another part of the body. These shunts have been specially developed by our doctors, who hold patents on these specialized valves. They are titrated and adjustable, so the exact amount of CSF needed to be drained can be personalized for each patient. The placement of the shunt will be recommended by the neurosurgeon based on your particular case. The fluid may be redirected by the following methods:

Ventricular shunt- the fluid will be drained into the peritoneal cavity or belly Lumbar shunt- the fluid will be drained into the peritoneal cavity or belly Right Atrium shunt- the fluid will be drained into your heart

The goal of the shunt is to continuously drain the correct amount of CSF so the patient has relief from all hydrocephalus symptoms.

What Are the Types of Hydrocephalus?

There are several different types of hydrocephalus. These types include

Congenital hydrocephalus Acquired hydrocephalus Communicating hydrocephalus Non-communicating hydrocephalus Hydrocephalus ex-vacuo Normal pressure hydrocephalus.

Congenital Versus Acquired Hydrocephalus

Congenital hydrocephalus is present at birth and may be caused by environmental influences during fetal development or by genetic factors. Acquired hydrocephalus develops at the time of birth or at some point afterward. This type of hydrocephalus can affect individuals of all ages and may be caused by injury or disease.

Communicating Versus Non-Communicating Hydrocephalus

Communicating hydrocephalus occurs when the flow of cerebrospinal fluid (CSF) is blocked after it exits the ventricles. This form is called communicating, because the CSF can still flow between the ventricles, which remain open.

Non-communicating hydrocephalus -- also called "obstructive" hydrocephalus -- occurs when the flow of CSF is blocked along one or more of the narrow pathways connecting the ventricles. One of the most common causes of hydrocephalus is "aqueductal stenosis." In this case, hydrocephalus results from a narrowing of the aqueduct of Sylvius, a small passageway between the third and fourth ventricles in the middle of the brain.

Hydrocephalus Ex-Vacuo and Normal Pressure Hydrocephalus

There are two other forms of hydrocephalus that do not fit into the categories mentioned previously and that primarily affect adults: hydrocephalus ex-vacuo and normal pressure hydrocephalus. Ex-vacuo hydrocephalus (or hydrocephalus ex-vacuo) occurs when there is damage to the brain caused by stroke or traumatic brain injury (TBI). In these cases, there may be actual shrinkage (atrophy, or wasting) of brain tissue. Normal pressure hydrocephalus can occur in people of any age, but it is most common in the elderly population. Normal pressure hydrocephalus may result from:

A subarachnoid hemorrhage Head trauma Infection Tumor Complications of surgery.

However, many people develop normal pressure hydrocephalus even when none of these factors are present. In these cases, the cause of the disorder is unknown.

Some of the causes in children for Macrocephaly (macrocephaly is diagnosed when the circumference of the head is more than two standard deviations above average for the child's age, sex, race, and period of gestation) in children may include:

Cerebral gigantism Hydranencephaly Megalencephaly - Megalencephaly: Also called macrencephaly, a condition in which there
is an abnormally large, heavy, and usually malfunctioning brain. By definition, the brain weight is greater than average for the age and gender of the child. Head enlargement may be evident at birth or the head may become abnormally large in the early years of life.

Cerebral cyst Subdural hematoma Cranioskeletal dysplasia Achondroplasia Sotos syndrome Increased brain size: Neurofibromatosis typeI Increased skull thickness due to chronic severe anemia Osteogenosis imperfecta Communicating hydrocephalus: Post-hemorrhage Postinfectious Non-communicating hydrocephalus: Arnold-Chiari malformation Aqueductal stenosis Mass lesion Ependymoma Astrocytoma Abscess

Intraventricular hemorrhage Subarachnoid hemorrhage

CRANIAL ENLARGEMENT?? DD??

1. Hydrocephalus
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Hydrocephalus is a condition characterized by excessive fluid in the brain. It is associated with cranial enlargement only when it affects children, whose skulls are still malleable enough to expand from the fluid's pressure. Adults, whose skulls are fully fused and much harder, experience intense headaches and cognition problems. Hydrocephalus is most often treated surgically, according to the National Institute of Neurological Disorders and Stroke.

Paget's Disease of Bone

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Paget's disease of bone involves abnormal bone growth and weakening of the bones. It occurs most commonly in the skull, pelvis, spine and legs. It is most common in older people and affects more men than women. It can be treated with medicine or surgery but, as with hydrocephalus, its causes are still unknown, according to MedlinePlus.

Gigantism
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Gigantism is abnormally large growth of the bones. It is caused by excessive amounts of growth hormone released in childhood when bone plates are still being formed. The most common cause of gigantism is a benign tumor on the pituitary gland. Gigantism can cause excessive growth of the head, including the facial features and jaw. It can be treated surgically or with radiation therapy, according to MedlinePlus.