Cerebrospinal Fluid (CSF) Disorders

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Cerebrospinal Fluid (CSF) is a clear watery fluid present in the ventricles of the brain and spinal cord and plays an essential role in the health of the central nervous system (CNS). It performs several important functions in your body, such as providing nutrition, cushioning the brain, absorbing shock for the CNS and removing cellular waste products.

CSF consists mainly of protein, glucose, electrolytes, antibodies, and white blood cells. CSF is devoid of red blood cells. Cerebrospinal fluid disorders occur when there are abnormalities in its composition, if the fluid builds up in the brain or spinal cord, or if it leaks into the skull base, like the nose or ear canals, leading to complications like infection.

What are Cerebrospinal Fluid Disorders?

Abnormalities in fluid dynamics lead to cerebrospinal fluid disorders. These abnormalities are well-known culprits for CSF disorders. Basically, there are two types of abnormalities:

1. CSF pathway problem (obstruction of CSF pathways)
2. CSF production/ absorption (communicative)

What are the types of Cerebrospinal Fluid Disorders?

Hydrocephalus

Hydro means “water,” and Cephalus means “brain and spinal cord.” Hence termed, hydrocephalus, refers to an abnormal build-up of CSF in the cavities of the brain (ventricles). This causes the cavities to enlarge, which, in turn, puts pressure on the brain.

It is also associated with the abnormal flow, absorption, and production of CSF. This results in an accumulation of CSF in the ventricles of the brain and spinal cord, also known as hydrocephalus.

Although it can occur at any age, it is more common among infants and the elderly.

Congenital Obstructive Hydrocephalus

This type of hydrocephalus is a congenital disability or malformation in the brain which involves the buildup of CSF in the subarachnoid space or obstruction of CSF pathways in the brain. Babies experience this shortly after being delivered. This increased intracranial pressure often manifests as a large head (Macrocephaly).

Another congenital disorder is a Chiari malformation (the lower part of the brain pushes down into the spinal canal) that causes the blockage of CSF outflow to the spinal canal and accumulation of CSF, which, in turn, increases the pressure inside the brain.

Communicating Hydrocephalus (non-obstructive hydrocephalus)

The term “communicating” refers to the fact that CSF is still able to flow between the ventricles as well as to the spinal canal, which remains open. However, the absorption of CSF back into the venous circulation is blocked or disturbed, resulting in the accumulation of CSF in the brain and the build-up of pressure. Communicating hydrocephalus is a very common complication in traumatic brain injuries, meningitis, subarachnoid haemorrhage and tumours.

Conversely, non-communicating hydrocephalus or obstructive hydrocephalus occurs when the flow of CSF is blocked along the passages connecting the ventricles.

Normal Pressure Hydrocephalus (NPH)

Normal pressure hydrocephalus develops over months when there is an abnormal build-up of CSF in the brain’s cavities because of the shrinkage of the brain in the elderly. There is no blockage or absorption problem in the brain or CSF pathways.

The relatively increased volume of CSF in the ventricles of the brain causes fluid to spill into the brain tissue along the ventricles, affecting various functions of the brain such as memory, movement of both legs and urinary bladder sphincter control. The pressure inside the ventricle is relatively normal or lower than normal in these patients; therefore, it is called normal pressure hydrocephalus.

NPH may be caused by subarachnoid haemorrhage, trauma, infection, a tumour or surgical complication. That being said, it may also be idiopathic, which means that there is no known cause.

The mean age of onset of Normal pressure hydrocephalus is over 70 years, and symptoms often involve recent memory loss, urinary dysfunction and magnetic gait (inability to lift the feet of the floor).

When should I see a doctor?

CSF anomalies can affect your brain and spinal cord function and can eventually lead to coma or sudden death. This makes prompt diagnosis and treatment crucial.

Moreover, sudden blockage of the CSF pathway with rising pressure inside the brain (raised ICP) can cause sudden death. Please make an appointment with us if you experience the following symptoms:

Gate apyrexia is characterised by impaired walking, shuffling, the tendency to fall backwards and forward, and unstable turns.
Early-onset dementia (memory loss)
The blurring of vision and photophobia
Seizures
Recurrent vomiting and headaches

How are Cerebrospinal Fluid Disorders caused?

Cerebrospinal fluid disorders can be idiopathic or secondary associated with CSF production, absorption, or pathway blockage.

Traumatic brain injury
Meningitis ( bacterial or Tuberculosis)
Subarachnoid haemorrhage ( aneurysmal bleeding)
Brain tumours
Congenital conditions like:
- Aqueductal stenosis
- Chiari malformation
- Dandy-walker syndrome (due to enlarged 4th ventricle)
Pseudotumor cerebri (benign intracranial hypertension)

What can I expect during my first consultation?

During your first consultation, I would ask various questions to get a better understanding of your condition, such as:

Personal and family history
Onset of symptoms
Duration of symptoms
Physical examination
Laboratory investigation
Any other conditions you may have
These questions will be followed by a physical examination and various diagnostic tests for an accurate diagnosis.

How are Cerebrospinal Fluid Disorders diagnosed?

In order to accurately understand and treat your condition, I typically run some tests, such as:

Cerebrospinal fluid studies

This involves a series of diagnostic tests to rule out any infectious diseases of the brain and spinal cord, tumours, autoimmune disorders, or bleeding in the brain.

CSF spectroscopy

CSF spectroscopy is a technique attributed to patients with subarachnoid haemorrhages. It detects bilirubin in CSF which is the constituent of blood.

MRI and CT scan

Magnetic resonance imaging and CT scans help provide detailed images that reveal any structural abnormalities in the ventricles.

Lumbar puncture

This diagnostic test involves collecting a sample of your cerebrospinal fluid to test for any infection, inflammatory condition, bleeding or certain cancers and also measure the CSF pressure.

CSF pressure measurement (ICP monitoring)

Manometers measure CSF pressure. Pressure-sensitive probes are used in nanometers to monitor intracranial pressure directly through the skull and put into the ventricles.

Can CSF Disorders be cured?

Thankfully, there are various treatments available that can re-establish normal levels of CSF. Sometimes, a patient may require multiple procedures to treat hydrocephalus. Conversely, CSF leaks may heal on their own.

How are Cerebrospinal Fluid Disorders Treated?

Treatment options largely depend on your current condition and the type of CSF disorder. These life-saving procedures often involve re-establishing the circulation and absorption of CSF fluid, either by removing the blockages or creating alternative pathways for the fluid to flow.

Some of these are either medical treatment or surgical treatment. Medical treatments serve as temporary measures to help lower pressure inside the brain or reduce CSF production. Surgical treatments are definitive treatments, and options are:

Endoscopic third ventriculostomy

It is a minimally invasive surgical procedure in which a small hole is indicated in the base of the 3rd ventricle to drain extra fluid from the brain to the basal cistern and lower the CSF pressure.

CSF diversion

These procedures generally involve the use of implanted shunts to divert the cerebrospinal fluid. The shunt systems drain the excess fluid from the brain to other parts of the body, where it is eventually absorbed as part of the normal circulatory process.

Ventriculoperitoaneal shunt

The ventriculoperitoneal shunt is a surgical procedure indicated in patients with increased CSF pressure. It includes a plastic tube through which the excess fluid is drained.

Ventriculoatrial shunt

It is a surgical procedure indicated in hydrocephalus in which placement of shunt cause flow of fluid from the brain toward the atrium (part of the heart).

Ventriculopleural shunt

It is a procedure in which a proximal catheter is placed on the skull, and a distal catheter is placed in the pleural cavities. It is an alternative form of ventriculoperitoneal shunt.

Lumbar peritoneal shunt

The lumbar peritoneal shunt is a procedure in which excessive fluid is drained from the brain through subarachnoid spaces to the peritoneal cavity.

Summary

Cerebrospinal fluid disorders are mainly related to CSF metabolism. Any abnormality in CSF metabolism that leads to excessive production or decreased absorption is responsible for these disorders. Sometimes, they are life-threatening, so they should be carefully diagnosed, and prompt treatment is required to avoid harmful consequences. Please do not hesitate to contact us if you suffer from any CSF disorder symptoms.

Dr Sein Lwin is an experienced Senior Consultant Neuro & Spine Surgeon and the Neurosurgical Director at the Advanced Brain and Spine Surgical Centre.

He is highly experienced in spine surgery and in minimally invasive approaches for spinal cord tumours and degenerative spine surgery. His specialised interests lie in endoscopic endonasal and open skull base surgery, pituitary tumours, vascular surgery, cranial nerve disorders and peripheral nerve conditions.

Urgent Care

Many neurological conditions may require urgent attention. If you require immediate care, please contact us.

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