Cerebrospinal Fluid (CSF) Disorders

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What are Cerebrospinal Fluid Disorders?

Cerebrospinal fluid (CSF) disorders occur when there are abnormalities in the CSF composition, such as if the fluid accumulates in the brain or the spinal cord or if it leaks into other areas of the body. CSF leaking to the skull base, such as the nose or ear canals, can lead to medical complications, including infections. Treatment for these disorders can vary from simple bed rest and hydration to medication and surgeries, depending on the type of disorder and its present condition.

Cerebrospinal fluid – a clear, watery fluid present in the ventricles of the brain and spinal cord, plays a vital role in maintaining the health of your central nervous system (CNS). It performs several critical functions in the human body, including providing nutrition, cushioning the brain, absorbing shock for the CNS and removing cellular waste products. Confined within the CNS by the dura mater, a protective membrane, CSF is devoid of red blood cells and consists mainly of protein, glucose, electrolytes, antibodies, and white blood cells.

What are the Types of Cerebrospinal Fluid Disorders?

There are two main types of abnormalities in fluid dynamics that lead to cerebrospinal fluid disorders.

1. CSF pathway problem (obstruction of CSF pathways)

2. CSF production/absorption (communicative)

Hydrocephalus

Hydro means “water,” and Cephalus means “brain and spinal cord.” Hydrocephalus, as the term implies, refers to an abnormal build-up of CSF in the cavities of the brain (ventricles). The extra fluids enlarge the ventricles, which in turn puts pressure on the brain. It is also associated with the abnormal flow, absorption, and production of CSF.

Excessive cerebrospinal fluid pressure can damage brain tissue and induce a variety of symptoms related to brain function. Although it can occur at any age, hydrocephalus is more common among infants and the elderly.

Congenital Obstructive Hydrocephalus

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

A Chiari malformation is another congenital disorder in which the lower part of the brain pushes down into the spinal canal. This blocks CSF outflow to the spinal canal and causes CSF accumulation, increasing pressure inside the brain.

Communicating Hydrocephalus (Non-Obstructive Hydrocephalus)

The term “communicating” refers to the fact that CSF can still flow between the ventricles and into the spinal canal, which remains open. However, CSF absorption back into the venous circulation is blocked or disrupted, leading to CSF accumulation in the brain and pressure build-up. Communicating hydrocephalus is a common complication of 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 occurs over months due to abnormal CSF build-up in the brain’s cavities caused by age-related brain shrinkage among the elderly. There are no blockages or absorption issues in the brain or CSF pathways.

The significantly higher volume of CSF in the ventricles of the brain causes fluid to leak into the brain tissue along the ventricles, impairing various brain functions such as memory, leg movement, and bladder sphincter control. It is known as normal pressure hydrocephalus, as the pressure inside the ventricles is relatively normal or lower than normal in these patients.

NPH may be caused by subarachnoid haemorrhage, trauma, infection, a tumour or a surgical complication. However, it could also be idiopathic, which means that there is no identifiable reason.

Normal pressure hydrocephalus typically develops at an age over 70. Its symptoms often include recent memory loss, urinary dysfunction, and magnetic gait (inability to lift the feet off the ground).

When Should I See a Doctor?

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

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

Gate apyrexia is characterised by impaired walking, shuffling, a tendency to fall backwards and forwards, 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 such as:
- 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 the first consultation, your doctor 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 inquiries will be followed by a physical examination and various diagnostic tests to ensure an accurate diagnosis.

How are Cerebrospinal Fluid Disorders Diagnosed?

In order to accurately understand and treat your condition, your doctor will 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 as well as to 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?

Nowadays, there are various treatments available to 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 CSF fluid circulation and absorption, either by removing the blockages or creating new pathways for the fluid to flow.

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

Endoscopic Third Ventriculostomy

It is a minimally invasive surgical procedure in which a small hole is made 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 natural circulatory process.

Ventriculoperitoaneal Shunt

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

Ventriculoatrial Shunt

It is a surgical procedure used to treat hydrocephalus in which the placement of a shunt causes the flow of fluid from the brain toward the atrium (part of the heart).

Ventriculopleural Shunt

It is a treatment 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 treatment in which excessive fluid is drained from the brain through subarachnoid spaces into the peritoneal cavity.

Summary

Cerebrospinal fluid disorders are mainly related to CSF metabolism. The conditions are caused by any anomaly in CSF metabolism, which leads to excessive production or reduced absorption. As they can be life-threatening at times, these conditions should be properly diagnosed and treated as soon as possible to prevent harmful outcomes. Whether you are seeking consultation regarding CSF disorder symptoms or brain tumour treatments, do not hesitate to reach out to us today!

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.