A type of cancer that begins to develop in the cerebrospinal fluid and leptomeninges is called leptomeningeal disease (LMD). Certain specific membranes that surround the spinal cord and brain are called leptomeninges. Unfortunately, there is no way to cure this type of cancer, but doctors may recommend some treatments to relieve the symptoms, prevent its spread, and improve your quality of life.
Furthermore, this type of cancer occurs quite rarely, and it is more likely to develop as the cancer progresses. For instance, about 5% of people with metastatic cancer are diagnosed with leptomeningeal disease. Sometimes, this condition is called leptomeningeal carcinomatosis or leptomeningeal metastases (LMM).
What Are The Symptoms of Leptomeningeal Disease?
Typically, LMD causes multiple nervous system symptoms. Check the most common of them below:
- Hearing or vision problems (such as double vision)
- Hearing loss (deafness)
- Back and leg pain
- Seizures
- Difficulty going to the bathroom
- Numbness or weakness in the legs or arms
- Nausea
- Vomiting
- Headaches
- Confusion
- Mood swings
- Behavioral changes
- Gait changes
Immediately contact your healthcare professional if any of the symptoms listed above occur. Otherwise, it may lead to serious complications.
Causes and Risk Factors
Mostly, LMD occurs when the cancer spreads from other parts of the body to the cerebrospinal fluid (CSF) and leptomeninges. In other words, this disease is an advanced and aggressive cancer that can cause serious complications.
In normal circumstances, leptomeninges are specific layers of a membrane that cover and protect the brain and spinal cord. There is also localized CSF, which is a clear fluid that surrounds the central nervous system. Moreover, CSF has direct contact with leptomeninges. Typically, this fluid is drained through a network of veins until something keeps it from draining.
In people with LMD, the cancer blocks the normal flow of CSF, which causes pressure on the brain. This condition is called hydrocephalus, and people who develop it may experience neurologic symptoms.
While anyone with metastatic cancer can develop leptomeningeal disease, there are some factors that may increase the risk. Check some examples below:
- Lung cancer
- Melanoma
- Non-Hodgkin lymphoma
- Acute lymphocytic leukemia (ALL)
- Breast cancer
- Small and non-small cell lung cancer
What Are The Possible Complications of Leptomeningeal Disease?
Those who develop LMD may also experience some complications, especially if the condition is poorly managed. Check some complications below:
- Hydrocephalus and ICP (increased intracranial pressure) – In people with leptomeningeal disease, the tumor reduces CSF outflow, which may lead to swelling and severe symptoms. These include nausea, vomiting, cognitive decline, headaches, and others.
- Cranial nerve deficits – This condition may also affect the cranial nerves, which may lead to diplopia, facial weakness, hearing loss, and swallowing problems (dysphagia).
- Spinal cord and nerve root compression – In some cases, the tumor may put pressure on the spinal axis and cause radicular and back pain, limb weakness that worsens over time, sensory loss, and bladder or bowel dysfunction.
- Vascular problems and strokes – ICP and the tumor can compress certain small blood vessels. As a result, it may lead to localized cerebral ischemia or infarction.
- Seizures – Cancerous cells may also cause focal or generalized seizures.
- Encephalopathy – This complication may cause altered mental status, behavioral and mood changes, and confusion.
The previous list does not contain a full list of LMD complications, but you can consult with your physician about measures to reduce the risk of experiencing them. Unfortunately, there is no way to prevent this condition because physicians do not fully understand what exactly causes it.
Diagnosis
Usually, the diagnosis starts with a physical examination and questions about your medical history and symptoms. Physicians may also perform some tests to rule out other conditions that cause similar symptoms to LMD. These include:
- Lumbar puncture (also known as a spinal tap) – During this procedure, physicians will collect CSF for testing.
- Genetic testing – This test requires a blood sample to check for abnormal genes that may contribute to LMD.
- MRI (magnetic resonance imaging) – This is an imaging test used to get detailed images of different structures and tissues in the body. In this case, physicians will perform MRI scans of the brain and spine.
Treatment
Unfortunately, there is no way to cure this disease. That’s why the treatment goal is to relieve the symptoms, prevent life-threatening complications, and improve your quality of life. Check below for the most common options recommended by doctors for people with LMD:
- Radiation therapy – This therapy uses powerful energy beams to destroy cancerous cells. While most of the time this energy comes from X-rays or protons, it may also come from other sources. In some cases, it is combined with chemotherapy to improve its effectiveness.
- Intrathecal chemotherapy – This treatment involves strong medicines that destroy cancerous cells throughout the body. It is often delivered directly to the CSF.
- Targeted therapy – Doctors often prescribe this option when the previous cancer treatments do not work. It also involves medicines that block a specific protein in the cancer cells, causing them to die.
Additionally, physicians may recommend the following options to manage the symptoms. For example, antiseizure medicines, corticosteroids to reduce inflammation, and a brain shunt to redirect CSF that builds up in the brain.
Frequently Asked Questions
Is leptomeningeal disease the same as brain cancer?
No. Leptomeningeal disease is not a primary brain cancer. It usually happens when a tumor from another part of the body (such as the breast, lungs, or skin) spreads to the cerebrospinal fluid (CSF) and the leptomeninges surrounding the brain and spinal cord.
Is leptomeningeal disease curable?
Unfortunately, there is currently no cure for leptomeningeal disease. However, treatments such as radiation therapy, intrathecal chemotherapy, targeted therapy, and supportive care can help relieve symptoms, slow disease progression, and improve quality of life.
How is leptomeningeal disease diagnosed?
Healthcare providers typically diagnose LMD using a combination of neurological examinations, MRI scans of the brain and spine, and cerebrospinal fluid analysis obtained through a lumbar puncture (also known as a spinal tap). Sometimes, repeated CSF testing is necessary because cancer cells may not appear in the first sample.
What cancers are most likely to cause leptomeningeal disease?
Leptomeningeal disease most commonly develops from metastatic breast cancer, lung cancer (including both small cell and non-small cell lung cancer), melanoma, certain types of leukemia, and lymphomas. However, it can occur with other advanced cancers as well.
Can leptomeningeal disease cause paralysis?
Yes. As the disease affects the brain, spinal cord, or nerve roots, it can lead to muscle weakness, numbness, difficulty walking, and, in severe cases, partial paralysis. Early treatment may help reduce neurological complications.
What is the life expectancy of someone with leptomeningeal disease?
The prognosis varies depending on the type of underlying cancer, the extent (stage) of disease, and how well the person responds to treatment. Without treatment, survival is often measured in weeks, while appropriate therapy may extend survival to several months or longer in some patients. Your healthcare provider can give the most accurate outlook based on your individual situation.
Can leptomeningeal disease return after treatment?
Yes. Even after successful symptom control or an initial response to treatment, leptomeningeal disease may recur or continue to progress. Regular follow-up appointments and imaging studies help healthcare providers monitor for changes and adjust treatment when necessary.
What symptoms require immediate medical attention?
Seek emergency medical care if you experience sudden severe headaches, seizures, confusion, loss of consciousness, new vision changes, difficulty speaking, rapidly worsening weakness, loss of bladder or bowel control, or difficulty breathing. These symptoms may indicate increased pressure in the brain or other serious complications.
Can leptomeningeal disease be prevented?
Currently, there is no proven way to prevent leptomeningeal disease. The best approach is to receive appropriate treatment for the underlying cancer and attend regular follow-up visits so healthcare providers can detect and manage complications as early as possible.
Does everyone with metastatic cancer develop leptomeningeal disease?
No. Most people with metastatic cancer do not develop leptomeningeal disease. It is considered an uncommon complication, occurring in approximately 5% of people with metastatic cancer, although the risk varies depending on the type of cancer and its stage. If you have any other questions, ask your healthcare provider.
