What is Leptomeningeal Disease and how is it treated?

A number of our members deal with leptomeningeal disease (LMD) at some point in their diagnosis. Read more on what LMD is and how it is treated.

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What is Leptomeningeal Disease and how is it treated?

Leptomeningeal disease (LMD) occurs when cancer cells migrate from the breast, lung, or other parts of the body to the cerebrospinal fluid (CSF) or meninges.  CSF is the liquid that circulates nutrients and chemicals to the brain and spinal cord.  Meninges is the layer of tissue the covers and protects the brain and the spinal cord.  CSF is found in between the layers of the meninges which helps to cushion the brain and the spinal cord and is produced in a brain cavity called ‘the ventricle’.  CSF is composed of blood and travels from the brain to the back of the brain, up and down the spinal cord, passes through the brain surfaces, and finally back to the bloodstream.  CSF is replaced several times a day by the body.  If the tumor cells survive in the CSF, then it is possible they may travel to many locations.  

MRI may be used to detect leptomeningeal disease but it may not always pick up the problem.  A procedure called “lumbar puncture” can be used to detect leptomeningeal disease as well.  In this procedure, a sample is taken from the fluid between two vertebrae in the lumbar part of the spine.     

The most common approach to treat LMD is to use surgery, radiation, or chemotherapy.  One method is to inject chemotherapy directly into the spinal fluid by lumbar puncture (spinal tap) or by implanting a device called an Ommaya.  This Ommaya is a ventricular access device that is implanted via surgery under the skin where it is connected via a thin tube with fluids that can access the spinal fluid easily.  A third method is by whole brain radiotherapy (WBRT).  Recent advances in immunotherapy have opened up more options for doctors such as T-cell specific targeting, immune checkpoint inhibitors, or targeted immunotherapy drugs like Ipilimumab.  

What type of symptoms are common in leptomeningeal disease?  

There are three types. 1. Symptoms due to pressure changes of the brain, such as nausea, headaches, confusion, seizures (uncontrolled muscle spasms), sleepiness, or lethargy (extreme lack of energy). 2. Symptoms because of the meningeal lining being infiltrated by cells.  Some of them present like having a neck or a back pain or headaches that starts from the neck. 3.  Lastly, some nerve problems may be present, but these vary widely due to where the nerve may innervate and the area affected in the brain.  Sometimes loss of bladder or bowel control or lack of coordination, or even numbness in the face can be due to leptomeningeal disease.  

A good depiction of CSF, Ommaya reservoir, and where leptomeningeal disease may occur.

http://europepmc.org/article/PMC/4939138

https://www.mountsinai.org/care/neurosurgery/services/brain-tumors/leptomeningeal-disease

https://www.mskcc.org/cancer-care/patient-education/leptomeningeal-metastases

https://www.uhn.ca/PatientsFamilies/Health_Information/Health_Topics/Documents/Learn_Leptomeningeal_disease.pdf 

http://europepmc.org/article/PMC/4939138

AUTHOR: Alice Chou

Kirk Smith