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Headaches caused by low cerebrospinal fluid (CSF) pressure, also known as low-pressure headaches or CSF leak headaches, are a distinct type of headache that occurs when the pressure of the CSF, which cushions the brain and spinal cord, drops below normal levels. This type of headache is often associated with a spinal CSF leak, which can be spontaneous or result from medical procedures.

About

• Headache due to presumed CSF leakage

Aetiology

• Spontaneous CSF Leak: often due to a small tear or hole in the dura mater, the membrane surrounding the brain and spinal cord.
• Post-Lumbar Puncture: lumbar puncture (spinal tap), where the needle puncture does not seal properly, leading to CSF leakage.
• Trauma: Head or spinal injuries can result in a tear in the dura mater, leading to a CSF leak and subsequent low-pressure headache.
• Surgical Procedures: surgeries involving the spine or brain can inadvertently cause a CSF leak.
• Connective Tissue Disorders: Marfan syndrome or Ehlers-Danlos syndrome can weaken the dura mater, making it more prone to tears and leaks.

Clinical

• Headache is a diffuse, throbbing pain
• Most severe in the occipital region but can affect the entire head
• Come on when the patient wakes up - worse when standing or sitting and improves when lying down
• Worse on standing in an upright posture and resolves on lying down
• Nausea, vomiting and neck pain and stiffness
• Tinnitus (ringing in the ears) or a sense of fullness in the ears may be present
• Cranial Nerve Palsies: diplopia

Investigations

• MRI: with gadolinium contrast is often used to detect signs of a CSF leak, such as meningeal enhancement, subdural fluid collections, or brain sagging.
• CT Myelography: This imaging technique involves injecting contrast into the CSF space to identify the exact location of the leak.
• Intrathecal Contrast-Enhanced MRI: This is another method used to visualize the CSF leak and its source.
• Radioisotope Cisternography: A less commonly used technique where a radioactive tracer is injected into the CSF to detect leaks.

Management

• Conservative Management: bed rest, hydration, and caffeine intake, can alleviate symptoms in some cases.
• Epidural Blood Patch: most common treatment for low CSF pressure headaches, particularly those following a lumbar puncture. A small amount of the patient's own blood is injected into the epidural space to form a clot and seal the leak.
• Surgical Repair: surgical repair may be necessary to close the dura mater defect and stop the CSF leak.
• Hydration and Caffeine: fluid intake and caffeine can help elevate CSF pressure temporarily and alleviate symptoms.
• Pain Management: Analgesics and antiemetics may be prescribed to manage headache and associated symptoms such as nausea and vomiting.