Chiari Malformation and Headache: Causes, Symptoms, and Treatments

Chiari malformation is a structural defect at the base of the skull and cerebellum that can lead to various symptoms, including severe headache.

This condition is often challenging to diagnose and can significantly impact quality of life. This guide provides an overview of Chiari malformation, its symptoms, causes, and when to seek medical attention.

What is Chiari Malformation?

Chiari malformation is a condition where the lower part of the brain, the cerebellum, herniates through an opening at the base of the skull, called the foramen magnum. This herniation can compress the brainstem and cerebellum, leading to a range of symptoms.

  • Structural Defect: The back of the skull is smaller than usual, causing part of the brain to sag through the opening at the skull base.
  • Brain Compression: Compression of the brainstem can affect vital functions like respiration, blood pressure, and coordination, while compression of the cerebellum affects balance.

Symptoms of Chiari Malformation

Chiari malformation can produce a wide variety of symptoms, including:

  • Headache: Often located at the back of the head and worsened by sneezing, coughing, or straining.
  • Dizziness and Balance Issues: Symptoms may include vertigo, ataxia (difficulty with balance), and veering to one side while walking.
  • Visual Disturbances: Double vision or blurred vision.
  • Neurological Symptoms: Weakness, numbness, or even loss of consciousness in severe cases.

Why Does Chiari Malformation Cause Headache?

Headache in Chiari malformation is thought to be caused by the obstruction of cerebrospinal fluid (CSF) flow:

  • Obstructed CSF Flow: The normal flow of CSF from the brain to the spinal cord is blocked, leading to increased pressure within the brain. Activities like coughing or sneezing can further increase this pressure, worsening headache.

Differences Between Chiari Malformation Headache and Migraine

While Chiari malformation can cause headache that may resemble migraine attacks, there are key differences:

  • Location: Chiari headache is often at the back of the head, while migraine attacks are usually one-sided or located around the temples.
  • Triggers: Chiari headache is worsened by activities like coughing, sneezing, or straining, which is less common for migraine attacks.
  • Coexistence: It’s not uncommon for people with Chiari malformation to also have migraine, but surgery for Chiari malformation is unlikely to cure migraine.

Diagnosis of Chiari Malformation

Chiari malformation is typically diagnosed through:

  • MRI Scans: An MRI can reveal the extent of brain herniation. A sagging of more than 5 millimeters is often considered diagnostic.
  • Radiologist Expertise: Diagnosis requires careful interpretation by a trained neuroradiologist, as Chiari malformation can be mistaken for other conditions.

Treatment Options for Chiari Malformation

Decompression Surgery: Involves removing a small section of bone at the back of the skull to relieve pressure. Effective primarily for headache at the back of the head that worsens with activities like coughing or sneezing. 

Managing Symptoms:

  • Pain Management: Medications and physical therapy may be used to manage symptoms.
  • Avoiding Triggers: Identifying and avoiding activities that worsen symptoms.

When To Talk With a Healthcare Provider​

You should consult a healthcare provider if you:

  • Experience severe headache, especially those that worsen with coughing, sneezing, or straining.
  • Have balance issues, dizziness, or visual disturbances.
  • Experience neurological symptoms like numbness, weakness, or episodes of loss of consciousness.
  • Suspect you have Chiari malformation but have not received a diagnosis or proper evaluation.

It is crucial to be evaluated by a neurologist or neurosurgeon who specializes in Chiari malformation, as not all doctors are well-versed in diagnosing or treating this condition.

This resource is based on HeadWise Episode 33, Chiari Malformation and Headache with Vince Martin, MD.
Published 10/24/2019

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