What are the symptoms of Chiari (Arnold-Chiari) malformation?

From the Guidelines

Chiari malformation symptoms typically include occipital or neck pain exacerbated by Valsalva maneuvers, peripheral motor and/or sensory defects, clumsiness, hyporeflexia or hyperreflexia, respiratory irregularities, central apnoeas, and lower cranial nerve dysfunction, as reported in a study published in 2019 1. The symptoms of Chiari malformation can vary widely between individuals, ranging from mild to severe, and some people with Chiari may not experience any symptoms at all.

• Common symptoms include:
  • Occipital or neck pain exacerbated by Valsalva maneuvers
  • Peripheral motor and/or sensory defects
  • Clumsiness
  • Hyporeflexia or hyperreflexia
  • Respiratory irregularities
  • Central apnoeas
  • Lower cranial nerve dysfunction It's essential to note that symptoms can worsen over time and can be exacerbated by activities that increase pressure in the head. If you suspect you or someone you know may have Chiari malformation, it's crucial to seek medical evaluation for proper diagnosis and treatment, as symptoms can overlap with other conditions. A neurologist or neurosurgeon can perform the necessary tests, including MRI scans, to confirm the diagnosis and recommend appropriate management strategies, as suggested in a study published in 2018 1.

From the Research

Symptoms of Chiari Malformation

The symptoms of Chiari malformation can vary, but common complaints include:

• Headache, often worsening with Valsalva maneuver 2
• Dizziness 3
• Numbness 3
• Ataxia, or loss of coordination 3
• Weakness, particularly in the arms and legs 3, 2
• Visual symptoms, such as:
  • Retro-orbital pain 2
  • Flashing lights or floaters 2
  • Blurred vision 2
  • Photophobia 2
  • Diplopia, or double vision 2
  • Transient vision loss 2
  • Peripheral vision loss 2
• Nystagmus, or abnormal eye movements 2
• Downbeat nystagmus, which worsens in certain gaze directions 2
• Difficulty swallowing, or dysphagia 2
• Gait abnormalities, or difficulty walking 3, 4
• Balance problems 3, 4
• Lumbar pain, or lower back pain 4
• Heaviness in the lower limbs 4
• Mental confusion 4

Less Common Symptoms

Some patients may experience less common symptoms, such as:

• Hemiparesis, or weakness on one side of the body 3
• Syringomyelia, or a fluid-filled cavity in the spinal cord 3, 5
• Cervical spondylosis, or wear and tear on the cervical spine 5
• Upper extremity neurologic deficits, including grip strength weakness 5

Post-Surgical Symptoms

Patients who have undergone surgery for Chiari malformation may still experience symptoms, including:

• Headache 6
• Neck pain 5, 6
• Persistent syringomyelia 5
• Cerebellar ptosis, or sagging of the cerebellum 6