Case report| Volume 57, P180-182, November 2018

Chiari I malformation with significant motor and autonomic dysfunction in an infant

Published:August 22, 2018DOI:


      • Chiari 1 malformations have variable presentation and demographics, however key patterns exist.
      • The unusually aggressive Chiari I malformation presentation in a 3-month-old is discussed.
      • Emergency surgical intervention due to the acute onset lead to full patient recovery.
      • Knowledge of unique cases may improve recognition and aid diagnosis.


      Quadriparesis and cardiovascular instability are not symptoms commonly associated with Chiari I malformation diagnoses, less so in a 3-month-old toddler. Chiari I malformations have a wide array of symptoms, varying with regard to both the nature of onset and the age of the patient presenting. This variation is illustrated in this report, presenting a novel description of a set of symptoms in a particularly young patient, a case lacking the common insidious nature of Chiari I malformation symptoms. Acute onset of cardiovascular instability, global hypertonia, hyperreflexia, and proximal upper limb weakness in a 3-month-old toddler are discussed. Parallels between the presented case and previous case reports are few, however some key features are distinguishable among similar cases. A successful posterior fossa craniectomy allowed the acutely unwell toddler to become a healthy, fully functional child. We believe this case contributes to elucidating the rare presentations of a curious pathology.


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