Acute and Sudden Presentations of the Chiari Malformations

Abstract

Chiari malformation type 1 (CM I) is a common neurological disease in both children and adults. While CM I patients commonly present with slowly progressive pain, signs of brainstem compression, or spinal cord compression from a syrinx, a small minority of patients present with rapid onset of neurological deficits. Compared to the general CM I population, these patients are more likely to have motor deficits, respiratory dysfunction, cranial nerve abnormalities, or even sudden death. While traumatic injury is a potential cause of such abrupt presentations, many patients develop acute neurological deficits without preceding trauma. Compared to the typically elective management of CM I, patients with acute deficits should generally undergo surgical treatment in an expedited or urgent manner, with particular attention also paid to managing any respiratory concerns prior to and following surgical intervention. With appropriate management, these patients often have substantial if not complete resolution of their presenting complaints. Therefore, while CM I is typically considered a chronic condition, physicians should remain aware of the important minority of patients who present acutely, ensuring they receive timely diagnosis and management.