Spontaneous Resolution of Aberrant Cerebellar Tonsil Movement in a Patient with Improving Chiari I Malformation

A genetic component in Chiari I malformation: Chiari 1 malformation in all five family members

“…This reduction in volume can lead to the herniation of the cerebellar tonsils through the foramen magnum, which causes many of the symptoms seen in CMI [13] . There is also a strong correlation between CSF flow and the severity of CMI symptoms [3] . In this case series, the resolution of symptomatic CMI was associated with the restoration of CSF flow; the herniation of the cerebellar tonsils was not resolved itself.…”

“…Cerebellar tonsil herniation also disrupts the flow rate of cerebrospinal fluid through the CMJ. This disturbance in flow is more closely related to the severity of symptoms than the amount of herniation itself [ 3 , 14 , 19 ]. The severity of symptoms for each of the patients in this family supports this finding, as the mother and father, both of whom experienced aberrant CSF flow, experienced the most intense symptoms compared to their 3 sons.…”

“…Symptomatic patients most commonly present with headaches; however, dizziness, disequilibrium, neck pain, difficulty sleeping, weakness, numbness in the upper or lower extremities, nausea, and blurred vision may also be present [2] . Cerebellar tonsillar herniation results in aberrant tonsillar pulsatile motion during the cardiac cycle, which interferes with cerebrospinal fluid (CSF) flow at the foramen magnum and causes symptoms [ 3 , 4 ]. Traditional treatment for symptomatic CMI involves posterior fossa decompression, which creates more space for the cerebellum, relieves pressure on the spinal cord, and restores normal CSF flow [5] .…”