Basilar Invagination With Chiari Type I Malformation and Atlanto-Axial Instability: A Rare Case Report

Basilar invagination (BI) and Chiari malformation type I (CM-I) are important anomalies involving the craniovertebral junction (CVJ) involving the skull base and occipitocervical region. The incidence of BI is rare involving < 1% of the general population worldwide. They present with varied and complex clinical-radiological features. We present a 36-year-old male who displayed complaints of persistent reeling sensation at our center. Clinical examination revealed bilateral cerebellar signs along with nystagmus and restricted neck movements. Imaging revealed evidence of BI with cerebellar tonsil herniation of ~14.7 mm. Atlantodens interval of 6 mm was noted. The unexpected findings of C1-C2 fusion and instability were also noted. We describe a rare case of BI with C1 prolapse into the foramen magnum along with CM-1 malformation and congenital fusion of C1-C2. We conclude that the treatment algorithm for these rare cases is not very well established and is individually dependent.