Chiari malformation and types of basilar invagination with/without syringomyelia

Surg Neurol Int. 2019 Oct 18:10:206. doi: 10.25259/SNI_469_2019. eCollection 2019.

Abstract

Background: Craniometric studies document different subtypes of craniocervical junction malformations (CCJM). Here, we identified the different types and global signs and symptoms (SS) that correlated with these malformations while further evaluating the impact of syringomyelia.

Methods: Prospective data concerning SS and types of CCJM were evaluated in 89 patients between September 2002 and April 2014 using Bindal's scale.

Results: The mean Bindal's scores of each type of CCJM were Chiari malformation (CM) = 74.6, basilar invagination Type 1 (BI1) = 78.5, and BI Type 2 (BI2) = 78. Swallowing impairment and nystagmus were more frequently present in the BI patients. Symptomatic burdens were higher in patients with syringomyelia and included weakness, extremity numbness, neck pain, dissociated sensory loss, and atrophy.

Conclusion: There were no statistically significant differences in SS between the different CCJM types. BI patients had more swallowing and nystagmus complaints versus CM patients, but there were no significant differences in clinical SS between BI1 and BI2 patients. Notably, those with attendant syringomyelia had a higher SS burden.

Keywords: Arnold–Chiari malformation; Basilar impression; Neurologic manifestations; Platybasia; Signs and symptoms.