Objective: To develop a scale for assessing the severity of cerebellar ataxia in patients with PCFT

Background: Cerebellar ataxia (CA) is prevalent in patients with posterior cranial fossa tumors (PCFT) both before and after surgery, affecting 50-90% of cases [1, 2, 3, 4, 5]. Despite its significance, there is a lack of standardized tools for assessing ataxia severity in this population, warranting further investigation.

Method: We examined 118 patients with PCFT aged from 4 to 60 years. All patients underwent a comprehensive clinical and paraclinical examination. We developed the Cerebellar Ataxia Severity Assessing Scale (CASAS) comprising 8 items to evaluate CA severity. The scale’s maximum score, 26, indicates the most severe ataxia, equivalent to cerebellar mutism. CA severity is categorized as follows: “0” for no CA, “1-7” for mild CA, “8-20” for moderate CA, and “>20” for severe CA. Additionally, we analyzed the distribution of ataxia severity across different age groups and histologic types of PCFT. The Scale for the Assessment and Rating of Ataxia (SARA) was used for comparison

Results: In the assessment of cerebellar ataxia (CA) severity among 118 patients with posterior cranial fossa tumors (PCFT) using the CASAS scale, results were compared with the Scale for the Assessment and Rating of Ataxia (SARA) for sensitivity and specificity evaluation. Both SARA and CASAS scores decrease with increasing age, indicating that younger patients generally experience more severe ataxia. The youngest age group (4-9 years) demonstrated the highest scores, while the oldest age group (35 years and older) exhibited the lowest scores. The distribution of patients by CA severity revealed that most fell within the mild to moderate ataxia categories, with medulloblastoma patients showing more severe ataxia compared to other histologic types of PCFT patients tend to exhibit more severe ataxia compared to other histologic types of PCFT.

Conclusion: Our study, utilizing the CASAS scale, revealed a predominant occurrence of no to mild CA in patients with posterior cranial fossa tumors. Moderate ataxia was observed in a smaller proportion, while severe ataxia was rare. Additionally, medulloblastoma emerged as associated with more severe ataxia. These findings underscore the efficacy of CASAS in assessing ataxia severity, offering valuable insights for clinical management in this patient population.

References: 1. Di Rocco C., Chieffo D., Pettorini B.L., Massimi L., Caldarelli M., Tamburrini G. Preoperative and postoperative neurological, neuropsychological and behavioral impairment in children with posterior cranial fossa astrocytomas and medulloblastomas: the role of the tumor and the impact of the surgical treatment. Childs Nerv Syst. 2010;26(9):1173–1188.
2. Sunderkaer S., Schmiegelow M., Carstensen H., Nielsen L.B.,Miller J., Schmiegelow K. Long-term neurological outcome of childhood brain tumors treated by surgery only. J Clin Oncol. 2003;21(7):1347–1351.
3. Piscione P.J., Bouffet E., Mabbott D.J., Shams I., Kulkarni A.V. Physical functioning in pediatric survivors of childhood posterior fossa brain tumors. Neuro Oncol. 2014;16(1):147–155.
4. Ilg W., Timmann D. Gait ataxia–specific cerebellar influences and their rehabilitation. Mov Disord. 2013 Sep 15;28(11):1566-75.
5. Lannering B., Marky I., Lundberg A., Olsson E. Long-term sequelae after pediatric brain tumors: their effect on disability and quality of life. Med Pediatr Oncol. 1990;18(4):304–310.

« Back to 2024 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/assessment-of-cerebellar-ataxia-severity-in-patients-with-posterior-cranial-fossa-tumors-using-casas-scale/