Brain Disorders (Sep 2023)
Investigating severity of symptoms associated with Posterior Fossa Syndrome as predictors of long-term cognitive and functional outcomes: A case series
Abstract
Purpose: Patients may experience Posterior Fossa Syndrome (PFS) also often referred to as Cerebellar Mutism Syndrome (CMS) following medulloblastoma resection. PFS/CMS is associated with delayed onset speech/language changes, motor impairments, and emotional lability. While initially considered a temporary condition, PFS/CMS symptoms impacting patients’ quality of life persist. Existing literature is limited by largely retrospective studies, with short follow-up, and exclusion of cognitive assessment during acute recovery. The current study reviews the history of patients with PFS/CMS acutely and throughout treatment who were matched on variables associated with poorer long-term cognitive functioning. Procedures: The Cognitive and Linguistic Scale (CALS) was used to evaluate two participants with PFS/CMS across the course of cancer-directed treatment. In addition, gold-standard measures of cognitive, academic, motor, language, and adaptive functioning were utilized following completion of treatment. Findings: Mutism duration varied between the two patients (Patient 1 = 88 days, Patient 2 = 28 days). The longer period of mutism was associated with lower CALS scores before radiation therapy, which persisted through pre-chemotherapy and end of treatment. The longer period of mutism and lower initial CALS score were associated with greater reading and math difficulties at the 1- and 2-years post-diagnosis evaluations, and were associated with significantly lower processing speed, visuomotor integration, and adaptive functioning at the 2-years post-diagnosis evaluation. Conclusions: Medulloblastoma survivors are at risk for cognitive late effects. Early assessment of cognitive skills during initial recovery from PFS/CMS (i.e., before mutism resolves) is feasible and may predict severity of PFS/CMS and long-term outcomes, thus providing earlier opportunity to guide caregiver education and advocate for support services.
