Ataxic-hypotonic cerebral palsy in a cerebral palsy registry: Insights into a distinct subtype
Jake P Levy1, Maryam Oskoui1, Pamela Ng1
1Faculty of Medicine (JPL), McGill University, Montreal, QC; Department of Pediatrics and Neurology and Neurosurgery (MO, MS), McGill University, Montreal, QC; Centre for Outcomes Research and Evaluation (MO, PN, MS), Research Institute of the McGill University Health Centre, Montreal, QC; Department of Pediatrics (JA), University of Alberta, Edmonton, AB; Janeway Children's Hospital (DB), St. John's, NL; Department of Paediatrics (DF), University of Toronto, Bloorview Research Institute, Toronto, ON; Departments of Pediatrics and Clinical Neurosciences (AK), Cumming School of Medicine, University of Calgary, AB; Centre de réadaptation Marie Enfant du CHU Sainte-Justine (LK), Montreal, QC; Centre hospitalier universitaire de Sherbrooke (NP), Sherbrooke, QC; BC Children's Hospital (EvR), Vancouver, BC; and IWK Health Centre (EW), Halifax, NS, Canada.
Objective:
To specifically report on ataxic-hypotonic cerebral palsy (CP) using registry data and to directly compare its features with other CP subtypes.
Methods:
Data on prenatal, perinatal, and neonatal characteristics and gross motor function (Gross Motor Function Classification System [GMFCS]) and comorbidities in 35 children with ataxic-hypotonic CP were extracted from the Canadian Cerebral Palsy Registry and compared with 1,804 patients with other subtypes of CP.
Results:
Perinatal adversity was detected significantly more frequently in other subtypes of CP (odds ratio [OR] 4.3, 95% confidence interval [CI] 1.5-11.7). The gestational age at birth was higher in ataxic-hypotonic CP (median 39.0 weeks vs 37.0 weeks, p = 0.027). Children with ataxic-hypotonic CP displayed more intrauterine growth restriction (OR 2.6, 95% CI 1.0-6.8) and congenital malformation (OR 2.4, 95% CI 1.2-4.8). MRI was more likely to be either normal (OR 3.8, 95% CI 1.4-10.5) or to show a cerebral malformation (OR 4.2, 95% CI 1.5-11.9) in ataxic-hypotonic CP. There was no significant difference in terms of GMFCS or the presence of comorbidities, except for more frequent communication impairment in ataxic-hypotonic CP (OR 4.2, 95% CI 1.5-11.6).
Conclusions:
Our results suggest a predominantly genetic or prenatal etiology for ataxic-hypotonic CP and imply that a diagnosis of ataxic-hypotonic CP does not impart a worse prognosis with respect to comorbidities or functional impairment. This study contributes toward a better understanding of ataxic-hypotonic CP as a distinct nosologic entity within the spectrum of CP with its own pathogenesis, risk factors, clinical profile, and prognosis compared with other CP subtypes.
