Cerebral palsy in Mulago hospital, Uganda : comorbidity, diagnosis and cultural adaptation of an assessment tool

Background and aim: Cerebral palsy (CP) is the most common form of chronic motor disability that begins in early childhood and persists throughout life. The clinical features, including motor function, comorbidities and nutritional status, have not been investigated in Uganda. In addition, no assessment tool to measure functional skill development and the level of independence performance in activities of daily living has been developed for these children. The overall aim of this thesis was to describe the neurological, anthropometric and brain imaging findings of Ugandan children with CP and to develop a culturally relevant assessment tool for measuring their functional performance.

Methods and participants: Five cross sectional studies (I-V) were carried out at the Mulago National Referral Hospital in Kampala and in varied rural and urban districts within Uganda. Three studies were conducted at the health facility (I-III), while two were conducted in the community (IV-V). Study I investigated the clinical types, motor function and comorbidities of children with CP. In Study II, this same cohort had their anthropometric measurements taken, as well as information about their clinical, feeding and perinatal history to determine their nutritional status and associated factors. Study III, performed on a sub sample of the original cohort, investigated the brain computed tomography (CT) scans and associated features. In Study IV, the Pediatric Evaluation Disability Inventory (PEDI) was translated and cross-culturally adapted to the Ugandan environment to create the PEDI-UG instrument. The psychometric properties of the new PEDI-UG instrument was validated in Study V.

Results: Bilateral spastic CP was the main clinical subtype (45%). Severe gross and fine motor function levels were more common in the bilateral spastic and dyskinetic CP subtypes. Signs of learning disability (75%) and epilepsy (45%) were the most common comorbidities. Speech and language impairments were associated with bilateral spastic CP and severe gross and fine motor dysfunction (Study I). More than half (52%) of the children with CP were malnourished, with being underweight (42%) presented as the most common form. Malnutrition was associated more with children 5 years of age or older, and those with a history of complications during the neonatal period (Study II). The distribution of brain image patterns differed from that seen in high income countries with more primary grey matter injuries (PGMI) (44%) and normal scans (31%) and very few primary white matter injuries (4%). PGMI were more common in children with a history of hospital admission following birth (Study III). In the culturally adapted PEDI-UG, overall 178 of the original 197 PEDI items (90%) were retained, with a number of modifications in the remaining items, to create the final 185-item PEDI-UG. (Study IV). Most activities of the culturally adapted PEDI UG (95%) showed acceptable fit to the Rasch model. In addition, the caregiver assistant rating scale was changed from a six-point to four-point rating scale (Study V).

Conclusions: There was a large proportion of severely affected children with CP in this cohort, with frequent malnutrition and more PGMI. These results suggest a different etiology of CP in infants born full-term between sub-Saharan Africa and high-income countries. Our findings could imply a higher occurrence of birth asphyxia, postnatally acquired infections or other varied insults around the last trimester period which may possibly benefit from improved emergency obstetric and postnatal care. The culturally adapted PEDI-UG instrument with a four categories caregiver assistant rating scale is appropriate, providing a valid measure of the functional performance of typically developing children from the age of 6 months to 7.5 years in Uganda and other similar African contexts.

List of scientific papers

I. Kakooza-Mwesige A, Forssberg H, Eliasson AC, Tumwine JK. Cerebral palsy in children in Kampala, Uganda: clinical subtypes, motor function and co-morbidities. BMC Research Notes 2015. 8: 166.
https://doi.org/10.1186/s13104-015-1125-9

II. Kakooza-Mwesige A, Tumwine JK, Eliasson AC, Namusoke HK, Forssberg H. Malnutrition is common in Ugandan children with cerebral palsy, particularly those over the age of five and those who had neonatal complications. Acta Paediatrica 2015, 104: 1259–1268.
https://doi.org/10.1111/apa.13089

III. Kakooza-Mwesige A, Byanyima RK, Tumwine JK, Eliasson AC, Forssberg H, Flodmark O. Grey matter brain injuries are common in Ugandan children with cerebral palsy suggesting a perinatal etiology in full-term infants. Acta Paediatrica. 2016 [Epub ahead of print].
https://doi.org/10.1111/apa.13352

IV. Kakooza-Mwesige A, Tumwine JK, Forssberg H, Eliasson AC. Uganda cross-cultural adaptation of the Pediatric Evaluation Disability Inventory (PEDI). [Submitted]

V. Amer A, Kakooza-Mwesige A, Hermansson L, Tumwine JK, Forssberg H, Eliasson AC, Jarl G. Psychometric Properties of the Ugandan Version of the Pediatric Evaluation of Disability Inventory (PEDI-UG). [Manuscript]