1. The Developmental Intervention Clinic UTH-PCOE Department of Paediatrics & Child Health, University Teaching Hospital 10 November 2015

2. About the D.I.C. Multidisciplinary assessment Early intervention – 0 to 7 years – 0 to 18 Epilepsy HIV SCD Parent/caregiver focused To support children and families with developmental disorders to reach their individual potential.

3. Multidisciplinary team Paediatric neurology Dr Ornella Ciccone Dr M Mathew Occupational therapyEdna Mwaanga PhysiotherapyKateule Banda Speech & language therapyCaitlyln Wessels Pezo Mumbi Cognitive/learning supportDr. Sylvia Kabaghe Behaviour supportAlice Kabwe Grollnek Psychosocial counsellingSandra Shanungu

4. The Process Referrals Intake Neurological assessment Therapy assessment Occupational therapy Speech & language Cognitive assessment Physiotherapy Communication skills Social skills Fine & gross motor development Educational skills Psychosocial assessment Psychosocial counseling Behaviour management Behavior screening Intervention

5. Early Intervention Parent/caregiver training Weekly support Rehabilitation Improvement of quality of life Majority of children with developmental disorders are being referred for further assessment under the age of 3 years. Very few referrals in the first year of life Early intervention: Minimizes sequale of neurological injury improves outcomes Improves quality of life 0 to 3y 4 to 7y 49% 7y + 30% 21%

6. Early Intervention 1.Cerebral Palsy 2.ASD 3.HIV 4.Epilepsy

7. Early Intervention: Cerebral Palsy 25% of DIC patients – 50%: birth asphyxia – 50%: other perinatal injury Majority of children with perinatal injury are referred in the first three years of life. Poor identification and intervention in the first year of life. 0 – 6 mo: 2% 6 mo – 1yr: 8%

8. Early Intervention: Cerebral Palsy OverviewIntervention PT, OT, SL, BX Dysphagia support Pharmacological Tx – AED – Baclofen, clonazepam, anti GERD etc.. Pychosocial support for the family Orthopaedic consultation and orthoptaedic AIDS Feeding challenges & malnutrition: 46% Associated epilepsy: 32%

9. Early Intervention: ASD OverviewIntervention SL, BX, OT – Individual tx – Group tx Psychosocial support – Understanding ASD – Connecting Autism parent support groups Strategies: – Functional communication – Managing challenging behaviour – Joint attention – Social skills 16 % of DIC patients 60 patients on active treatment Existing communication skills/verbal: 38% No functional communication skills/non verbal: 61%

10. Early Intervention: HIV Related Overview 12% of DIC patients have HIV related neurological concerns – Underestimate (excludes consultations done in ART clinic) Late referral

11. Early Intervention: HIV related RVD Encephalopathy Global developmental delay Gross motor impairment Speech impairment Cognitive impairment Behavioural challenges Learning disabilities Symptomatic epilepsy Sequalae of TBM & Meningocephalitis Gross and fine motor impairment Hearing impairment Cortical blindness Acquired aphasia Hydrocephalus Symptomatic epilepsy Learning disabilities and MR 40% 20% 10 % 20 % Epilepsy as presenting manifestation 10 % Miscellaneous Sequelae of cerebral malaria Sequelae of acute hypoxic brain damage 2 nd to profuse bleeding Peripheral neuropathy Sleep disorder CP 2 nd to HIE Global developmental delay 2 nd to malnutrition & poor stimulation Pseudo-seizures (possibly disclosure related) ASD (RVD related?)

12. Early Intervention: HIV related Intervention Integrated care with ART clinic Pharmacological intervention – AEDs – Psychotropic PT, OT, SL, BX Learning support Psychosocial counselling New initiatives Routine developmental screening – 0 to 5 years – PCOE - ART clinic Goal: early identification and referral of developmental concerns

13. Early Intervention: Epilepsy 20% of DIC referrals have epilepsy – 90% with symptomatic epilepsy Total epilepsy patients with bx & cog comorbidities: 51% – 50% have both BX & COG comomorbidiies: 38% Behavioural challenges in epilepsy – hyperactivity, anxiety, aggression, autistic features and personality disorders, depression. – Comorbidities have the greatest impact on quality of life Intervention – AED – Investigation on comorbidities – Psychosocial support ( & psychotropic drug) – Behavioural and learning support

14. O Ciccone, A. Kabwe, C. Boniver J Pediatr Epilepsy 2015;4:85–89 Referral to D.I.C – 2yr 7 mo, male – Behavioral disorder & suspected seizures Parent reported concerns Severe hyperactivity Short attention Tantrums Poor communication Pica *started at 1yr 6mo History: – Uneventful antenatal/perinatal hx Family History: – No epilepsy – No BX disorder Developmental milestones – Mostly within normal limits – Speech and language delay Lennox–Gastaut Syndrome and Behavioral Disorder: A Case Report of Unrecognized Epilepsy in Infancy.

15. Case Report: Lennox Gastaut Syndrome & BX disorder Anamnesis revealed a past History : – infantile spasms from the age of 6 months.  daily episodes of staring gaze, sudden flexion of the head and trunk associated with abduction and elevation of upper limbs. – Sporadic episodes of confusional state and drowsiness lasting for hours. Mother report: – Sought medical attention several times for “abnormal movements” & “strange behavior” – No diagnosis – Never started treatment

16. D.I.C. Assessments Paediatric Neurology Evaluation Behavioural screening Speech & Language assessment Observations Hyperactivity Short attention span Prompt and reinforcer dependent Little spontaneous vocal output No response to name Inconsistency in following simple 1-step directions Pica (styrofoam) Episodes of “head nodding” Neurological exams otherwise normal Case Report: Lennox Gastaut Syndrome & BX disorder

17. Sleep: slow spike and wave discharges at 1.5 Hz. Case Report: Lennox Gastaut Syndrome & BX disorder

18. After awakening: high voltage slow complex clinically associated with episodes of “head nodding,” repeated every 10–20 seconds, for five times. Case Report: Lennox Gastaut Syndrome & BX disorder

19. Sleep: brief paroxysms of fast activity Case Report: Lennox Gastaut Syndrome & BX disorder

20. EEG findings: Consistent with Lennox Gastatut Other investigations (pica): No iron deficiency anemia, SCD, or worm infestation Blood lead level testing was not available MRI/CT not done (machine not functioning) Behavior/Communication Findings: DSM-IV for attention deficit hyperactivity disorder: – Inattention score: 9/10 – Hyperactivity & impulsivity: 6/8 Protest and tantrum behavior, common in nonverbal children Cognitive functioning: (not formally assessed) developmental and behavioral evaluation  cognitive level was not appropriate for age Case Report: Lennox Gastaut Syndrome & BX disorder

21. Intervention: – Started on Sodium valproate – Follow up for seizure control – D.I.C. Home program Behavior Communication At 2 & 4 week follow up: – Significant seizure reduction – Resolution of pica – Increased ability to respond to his name – Improvements: Attention Imitation Following 1-step directions. Vocabulary (from 2 to 10 single word utterances)

22. Challenges in early identification of epilepsy with behavioural disturbances – Family focused on “strange behaviour” – Health care personnel (local clinic) could not recognize a less common form of epilepsy associated with behavioral disorder. Possible unrecognized & untreated West Syndrome from the age of 6months  Later developed different types of seizures (tonic, atonic, and atypical absences) consistent with a diagnosis of LGS. Confusiontal state with drowsiness for several hours = possibly episodes of non-convulsive status epilepticus (absence status epilepticus) Case Report: Lennox Gastaut Syndrome & BX disorder

23. Late Tx Cog/BX dysfunction – No antiepileptic treatment from the onset of infantile spasms until 2yr. – worsened the cognitive and behavioural dysfunction associated with the uncontrolled epileptogenic activity. Early Diagnosis & TX – Could have minimised associated communication & behavioural disorders – Initiation of treatment in this patient was associated with: Significant seizure reduction Resolution of pica Some improvement in communication and behaviour.

24. Thank you ! 2° part

25. D.I.C. Services: School Age Children? Early intervention 0 to 7yrs Birth to primary school Exceptions: – HIV – Epilepsy – SCD What about children over 7 years?

26. Services for school age children: D.I.C. & Dept of Paeds Suggested D.I.C. support to Dept. of Paediatrics: 1.Training in neurological evaluation of school age children 2.Provision of referral options/resources 3.Dr. Ciccone & Dr. Matthew available for ward/clinic neurolgoical consultations What are we currently doing for school going children? Referral to other service providers Advice/support for school placement

27. Services for school age children: D.I.C. & Dept of Paeds ?Questions ? Suggestions ?Feedback

28. Acknowledgements Dr Kankasa, Director, UTH-HAP PCOE Staff Department of Paediatrics & Child Health, UTH Children & families served at the D.I.C.