Case Presentation

1. Case Presentation Progressive Weakness in an 8 year-old

2. History • 8yr old boy • Coughing and shortness of breath • Fever • Non-productive cough • No TB contacts • Unable to walk for 1mth, with generalised progressive and symmetrical weakness

3. History continued • Unable to feed himself or use upper limbs • Loss of bladder and bowel function • Not able to attend school for one month • No obvious weight loss • Not tested for HIV • No history of trauma

4. Past Medical History • Previously admitted to for abdominal mass and had laparotomy • Mother not sure of the findings • Histology unobtainable

5. Birth History • Born in Ladysmith • Full Term/ NVD • Uneventful pregnancy • Not tested for HIV in pregnancy

6. Immunisations • Up to date ( Including polio)

7. Growth and development • Normal milestones • No developmental delays • Currently Grade IV pupil and doing well

8. Family History • No family history of neurological disorders or malignancies • No TB Contacts • Mom lives in Johannesburg • Two children (including patient), both live in Ladysmith

9. Examination • Weight 21kg (< 3rd centile) • Pyrexial 38.6 • Acutely ill • Saturation 86% in room air and improved to 99% on polymask • Generalised shotty lymph nodes • Hydration good • Mild pallor • No jaundice • Early clubbing

10. Respiratory • Chest :No features of chronicity • Moderate respiratory distress with intercostal and subcostal recession • Features of consolidation bilaterally in the lower zones • Very weak cough

11. Cardiovascular System • No features of cor pulmonale • Normal heart size • Normodynamic • Normal heart sounds • No cardiac murmurs

12. GIT • Old laparotomy scar visualised • No distension • 2cm hepatomegaly • Spleen not enlarged • Extensive bed sores over the sacral area extending to the buttocks. Septic at the base with thick slough • Decreased anal tone • Constant dribbling of urine but no palpable bladder

13. Neurologically • Glasgow Coma Scale 15/15 • Alert and orientated • Speech and intellect normal • Cranial nerves intact • Obvious muscle wasting • Upper limbs: Motor deficit level C4 • Sensory deficit at level T2/3 • Lower limbs: Power 0/5 • Upper motor neuron signs • Plantars upgoing • Absent sensation

14. Neurological/Musculoskeletal • Truncal Ataxia • No other features of cerebellar disease • Musculoskeletal: early contractures of the hands • No joint deformities

15. Summary • 8year old child • Presenting with history of progressive, symmetrical weakness and paralysis for 1 mth (but probably longer in view of the extensive bed sores) • Features of high spinal lesion at ± C3/4 • Concomitant multilobar pneumonia (?weak diaphragm) • No TB contact

16. Differential Diagnosis • TB Spine • Neuroblastoma • Other spinal tumours • Transverse myelitis • Subacute combined degeneration of the cord

17. Investigations • FBC: WCC 17.6 Hb 10.8 Plt 342 • Diff: N85% M8% L7% • ESR: 87 CRP: 216 • U&E:133/3.4/96/18/4.2/26 • Total protein: 76 Albumin 34 • LFT Normal CMP Normal • Vit B12 Normal

18. Investigations • CSF Protein >5 Glucose 3.3 Chloride122 • Polys 0 Lymphs 3 RBCs > 1000 • CSF ADA 13.4 • No growth • Latex Negative • HIV Elisa Positive • CD4 Count 24% • Viral load 28000 Log 4.45 • Lipase 23 (normal) • Blood cultures Negative

19. CXR • Consolidation of the R middle and lower lobes • No significant hilar adenopathy but no lateral film to confirm

20. MRI Scan

21. MRI Scan • Report: Intramedullary Lesion involving the spine from C3 to C7. • ?Astrocytoma

22. Progress in the Ward • Started on i.v. antibiotics (Rocephin and cloxacillin) • High dose dexamethasone started • Chest physiotherapy • TB work-up Negative • Ultrasound abdomen Normal • Seen by Neurosurgeons – Biopsy done

23. Biopsy Results • Preliminary Report: Grade II Astrocytoma • Further staining being done to rule out infective process especially TB in view of the HIV status

24. Summary • 8yr old boy • HIV Positive – Clinically stage IV (presence of malignancy) • High spinal mass lesion – Astrocytoma on biopsy • TB work up Negative • Pneumonia - treated

25. Intraspinal Malignancies in Childhood • Rare in children • 20:1 to 5:1 incidence to intracranial tumours reported in children. • Intramedullary tumours account for 10% of CNS malignancies in childhood.

26. Classification • Intramedullary tumours: • Astrocytomas – 60% • Ependymomas – 20% • Other less common tumours are haemangioblastomas and gangliomas.

27. Intradural Extramedullary Tumours: • Drop metastases (leptomeningeal spread) from primary brain tumours • Neurofibromas • Extramedullary Tumours: • Direct extension from neuroblastomas, • Histiocytosis • Lymphoma • Sacrococcygeal Teratomas

28. Astrocytomas • Most common intramedullary tumours • Predilection for the cervical cord • Any portion of the spine may be involved. • 60% of cases the entire cord may be involved by poorly differentiated tumours (Holocord tumour). • Males = Females incidence • Usually present around 10years of age

29. Astrocytomas • Arise from astrocytes • Range from relatively benign to malignant tumours • Grade I astrocytomas, spongioblastomas and pilocytic astrocytomas are relatively benign. • High grade astrocytomas: Grade IV and glioblastoma multiforme are more malignant • Cystic or solid • Mixed solid and cystic components. • MRI Scan is the imaging modality of choice for diagnosing these tumours

30. Treatment • Multidisciplinary team involvement • Modalities include radical surgery, chemotherapy and/or spinal radiotherapy. • CUSA – Cavitrone Ultrasonic Apparatus has been used with good results and less neurological deficits. This destroys tissue by ultrasonic vibration, emulsifies the debris and sucks it away.

31. Our Patient • Started on HAART since clinically Stage IV • Discussion with Oncologists for possible radiotherapy once histology confirmed.