Approach to child with acute white matter.pptx

This presentation talks about acute white matter in children

1. Approach to a Child with Acute W hite M atter D isorders Dr. Inaam Gashey P rofessor of pediatric neurology G afer Ibn Auf Specialized Hospital

2. Part of the talk……….. White matter structure and functions Acute disseminated encephalomyelitis(ADEM) Optic neuritis (ON) Transverse myelitis (TM) Neuro - myelitis optica (NMO) Multiple sclerosis(MS)

3. EMBRYOGENESIS PROLIFERATION MIGRATION , MATURATION NEURONES 8 weeks 22 weeks 16 weeks Birth MYELINATION Axonogenesis Dendritogenesis Synaptogenesis Oligodendrocyte maturation Progenitor Pre oligodendrocyte myelinating Oligodendrocyte Pluripotent cells ZSV Myelin sheaths formation OLG Maturation and CNS Myelination latest event of the brain development cytokines Nerve influx neurotransmitters Hormones (T3) Astro/glia contact PROLIFERATION MIGRATION MATURATION OLIG

4. FETAL in months FIRST YEAR in months YEARS 6 9 3 6 9 12 Sensory roots Medial lemniscus Stato-Acoustic system Tectum and tegmentum Motor roots Inferior cerebellar peduncle 3 6 9 12 15 Somesthetic radiations Optic Nerve and tract Superior cerebellar peduncle Medium cerebellar peduncle Optic radiations Acoustic radiations Pyramidal tracts Reticular formation Fronto-pontine tracts 18 Anterior before posterior CNS . Spinal cord before brain . CNS myelination- LONG

5. White matter functions White matter constitute 60% of the brain Allows communication to and from grey matter areas, and between the grey matter and the parts of the body. Then grey matter serves to process information Develops throughout 20s and peak in middle age

6. Case scenario 1

7. History A 4 -year- old male child presents to the ER with seizures, confusion and irritability. He is normally developing child with average school performance and good social interaction. Mum gave history of common cold 10 days ago for which he was treated at home and recovered fully. Otherwise no history of concern

8. For the last week he became clumsy with frequent falls and had mild fever . For the last few hours before presentation to ER he had generalized tonic clonic seizures that continued for almost half an hour , after which he became confused with severe agitation and irritability .

9. No symptoms referable to other systems Parents are second degree cousins. No family history of developmental delay or regression. Mother had repeated first trimestric miscarriage. He is the eldest child having one male sibling a/w Otherwise no issues of concern .

10. What are the possibilities ?

11. Meningoencephalitis Cerebral malaria Acute Disseminated Encephalomyelitis (ADEM) Acute presentation of Neurodegenerative disorders. Epileptic encephalopathy

12. Examination C onfused, not oriented , Glasgow coma scale 10. Blood pressure 110/70 Pulse 110/min RR 38/min Temp 37.9 o C Negative Kernig's sign

13. Cranial nerves / intact Right UL:-hypotonia, hyporeflexia , power Grade 3+ Left UL/ normal Bladder / full Right & left - Lower limb:- hypotonia, hyporeflexia , power Grade 3+ Up going plantar response Examination of other systems reveals no abnormalities.

14. What are the possibilities ?

15. Meningoencephalitis ADEM Neurodegerative disorders Epileptic encephalopathy(LKS)

16. Investigations CBC / CRP MRI/ brain

17. 17

18. Treatment Intravenous immunoglobulin 2g/kg/5days Pulse methylprednisolone 30mg/kg/day for 5 days Continue with oral steroids for at least 8 weeks

19. Acute disseminated encephalomyelitis (ADEM) Is an immune-mediated inflammatory demyelinating condition that predominately affects the white matter of the brain and spinal cord. The disorder manifests as an acute-onset encephalopathy associated with poly-focal neurologic deficits.   ADEM bears a striking clinical and pathological resemblance to other acute demyelinating syndromes (ADS) of childhood, including multiple sclerosis (MS).

20. Full recovery is seen in 50 to 70% of cases, ranging to 70 to 90% recovery with some minor residual disability  A verage time to recover is one to six months The mortality rate may be as high as 5%-10%

21. Prognosis Poorer outcomes are associated with unresponsiveness to steroid therapy, unusually severe neurological symptoms, or sudden onset . Children tend to have more favorable outcomes than adults, and cases presenting without fevers tend to have poorer outcomes ADEM can progress to MS

22. Approach to a child with acute loss of vision.

23. History 14 years old female Presented with acute loss of vision and headache . She was quite well before her presentation. No family history of similar condition Not preceded by any viral infection.

24. Examination Well grown, pubertal female Intact higher functions Normal vital signs Neurological examination was normal apart from:- Right pupil: dilated, fixed, with absent direct and consensual reflexes and no perception of light Left eye:- sluggish reaction to light

25. Funduscopy Papillitis with hyperemia and swelling of the disk, blurring of disk margins, and distended veins is seen in one-third of patients with optic neuritis Two-thirds of these patients have retrobulbar neuritis with a normal funduscopic examination

26. Differential Diagnosis Optic neuritis Retrobulbar neuritis Multiple Sclerosis Vasculitis(Sickle cell disease, SLE, autoimmune disorders……)

27. Investigations MRI with orbital cuts with gadolinium contrast V isual evoked response  — A delay in the P100 of the visual evoked response (VER) is the electrophysiologic manifestation of slowed conduction in the optic nerve as a result of axonal demyelination 

28. Methylprednisolone 30mg/kg/day (maximum 1 gram per day)for 3-5 days Oral prednisolone 2mg/kg/day for 6-8 weeks. IVIG Plasmapheresis Treatment

29. Optic neuritis I s an inflammatory, demyelinating condition that causes acute, usually monocular, visual loss. It is highly associated with multiple sclerosis (MS). Optic neuritis is the presenting feature of MS in 15 to 20 percent of patients and occurs in 50 percent at some time during the course of their illness.

30. Optic neuritis usually comes on fairly quickly and can cause eye pain and vision loss within a few days or weeks. The visual field defect in optic neuritis is typically characterized as a central scotoma

31. Other possible causes of optic neuritis. H erpes zoster A utoimmune disorders (e.g. SLE) Methanol poisoning B12 deficiency Diabetes M S inus thrombosis Others ……..

32. Approach to a child who suddenly stop walking

33. History A 13 year old male , from Jazeera State, who was quite well before , Presents to the ER with difficulty walking and urine retention for the last 10 days. Condition was progressive .

34. Examination Stable vital signs Normal respiratory rate and pattern Intact cranial nerves LL Paraparesis- power grade III- hypotonia and hyporeflexia Sensory level(L1 ) Planter response- up Full bladder Back: no kyphoscoliosis – no area of tenderness Upper limbs – normal Cranial nerves – intact Higher function- Intact Fundus Normal

35. Differential diagnosis Guillian Barre syndrome . Poliomyelitis . Spinal cord compression * Epidural (metastases, haematoma, abscess, bony compression) *Intradural (neurofibroma) *Intramedullary ( ependymoma, glioma, hydromyelia ) Spinal cord pathology:- *Transection (Trauma). * Spinal cord inflammation (Transverse myelitis ) * Vascular pathology (Anterior Spinal Artery occlusion, vascular malformations) *Discitis .

36. MRI spine with contrast

37. Acute Transverse Myelitis(ATM) Acute transverse myelitis (ATM), an inflammatory myelitis, is one of the causes of acute transverse myelopathy. The three main categories in the differential diagnosis of ATM are demyelination, including multiple sclerosis (MS), neuromyelitis optica (NMO), and idiopathic transverse myelitis .

38. Demyelination of one or more segments of the spinal cord resulting in acute or sub acute onset of symptoms and signs of severe cord dysfunction with motor, sensory and sphincter disturbance. One third – full recovery

39. Treatment IVIG + Pulse methyl + oral steroids Physical therapy Occupational therapy Psychotherapy

40. Pediatric MS Multiple sclerosis (MS) is typically considered to be a disease of young adults. However , pediatric MS, defined as onset of MS before the age of 18, is increasingly recognized and accounts for approximately 5 percent of cases and previously known as early onset MS (EOMS) and juvenile MS. Etiology is a complex interplay between environment, genetics, infection and vitamin D deficiency.

41. It present as relapsing-remitting course with first attack usually consisting of optic neuritis, transverse myelitis, acute disseminated encephalomyelitis and monofocal or polyfocal neurological deficits.  Signs are often discrete and isolated and include unilateral optic neuritis , weakness , spasticity and sensory symptoms.

42. MRI/Brain in MS

43. Immunomodulatry drugs Steroids IVIG I nterferon β Azathioprine Rituximab Others

44. Take home message Do not miss a treatable condition