A 12 years old boy with behavioral disorders

1. A 12 years old boy with behavioral disorders F.Ahmadabadi Child neurologist

2. Problems list • Behavioral disorder • Seizure • Delirium • MRI involvement(Frontal lobe) • CSF PCR (Enteroviral involvement) • EEG Changes(Frontal lobe ) • Hypo pigmented Patch

3. Sudden onset Behavioral disorder • Psychogenic (PTSD-Acute stress disorders-Psychosis) • Epilepsy • Brain injury • Trauma • Metabolic (Hepatic) • Drugs • Infections(direct-Immune)

4. Definitions Encephalopathy defined as: • Two of following findings • Seizure • Loss of consciousness • Behavioral disorders Encephalopathy+ Inflammatory changes in CSF  Encephalitis

5. Encephalitis • Most viruses reach the CNS hematogenously,except Rabies and HSV1. • Viruses induce neurologic signs and symptoms by damaging neural cells directly or by stimulating host-dependent immune responses • HSV…………….Direct damage………….…Hemorrhagic necrosis • JC ……………...oligodendrocytes………..Progressive demyelination • Induction of Immune system….………ADEM (10-15%of Encephalitis)

6. Types of involvement • Aseptic Meningitis • Encephalitis • Guillain-Barre Syndrome • Bells palsy • Acute cerebellar Ataxia • Myositis • Myelopathy

7. Viruses Associated with Aseptic meningitis and Encephalitis • Meningitis • Nonpolio enterovirus • HSV 1,2 • Adenovirus • LCMV • EBV • HIV • St Luis • Mumps Virus • Colorado tick fever • Encephalitis • St Luis • Japanese Encephalitis • West Nile • EBV • Rabies • HIV • VZV • CMV • Enterovirus 71 • Mumps Virus

8. Diagnostic Evaluation • CSF/A • EEG • CSF/C • CSF/PCR • Brain MRI • EMG/NCV

9. CSF Analysis • Lymphocytic Pleocytosis(5-500 cells/ml) • Mildly elevation of Prt(50-200 mg/dl) • Normal Glucose • 5-15%patients with HSV encephalitis have an entirely normal initial CSFprofiles.

10. EEG Findings • Slowing • Epileptiform discharges • HSV: Slowing+lateralizing+localized sharp waves(Temporal) • 40-50% of La crosse or eastern equine encephalitishave focal and lateralizing changes in EEG

11. Imaging(MRI ) • HSV (non neonatal): • T2 prolongation in medial temporal lobe-orbitofrontalregion or cingulate gyrus+ cortical enhancement(GDM) • Neonatal HSV • Edema(focal or diffuse) • Japanese or EBV encephalitis • Basal ganglia • Neuroinvasive West Nile • Substantia nigra • ADEM • Diffuse white matter involvement

12. Cont, • In polio and coxsackie virus infections, T2-weighted MRI may show hyperintensities in the midbrain and anterior horn of spinalcord. • In EBV infection hyperintensities in the basal ganglia and thalami may be observed on T2-weighted MRI • MRI of Japanese encephalitis can show hyperintensities in bilateral thalami, brainstem and cerebellum.

13. Herpes Virus • Neurologic disease during in utero-Perinatal-Postnatal • HSV1-2(Neonatal infection-Encephalitis) • CMV9Intrauterine infection) • EBV(GBS-Encephalitis) • HHV6-7(FC) • Varicella (Ataxia-Encephalitis) • Oral transmission(HSV1) • Hemiparesia-Dysphasia-visual field defect-Seizure(40%) • Predilection for frontotemporal area. • Bells palsy- Behaviorial disturbance-Recurrent aseptic meningitis-Necrotizing myelopathy of childhood

14. Herpes Virus Cont, • Diagnosis: DNA PCR - MRI - EEG • Treatment: • 20mg/kg/q 8h for 21 days in encephalitis repeat PCR (CSF) on 21th day and if it remains positive should be continuedfor another 7 days.

15. Picorna viruses • Members of this family specially enterovoruses are important causes of neurologic syndromes. • Aseptic meningitis-GBS-Acute ataxia-Acute Hemiplegia-Opsoclonus-Myoclonus-Encephalitis may be seen due to these. • Aseptic meningitis is the most common neurologic manifestation of NPE s. • Occasionally non polio infection cause an acute encephalitic presentation with Fever-Somnolence – Coma or focal deficits as like as HSV.(71) • Children with agammaglobulinemia can have fetal persistent condition and may have progressive cortical atrophy.

16. Picorna viruses cont, • Traetment: • Supportive cares • Severe nonpolio encephalitis Pleconaril • AgammaglobulinemiaIVIG • Outcome in Myocarditis or encephalitis (71) is less favourable

17. Abstract • We report a 4-year-old, left-handed male with focal coxsackievirus A3 encephalitis who presented with seizures and acquired aphasia. Electroencephalography exhibited focal spike discharges over the right frontal regions, but cranial magnetic resonance imaging did not reveal any structural abnormalities. However, brain single-photon emission computed tomography performed during the acute phase disclosed focal hypoperfusion in the right frontal lobe, consistent with decreased regional cerebral blood flow in the territory of some branches of the right cerebral anterior artery. Without specific treatment, the patient recovered completely within 1 month, when brain single-photon emission computed tomography images returned to normal and cranial magnetic resonance imaging still demonstrated no abnormalities. The present case suggests the possible role of transient local cerebral vasculitis in the pathogenesis of focal enterovirus encephalitis.

18. Abstract • MR imaging studies of 20 patients showed hyperintensity in the brain stem and spinal cord in 15 patients, as seen on T2-weighted images. The major CNS lesions were in the medulla oblongata, pons, midbrain, and the dentate nuclei of the cerebellum. In some cases, the lesions involved the spinal cord (three cases) as well as the thalamus (two cases) and putamina(one case). Five patients had normal MR imaging results. The presence of EV 71 was detected in specimens from 18 patients. • CONCLUSION: Because EV 71 was identified in 18 patients, and no other virus was detected, EV 71 was determined to be the major causative agent of this encephalomyelitis. Brain stem and cervical spinal cord involvement are characteristic findings of enteroviral encephalomyelitis.

19. Abstract Limbic encephalopathy • Symptoms & Signs: • Subacute memory loss,behaviorialdisorders,Seizures,High signal in MTLS(MRI),CSF variable ,Hyponatremia • Median age:65 years, M/F= 2:1 • May be a paraneoplastic syndrome but due to voltage-gated potassium channelopathy.(VGKC)