Adems
Download as PPTX, PDF0 likes133 views
1. The document describes a case of a 4-year old boy who presented with slurred speech and difficulty swallowing along with fever and altered mental status. 2. MRI of the brain showed lesions in multiple areas including the basal ganglia, pons, midbrain, medulla, thalami and frontal cortex. CSF analysis was normal. 3. The boy was diagnosed with Acute Disseminated Encephalomyelitis (ADEM), an initial inflammatory demyelinating condition affecting multiple areas of the brain, and treated with steroids which improved his symptoms.
Adems
- 1. Acute Disseminated Encephalomyelitis (ADEM) Dr. Pawan Kumar Barolia MBBS MD (pediatrics) Fellowship in intensive care pediatrics
- 2. Case • A 4-year-old boy was brought to our Hospital with complaints of slurring of speech and difficulty in swallowing and fever for 2 days. He was evaluated at emergency and his GCS was 10/15 (E4V2M4). On examination, he was in altered sensorium; tone was increased in upper and lower limb with brisk deep tendon reflexes. Plantars were upgowing and cranial nerve examination was normal. No meningeal sign was present. He was started on with IV antibiotic (ceftriaxone), acyclovir and supportive measures. Prior to this he had fever for 5 days, 8 days back along with mild cough and cold. On Day 4 of fever child had one episode of seizure.
- 3. • He was admitted in the intensive care unit of a local hospital and managed. MRI of brain was done which was unremarkable except for mildly dilated lateral and third ventricle. CSF cytobiochemistry was normal. He was managed with IV Ceftriaxione and IV Acyclovir IV Phenytoin and other supportive measures. Gradually he improved and was discharge in a stable condition after 6 days Two days later patient developed slurring of speech and difficulty in swallowing with fever for which he was admitted in our hospital
- 4. • Initial investigations revealed Hb - 11.8 gm/dl, TLC - 15200/mcl, platelet - 3.13 lac. RFT and LFT were normal. CRP was 17 mg/L, Typhi dot, Widal test, Blood C/S and Malarial antigen test was negative. MRI of brain was done which showed inhomogeneous area of increased signal in T2 weighted images in basal ganglia, pons, midbrain, medulla, thalami, and frontal cortex. Fundoscopy was normal. Lumbar puncture revealed CSF cell count was 3 cells (100% lymphocytes), Protein - 32 mg/dl and Glucose - 56 mg/dl (capillary blood glucose was 86 mg/dl) LDH - 12 U/L
- 5. • CSF Bacterial antigen test causing encephalitis (Streptococcus Group B, H. influenzae b, S. pneumoniae, N. meningitidis, E. coli, herpes IgM test and herpes PCR were negative. EEG revealed generalized cerebral dysfunction with no definitive irritable foci. Diagnosis of ADEM was considered. Child was started on IV methylprednisolone (500 mg) pulse therapy for 3 days, following which he was started on oral steroid therapy. He showed good response and speech improved. Gradually his sensorium became better. By Day 5, he started responding to commands..
- 6. • On day 6 visual evoked potential was done which revealed perception of light bilaterally. Power in upper and lower limb gradually improved • He was discharged and at the time of discharge he was conscious oriented with GCS of E4 V5 M6. He was seen on follow up after 7 days and neurological examination revealed alert child with mild slurring of speech
- 7. MRI T2 weighted image showing areas of hyper intensity involving brainstem MRI T2 weighted image showing areas of hyper intensity involving caudate nucleus and thalamus
- 8. Acute Disseminated Encephalomyelitis (ADEM) • an initial inflammatory, autoimmune, • Demyelinating event with multifocal neurologic deficits, • Typically accompanied by encephalopathy. • ADEM can occur at any age • mean age between 5 and 8 yr • slight male predominance • Incidence ranges from 0.07-0.4 per 100,000 per yr in the pediatric population
- 9. • Molecular mimicry induced by infectious exposure or vaccine may trigger production of CNS autoantigens • Many patients experience a transient febrile illness in the month prior to ADEM onset • Preceding infections - influenza, Epstein-Barr virus, cytomegalovirus, varicella, enterovirus, measles, mumps, rubella, herpes simplex, and Mycoplasma pneumoniae. • Postvaccination - smallpox, measles, mumps, rubella, Japanese encephalitis B, pertussis, diphtheria-polio-tetanus, and influenza.
- 10. Clinical features • Initial symptoms of ADEM may include lethargy, fever, headache, vomiting, meningeal signs, and seizure, including status epilepticus • Encephalopathy is a hallmark of ADEM • Ongoing confusion to persistent irritability to coma • Focal neurologic deficits can be difficult to ascertain in the obtunded or very young child • Visual loss, cranial neuropathies, ataxia, motor and sensory deficits, plus bladder/bowel dysfunction with concurrent spinal cord demyelination.
- 11. Neuroimaging • Head CT may be normal or show hypodense regions • Cranial MRI, the imaging study of choice • typically exhibits large, multifocal and sometimes confluent or tumefactive T2 lesions with variable enhancement within white and often gray matter of the cerebral hemispheres, cerebellum, and brainstem • Deep gray matter structures (thalami, basal ganglia) are often involved although this may not be specific to ADEM • Spinal cord may have abnormal T2 signal or enhancement, with or without clinical signs of myelitis
- 12. • MRI lesions of ADEM typically appear to be of similar age but their evolution may lag behind the clinical presentation • Serial MRI imaging 3-12 mo following ADEM shows improvement and often complete resolution of T2 abnormalities although residual gliosis may remain • No biologic marker for ADEM • CSF - pleocytosis with lympho or monocytic predominance • CSF protein can be elevated, especially in repeat studies • Up to 10% of ADEM - oligoclonal bands in the CSF and/or elevated CSF immune globulin production
- 13. Investigations • Electroencephalograms (EEG) often show generalized slowing, consistent with encephalopathy • Polyregional demyelination of ADEM can also cause focal slowing or epileptiform discharges • D/D for ADEM is broad but can be narrowed by careful history, appropriate laboratory evaluations, and MRI. • Empirical antibiotic and antiviral treatment should be considered while infectious evaluations are pending
- 14. Investigations • Follow-up MRI examinations 3-12 mo after ADEM should show improvement • New or enlarging T2 lesions should prompt re-evaluation for other etiologies such as MS, leukodystrophies, tumor, vasculitis, or mitochondrial, metabolic, or rheumatologic disorders
- 15. Treatment • High dose intravenous steroids are commonly employed (typically methylprednisolone 20-30 mg/kg per day for 5 days with a maximum dose of 1,000 mg per day) • An oral prednisone taper over 1 mo may prevent relapse • Other treatment options include intravenous immune globulin (IVIG; usually 2 g/kg administered over 2-5 days) • Plasmapheresis (typically 5-7 exchanges administered every other day)
- 16. Prognosis • Many children experience full recovery after ADEM • some are left with residual motor and/or cognitive deficits • ADEM is usually a monophasic illness but demyelinating symptoms can fluctuate for several months • Repeated bouts of demyelination more than 3 mo after ADEM later raise the question of MS versus repeated ADEM.