1. POSTER TITLE: CLINICAL AND RADIOLOGICAL PROFILE, TREATMENT AND OUTCOME OF PEDIATRIC ACQUIRED DEMYELINATING DISORDERS OF CENTRAL NERVOUS SYSTEM
PRESENTER NAME:Deepthi Shetty, Vykuntaraju K Gowda, Basavaraj GV, Naveen Benakappa, Asha Benakappa
INTRODUCTION AND KEYWORD:Demyelinating disorders are rare childhood disorders. These include acute disseminated encephalomyelitis(ADEM), Multiple sclerosis(MS), Transverse myelitis, optic neuritis and Neuromyelitis optica. These disorders culminate in various physical and cognitive disabilities. Timely diagnosis and institution of appropriate treatment determines prognosis and outcome.
KEYWORDS: Demelinating disorders, ADEM, Multiple sclersois, Transverse myelitis, Optic neuritis, Neuromyelitis optica
AIM:To study clinical profile, investigative and radiological features, treatment and outcome of children with demyelinating disorders.
MATERIALS AND METHODS:A retrospective chart review of children with demyelinating disorder admitted in tertiary care pediatric hospital of Bangalore between Jan 2013 and Aug Demographic data, clinical features, CSF findings, radiological features, treatment received and outcome were collected and results analysed.
RESULTS: Among 26 patients of demyelinating disorders, majority were ADEM-13(50%), followed by transverse myelitis-7 (26.92%), multiple sclerosis 3 (11.54%), optic neuritis- 2 (7.69%) and NMO- 1 (3.85%). Median age of presentation was 8.5years. Among 26 patients, 12 were male (46.15%).
In ADEM, median age was 6 years. Among 13 patients, 12(92.13%) had pyramidal features like hemiparesis(6/12, 50%) with or without facial palsy, quadriparesis(4/12, 33.3%) with or without facial palsy and paraparesis(2/12, 16.6%). Encephalopathy was seen at presentation in 10 (76.92%) patients, during hospital stay all patients developed encephalopathy. Convulsion was present in 4 patients(30.77%), neurogenic bladder in 3 patients(23%), sensory involvement in 2(15.38%) patients. CSF analysis was done in 9 patients, which was found to be normal in 4 patients and abnormal in 5 patients ( i.e.,pleocytosis, increased protein). MRI brain done shows hyperintensities in subcortical white matter of supratentorial region, basal ganglia, thalamus, cerebellum, corpus callosum, cervical- thoracic cord. Basal ganglia involvement was found in 5 (41.6%) patients and spinal cord involvement in 2 (11.6%) patients. Two patients had Tumefactive ADEM. 10 recovered with residual disability in 3 patients after steroid therapy.
In MS, median age was 9 years, 2 were female, . Fever preceded most of the events in all 3 with encephalopathy in all 3 patients during acute events( during 1st episode in 2 patients and during 2nd episode in other patient). Other features include convulsions(2/3, 66.67%), left hemiparesis(2/3, 66.67%), meningism(2/3, 66.67%), neurogenic bladder(1/3, 33.33%), paresthesia(1/3). Eye involvement was seen in 2 patients. Recurrent history present in all 3 patients. CSF pleocytosis with positivity for oligoclonal bands is seen in 1 patient. MRI brain done shows multiple demyelinating plaques involving supratentorial, infratentorial, corpus callosum, cerebellum, brainstem, cervical cord with new lesions in repeat MRI. 1 patient died and 2 patients improved.
In transverse myelitis, median age was 11 years. 5 had cervical and 2 thoracic cord involvement. . Neurogenic bladder present in all patients , sensory disturbances in 4(57.14%) patients. 6 patients recovered with 1 death.
A patient with NMO presented with headache and diminution of vision. , CSF was positive for antibody to aquaporin 4. Child improved with steroids and IVIG.
Two patients with optic neuritis presented with headache and diminution of vision. were treated with steroids and they recovered.
CONCLUSION:Demyelinating disorders are not uncommon in children. Most common is ADEM. Multiple sclerosis is not rare in children, accounting for 11% in present study. Early diagnosis and treatment with steroids improves outcome.
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Shelfali Gulati, Biswaroop Chakrabarty, Atin Kumar, Puneet Jain et al. Acquired demyelinating disorders of central nervous system: A pediatric cohort. Ann Indian Acad Neurol Sep; 18(Suppl 1): S48-S55
Mahesh Kamate, Vivek Chetal, Venkatesh Tonape, Niranjana Mahantshetti, Virupakshi Hattiholi. Central nervous system inflammatory demyelinating disorders of childhood. Ann Indian Acad Neurol Oct-Dec; 13(4):