Clinical and laboratory features of childhood acute disseminated encephalomyelitis (CADEM): Experience at the children's hospital-Lahore

Background: the central nervous system (cns) acquired demyelinating disorders of childhood include both self-limited and lifelong conditions,which can be indistinguishable at the time of initial presentation study type: clinical observational period: from 1 st jan 2006 to 31 st dec 2008. place: department of neurosciences,the children's hospital,lahore,pakistan. aim of study: to present clinical manifestations of childhood acute disseminated encephalomyelitis (cadem) with neuroimaging correlation,response to treatment and outcome.patient and methods: over the period of three years (2006-2008),25 cases among the 40 children admitted in neurology wards with clinical diagnosis of disseminated demyelination of nervous system had confirmed cadem on mri scans of brain. epidemiological variables,clinical course,risk factors for mortality,cerebrospinal fluid (csf) analysis,electroencephalogram (eeg),neuroradiological findings and treatment data were analyzed. results: data were analyzed on 40 children admitted with clinical picture of non-specific encephalopathy. of these 25 (14 males,11 females) children were found to have cadem,who constituted the study population. mean age was 8.8 years with a male/female ratio of 1.2. a polysymptomatic presentation occurred in 80% and 76% of the cases had evidence of antecedent febrile illness. impaired consciousness (100%),motor signs (80%),seizures (50%) and nuchal rigidity (30%) were the most common neurological findings. investigation abnormalities were: evidence of inflammation (62%),csf (56%),eegs (100%),cts brain (50% of done) and mris brain (100%). patients were administered steroid pulse therapy in 20 cases (75% improved) and immunoglobulin in 10 cases (60% improved). 4 patients died and acute hemorrhagic encephalomyelitis (ahem) was the highest mortality risk among these patients. all patients had some neurological morbidity at the time of discharge,however,majority of them are improving. two patients in our series relapsed but are stable on oral azathioprine therapy. conclusion: cadem is a potentially severe demyelinating cns disorder with predominantly encephalytic clinical picture and is treatable. mri brain is the imaging of choice and documentation of ahem is of grave prognosis among these patients. further studies are required to understand cadem pathogenesis,to improve diagnostic and treatment strategies.