الفهرس 
Acute Disseminated EncephalomyelitisOnly 14 pages are availabe for public view
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Abstract
Summary
This partially prospective and partially retrospective study assessed children who presented with ADEM over a period of 19 months (from February 2014 to September 2015). The children were followed up till December 2015. Data of already registered cases of ADEM at the paediatric neurology clinic at Ain Shams University hospitals, over the preceding 5 years, was re-evaluated and described. Patients’ outcome at follow up was correlated with their initially collected data.
Patients with CNS illness not fulfilling diagnostic criteria of ADEM, as well as known cases of collagen disorders, vasculitides, hemophagocytic syndrome or other conditions with similar clinical presentation to ADEM were excluded.
Our study enrolled a total of 21 patients; 14 new and 7 old ones. There were 11 males and 10 females with a mean age of 4.4 years.
• As regards the demographic data of patients, fifty two percent of the studied subjects were males and 48% were females. 90% of the patients were under 10 years old at onset of disease.
• More than half of the cases (52%) occurred in spring, followed by summer, where 24% of the cases were reported.
• About 38% of the cases were post vaccinal, and 42.9% were post infectious. The vaccine most implicated in precipitating disease was the OPV; which was alone responsible for 66.6% of the post vaccinal cases. The commonest infection, according to history given by the caregiver, was unspecified URTI at 44%, followed closely by GE at 33%, and lastly acute follicular tonsillitis at 22%.
• As regards the clinical presentation, all patients had altered mental status at presentation. Seizures were present in 71.4%. The commonest seizure type was GTC. Fever was seen in 71.4% of the patients.
• As regards findings on clinical examination, pyramidal signs were observed in all patients, followed by cranial neuropathy (42.8%), and then extrapyramidal signs occurring in one third of the patients. Meningeal irritation signs were seen in 9.5%, and headache in 4.8%.
• Lateralization was observed in 19% of patients. Three out of 4 patients who showed lateralization did so in the form of hemiplegia.
• The commonest cranial neuropathy observed was pseudobulbar palsy, seen in 9 out of 7 patients.
• Among patients with extrapyramidal manifestations, the vast majority (62%) had dystonia, followed by choreo-athetosis seen in 25%. Ataxia was the least commonly found movement disorder, seen in only 1 patient (13%).
• As regards the CBC, it is of note that 47% of patients were lymphopenic, 35% were anemic and a striking 70% had microcytosis. About 30% had thrombocytosis whereas only 1 patient (5.9%) was thrombocytopenic.
• CRP was positive in 35% of patients and serum sodium was low (hyponatemia) in almost two thirds of the studied subjects.
• As regards the CSF analysis, CSF was positive for cells in only 25% of the cases. The cells were mostly lymphocytes, and their count was low with a mean of 5 cells permm3. CSF glucose was normal for all cases. CSF proteins were elevated in 50% of the cases and low in only one patient (6.2%). CSF oligoclonal bands were negative in all studied cases.
• As regards neuroimaging findings, CT scanning was abnormal in only 10% of the patients for whom CT was ordered as the primary neuroimaging modality (10 patients). A striking 90% of these patients had completely normal CT brain.
• MRI Lesions were typically bilateral and asymmetrical (86%). The juxta-cortical and deep white matter were almost invariably involved (about 80% of cases), followed by the thalami, which were involved in 30% of the cases. Cortical grey matter and periventricular white matter, as well as basal ganglia and cerebellum, were involved in 19% of the cases. The commonest pattern was that of patchy and rather small lesions (71.4%), followed by confluent/ larger areas seen in 19% of cases.48% of cases had diffusion restriction in their MRI lesions. Contrast enhancement was seen in the MRIs of only 14% of cases. A positive mass effect and/or diffuse cerebral edema were seen in association with the demyelinating plaques in two cases. Both of these patients died.
• As regards EEG, it was completely normal in one third of the cases for which EEG was available (n=15), and was abnormal in two thirds. Among patients who demonstrated an abnormal EEG (n=10), the commonest finding was diffuse background slowing, observed in 90% of the cases. One patient had generalized epileptiform discharge, another had generalized status epilepticus, and a third had epilepsia partialis continua.
• As regards treatment, among the seven old patients 5 out of 7 did not receive immune-modulating therapy. All new patients were given some form of immunomodulation. However, contrary to consensus, 48% of total and upwards of 60% of those who were actually treated received IVIG as a first line of treatment in the lieu of steroids. 28% of the total number of patients – 37% of those actually treated – received steroids as a first line of treatment
• All six patients who received corticosteroids showed prompt improvement with no need for further therapy.
• Out of the 10 patients who received IVIG as first line treatment, 6 patients (60%) failed treatment (remained disturbed for 7 or more days after starting treatment), and received steroids as second line treatment. They improved shortly afterwards. Only 2 patients (20%) showed adequate response to IVIG alone, whereas another 2 patients (20%) died a few days into treatment.
• All in all, 8 out of 14 surviving patients who received treatment (58%) had no sequelae at 12 weeks, versus only one patient (20%) of the untreated group. Moreover, that one untreated patient with no sequelae had recurrent ADEM a year later.
• Among the treated group, 2 patients died. And while no deaths were recorded among the untreated group, this group exclusively included the recorded data of old cases; which means that the data of those patients who might have died would not be available.
• The commonest sequels were motor and cognitive deficits, seen in 8 (42%) of patients with sequelae. Four patients (21%) had behavioral disturbances.
• The most common (56%) motor sequelae were focal deficits – hemiparesis- which where mild for the most part. Severe spastic quadriparesis was seen in 2 patients (22%).
• MRI was available at 3 months for all surviving new patients, and 6 out of seven old patients. In 8 out of 18 patients (45%), the MRI became completely normal. 6 out of 18 (33%) of the cases showed regression in their lesions without total resolution, while in 22% of the cases (4 patients) the lesions remained stationary with no interval regression.
• Resolution was the commonest fate in MRI of treated cases, seen in 8 out of 14 cases (57%). None of the untreated cases demonstrated resolution on MRI obtained after 3 months. Conversely, persistence of lesions on MRI was seen in 3 out of 4 (75%) of the untreated patients, versus only 1 of the 14 treated patients (7%).
• Among the new treated patients, one of the patients whose 3 months MRI didn’t demonstrate total resolution had a follow up MRI after 6 months and then at 1 year. His MRI showed further regression.
• Among the old treated patients (n=2), one of them had resolution of the lesions after more than one year, although he continued to demonstrate combined motor and cognitive deficits. The other child’s MRI demonstrated a stationary course of the lesions, yet her clinical deficits were less than those of the first child.
• Three out of the five old cases (60%) who did not receive treatment demonstrated encephalomalacia and gliosis on their 1 year MRI follow up, and demonstrated variable clinical outcomes from mild focal motor deficits to profound global delay and seizures.
• In one patient – namely the patient who had recurrent ADEM- the MRI showed progression of the lesions previously noted. The girl, however, made a complete clinical recovery with no sequelae.
• None of these untreated cases showed further resolution or regression of their previously noted lesions in the 3 months MRI study.