Abstract:

Introduction: Acute disseminated encephalomyelitis (ADEM) is a demyelinating central nervous system disorder. The experience of children in our area is limited.

Purpose: We describe a cohort of consecutive children with ADEM.

Methods: Clinical and MRI of brain characteristics, treatment, and outcome of a series of ADEM hospitalized in PICU, Oran University Hospital center (Algeria) from January 2011 to May 2016 were reviewed.

Results: There were 07 males and 03 females. The mean age (median) at onset was 48 months [range, 6 –132 months]. All children had a prodromal event (infectious 08 cases or vaccination 02 cases). The mean interval between the febrile prodrome and the beginning of neurologic disturbance was 14 days (range, 3 to 30 days). Limb weakness (02 cases), ataxia (02 cases), ophthalmoplegia (02 cases), and acute hemiparesis (02 cases) were the most prominent initial findings. Aphasia was noticed in one patient. the Seizure was observed in 07 patients. Consciousness disturbance (n=5) evolved into a coma with cardiomyopathy of stress in one patient. CSF lymphocyte pleocytosis was found in 3 patients (9 – 208 cells/mm3). Initial MRI showed a deep gray matter involvement in 6 cases. Electroencephalogram (EEG) showed slowing of background activity in 04 patients. 05 patients were treated with IV immunoglobulin alone and 03 with high-dose IV methylprednisolone pulse. Among 10 patients, five had long-term neurological squeals.

Conclusion: We conclude that early diagnosis and prompt treatment of ADEM will probably reduce morbidity and the seizures are not uncommon in ADEM.

Keywords: Encephalopathy, ADEM, Brain Magnetic Resonance Imaging, Children, Outcome.

ABSTRACT:

Introduction: Refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE) are serious and frequent neurological emergencies in children associated with high morbidity and mortality. Despite this, we note the absence of published Algerian data. The aim of this work is to determine the demographic data, the etiologies, and the factors of progression in RCSE and SuperRCSE as well as the short-term prognosis.

Design: Prospective observational study with retrospective analysis of all the episodes of RCSE and Super-RCSE treated between January 1, 2008, and April 30, 2016. Setting: Pediatric Intensive Care Unit, University Hospital of Oran, Algeria. Patients: RCSE is defined as seizure activity that persists after administration of a first-line benzodiazepine (BZD) and a second-line antiepileptic drug (AED). Super-RCSE is defined as «SE that has continued or recurred despite 24 hours of general anesthesia. » Factors independently associated with Refractory and Super-RCSE were established through univariate and multivariable analyses. The outcome was evaluated using the Glasgow Outcome Score.

 Results: Among 443 patients with status epilepticus, 140 patients (31.6%) were afflicted with refractory status epilepticus and 25 (5.6%) with SRSE. The mean age of the entire cohort was 40.65 ± 2.16 months [1 – 180 months].135 patients (33.8%) had suffered previous seizures; RCSE was related to younger age (< 3 years) (OR 1.76; 95% CI 1,018 – 3,058) p=0.043, duration of status epilepticus >2 h (OR 3.096; 95% CI 1, 77 – 5, 39) p= 10⎺⁴, acute symptomatic etiologies (OR 3.609; 95% CI 2,112 – 6.166), Severe consciousness impairment (GCS < 13/15) before treatment (OR 18.55; 95% CI 8,202 – 41.972) p= 10⎺⁴ and Non respect of the therapeutic protocol upstream of PICU (OR 3.659; 95% CI 1,926 – 6.949) p= 10⎺⁴. Super-RCSE was independently associated with age greater than 3 years (OR 3.28; p=0.018), indeterminate etiology (OR 6.628; p=0.009), encephalitis (OR 4. 782; p=0.003) and use of Vasopressor medications (OR 4.239; p=0.007). Overall mortality was 83 (20.75%).

Conclusion The etiologies of CSE are heterogeneous, with a significant incidence of RCSE in the acute symptomatic group. Encephalitis was the determinant of the progression of CSE to Super-RCSE. The therapeutic success of which depends on whether or not the cause of CSE can be treated.

Keywords: Refractory status epilepticus; Super-Refractory status epilepticus; Etiology; PICU; Outcome; Children. Abbreviations: SE: Status Epilepticus, RCSE: Refractory Convulsive Status Epilepticus, NRCSE: Non-Refractory Convulsive Status Epilepticus, Super-RCSE: Super-Refractory Convulsive Status Epilepticus, BZD: Benzodiazepine, AED: Antiepileptic Drogue, CNS: Central Nervous System