RESUMEN
BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder of the central nervous system. Little information is available about the clinical and neuroradiological profile or the follow-up of this disease in Tunisian children. AIM: To determine the clinical, laboratory, and radiological features and the outcome of ADEM in children admitted to the pediatrics department of a university hospital in Tunisia. METHODS: All children ≤ 18 years old presenting with ADEM and admitted to the tertiary referral center for pediatrics at Sahloul University Hospital from January 2000 to December 2020 were included in the study. The diagnosis of ADEM was confirmed according to the international pediatric multiple sclerosis study group criteria. RESULTS: A total of 20 patients (13 girls and 7 boys) fulfilled the diagnostic criteria for ADEM. The mean age at diagnosis was 5.6 years. The clinical presentation included polyfocal neurological signs such as cranial hypertension (45%), seizures (35%), and motor weaknesses (55%). Pyramidal tract signs and cranial nerve palsies were noted in 55% of cases. Brain magnetic resonance imaging showed particular features, namely, a relapsing tumor-like form in one case, and optic neuritis and demyelinating lesions of the white matter in the brain and the spinal cord with gadolinium cerebral ring-like enhancement in another case. The treatment consisted of intravenous immunoglobulin in 16 cases (80%) and corticosteroid in 19 cases (95%). Plasmapheresis was used for one patient. Complete recovery was observed in 12 patients (60%); 19 patients (95%) had a monophasic course of the disease while only one patient developed multiphasic ADEM. CONCLUSIONS: ADEM remains a difficult diagnosis in children. Nevertheless, after prompt diagnosis and adequate treatment, most children with ADEM have a favorable outcome with restitutio ad integrum.
Asunto(s)
Encefalomielitis Aguda Diseminada/diagnóstico , Adolescente , Corticoesteroides/uso terapéutico , Niño , Preescolar , Encefalomielitis Aguda Diseminada/clasificación , Encefalomielitis Aguda Diseminada/epidemiología , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Lactante , Masculino , Pediatría/métodos , Pediatría/estadística & datos numéricos , Estudios Retrospectivos , Túnez/epidemiologíaRESUMEN
Kawasaki disease is a generalized systemic vasculitis, which primarily affects medium-sized arteries. Kawasaki disease shock syndrome is a rare but severe presentation of this disease. This report describes a case of delayed diagnosis of Kawasaki disease shock syndrome in a 13-year-old boy who presented with cervical adenophlegmon, persistent fever, injected conjunctiva, rash, and hypotension. Echocardiography revealed the presence of bilateral coronary aneurysms. Early recognition of Kawasaki disease shock syndrome can be difficult; however, delay in diagnosis and treatment can increase the risk of coronary artery disease.