RESUMO
A 2-year-old child who acutely developed hemiplegia and seizure was found to have moyamoya disease and heterozygous protein S deficiency. This case report should alert physicians to the possible coexistence of moyamoya disease and protein S deficiency, even in the case of typical moyamoya disease. The intimate relationship between the two require further study.
Assuntos
Doença de Moyamoya/diagnóstico , Deficiência de Proteína S/diagnóstico , Angiografia Digital , Encéfalo/irrigação sanguínea , Encéfalo/patologia , Angiografia Cerebral , Pré-Escolar , Triagem de Portadores Genéticos , Humanos , Imageamento por Ressonância Magnética , Masculino , Doença de Moyamoya/genética , Deficiência de Proteína S/genética , Tomografia Computadorizada por Raios XRESUMO
Between 1980 and 1989, 21 children suffering from intractable seizures other than infantile spasms were treated with intramuscular ACTH at the Children's Hospital Camperdown. Five patients had two courses of ACTH therapy, 24% of patients had a good response (group A), 56% responded transiently (group B) and 20% did not respond (group C). Group A had normal development and no neurological deficits prior to seizures. A favourable response was not observed in patients with partial seizures, 90% of the patients who responded had a recurrence of seizures. Mean time to recurrence was 9.0 +/- 7.3 months in group A and 1.6 +/- 2.0 months in group B. Hypokalaemia, hypertension and infection were found in 42.9%, 33.3% and 19.1% respectively. ACTH also had effects on concurrent anti-epileptic drug levels.
Assuntos
Hormônio Adrenocorticotrópico/uso terapêutico , Convulsões/tratamento farmacológico , Adolescente , Hormônio Adrenocorticotrópico/efeitos adversos , Criança , Pré-Escolar , Resistência a Medicamentos , Feminino , Humanos , Lactente , Masculino , Convulsões/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
Hearing loss is one of the serious complications of bacterial meningitis. Conventional audiometry and auditory brainstem response (ABR) methods were used to detect this complication in eighteen children who recovered from bacterial meningitis treated at Ramathibodi Hospital from January 1983 to December 1987. Six patients (33%) were found to have persistent bilateral sensorineural hearing loss. Among them, the causes of meningitis were: Hemophilus influenzae (3 patients), Streptococcus pneumoniae (1 patient), Streptococcus agalactiae (1 patient) and Escherichia coli (1 patient). Various clinical and demographic factors were examined in relation to the hearing loss, but no significant correlation was observed. Since meningitis often affects small children and makes conventional audiometry tests difficult, ABR was found to be a more effective method for testing this group of patients.
Assuntos
Infecções Bacterianas/complicações , Perda Auditiva Neurossensorial/etiologia , Meningite/complicações , Audiometria , Infecções Bacterianas/líquido cefalorraquidiano , Criança , Pré-Escolar , Potenciais Evocados Auditivos , Feminino , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/epidemiologia , Hospitais Urbanos , Humanos , Lactente , Masculino , Meningite/líquido cefalorraquidiano , Fatores de Risco , TailândiaRESUMO
Moyamoya disease is a cerebrovascular disorder characterized by occlusion of the internal carotid arteries with cerebral angiographic finding of smoke-like collateral vessels. Typical moyamoya blush can also be seen in immediate enhanced CT scan of the brain. The disease is rare in non Japanese. The present communication reports four Thai children aged 3-12 years, presented at the Department of Pediatrics, Ramathibodi hospital with recurrent hemiparesis. In one patient, brain biopsy was almost carried out. The disease should be suspected in every child with recurrent cerebral ischemic episodes. Cerebral angiogram and immediate enhanced CT scan are the two most useful investigation procedures. Either of them should be selected first for diagnosis before other unnecessary invasive investigations are done.