RESUMO
INTRODUCTION: Episodes of recurrent bacterial meningitis can occur in patients due to either congenital or acquired disorders. Congenital deformity of the bony labyrinth can be linked to a fistulous tract communicating it with the intracranial subarachnoid space. Mondini deformity is a frequent malformation in congenitally deaf patients. CASE REPORT: We report the case of an adolescent with a history of being unable to hear in one ear who, from the age of 10 years, began to suffer repeated bacterial meningoencephalitis with microbiological recovery of Streptococcus pneumoniae on three occasions. The type of germ recovered in the cerebrospinal fluid (CSF) and the history of congenital deafness that was detected when the patient was 3 years old were the diagnostic clues to the possible anomaly of the inner ear with a CSF fistula. The clinically proven CSF rhinorrhea contributed to the diagnosis of an ear anomaly with a fistula. Computerised axial tomography and magnetic resonance studies of the petrous portion of the temporal bone revealed the malformation that was later found and closed during the surgical intervention on the affected ear. The clinical absence of rhinorrhea, a year's progression without new infections after operating on the patient and post-surgery imaging studies were all proof that the fistula had closed. CONCLUSIONS: Mondini dysplasia with CSF fistula must be included as a possible diagnosis when faced with a patient with recurrent bacterial meningoencephalitis. Imaging studies, especially magnetic resonance, enable the clinician to check the diagnosis and the CSF fistula can be closed with ear surgery.
Assuntos
Surdez/etiologia , Orelha Interna/anormalidades , Doenças do Labirinto , Meningites Bacterianas , Adolescente , Rinorreia de Líquido Cefalorraquidiano , Criança , Pré-Escolar , Surdez/patologia , Orelha Interna/cirurgia , Feminino , Fístula/diagnóstico , Fístula/patologia , Fístula/cirurgia , Humanos , Doenças do Labirinto/líquido cefalorraquidiano , Doenças do Labirinto/diagnóstico , Doenças do Labirinto/patologia , Meningites Bacterianas/líquido cefalorraquidiano , Meningites Bacterianas/complicações , Meningites Bacterianas/diagnóstico , Meningites Bacterianas/patologia , RecidivaRESUMO
INTRODUCTION: Myasthenia gravis (MG) shows specific clinical features in children. It is essential to know this and also the use of diagnostic techniques used in infancy and childhood for correct diagnosis. OBJECTIVES: To analyze the clinical behaviour of this disorder and the use of complementary tests in the diagnosis of paediatric patients. PATIENTS AND METHODS: We studied a group of 12 children diagnosed as having MG, who were admitted to the Instituto de Neurolog a y Neurocirug a de Ciudad de La Habana (Cuba) between March 1997 and June 2001. Data were obtained from the clinical histories regarding the clinical picture, anticholinesterase test, repetitive stimulation test (RST), simple fibre test (SFT), computerized axial tomography (CAT) of the mediastinum and the treatment given in each case. RESULTS: Juvenile myasthenia gravis (JMG) presented in 91% of the patients studied. The average age of onset of JMG was 7.45 years, with no difference in presentation in the two sexes. The extrinsic muscles of the eye were most affected and the form with generalized clinical involvement predominated at the time of admission. The RST was positive in four of the nine patients in whom it was done (44%) and the SFT was positive in the six cases in which it was done. No changes were found in the mediastinum on CAT scanning. Mestinon and prednisone were the most commonly used drugs. CONCLUSION: In our group JMG was the most frequent form seen. Neurophysiological studies were very useful diagnostic tools.
Assuntos
Miastenia Gravis/diagnóstico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Mediastino/anatomia & histologia , Miastenia Gravis/fisiopatologia , Testes Neuropsicológicos , Músculos Oculomotores/fisiopatologiaRESUMO
INTRODUCTION: The term progressive myoclonic epilepsy (PME) includes a groups of heterogeneous conditions, with genetic causes, characterized by having different types of seizures, basically myoclonic, and other neurological findings due to a progressive lesion of the central nervous system. OBJECTIVE: To demonstrate the aetiology and clinico-encephalographic changes seen in patients with PME. PATIENTS AND METHODS: A retrospective, descriptive study was done of patients attended for PME in the Instituto de Neurología y Neurocirugía de Cuba between 1990 and 1995. Eighteen patients were included. All were interviewed and had a physical examination, EEG and the specific complementary tests for each aetiology. RESULTS: There was a predominance of neural ceroid lipofuschinosis in 10 patients (55.5%), and in 9 of these the illness started before the age of 9 years. The second most frequent condition was myoclonic epilepsy with red-torn fibres (16.6%) and Unverricht-Lundborg disease (16.6%). The latter began in late childhood or adolescence. The most marked clinical characteristics were epilepsy, which was difficult to control and intellectual deterioration in 100%, followed by cerebellar signs in 88.8%. Myoclonias were the commonest type of seizures (94.4%) and many children presented with prior tonic-clonic seizures (88.8%). CONCLUSION: Response to treatment was poor but the best results were obtained using valproate either alone or associated with benzodiazepines.
Assuntos
Epilepsias Mioclônicas Progressivas/diagnóstico , Adolescente , Adulto , Antidepressivos/uso terapêutico , Criança , Pré-Escolar , Eletroencefalografia , Feminino , Humanos , Lactente , Masculino , Epilepsias Mioclônicas Progressivas/tratamento farmacológico , Estudos Retrospectivos , Ácido Valproico/uso terapêuticoRESUMO
1,052 medical histories of patients discharged from "Pedro Borrás Astorga" Pediatric Teaching Hospital with a diagnosis of non-urlean viral meningoencephalitis are reviewed. These cases occurred in the period October 1985-March 1986 and they were followed regularly on an outpatient basis for 1 year. The discharges were comparatively analyzed as regards months in the semester, age, sex, nutritional status, personal pathologic history, time from onset of disease to hospitalization, symptoms and signs of onset, diagnostic impression at hospitalization, evolution, and complications.
Assuntos
Meningoencefalite/microbiologia , Viroses , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
1,052 medical records of patients discharged from "Pedro Borrás Astorga" Pediatric Teaching Hospital, with a diagnosis of non-urlean viral meningoencephalitis in the period ranging from October 1, 1985 to March 31, 1986, were reviewed. In this study the medical records of three deaths were particularly analyzed by means of the data of highest interest such as: age, sex, color of skin, length of stay, diagnostic impression at admission, motive of admission, course of the disease, physical examination and complementary procedures, and particularly anatomopathologic results. Conclusions are provided.