RESUMO
Se evaluaron 18 biopsias de nervio diagnosticadas como neuropatía por lepra y realizadas en el Instituto Nacional de Neurología y Neurocirugía de La Habana, Cuba, en el transcurso de toda su historia, y se revisaron las historias clínicas de todos los pacientes ingresados en el centro. La muestra se caracterizó por edad, sexo, cuadro clínico neurológico, nervios más comprometidos, lesiones de piel, estudios electrofisiológicos y descripción del estudio anatomopatológico. La mayoría de los pacientes eran del sexo masculino y sus edades en el 100/100 eran las reconocidas como laboralmente activas. El patron neuropático más frecuente fue el mononeuropático múltiple y la calidad, sensitiva pura o mixta, fue predominantemente sensitiva. Los nervios más afectados fueron el cubital en 10 pacientes y el mediano en 6. Las lesiones de piel más frecuentes fueron las máculas anestésicas. La anatomía patológica mostró infiltrado inflamatorio en todos los pacientes y el bacilo estuvo ausente sólo en uno
Assuntos
Hanseníase , Manifestações Neurológicas , Manifestações Cutâneas , CubaRESUMO
INTRODUCTION: Behçet's disease is a multisystemic illness in which neurological abnormalities are seen in 5-48% of cases. CLINICAL CASES: We present 3 patients (2 men and 1 women) diagnosed as having Behçet's disease, according to the criteria of the 'International Study Group for Behçet's Disease' of 1990. We describe the clinical findings in these patients, the results of investigations done (MR or CT, evoked potentials-PEV, PESS, PEATC-EEG, ECN, CSF and neuropsychological tests), their course and response to treatment with hyperimmune human gammaglobulim at a dose of 400 mg/kg body weight in 2 patients. All three patients had repeated episodes of meningitis and alterations of the cranial nerves; two patients had epileptic seizures and two had signs of cerebellar disorders and dementia at some time during their illness. One patient died and the other two survived with severe disabilities. Cranial CT showed a cerebral infarct in one patient; evoked potentials showed axon damage in the two patients in whom this was studied. In the only patient in whom ECN was done, signs of axonal polyneuropathy were found. In all three patients there was pleocytosis in the CSF. In the patients in whom hyperimmune human gammaglobulin was given, the results were: one improved after this treatment; in the other there was clinical deterioration, but this was considered to be secondary to a curettage done the day before. CONCLUSION: The presence of neurological findings in Behçet's disease not caused by cerebral vein thrombosis worsens the prognosis of these patients.
Assuntos
Síndrome de Behçet/fisiopatologia , Adulto , Potenciais Evocados , Feminino , Humanos , Masculino , Testes Neuropsicológicos , PrognósticoRESUMO
INTRODUCTION: Neuropathy due to leprosy is the most frequent cause of peripheral nervous system disorders due to an infective agent, one of the commonest aetiologies of peripheral neuropathy, and also one of the few peripheral neuropathies which is curable. Hansen's disease, initially and predominantly involves the skin, so it is not usually seen by a neurologist; a neurologist sees cases which are difficult to diagnose and often after the condition has been ruled out by other doctors from other medical specialties. PATIENTS AND METHODS: In the National Institute of Neurology and Neurosurgery of La Habana, Cuba, 18 nerve biopsies were studied (during its history), that had been diagnosed as leprous neuropathy. We reviewed the clinical histories of all the patients admitted to this centre. The sample was characterized by: age, sex, neurological clinical picture, nerves most affected, skin lesions, electrophysiological studies and a description of the anatomopathological findings. RESULTS AND DISCUSSION: Most patients were male and all (100%) were adults of 'working age' (19-65 years). The most frequent neuropathic pattern was multiple mono-neuropathy (78%) and the quality, purely sensitive or mixed was predominantly sensitive (100%). The nerves most affected were the ulnar (10 patients) and median (6 patients). The most frequent skin lesions were anesthetic maculae. Anatomo-pathological study showed inflammatory infiltration in all patients and the bacillus was absent in only one case.
Assuntos
Hanseníase/complicações , Hanseníase/epidemiologia , Doenças do Sistema Nervoso Periférico/epidemiologia , Doenças do Sistema Nervoso Periférico/etiologia , Adulto , Idoso , Cuba/epidemiologia , Eletromiografia/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/diagnóstico , Debilidade Muscular/etiologia , Parestesia/diagnóstico , Parestesia/etiologia , Doenças do Sistema Nervoso Periférico/diagnóstico , Estudos RetrospectivosRESUMO
INTRODUCTION: Head injury is the commonest cause of symptomatic or secondary epilepsy and one of its most serious sequelas. Typical absence seizures are well defined clinically and electroencephalographically and are seen in age-related idiopathic epilepsies. There are very few descriptions of seizures of typical absences that were symptomatic of tumors or other structural lesions. CLINICAL CASE: We describe the case of a nine year old boy who had had a severe head injury at the age of four years. When he was seven years old he started to have seizures with all the clinical and electroencephalographic features of typical absences. CONCLUSIONS: In this case, taking the age of the patient into account, the APF, APP, electroclinical characteristics of the seizures, neurological and clinical condition, the problem was to decide whether the seizures were idiopathic or symptomatic of a cerebral lesion. This was important for treatment and prognosis. The answer could only be obtained by follow-up and assessment of the response to specific treatment for petit mal.
Assuntos
Lesões Encefálicas/complicações , Epilepsia Tipo Ausência/diagnóstico , Epilepsia Tipo Ausência/etiologia , Criança , Eletroencefalografia , Epilepsia Tipo Ausência/tratamento farmacológico , Humanos , Masculino , Índice de Gravidade de Doença , Sono REM/fisiologia , Ácido Valproico/uso terapêutico , Vigília/fisiologiaAssuntos
Cuba/epidemiologia , Debilidade Muscular/diagnóstico , Debilidade Muscular/etiologia , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/epidemiologia , Doenças do Sistema Nervoso Periférico/etiologia , Eletromiografia/métodos , Estudos Retrospectivos , Hanseníase/complicações , Hanseníase/epidemiologia , Parestesia/diagnóstico , Parestesia/etiologiaRESUMO
Introducción. El traumatismo craneoencefálico (TCE) es la causa más frecuente de epilepsia sintomática o secundaria, que, a su vez, constituye una de las secuelas más serias del TCE. Las crisis de ausencia típica están bien definidas clínica y electroencefalográficamente, y se observan en las epilepsias idiopáticas relacionadas con la edad; muy raramente se han descrito crisis de ausencia típica sintomáticas a tumores u otras lesiones estructurales. Caso clínico. Describimos el caso de un niño de 9 años de cada con antecedentes de TCE grave sufrido a los 4 años. A los 7 comienza a presentar crisis con todas las características clínicas y electroencefalográficas de las crisis de ausencia típicas. Conclusiones. En el caso que nos ocupa, y teniendo en cuenta la edad del paciente, los APF, los APP, las características electroclínicas de las crisis y el cuadro clínico neurológico, la problemática reside en precisar si estas crisis son idiopáticas o sintomáticas a la lesión cerebral, con la importancia que tiene esta precisión desde el punto de vista terapéutico-pronóstico. La respuesta sólo la puede dar el seguimiento evolutivo del paciente y la respuesta al tratamiento anti "petit mal" específico(AU)