RESUMO
Movement disorders and epilepsy rarely occur in the early stage of Creutzfeldt-Jakob disease (CJD) but have not been reported concurrently. We report on a 47-year-old patient with probable CJD who presented with generalized chorea and focal dystonia with myoclonic jerks on the right hand. Myoclonic jerks progressed to epilepsia partialis continua within 5 days of admission to the hospital. The diagnosis of our patient was compatible with probable CJD on the basis of clinical course, electroencephalogram, and diffusion-weighted magnetic resonance imaging findings, and presence of 14-3-3 protein in cerebrospinal fluid. To our knowledge, this is the first report of a case developing both movement disorders and epilepsia partialis continua in the early stage of the disease.
Assuntos
Síndrome de Creutzfeldt-Jakob/epidemiologia , Epilepsias Parciais/epidemiologia , Transtornos dos Movimentos/epidemiologia , Gânglios da Base/patologia , Núcleo Caudado/fisiopatologia , Síndrome de Creutzfeldt-Jakob/patologia , Diagnóstico Diferencial , Eletroencefalografia , Epilepsias Parciais/diagnóstico , Epilepsias Parciais/fisiopatologia , Feminino , Giro do Cíngulo/fisiopatologia , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Transtornos dos Movimentos/diagnóstico , Transtornos dos Movimentos/fisiopatologia , Putamen/fisiopatologia , Índice de Gravidade de Doença , Tálamo/fisiopatologiaRESUMO
We report on a 63-year-old man presenting with hemichorea on his right side. Magnetic resonance imaging revealed a left putaminal cavernoma. To our knowledge this is the first report of such a case caused by contralateral putaminal cavernous angioma.
Assuntos
Doenças dos Gânglios da Base/diagnóstico , Neoplasias Encefálicas/diagnóstico , Coreia/etiologia , Dominância Cerebral/fisiologia , Hemangioma Cavernoso do Sistema Nervoso Central/diagnóstico , Putamen , Doenças dos Gânglios da Base/fisiopatologia , Neoplasias Encefálicas/fisiopatologia , Córtex Cerebral/patologia , Córtex Cerebral/fisiopatologia , Coreia/fisiopatologia , Lateralidade Funcional/fisiologia , Hemangioma Cavernoso do Sistema Nervoso Central/fisiopatologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Exame Neurológico , Putamen/patologia , Putamen/fisiopatologiaRESUMO
The nature and intensity of the association of myasthenia gravis (MG) with distinct human leukocyte antigens (HLA) haplotypes differ between ethnic populations. The aims of the present study were to examine the relationship between HLA class I and II haplotypes and MG; to show the HLA associations with various MG subsets; and to investigate the association between MG and clinical subgroups of multiple sclerosis (MS) regarding HLA haplotypes. A total of 66 patients with MG were enrolled onto the study. The mean age at onset was 42.01 years. A total of 122 clinically definite MS patients and 188 healthy subjects were examined as control groups. The present study clearly showed associations with HLA-DR3, -B8, -A1, and -A2 in MG. In patients with early-onset MG, associations with HLA-DR3, -B8, and -A2 were stronger. When compared with MS, in the MG group, there was still a strong association with -B8, -DR3, and -A1. In subgroup analysis, there was no difference between MG and primary progressive MS patients. On the basis of the presence of anti-AChR antibodies, there was a statistically significant association with HLA-DR3. On the basis of presence of thymoma, no HLA allele showed clear associations in MG patients with thymoma. This is the first study to examine the relationship between HLA haplotypes and MG in the Turkish population and to compare MG with another autoimmune disease, MS, on the basis of the HLA haplotypes. Further investigations with a larger population are required to explain this finding.
Assuntos
Antígenos HLA/genética , Esclerose Múltipla/genética , Miastenia Gravis/genética , Adolescente , Adulto , Idoso , Testes Imunológicos de Citotoxicidade , Feminino , Frequência do Gene/genética , Genótipo , Antígenos HLA/imunologia , Antígenos HLA-A/genética , Antígenos HLA-A/imunologia , Antígenos HLA-B/genética , Antígenos HLA-B/imunologia , Antígenos HLA-DR/genética , Antígenos HLA-DR/imunologia , Teste de Histocompatibilidade , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/imunologia , Miastenia Gravis/imunologia , TurquiaAssuntos
Blefarospasmo/fisiopatologia , Distúrbios Distônicos/fisiopatologia , Estimulação Elétrica/métodos , Córtex Motor/fisiopatologia , Torcicolo/fisiopatologia , Estimulação Magnética Transcraniana , Adulto , Idoso , Estudos de Casos e Controles , Eletromiografia/instrumentação , Eletromiografia/métodos , Potencial Evocado Motor/fisiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculos/fisiologia , Inibição Neural/fisiologia , Tempo de ReaçãoRESUMO
We studied the excitability of the motor cortex in patients with migraine without aura (MWOA) (n = 20) and with chronic migraine (CM) (n = 20) using transcranial magnetic stimulation (TMS). By using a 90-mm circular coil placed over the vertex and recording of the first dorsal interosseous muscle, we measured thresholds, latencies and amplitudes of motor evoked potentials and duration of cortical silent periods in patient groups and in controls (n = 20). No differences were found between groups for threshold, latency and amplitude values. However, the duration of the cortical silent period was longer in CM patients, being significantly different from both controls and MWOA. We suggest that either this difference in cortical excitability may develop during transformation from MWOA to CM or different pathophysiological mechanisms may play a role in these two headache syndromes.