Asunto(s)
Anticuerpos Monoclonales Humanizados , Miastenia Gravis , Humanos , Anticuerpos Monoclonales Humanizados/uso terapéutico , Anticuerpos Monoclonales Humanizados/farmacología , Inactivadores del Complemento/uso terapéutico , Inactivadores del Complemento/farmacología , Miastenia Gravis/tratamiento farmacológico , Resultado del TratamientoRESUMEN
Pregnancy was not associated with deterioration of HAM nor was HAM associated with adverse pregnancy outcome in this case. These findings suggest that women with HAM/TSP, even those who use a wheelchair, should not be discouraged from pregnancy.
Asunto(s)
Mapeo Encefálico/métodos , Núcleos Cerebelosos/anomalías , Núcleos Cerebelosos/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Epilepsias Mioclónicas Progresivas/diagnóstico por imagen , Adulto , Mapeo Encefálico/normas , Femenino , Humanos , Imagen por Resonancia Magnética/normas , Epilepsias Mioclónicas Progresivas/fisiopatologíaRESUMEN
A 34-year-old man was admitted with his unsteady gait, difficulty in speech and a paroxysmal severe headache accompanied with sensori-motor disturbance of the right extremities and aphasic symptom. His family history was unremarkable. His unsteadiness has progressed very slowly from childhood. He noted to be inarticulate at the age of 18 years. At the age of 33 years, he suddenly had an attack of severe throbbing headache, which was mainly left parietal, with nausea and photophobia. During the headache, his right extremities were paralyzed and he became aphasic. He had lost a partial memory of the event All these symptoms had gone within 24 hours. Thereafter, the same headache occurred about once a month. Neurological examination revealed a mild truncal ataxia and ataxic dysarthria. Electroencephalography (EEG) showed intermittent delta waves restricted over the left fronto-temporal region. Brain MRI showed a moderate atrophy of superior cerebellar vermis and anterior cerebellar lobe. The diagnosis of sporadic hemiplegic migraine (SHM) with cerebellar ataxia was made. Our case was very similar to familial hemiplegic migraine (FHM) 1, of which some families are accompanied with transient amnesia, cerebellar ataxia and EEG abnormality. Although we did not detect any mutations in CACNA1A gene previously reported in FHM1, our case might share same pathogenesis with FHM1.