RESUMEN
Pernicious anemia is uncommon in Africa. The purpose of this report is to describe a case of pernicious anemia observed in Madagascar. The revealing manifestation was encephalomyelitis with combined medullar sclerosis that responded favorably to vitamin B12 replacement therapy. Clinical symptoms included paresthesia associated with allodynia of all four extremities and with tetrapyramidal syndrome, medullar ataxia and minor cognitive disturbances ongoing for 5 months. Hemogram testing revealed macrocytic anemia. Serum cobalamin level was low. Anti-intrinsic factor antibody was detected. Spinal cord magnetic resonance imaging showed diffuse high-signal intensity along the posterior spinal cord extending from C1 to C4. Vitamin B12 replacement therapy led to full regression of clinical signs after six weeks. Association of central nervous system involvement with macrocytic anemia suggests vitamin B12 deficiency and pernicious anemia should be suspected. This disease can be considered as a curable form of myelitis in Africa and Madagascar.
Asunto(s)
Anemia Perniciosa/diagnóstico , Encefalomielitis/etiología , Anemia Perniciosa/tratamiento farmacológico , Encefalomielitis/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Clima Tropical , Vitamina B 12/uso terapéutico , Vitaminas/uso terapéuticoRESUMEN
The authors report a patient who had five relapses of encephalopathy with seizures and aseptic meningitis that coincided with the end of the menstrual cycle. High titers of antithyroid antibodies and the patient's response to corticosteroids suggested Hashimoto's encephalopathy. Pharmacologic suppression of the menstrual cycle made it possible to withdraw corticosteroids.
Asunto(s)
Encefalopatías/etiología , Ciclo Menstrual/fisiología , Tiroiditis Autoinmune/complicaciones , Adulto , Antiinflamatorios/uso terapéutico , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Encefalopatías/patología , Encefalopatías/fisiopatología , Femenino , Humanos , Imagen por Resonancia Magnética , Ciclo Menstrual/efectos de los fármacos , Prednisolona/uso terapéutico , Recurrencia , Convulsiones/etiología , Convulsiones/fisiopatología , Tiroglobulina/análisis , Tiroiditis Autoinmune/patología , Tiroiditis Autoinmune/fisiopatología , Tomografía Computarizada por Rayos XAsunto(s)
Cateterismo/efectos adversos , Embolia Intracraneal/etiología , Venas , Adulto , Infarto Cerebral/etiología , Trombosis Coronaria/complicaciones , Trombosis Coronaria/diagnóstico por imagen , Trombosis Coronaria/etiología , Remoción de Dispositivos , Ecocardiografía Transesofágica , Humanos , Masculino , Trastornos del Habla/etiologíaRESUMEN
Pallido-luysio-nigral atrophy (PLNA) is a rare neurodegenerative disease in which the clinical and radiologic correlates have not yet been clearly established. A 62-year-old man insidiously developed dystonic postures, choreoathetoid movements, slowness, and stiffness, which initially affected the right hand and foot and progressively spread to the entire right side. T2-weighted magnetic resonance imaging showed increased signal intensity in both left and right medial pallida and in the left substantia nigra. Tests using HMPAO-SPECT and FDG-PET demonstrated left cortical hyperperfusion and hypermetabolism, whereas the left lenticular nucleus was slightly hypometabolic. At age 65, abnormal movements and postures involved all four limbs and the axis causing major gait disturbances, and facial and bulbar muscles atrophied resulting in dysarthria, dysphagia, and impaired breathing. Diffuse amyotrophy and fasciculations also appeared. Death occurred at age 66, 4 years after onset. At autopsy, severe bilateral neuronal loss and gliosis restricted to the pallidum, the subthalamic nucleus, the substantia nigra, and the hypoglossal nucleus were noted, accounting for the diagnosis of PLNA with lower motor neuron involvement. Progressive hemidystonia with adult onset represents an unusual clinical presentation for this disorder. Moreover, this observation indicates that a diagnosis of PLNA should be considered for specific magnetic resonance imaging, SPECT, and/or PET data, and suggests that in PLNA, pallidal dysfunction might play a key role in the dystonic presentation.