RESUMO
Wernicke's encephalopathy (WE) is a neuropsychiatric condition generally caused by acute thiamine deficiency and classically involves the triad of altered mentation, ataxia and ophthalmoplegia. It is most common among alcoholics, but several other causes have been identified, including total parenteral nutrition (TPN) use. We present eight cases of WE in patients undergoing allogeneic BMT, where thiamine deficiency was caused by a lack of vitamin supplementation during TPN administration. Clinically, WE presented as a severe refractory metabolic acidosis, preceded by 'raspberry tongue', and ophthalmologic and neurologic dysfunction. The sites most affected were the periventricular structures and the thalamus, and no mammilary bodies lesions were found.
Assuntos
Transplante de Medula Óssea/efeitos adversos , Encefalopatia de Wernicke/etiologia , Acidose/etiologia , Acidose/patologia , Artérias , Encéfalo/patologia , Coma/etiologia , Coma/patologia , Endotélio/irrigação sanguínea , Endotélio/patologia , Hemorragia/etiologia , Hemorragia/patologia , Humanos , Doença Iatrogênica/epidemiologia , Bulbo/irrigação sanguínea , Bulbo/patologia , Transplante Homólogo , Encefalopatia de Wernicke/patologiaRESUMO
Alcohol abuse is one of the most serious problems in public health and the Wernicke-Korsakoff syndrome is one of the gravest consequences of alcoholism. The pathology is often undiagnosed in its less evident presentations, therefore an accurate diagnostic approach is a critical step in treatment planning. Treatment is based on restoration of thiamine, although this is insufficient to prevent the psychological decline of a great number of patients. The cognitive impact of the pathology is derived from the interaction of alcoholic neurotoxicity, thiamine deficiency and personal susceptibility. In this article, the literature concerning Wernicke-Korsakoff syndrome is reviewed.
Assuntos
Transtorno Amnésico Alcoólico/fisiopatologia , Doenças Metabólicas/fisiopatologia , Encefalopatia de Wernicke/fisiopatologia , Transtorno Amnésico Alcoólico/etiologia , Transtorno Amnésico Alcoólico/história , Transtorno Amnésico Alcoólico/patologia , Transtorno Amnésico Alcoólico/terapia , Alcoolismo/complicações , História do Século XIX , Humanos , Doenças Metabólicas/etiologia , Doenças Metabólicas/história , Doenças Metabólicas/patologia , Doenças Metabólicas/terapia , Prognóstico , Síndrome , Tiamina/uso terapêutico , Deficiência de Tiamina/complicações , Encefalopatia de Wernicke/etiologia , Encefalopatia de Wernicke/história , Encefalopatia de Wernicke/patologia , Encefalopatia de Wernicke/terapiaRESUMO
Wernicke's encephalopathy is a disease that when not diagnosed in time, can lead to severe CNS damage and death. It is characterized by specific clinical and radiologic findings; however, there are few reports dealing with the usefulness of neurophysiologic studies in this syndrome. We present a patient with Wernicke's encephalopathy, diagnosed postmortem on autopsy, in whom the neurophysiologic studies showed abnormalities in all the different pathways tested. This suggests that EEG and evoked potentials may be useful tools in the diagnostic workup of this entity. We propose performing these tests when this syndrome is being considered.
Assuntos
Encéfalo/patologia , Eletroencefalografia , Encefalopatia de Wernicke/diagnóstico , Adulto , Feminino , Humanos , Imageamento por Ressonância Magnética , Encefalopatia de Wernicke/patologiaRESUMO
A morphometric study was made of the neurons in the medial mammillary nucleus from 25 cases of Wernicke's encephalopathy (WE) and from 15 other cases (control group) in which hepatic or brain disease was excluded and the risk factors associated with WE were not present. The whole area of the mammillary body was also measured in all cases from both groups. The cases of WE were divided into acute and chronic. A highly significant reduction in the area of the mammillary body was noted in the chronic WE group, when compared to the control and acute WE cases. Although no significant differences were detected between the three groups with regard to the total number of neurons of medial mammillary nucleus, cell density was significantly higher in the chronic WE group, when compared to the control and acute WE cases. A significant reduction was noted in the nuclear volume of the neurons in the chronic cases of WE, when compared to the acute WE cases. The positive correlation between the area of the mammillary body and the nuclear volume of the neurons in the medial mammillary nucleus was highly significant for the WE group and significant for the control and chronic WE groups.(ABSTRACT TRUNCATED AT 250 WORDS)
Assuntos
Corpos Mamilares/patologia , Encefalopatia de Wernicke/patologia , Doença Aguda , Adolescente , Adulto , Idoso , Contagem de Células , Núcleo Celular/ultraestrutura , Doença Crônica , Feminino , Humanos , Cariometria , Masculino , Pessoa de Meia-Idade , Neurônios/patologiaRESUMO
Numa série de 36 casos de encefalopatia de Wenicke autopsiados, 11 haviam desenvolvido coma. Em nenhum desses pacientes comatosos o diagnóstico foi estabelecido durante a vida. Seis desses pacientes eram homens e 5 mulheres, com idades entre 26 a 50 anos (média de 36,6). História de alcoolismo crônico foi positiva em 7 casos, sinais de desnutriçäo grave ocorreram em 3; uma paciente apresentava câncer gástrico e uma, hiperemese gravídica. Dois pacientes foram admitidos ao hospital após terem sido encontrados em coma em suas residências. O exame neuropatológico revelou alteraçöes macroscópicas nos corpos mamilares em 8 casos - atrofia, descoloraçäo e espongiose. Atrofia da porçäo anterior e superior do vermis do cerebelo foi observada em um caso. Em 5 casos havia hemorragias petequiais no diencáfalo, principalmente próximas às paredes do terceiro ventrículo. A microscopia revelou além das hemorragias, proliferaçäo glial, hipertrofia do endotélio vascular e necrose de neurônios e mielina. Mielinólise pontina central ocorreu em um paciente. A encefalopatia de Wernicke é pouco diagnosticada clinicamente. O coma pode mascarar as outras manifestaçöes clínicas da doença ou pode constituir sua única manfiestaçäo. Embora seja um sinal de mau prognóstico, o coma pode ser revertido pela administraçäo de tiamina. É enfatizado que todo paciente em coma de causa näo estabelecida deve receber altas doses de tiamina
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Coma/diagnóstico , Encefalopatia de Wernicke/patologia , Alcoolismo , Coma/etiologia , Encefalopatia de Wernicke/complicações , Encefalopatia de Wernicke/mortalidadeRESUMO
O autor apresenta dados neuropsicológicos clínicos sobre os distúrbios associados ao alcoolismo crônico e discute o uso de exame neuropsicológico nestes pacientes
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Alcoolismo/complicações , Transtornos Cognitivos , Transtorno Amnésico Alcoólico/diagnóstico , Encefalopatia de Wernicke/diagnóstico , Encefalopatia de Wernicke/etiologia , Encefalopatia de Wernicke/patologiaRESUMO
Eleven out of 36 autopsied cases of Wernicke's encephalopathy had developed coma. None of these patients had the diagnosis during life. There were six men and five women with ages ranging from 26 to 50 years (mean 36.6). Seven of these patients were heavy drinkers, three exhibited signs of severe malnutrition, whereas one was being evaluated for a disseminated gastric cancer and one was in treatment of hyperemesis gravidarum. Two patients were brought to the hospital after found unconscious at home. Neuropathological examination disclosed gross changes in the mammillary bodies in eight cases and microscopic changes in all cases. In one case there was atrophy of the anterior superior part of the vermis. Petechial hemorrhages were observed particularly in the walls of the third ventricle. Microscopically there were in addition to hemorrhages, glial proliferation, endothelial hypertrophy and necrosis of nerve cells and myelin. Central pontine myelinolysis was observed in one case. Wernicke's encephalopathy is a clinically underdiagnosed condition. Coma may mask its classical clinical picture or even be the sole manifestation. Although coma points to a poor outlook it may be reversed by thiamine administration. Any patient with coma of unknown etiology should be given parenteral thiamine.
Assuntos
Coma/patologia , Encefalopatia de Wernicke/patologia , Adulto , Alcoolismo/complicações , Coma/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Encefalopatia de Wernicke/complicações , Encefalopatia de Wernicke/mortalidadeRESUMO
A case of Wernicke's encephalopathy associated with promyelocytic leukemia found at autopsy is reported. The patient was 30 years old and was undergoing chemotherapeutic treatment when she had a memory deficit for recent events (Korsakoff's syndrome) which persisted for 6 months, until death. The neuropathologic examination showed typical, old lesions that characterize Wernicke's encephalopathy, but only in the mamillary bodies. This case is compared with three other cases of Wernicke's encephalopathy associated with leukemia previously described in the literature. Comment is made on the cliniconeuropathologic picture and risk factors in leukemic patients that may favor the appearance of Wernicke's encephalopathy.
Assuntos
Transtorno Amnésico Alcoólico/diagnóstico , Leucemia Promielocítica Aguda , Encefalopatia de Wernicke/diagnóstico , Adulto , Encéfalo/patologia , Diagnóstico Diferencial , Feminino , Humanos , Leucemia Promielocítica Aguda/patologia , Encefalopatia de Wernicke/patologiaRESUMO
La encefalopatía de Wernicke, se debe al déficit de tiamina, relacionándose principalmente con alcoholismo y malnutrición. Clasicamente se considera diagnóstica la triada constituída por alteraciones de conciencia, transtornos motores oculares y ataxia. Estudios recientes demuestran que el 80
Assuntos
Tiamina/uso terapêutico , Deficiência de Tiamina , Encefalopatia de Wernicke/patologia , Alcoolismo/complicações , Furosemida/uso terapêutico , Antibacterianos/uso terapêutico , Fenitoína/uso terapêuticoRESUMO
En un estudio prospectivo de 175 alcohólicos autopsiados en un período de 2 años se investigó la frecuencia de 4 entidades neuropatológicas asociadas a alcoholismo crónico y su relación con otras enfermedades. La más frecuentemente encontrada fue la atrofia del vermis cerebeloso en su parte superior y en los ejemplos en los que se consideró acentuada, se pudo establecer una correlación clara entre duración e intensidad del alcoholismo. La encefalopatía de Wernicke se diagnosticó en 13 encéfalos y fue en todos de tipo crónico y confinada a los tubérculos mamilares. Hubo 4 casos de mielinolisis central del puente. En ninguno se pudo probar la existencia de elevación brusca del sodio a partir de niveles de hiponatremia. No hubo en esta serie ejemplos de necrosis cortical laminar de Morel. En los resultados se presentan también las enfermedades asociadas directa e indirectamente al alcoholismo. Otras enfermedades diagnosticadas en la autopsia son las frecuentemente encontradas en el nivel socioeconómico del Hospital General de México, S. S