1 - Departmento de Pediatria, Hospital Central do Funchal, Madeira
2 - Departamento de Neurorradiologia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
3 - Neuropediatria, Departamento de Pediatria, Hospital Prof. Dr. Fernando Fonseca, Lisboa
4 - Unidade de Cuidados Intensivos Pediátricos, Departamento de Pediatria, Hospital Prof. Dr. Fernando Fonseca, Lisboa
5 - Unidade de Cuidados Intensivos Pediátricos, Área de Pediatria, Hospital de Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
6 - Unidade de Neuropediatria, Hospital de Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
- Publicação no Neurology Clinical Practice, Sept 23, 2020
Resumo: Frequency of pediatric WE is estimated to be similar to WE in adults, although it is an underdiagnosed condition. In our reports, the nutritional deficit (caused by persistent vomiting and prolonged partial parenteral feeding) triggered signs and symptoms of WE. Therefore, pediatric WE should be considered as a differential diagnosis in all patients at risk for nutritional deficiency, increasing clinical suspicion and early treatment. Although typical of adult alcoholics, WE still remains under- diagnosed in pediatric and nonalcoholic population. The classic triad of encephalopathy, ophthalmoparesis and ataxia is present in only 16% of WE.1,4 Therefore, it is extremely important to have a high level of clinical suspicion, especially in conditions that could lead to thiamine deficiency. In situations of prolonged parenteral nutrition, thiamine supplementation is crucial. Brain MRI is helpful in diagnosis and follow-up, with high specificity and medium sensitivity in WE.2 Typical findings consist of symmetrical lesions with T2/FLAIR hypersignal in the thalamus, mammillary bodies, periaqueductal area, and tectal plate. These findings were present in both cases. Symmetric hyposignal in the same areas may be visible in the T1-weighted sequence. Atypical findings, as lesions in putamina and cerebral cortex, may also be seen. A few weeks after the onset of encephalopathy, there will often be atrophy of mammillary bodies, a phenomenon well described in alcoholic patients, and this was also noted in case 1.5 After treatment, neuroimaging changes may completely resolve, as documented in case 2, or persist. The latter is associated with worse prognosis. Although highly valuable, neuroimaging should not delay the beginning of treatment when there is high clinical suspicion. Clinical condition is sufficient to establish a diagnosis and justify emergent treatment, even before laboratory confirmation. If proper treatment is not started early, permanent neurologic deficit and death may occur. There are no defined guidelines for the treatment of pediatric WE. However, guidelines in WE in adults may guide the adapted therapeutic regimens. In patients at risk for WE oral absorption of thiamine is unreliable and intravenous treatment is recomended with 200 mg tid.
Palavras Chave: early treatment, intravenous, thiamine, Wernicke Encephalopathy.