1 - Andreia Forno, MD, Department of Pediatrics, Hospital Central do Funchal, Madeira, Portugal
2 - Bruno Cunha, MD, Department of Neuroradiology, Centro Hospitalar Lisboa Central, Lisbon, Portugal
3 - Catarina Luís, MD, Neuropediatrics, Department of Pediatrics, Hospital Prof. Dr. Fernando Fonseca, Lisbon, Portugal
4 - Ana Castro, MD, Pediatric Intensive Care Unit, Department of Pediatrics, Hospital Prof. Dr. Fernando Fonseca, Lisbon, Portugal
5 - Marta Moniz, MD, Pediatric Intensive Care Unit, Department of Pediatrics, Hospital Prof. Dr. Fernando Fonseca, Lisbon, Portugal
6 - Carlos Escobar, MD, Pediatric Intensive Care Unit, Department of Pediatrics, Hospital Prof. Dr. Fernando Fonseca, Lisbon, Portugal
7 - Sérgio Lamy, MD, Pediatric Intensive Care Unit, Hospital de Dona Estefˆania, Centro Hospitalar Lisboa Central, Lisbon, Portugal
8 - Andreia Pereira, MD, Department of Neuropediatrics, Hospital de Dona Estefˆania, Centro Hospitalar Lisboa Central, Lisbon, Portugal
9 - Ana Martins, MD, Department of Neuropediatrics, Hospital de Dona Estefˆania, Centro Hospitalar Lisboa Central, Lisbon, Portugal
10 - Carla Conceição, MD, Department of Neuroradiology, Centro Hospitalar Lisboa Central, Lisbon, Portugal
- 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 extremelyimportant to have a high level of clinical suspicion, especially in conditions that could lead to thiamine deficiency. In situations of prolonged parenteral nutrition, thiamine sup- plementation 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 confir- mation. If proper treatment is not started early, permanent neurologic deficit and death may occur. There are no de- fined 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: Wernicke, encephalopathy,children.