Endocrine Abstracts

Introduction: Gayet-Wernicke encephalopathy (WE) is a rare neurological disorder, caused by thiamine (vitamin B1) deficiency. We report a case of tertiary adrenal insufficiency revealing Gayet-Wernicke encephalopathy.

Case presentation: A 45-year-old women was admitted with abdominal pain, vomiting and weakness. Her medical history was significant for a long-term self-medication with corticosteroids and chronic vomiting. Clinical examination revealed a cushingoid appearance (discrete facial erythrosis, protuberant abdomen, skin fragility). The Laboratory results revealed a natraemia of 136 mmol/l, a kalaemia of 4 mmol/l, and an 0800 h serum cortisol level of 50 ng/ml. The diagnosis of tertiary adrenal insufficiency was made and treatment with parenteral hydrocortisone hemisuccinate was started. The evolution was characterized by the development of nystagmus, confusional state and flaccid tetraparesis. Brain MRI was consistent with Gayet-Wernicke encephalopathy. Parenteral vitamin B1 was initiated. As a result the symptoms of nystagmus and confusion subsided, but the patient retained motor sequelae.

Discussion: Wernicke encephalopathy (WE) is an acute neurological condition resulting from thiamine deficiency, most often secondary to chronic alcoholism, but can also occur in the context of chronic vomiting. WE is characterized by a clinical triad of ophthalmoparesis with nystagmus, ataxia and confusion. Brain MRI may reveal hyperintense signaling in the periventricular thalamus, mammillary bodies and periaqueductal gray matter. Early and adequate treatment with thiamine is necessary to avoid death or progression to permanent brain damage.

Conclusion: Our case illustrates the seriousness of self-medication with corticosteroids ranging from a simple tertiary adrenal insufficiency to a serious pathology such as Gayet-Wernicke encephalopathy.

References: Wernicke Encephalopathy.Vasan S, Kumar A.2021 Aug 11. In: StatPearls.

Thiamine in the treatment of Wernicke encephalopathy in patients with alcohol use disorders.Latt N, et al. Intern Med J. 2014.