Wernicke's encephalopathy — causes to consider

Abstract

Wernicke's encephalopathy (WE) is a thiamine deficiency disorder and is characterized clinically by the triad of ocular abnormalities, ataxia and disturbances of consciousness. We report on 3 patients with WE, of whom 2 had insufficient thiamine substitution. In the first patient symptoms disappeared during thiamine substitution. In the second patient acute WE was the terminating event in the sequence of parenteral nutrition, lactic acidosis and cardio-pulmonary decompensation. Possibly due to hereditary deficits WE developed in the third patient despite sufficient thiamine substitution. Attention to thiamine deficiency should be paid in all patients with history of alcoholism, malnutrition, malabsorption, tumors, inflammation, other severe diseases and in parenteral hyperalimentation. In order to prevent WE thiamine should be substituted with at least 100 mg/day i.v. or i.m.

Magnetic resonance imaging in a case of Wernicke's encephalopathy

“…In fact, some authors report that in the chronic form, lesions can be restricted to mammillary bodies and the thalamus, as in our case [3], but the periaqueductal region, midbrain, reticular formation and tectal plate can also be commonly involved [4].…”

“…Neuropathologically, blood-brain-barrier dysfunction and oedema-associated local hypoperfusion cause a neuronal loss that can be demonstrated in selected brain areas including the periventricular regions, the medial thalamic nuclei, the massa intermedia, the floor of the third ventricle and the mammillary bodies [3,4].…”

“…Among vitamins, thiamine plays an important role as a coenzyme both in the Krebs and in the pentose-phosphate cycles. In its absence, glucose metabolism is impaired, causing pyruvate and lactate accumulation and adenosine triphosphate stores depletion [2].Clinically, this condition is associated with a highoutput haemodynamic status, known as shoshin-beriberi, and with the occurrence of Wernicke's encephalopathy (WE) which is characterized by the triad of ophthalmoplegia, ataxia and consciousness disturbances.Neuropathologically, blood-brain-barrier dysfunction and oedema-associated local hypoperfusion cause a neuronal loss that can be demonstrated in selected brain areas including the periventricular regions, the medial thalamic nuclei, the massa intermedia, the floor of the third ventricle and the mammillary bodies [3,4].At present, MR imaging is considered the most valuable imaging technique to diagnose WE, since cranial CT normally fails to demonstrate significant density alterations and is positive only in exceptional cases [5].We describe the case of a patient who developed a full-blown WE due to a prolonged post-operative starvation. …”

Acute lactic acidosis with Wernicke's encephalopathy due to acute thiamine deficiency

“…Unless thiamine is rapidly supplemented, refractory acidosis and death may occur in few hours or a day [3]. Many situations, such as chronic alcoholic intoxication, overfeeding after starvation, gastroplasty, hyperemesis gravidarum, anorexia nervosa, total parenteral nutrition without thiamine supplementation may induce thiamine deficiency [4]. In our patient the fast and dramatic improvement in both confusion and lactic acidosis after thiamine administration strongly favors this diagnosis [5].…”

Wernicke's encephalopathy: Unusual manifestation on MRI

“…Also recognized predisposing conditions other than alcoholism include chronic dietary deprivation, and impaired absorption or intake of dietary nutrients [2]. Neuropathologically, blood-brain-barrier dysfunction and edema-associated local hypoperfusion cause a neuronal loss that can be demonstrated in selected brain areas including the periventricular regions, the floor of the third ventricle and the mammillary bodies [3]. At present, MRI is considered the most valuable imaging technique to diagnose WE.…”