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Thiamine deficiency in amyotrophic lateral sclerosis
  1. Sarah Jesse1,
  2. Dietmar R Thal2,
  3. Albert C Ludolph1
  1. 1Department of Neurology, University of Ulm, Ulm, Germany
  2. 2Laboratory of Neuropathology, Institute of Pathology, University of Ulm, Ulm, Germany
  1. Correspondence to Professor Albert C Ludolph, Department of Neurology, University of Ulm, Oberer Eselsberg 45, Ulm 89081, Germany; Albert.Ludolph{at}rku.de

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Introduction

Thiamine deficiency is related to malnutrition and causes severe neurological disorders such as Wernicke encephalopathy (WE). In cases of deficient thiamine intake, thiamine storages last for a maximum of 2–4 weeks. There are well-known risk factors for WE including medication, parenteral application of carbohydrates, malabsorption, reduced intake, emesis and catabolism.

Amyotrophic lateral sclerosis (ALS) is a prototype motor neuron disease (MND) with degeneration of anterior horn cells in the spinal cord, motor nuclei of cranial nerves, and pyramidal cells of the motor cortex. This causes progressive pareses of skeletal, intercostal and diaphragmal muscles, leading to respiratory insufficiency and dysphagia, which results in weight loss and malnutrition.

In this report, we highlight neuropathological signs of WE in two patients with ALS/MND which were unexpectedly found at autopsy. These findings prompted a prospective assessment of thiamine status in patients with ALS/MND in the University of Ulm Neurology Department.

Case 1

The patient reported dysarthria, dysphagia, weight loss, fasciculations and breathlessness during exercise. At an external hospital, ALS/MND was diagnosed and the patient visited our hospital to obtain a second opinion.

Clinical exploration and further diagnostics confirmed the diagnosis of ALS/MND. In the re-evaluation of disease symptoms, we found generalised amyotrophy, pareses and cachexia. The patient refused percutaneous endoscopic gastrostomy (PEG) and non-invasive ventilation treatments and received home care until death.

Macroscopic neuropathological examination revealed a reddish colouration of the corpora mammillaria and periventricular thalamus. Multiple fresh microscopic haemorrhages were observed in the mammillary body and thalamus, showing a pattern of acute WE (figure 1A, B). No siderophages could be detected by Prussian blue staining (figure 1C) as evidence for earlier bleeding into these regions. No fresh bleeding was found in other locations of …

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