Case Presentation:
A 48 year-old male with a past medical history of morbid obesity, hypertension, and poorly nutritious diet consisting strictly of “pizza and chips” who presented after he was found unresponsive and incontinent of urine at home. He reportedly did not consume alcohol. Four weeks prior to presentation, he developed an acute onset of abdominal cramping, nausea, vomiting, diarrhea, anorexia, and poor oral intake. A week later, he developed unsteady gait, worsening fatigue and slept for most of the day. He reportedly had not left his bed the previous 48 hours prior to presentation. Initial work-up was largely negative. Patient was afebrile with stable vitals. Neurologic exam was limited on account of patient’s lethargy and confusion, but notable for spontaneous movement of all extremities, normal deep tendon reflexes, and absence of nystagmus. Labs were largely unremarkable, with normoglycemia, and negative toxicology screen. Computerized tomography (CT) scan of the head, electroencephalogram, and cerebrospinal fluid analysis were unremarkable. Within 24 hours the patient became more obtunded and was intubated for airway protection. Subsequent magnetic resonance imaging study of his brain demonstrated bilateral thalamic and intraventricular hemorrhages. CT venogram and cerebral angiogram ruled out cerebral venous thrombosis and arteriovenous malformation, respectively. A blood thiamine level was markedly low at 37 nmol/L (normal reference range: 78 to 185), confirming the diagnosis of severe thiamine deficiency. He was treated with an external ventricular drain and IV thiamine. The following weeks showed gradual improvement in alertness, however, expressive aphasia and unsteady gait were among persistent remaining deficits.
Discussion:
This is a case of Wernicke encephalopathy complicated by bilateral thalamic hemorrhage in a non-alcoholic patient. Bilateral thalamic hemorrhage is a rare complication of severe thiamine deficiency with only a few other cases reported in the literature. Interestingly, in this case, a common gastroenteritis infection precipitated this near-fatal complication. Thiamine is ubiquitous in most foods and adequate stores are easily obtained with a typical western diet. The half-life is 9-18 days and deficiency can result from poor nutrition and/or high catabolic states. Classically, thiamine deficiency is associated with alcoholics who present with Wernicke encephalopathy, which is characterized by a triad of confusion, ophthalmoplegia, and ataxia. In our case, it is possible the patient had a poor reserve of thiamine due to the lack of diversity in his diet. A bout of gastroenteritis likely further precipitated acute thiamine deficiency from poor oral intake and rapid depletion of previously compromised reserves. His initial clinical signs of thiamine deficiency were new onset gait disturbance, severe fatigue, and persistent anorexia that further exacerbated the deficiency. This ultimately led to the severe complication of bilateral thalamic hemorrhage.
Conclusions:
Some symptoms of thiamine deficiency, such as anorexia and fatigue, can perpetuate those very symptoms into an ever-worsening deficiency. Due to the short half-life of thiamine (9-18 days), clinicians should consider the possibility of deficiency when evaluating any patient with a history of poor oral intake over a period of weeks, regardless of history of alcohol use. Thalamic hemorrhage is a rare and life threatening complication of severe thiamine deficiency.
