A 60-year-old patient with regular heavy alcohol intake is admitted to ICU in a stuporous state after a two­ week history of difficulty in walking and repeated falls. On examination, his Glasgow Coma Score is E2M4V3, with bilateral nystagmus and limited outward movement of both eyes on turning his head. There is no neck stiffness, asterixis or focal neurological deficit. A CT scan of the brain shows generalised cerebral atrophy.

 
a)    What is the diagnosis? (15% marks)

b)    What is the specific treatment for this condition? (10% marks)
 


 

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College answer

1. Wernicke’s Encephalopathy

2. IV Thiamine in high doses (500mg thrice a day x 2 days; 250mg daily thereafter) followed by IV    Glucose

Discussion

Those trainees who are wondering what the scientific definition of "stuporous" is will be enouraged to learn that there isn't one. Merriam-Webster defines it as 

"a condition of greatly dulled or completely suspended sense or sensibility; specifically : a chiefly mental condition marked by absence of spontaneous movement, greatly diminished responsiveness to stimulation, and usually impaired consciousness".

One would think that this sounds very colloquial and 19th century (where a young lady might have been accused of having greatly dulled or completely suspended sense or sensibility for rejecting a wealthy suitor). A slightly more modern and medical-sounding definition can be found in the 2007 edition of Plum & Posner's Diagnosis of stupor and coma (p.7):

 "Stupor, from the Latin ‘‘to be stunned,’’ is a condition of deep sleep or similar behavioral unresponsiveness from which the subject can be aroused only with vigorous and continuous stimulation. Even when maximally aroused, the level of cognitive function may be impaired. Such patients can be differentiated from those with psychiatric impairment, such as catatonia or severe depression, because they can be aroused by vigorous stimulation to respond to simple stimuli."

Anyway. This is clearly Wernicke's encephalopathy. Recall its features, of which this patient has several:

  • Encephalopathy
    • Rarely, stupor and coma
    • Most often, confusion and impaired memory
  • Ataxia
    • Usually, there are no upper limb or speech cerebelar signs
    • This is because only the anterior and superior vermis are affected
    • The lower limb cereballar signs conspire with vestibular damage and thiamine-associated polyneuropathy(i.e. "I can't feel my legs").
  • Eye signs
  • Hypothermia
    • due to impairment of thermoregulation: the hypothalamus is damaged
  • Hypotension
    • due to heart failure, "wet Beri-Beri"

The specific treatment is  IV thiamine. There is a disagreement as to how much is actually enough (The college answer to Question 13.3 from the second paper of 2013 suggests 100mg IV daily is a big enough dose, whereas Cook et al (1998) recommended 1g daily).

References

Johnson, J. "Stupor: a review of 25 cases." Acta Psychiatrica Scandinavica 70.4 (1984): 370-377.

Flynn, Alexandra, et al. "Wernicke’s Encephalopathy: Increasing Clinician Awareness of This Serious, Enigmatic, Yet Treatable Disease." The primary care companion for CNS disorders 17.3 (2015).

Thomson, Allan D., and E. Jane Marshall. "The natural history and pathophysiology of Wernicke's encephalopathy and Korsakoff's psychosis." Alcohol and Alcoholism 41.2 (2006): 151-158.

Gussow, Leon. "Myths of toxicology: thiamine before dextrose." Emergency Medicine News 29.4 (2007): 3-11.

Isenberg-Grzeda, Elie, Haley E. Kutner, and Stephen E. Nicolson. "Wernicke-Korsakoff-syndrome: under-recognized and under-treated." Psychosomatics 53.6 (2012): 507-516.\

Watson, A. J. S., et al. "Acute Wernickes encephalopathy precipitated by glucose loading." Irish journal of medical science 150.1 (1981): 301-303.

Kissoon, Niranjan. "Thiamine before glucose to prevent Wernicke encephalopathy: examining the conventional wisdom." JAMA 279.8 (1998): 583.

Day, Ed, et al. "Thiamine for prevention and treatment of Wernicke‐Korsakoff Syndrome in people who abuse alcohol." The Cochrane Library (2013).

Ambrose, Margaret L., Stephen C. Bowden, and Greg Whelan. "Thiamin Treatment and Working Memory Function of Alcohol‐Dependent People: Preliminary Findings." Alcoholism: Clinical and Experimental Research 25.1 (2001): 112-116.

Cook, Christopher CH, Phillip M. Hallwood, and Allan D. Thomson. "B Vitamin deficiency and neuropsychiatric syndromes in alcohol misuse." Alcohol and Alcoholism 33.4 (1998): 317-336.