Table 1.
Source | Country, year(s) of data collection | Number of cases and institution or type of setting (e.g., hospital and community) | Overall characteristics: age, gender, and underlying condition(s) | Likely cause of thiamine deficiency | Laboratory confirmation | Symptoms and clinical presentation | Treatment: supplementation dose and effect on symptoms (resolution) | Other notes (including the presence or absence of infection) |
---|---|---|---|---|---|---|---|---|
Alligier et al.12 (Published article) “A series of severe neurologic complications after bariatric surgery in France: the NEUROBAR Study” |
France, 2010–2018 | 38 cases with neurologic complications after bariatric surgery, of which 14 had confirmed thiamine deficiency | 34 females, 4 males; median age = 39 years; median BMI = 43 kg/m2; 34% received gastric bypass and 45% sleeve gastrectomy, with neurologic complications observed 6 months (median) after surgery | Gastrointestinal symptoms and surgical complications after bariatric surgery (e.g., vomiting and limited oral energy intake) | Thiamine deficiency was confirmed in 14 cases (values not provided) | 10 cases had encephalopathy, 15 had peripheral neuropathy, 12 had both, and 1 had a pyramidal syndrome | 15 patients received IV, and 2 received oral thiamine supplementation; neurologic symptoms were completely resolved in 9 cases; 2 patients died | No reported infection |
Kohnke and Meek18 (Published article) “Don't seek, don't find: the diagnostic challenge of Wernicke's encephalopathy” |
United Kingdom, 2020 | One case admitted to hospital (department of surgery) | 26‐year‐old woman had bariatric surgery 6 weeks before | Persistent vomiting for 6 weeks after gastric sleeve surgery | Not known (“inconclusive biochemistry” assessed after onset of treatment) | Presented with nystagmus, imbalance, and gait disturbance, interfering with activities of daily living | 250 mg IV thiamine (3×/day), later raised to 500 mg (3×/day) for several weeks, resulting in a marked improvement of symptoms | No reported infection |
Zafar30 (Published article) “Wernicke's encephalopathy following Roux‐en‐Y gastric bypass surgery” |
Saudi Arabia, 2015 | One case admitted to hospital | 40‐year‐old male had Roux‐en‐Y gastric bypass surgery (for weight loss) 3 months before and reported repeated vomiting since then | Repeated vomiting (and likely little oral food intake) following weight loss surgery | No | Presented with confusion and difficulty in maintaining balance while walking; assessment: nystagmus and ataxic gait (Wernicke encephalopathy) | 500 mg IV thiamine (3×/day), then 250 mg/day, resulted in improved symptoms (“almost completely normal” on 3‐month follow‐up visit) | No reported infection |
Isenberg‐Grzeda et al.15 (Published article) “Nonalcoholic Thiamine‐Related Encephalopathy (Wernicke–Korsakoff Syndrome) Among Inpatients With Cancer: A Series of 18 Cases” |
U.S., 2013–2014 | 18 cancer inpatients with Wernicke–Korsakoff syndrome referred to psychiatry service | Median age = 65 years; 33% women; 61% with solid tumors and 39% with hematologic malignancies | Disease‐related malnutrition: decreased availability/intake (e.g., nausea), accelerated usage (e.g., infection and fever), impaired use (e.g., fluorouracil and metronidazole), and excessive loss of thiamine | Yes, in 89% of patients: serum thiamine was <7 nmol/L (normal: 9–14 nmol/L) | Presented with cognitive signs and symptoms; for example, altered mental status (100%), cerebellar signs and symptoms (39%), and ocular signs and symptoms (17%). No/little vitamins B9 and B12 deficiency | Most received 500 mg IV thiamine (3×/day), initiated on average 18 days after symptom onset; 17% had complete resolution of symptoms, and 83% had residual symptoms at the time of last follow‐up | 50% of patients had an infection |
Cui and Qiu2 (Published article) “Thiamine Deficiency (Beriberi) Induced Polyneuropathy and Cardiomyopathy: Case Report and Review of the Literature” |
U.S., 2014 | One case admitted to the ER | 20‐year‐old female with papillary thyroid carcinoma and dysphagia caused by radiation injury; low body weight | Significantly decreased oral intake due to dysphagia | Yes, thiamine was 7 nmol/L (normal 9–14 nmol/L) | Presented with bilateral lower extremity weakness and paresthesia, inability to walk, chest palpitations, and shortness of breath; assessment: ptosis, nystagmus, tachycardia, and lactic acidosis | IV thiamine supplementation for 5 days and tube feeding after hospital discharge resulted in the improvement of all symptoms at 3‐month follow‐up visit | No reported infection |
Jung et al.11 (Published article) “Wernicke's Encephalopathy in Advanced Gastric Cancer” |
Korea, 2009 |
Two cases admitted to hospital |
Case 1: 48‐year‐old woman with advanced GC, receiving intermittent home parenteral nutrition (HPN), suffered a 20‐kg weight loss over 2 months Case 2: 58‐year‐old woman with advanced GC and a 15‐kg weight loss over 2 months |
Case 1: disease‐related malnutrition (with marked weight loss after chemotherapy) Case 2: disease‐related malnutrition (with marked weight loss after chemotherapy) |
Case 1: No Case 2: No |
Case 1: presented with dizziness and diplopia; assessment: nystagmus and gaze disturbance (Wernicke encephalopathy) Case 2: presented with sudden disorientation, confusion; assessment: gaze limitation and mild ataxia (Wernicke encephalopathy) |
Case 1: daily parenteral injection of thiamine 100 mg for 17 days resulted in improved symptoms Case 2: parenteral injection of thiamine (100 mg for 4 days), but patient had recurrent seizure attack and aggravation, resulting in death on hospital day 6 |
Case 1: no reported infection Case 2: no reported infection. Thiamine replacement started 3 days after neurologic symptoms and was ineffective |
Helali et al.31 (Published article) “Thiamine and Heart Failure: Challenging Cases of Modern‐Day Cardiac Beriberi” |
U.S., 2018 | Two cases admitted to the ER |
Case 1: 68‐year‐old homeless obese man (BMI = 33 kg/m2) Case 2: 63‐year‐old obese man (BMI = 39 kg/m2) was severely limiting caloric intake to encourage weight loss |
Case 1: food insecurity Case 2: restrictive diet (less than 1 meal/day, mostly convenience foods) |
Case 1: Yes, a random nonfasting level of 12 nmol/L, a few days after admission (normal: 8–30 nmol/L) Case 2: Yes, undetectable |
Case 1: presented with progressive dyspnea and swollen legs; assessment: cardiomegaly and anemia; multiple hospital visits in the following 3 months, with new neurocognitive deficits and bilateral cranial nerve 6 palsies Case 2: presented with dyspnea and altered mental status; assessment: tachycardia, tachypnea, and severe heart failure |
Case 1: 100 mg oral thiamine/day improved cardiac and cognitive function after 16 days Case 2: heart failure improved significantly after 14 days of IV thiamine |
Both cases: no reported infection. Authors suggest that patients who present with an unexplained cardiomyopathy should be evaluated for thiamine deficiency |
Misumida et al.32 (Published article) “Shoshin Beriberi Induced by Long‐Term Administration of Diuretics: A Case Report” |
U.S., 2014 | One case admitted to the ER; Transferred to ICU on second day | 61‐year‐old man with a history of heart failure (receiving furosemide and trichlormethiazide therapy for 6 months), diabetes and stage 3 chronic kidney disease. BMI = 29 kg/m2 | Chronic diuretic therapy | Yes, plasma thiamine concentration of 11 mg/dL (normal range: 20–50 mg/dL) | Presented with dyspnea; assessment: edema in legs, cardiomegaly, pulmonary vascular congestion, and severe metabolic acidosis | IV thiamine supplementation (100 mg/day) resolved all symptoms and patient was discharged on day 15 (on “oral vitamin pills”) | No reported infection (absence of fever and leukocytosis, and negative results of serial blood cultures) |
Romanski and McMahon33 (Published article) “Metabolic Acidosis and Thiamine Deficiency” |
U.S., 1999 | One case admitted to medical center | 19‐year‐old woman, BMI = 13 kg/m2, with persistent, unexplained GI symptoms and receiving HPN | Absence of multivitamins, most significantly thiamine, in HPN formula (no multivitamins or trace elements were provided for 19 days) | No, test was ordered but not completed; reason not provided | Presented with nausea, vomiting, diarrhea, and abdominal pain; assessment: very low BMI, hyperglycemia, and metabolic acidosis | IV thiamine supplementation (100 mg daily for 2 days), followed by daily administration of 50 mg orally for the next 14 weeks, resulted in a dramatic clinical improvement | No reported infection |
Koike et al.34 (Published article) “Myopathy in thiamine deficiency: analysis of a case” |
Japan, 2006 | One case admitted to hospital | 26‐year‐old woman with a particular dietary pattern (ate mostly white rice and drank coffee, disliked meat and vegetables); BMI = 24.5 kg/m2 | Monotonous diet | Yes, total thiamine in whole blood was 16 ng/mL (normal, 20–50 ng/mL) | Presented with walking difficulties, leg edema, and myalgia; assessment: moderate cardiomegaly with pulmonary congestion and axonal neuropathy | 75 mg oral dose of fursultiamine (daily) resulted in dramatic decrease in cardiomegaly, pleural effusions, and edema in the legs, followed by improvement of neurologic symptoms, muscle strength, and myalgia | No reported infection |
Shible et al.13 (Published article) “Dry Beriberi Due to Thiamine Deficiency Associated with Peripheral Neuropathy and Wernicke's Encephalopathy Mimicking Guillain–Barré syndrome: A Case Report and Review of the Literature” |
U.S., 2019 | One case admitted to hospital and transferred to intensive care unit | 56‐year‐old woman with history of gallstone pancreatitis and malnutrition; on HPN until 6 months prior to admission, then returned to normal diet. BMI unknown | Underlying severe protein‐calorie malnutrition and duration of critical illness | Yes, but measured after four doses of thiamine therapy (serum level: 104 nmol/L, reference range: 70–180 nmol/L) | Presented with paresthesia of the lower limbs, arms and neck; assessment: unresponsive to verbal stimuli, Hb 9.4 g/dL, and Wernicke encephalopathy | High‐dose IV thiamine (500 mg every 8 h) resulted in mental status improvement within 48 hours | Reported infection (septic state). Initial diagnosis was Guillain–Barré syndrome (symptoms and signs of dry beriberi can mimic those of the Guillain–Barré syndrome) |
Koike et al.17 (Published article) “Rapidly developing weakness mimicking Guillain–Barré syndrome in beriberi neuropathy: two case reports” |
Japan, 2007 | Two cases admitted to hospital |
Case 1: 46‐year‐old man had a gastrectomy 3 years ago to treat cancer Case 2: 33‐year‐old man with a particular dietary pattern (did not like meat or vegetables, preferring white rice and noodles with no side dishes); heavy outdoor work |
Case 1: disease‐related malnutrition (BMI or weight loss not reported) Case 2: monotonous diet |
Case 1: Yes, total thiamine in whole blood was 15 ng/mL (normal: 20–50 ng/mL) Case 2: Yes, total thiamine in whole blood was 7 ng/mL (normal: 20–50 ng/mL) |
Case 1: presented with weakness in lower extremities; assessment: axonal neuropathy; later developed progressive weakness, lactic acidosis, and heart failure Case 2: presented with weakness of limbs (unable to walk); assessment: severe sensory deficits in legs and mild sensory loss in hands |
Case 1: 100 mg IV thiamine resulted in gradual improvement of all symptoms Case 2: 100 mg IV thiamine resulted in gradual improvement of all symptoms |
Both cases: no reported infection and symptoms mimicked Guillain–Barré syndrome (which was initially considered as a diagnosis) |
Solorzano and Guha14 (Published article) “Wernicke's Encephalopathy: Under Our Radar More Than it Should Be?” |
U.S., 2016 | One case admitted to hospital | 30‐year‐old woman with abdominal sepsis due to choledocholithiasis, on parenteral nutrition due to poor oral intake (caused by nausea, vomiting, and abdominal pain) | Thiamine was not included in the parenteral nutrition formulation | Yes, low levels (values not provided) | Presented with deteriorated mental status; assessment: miotic pupils and roving eye movements, and Wernicke encephalopathy | 500 mg IV thiamine, 3×/day for 3 days, then oral 50 mg/day resulted in symptom improvement, although ataxia and memory issues persisted 2 months later | Reported complex intra‐abdominal infections |
Ruiz et al.35 (Conference abstract) “Acute polyneuropathy and Wernicke encephalopathy due to thiamine deficiency” |
Italy, 2019 | One case admitted to the ER | 59‐year‐old woman with cancer (adenocarcinoma of the extrahepatic biliary tree) | Disease‐related malnutrition | Yes, low serum thiamine level (34 nmol/L, normal values 66–200) |
Admission: subacute onset of confusion, amnesia for recent events and confabulation. Later: nystagmus, hypoesthesia, and severe flaccid quadriparesis |
200 mg of IV thiamine hydrochloride/day, for 3 weeks Strength improvement, distal lower limb paresthesia, reduced reflexes at upper limbs and areflexia at lower limbs. Amnestic‐confabulatory syndrome persisted |
No reported infection |
Murase et al.36 (Conference abstract) “Shoshin beriberi in a young man living on Japanese rice balls” |
Japan, year not reported | One case admitted to ER | 24‐year‐old single man, living alone, no underlying conditions (BMI = 17.4 kg/m2) | Food insecurity (subsisting on balls of polished rice in preceding 4 years) due to financial problems | Yes, 17 ng/mL (normal: 24–66 ng/mL) | Presented with chest pain and shortness of breath; assessment: systemic edema, central cyanosis, hyporeflexia, lactic acidosis, and moderate cardiomegaly | Hemodynamic parameters improved dramatically in only 3 h after thiamine administration (dose not reported) | No reported infection |
Bruera et al.37 (Clinical communication to the editor) “The Malnourished Heart: An Unusual Case of Heart Failure” |
U.S., 2017 | One case admitted to hospital | 66‐year‐old woman with no past medical history, but relied on a diet “of processed cheese chips and vanilla cake” for 2 years; BMI = 17 kg/m2 | Monotonous diet that led to multiple nutritional deficiencies | Yes, low levels (values not provided) | Presented with worsening dyspnea, lower extremity edema, and orthopnea; assessment: heart failure, periodontal disease, patchy hair loss, low HB (10.3 g/dL), deficient for vitamins B1, B6, C, and D | After injections of thiamine and other vitamins + multivitamin tablets, the heart failure symptoms resolved completely and periodontal disease improved | No reported infection |
Mates (2020) (Personal communication, unpublished dataa from Dr. Elisabeth Mates, MD, attending hospitalist physician at the VA Sierra Nevada Healthcare System) |
USA, 2018–2020 | 33 cases admitted to hospital | Veteran patients (mean age = 75 years old) from the greater Reno area, Nevada, with wide range of underlying acute and chronic illnesses | Disease‐related malnutrition (illnesses or conditions that led to reduced appetite and nutritional intake, e.g., cancer, cholecystitis, and pancreatitis) |
Yes, all patients had a plasma thiamine level ≤7 nmol/L (normal: 8–30 nmol/L); note: takes 7–10 days to obtain test result |
Wide‐ranging symptoms; no single symptom stands out other than many had weakness and “hospital delirium” | Treated patients (<50%) had demonstrable improvement in the neurologic symptoms and general weakness; some went from needing nursing home level of care to be discharged home after treatment | Pending subsequent prospective study (n = 400) to determine the prevalence of thiamine deficiency in hospitalized patients |
BMI, body mass index; ER, emergency room; GC, gastric cancer; GI, gastrointestinal; HPN, home parenteral nutrition; ICU, intensive care unit; IV, intravenous; WB, whole blood.
Manuscript in preparation for peer review; data included in this table reflect the information provided by Elisabeth Mates, MD, an attending hospitalist physician at the VA Sierra Nevada Healthcare System.