Peripheral neuropathy mimicking Guillain-Barre syndrome in a patient with severe refeeding syndrome: a case report

doi:10.21203/rs.3.rs-3470382/v1

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Case Report

Peripheral neuropathy mimicking Guillain-Barre syndrome in a patient with severe refeeding syndrome: a case report

https://doi.org/10.21203/rs.3.rs-3470382/v1

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Background:

Refeeding syndrome (RS) is a potentially serious condition and frequently ignored without uniform definition. Severe refeeding syndrome may present with symptoms of thiamine deficiency (TD) with poor outcome. Prevention in high-risk populations and timely treatment of clearly diagnosed patients may improve outcomes.

Case presentation:

A 58-year-old patient with fulminant myocarditis who received veno-arterial extracorporeal membrane oxygenation (VA-ECMO) ran into severe RS during the recovery period after ECMO was successfully removed. Considering thiamine deficiency as a result of severe refeeding syndrome, patients were given empiric thiamine 100mg intramuscularly once a day. The peripheral neuropathy mimicing Guillain-Barre syndrome (GBS), cardiac insufficiency, and hyperlactemia were improved after 5 days following thiamine supplementation.

Conclusion:

Refeeding syndrome often occurs, and it is very harmful. The clinical manifestations of refeeding syndromes are diverse. Proper management of refeeding syndrome is very important to improve patient outcomes.

Refeeding syndrome

thiamine deficiency

peripheral neuropathy

cardiac insufficiency

hyperlactemia

Refeeding syndrome (RS) is described as a series of metabolic and electrolyte disorders because of the restart and/or increased provision of calories after a period of absent or decreased caloric intake¹. When fasting or nutrient intake is insufficient, electrolyte and vitamin reserves are depleted, with or without a decrease in serum levels. When nutrient intake is restarted, the demand for electrolytes and thiamin greatly increases as they are necessary components for metabolism and intracellular substance synthesis. If timely supplementation is not possible, there will be a significant decrease in levels, namely hypophosphatemia, hypokalemia, hypomagnesemia and thiamine deficiency, and corresponding symptoms will appear¹. The electrolytes’ levels can be dynamically monitored and promptly and effectively supplemented. While thiamine is not routinely monitored in the clinic, it is easy to ignore². Thiamine is a vital cofactor which is involved in energy metabolism and intracellular substance synthesis³. Exogenous supplementation is the only way to get this vitamin because it cannot be synthesized in the human body and can only be kept for 30mg in skeletal muscle, heart, brain, liver, and kidney. The daily requirement is 1.1 to 1.2mg⁴. Thiamine deficiency can lead to a variety of illnesses, including wet beriberi (cardiac failure), dry beriberi (peripheral neuropathy), Wernicke encephalopathy, Korsakoff Psychosis, and gastrointestinal beriberi³.

This case describes a patient with fulminant myocarditis who experienced refeeding syndrome during the recovery period after successful ECMO withdrawal, with some disorders including significantly elevated myoglobin, zero grading muscle strength, ventilator weaning failure, and slightly elevated lactic acid level, which were diagnosed as peripheral neuropathy mimicing Guillain-Barre syndrome (GBS), cardiac insufficiency, and hyperlactemia. The aforesaid conditions gradually improved with thiamine supplementation, and the patient was successfully discharged from the ICU. Refeeding syndrome is a neglected but potentially serious condition, especially with thiamine deficiency^1,8. This case can provide a more understanding of the role of thiamine supplementation in the prevention and treatment of refeeding syndrome.

A 58-year-old female patient with no history of disease had a cold with a fever that reached 38.5°C three days before admission, and was admitted to the hospital for progressively acute tachypnea. Her blood pressure was 146/77mmHg at admission, her heart rate was 104 beats per minute, her respiratory rate was 40 beats per minute, and she had a 90% oxygen saturation on ambient air. A physical examination revealed pale skin, cold limbs, shortness of breath, and wet rale in the lower lobes of both pulmonary lobes. Sinus tachycardia, complete right bundle branch block, left anterior branch block, prolonged Q-T interval, and S-T change were all seen on the electrocardiogram. At the same time, cardiac markers (troponin I 27.72ng/mL, myoglobin 155.85ng/mL, creatine kinase-MB 100.00ng/mL) were clearly abnormal. Bedside echocardiography showed abnormal ventricular wall movement and decreased overall systolic ability of left ventricle (EF 46%). Because of the rapid circulation deterioration with lactic acid increased to 4.3mmol/L, the patient was implemented tracheal intubation before undergoing the coronary angiogram. Myocarditis was considered in the absence of coronary artery disease. Even though norepinephrine and epinephrine were injected at maximum dose, circulation could not be maintained, and lactic acid levels steadily rose to 27mmol/L. VA-ECMO was used after a sudden cardiac arrest and ran for 132 hours without bleeding, thrombosis, ischemia, or other complications. Renal failure and volume overload necessitated the concurrent use of continuous renal replacement therapy (CRRT).

Next, the patient was awake and conscious after reducing sedation and analgesia, but her limb muscle strength was zero, and no cerebral ischemia or bleeding was discovered. At the same time, circulation was still erratic, arrhythmias were intermittent, and a little dosage of norepinephrine was required to keep blood pressure stable. Despite volume optimization with CRRT, levosimendan for cardiac function adjustment, and arrhythmia management, lactic acid levels remained around 3.0mmol/L, and respiratory function exercise could not be tolerated in the absence of lung illnesses. Take into account that the patient's heart function remains weak. Simultaneously, nutritional therapy was initiated two days after withdrawal, and myoglobin levels rose on the third day, accompanied by a drop in blood phosphorus from 1.4mmol/L to 0.94mmol/L(Because of kidney failure and daily supplementation, serum phosphorus levels did not fall below the normal range), indicating rhabdomyolysis due to refeeding syndrome. As a result, empiric thiamine 100mg intramuscular injections were given once a day, a tracheotomy was performed, CRRT was continued for volume optimization and myoglobin clearance. While myoglobin levels were elevated after 5 days of supplementation, muscular strength was restored to level 2 and lactic acid levels were normal. Meanwhile, the patient was successfully taken off the invasive ventilator and replaced with high-flow oxygen therapy delivered via a tracheotomy tube. Given the effectiveness of thiamine supplementation, thiamine deficiency can be diagnosed. Then the patient was transferred out of ICU one month after onset with stable circulation, sinus rhythm, no vasoactive medications, appropriate urine volume with extended intervals of CRRT and occasional low-dose diuretic, and muscle strength of grade 3–4.

The American Society for Parenteral and Enteral Nutrition (ASPEN) suggested a unifying definition for RS and its severity categorization in 2020 as follows: a decrease in any 1, 2, or 3 of serum phosphorus, potassium, and/or magnesium levels by 10–20% (mild), 20–30% (moderate), or > 30% and/or organ dysfunction resulting from a decrease in any of these and/or due to thiamin deficiency (severe), occurring within 5 days of reintroduction of calories¹.

Thiamine, often known as Vitamin B1, is a water-soluble, serves as a catalyst of PDH in the reactions of pyruvate to acetyl coenzyme A and α-ketoglutarate to succinyl coenzyme A in the Krebs cycle, contributing to the synthesis of ATP and some neurotransmitters⁴. Because it is an exogenous vitamin with a poor storage capacity in the body, shortage is common due to insufficient intake or increased use, and illnesses such as Wernicke-Korsakoff syndrome, wet beriberi, dry beriberi, and infantile beriberi can develop². However, because the methods for determining thiamine nutrition status are costly and no-real, they are rarely used in clinical practice, and the diagnosis is usually made based on the patient's clinical condition, risk factors, and satisfactory response to the therapeutic test⁹. Malnutrition, refeeding syndrome, gastrointestinal surgery, alcoholism, diuretic furosemide, and hemodialysis are all well-known risk factors for thiamine deficiency^4,5. Thiamine deficiency was also diagnosed clinically in this case, with risk factors including insufficient intake, RS, hemodialysis, and diuretics, as well as the efficacy of thiamine supplementation.

The dry beriberi is characterized by distal, sensory-motor, and symmetrical peripheral polyneuropathy, and patients typically mention gradual weakness of the lower limbs¹⁰. However, some cases may have a quicker onset and mimic other paresthesias, such as Guillain-Barre syndrome¹¹. There have been several case reports of patients who were misdiagnosed as GBS, and showed a good response to thiamin replacement^{6, 12}. In this case, the patient was conscious after stopping the sedative, but her limbs were entirely immobile. We suspected a cerebrovascular event at first, but a cranial CT revealed no bleeding or infarction. The use of analgesic-sedative and muscle relaxant medicines for a long time and in big amounts may then lead to this illness. At the same time, rhabdomyolysis linked to the refeeding syndrome has been reported for myasthenia. GBS was also suspected. After supplementation of thiamine for the treatment of the refeeding syndrome, the patient's symptoms were quickly improved, so TD can be considered.

The symptoms of wet beriberi, including decreased vascular tone, congestive heart failure, and even cardiac arrest¹³. The clinicians usually confuse these symptoms with other cardiac disease¹⁴. A systematic review and meta-analysis concluded that patients with heart failure are at risk of thiamine deficiency and that supplementation was likely beneficial in improving left ventricular function as well as quality of life¹⁵.In this case, the main manifestation of the patient’s cardiovascular symptoms was cardiac insufficiency with an approximal normal ejection fraction that resulting in intolerance of the increased oxygen demand from the respiratory function exercise. The symptom was thought about poor heart function due to fulminant myocarditis before awareness of TD, and were predicted to improve for a long time, so the patient was carried out tracheotomy. In fact, 4 days after tracheotomy and 5 days after thiamine supplementation, the patient succeeded to wean ventilator. If TD was recognized at an early stage, tracheotomy may avoid, then the harm to the patient would be reduced, and the time for recovery would be shortened.

Thiamine deficiency can also lead to elevated lactate. Thiamine is a cofactor of the pyruvate dehydrogenase in Kreb’s circle. Clémence Didisheim et reported a case with severe lactic acidosis in a critically ill child, which normalized within 12 hours after thiamine supplementation¹⁶. In this case, there were no reasonable explanation for the mild elevated lactic acid level ranged from 2 to 3mmol/L without abnormal liver function after stable circulation. In the retrospect, the level was gradually restored to normal after 3 days for thiamine supplementation. This recognition may reveal that the reason of increased lactate, indicate the presence of thiamine deficiency, and support the significance of thiamine supplementation.

In this case, the improvement of clinical symptoms after thiamine supplementation plays a decisive role in the rehabilitation of patients after diagnosed with refeeding syndrome. Therefore, more attention should be paid on refeeding syndrome and thiamine deficiency in clinical practice. Moreover, there are controversies over routine thiamine supplementation, and more prospective studies are needed.

Refeeding syndrome

thiamine deficiency

VA-ECMO /ECMO

veno-arterial extracorporeal membrane oxygenation

GBS

Guillain-Barre syndrome

CRRT

continuous renal replacement therapy

Ethical Approval and consent to participate

The case report was approved by the Medical Ethics Committee of Chongqing General Hospital. Informed consent was obtained from the patient.

Consent for Publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for Supplementary material.

Availability of data and materials

Not applicable.

Competing interests

The authors declare that they have no competing interests

Funding

None of the authors received funding for this study.

Authors’ contributions

JY and TL designed the study. TL, MZ, LC, HL, XY, FL collected and interpreted the data. TL drafted the manuscript. JY modified the manuscript and finally approved the version to be published. All authors read and approved the final manuscript.

Acknowledgements

Not applicable.

Author information

Authors and Affiliations:

Department of Intensive Care Unit, Chongqing General Hospital, Chongqing, China

Corresponding author

Correspondence to Jin Yang

Department of Intensive Care Unit, Chongqing General Hospital, Chongqing, China

E-mail：[email protected]

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No competing interests reported.

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Peripheral neuropathy mimicking Guillain-Barre syndrome in a patient with severe refeeding syndrome: a case report

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Abstract

Background

Case presentation

Discussion

Conclusion

Abbreviations

Declarations

References

Additional Declarations

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