Annals of Indian Academy of Neurology
  Users Online: 1854 Home | About the Journal | InstructionsCurrent Issue | Back IssuesLogin      Print this page Email this page  Small font size Default font size Increase font size


 
Table of Contents
CASE REPORT
Year : 2013  |  Volume : 16  |  Issue : 4  |  Page : 687-689
 

"Dry" and "wet" beriberi mimicking critical illness polyneuropathy


1 Department of General Medicine, K. S. Hegde Medical Academy, Deralakatte, Mangalore, Karnataka, India
2 Department of Neurology, K. S. Hegde Medical Academy, Deralakatte, Mangalore, Karnataka, India
3 Department of Nephrology, K. S. Hegde Medical Academy, Deralakatte, Mangalore, Karnataka, India
4 Department of Cardiology, K. S. Hegde Medical Academy, Deralakatte, Mangalore, Karnataka, India

Date of Submission16-Feb-2013
Date of Decision26-Feb-2013
Date of Acceptance26-Mar-2013
Date of Web Publication25-Oct-2013

Correspondence Address:
S Rama Prakasha
Department of General Medicine, K. S. Hegde Medical Academy, Deralakatte, Mangalore - 575 018, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.120467

Rights and Permissions

 

   Abstract 

Three cases with manifestations of right heart failure, shock, metabolic acidosis, and renal failure in varying combination were admitted with paraparesis. Nerve conduction study suggested predominantly motor and mainly axonal type of neuropathy. Rapid reversal of shock, acidosis, and multi-organ dysfunction with timely infusion of thiamine was followed by the complete neurological recovery.


Keywords: Beri-beri, critical illness polyneuropathy, multiorgan failure, thiamine


How to cite this article:
Prakasha S R, Mustafa A S, Baikunje S, Subramanyam K. "Dry" and "wet" beriberi mimicking critical illness polyneuropathy. Ann Indian Acad Neurol 2013;16:687-9

How to cite this URL:
Prakasha S R, Mustafa A S, Baikunje S, Subramanyam K. "Dry" and "wet" beriberi mimicking critical illness polyneuropathy. Ann Indian Acad Neurol [serial online] 2013 [cited 2019 Oct 20];16:687-9. Available from: http://www.annalsofian.org/text.asp?2013/16/4/687/120467



   Introduction Top


Beriberi is the clinical disease associated with thiamine deficiency and sub-acute thiamine deficiency will have symptoms of peripheral edema and mixed motor and sensory neuropathy. A more rapid form of wet beriberi is termed as acute fulminant cardiovascular beriberi or Shoshin beriberi, which manifests in the form of shock, severe metabolic acidosis, and renal failure. In individuals without risk-factors for thiamine deficiency, the diagnosis of this condition is often missed.

Critical illness polyneuropathy (CIP) is an acute neuromuscular disorder of severely ill patients in the setting of systemic inflammatory response syndrome, sepsis, and multiple organ failure. In a patient with tachycardia, tachypnoea, and multiorgan dysfunction, weakness may be attributed to CIP, severe electrolyte disturbance, critical illness myopathy, pre-existing neuropathy or possibly Guillain-Barré syndrome (GBS). In this case series, we describe three patients who had the above manifestations and all had an uneventful recovery on treatment with intravenous thiamine.


   Case Reports Top


Case 1

A 44-year-old non-alcoholic male was admitted with dyspnea, vomiting, upper abdominal pain, oliguria, and weakness of both the lower limbs of 3 days duration. He was admitted thrice in the previous 1 month for symptoms of heart failure at a peripheral hospital, with partial recovery each time upon conservative treatment. On admission, patient had tachypnea, unrecordable blood pressure, heart rate 136/min, elevated Jugular Venous Pressure (JVP), and bilateral pitting edema of legs. Systemic examination revealed right sided S3, systolic murmur of 3/6 intensity at the lower left sternal edge and 3 cm tender hepatomegaly. Bilateral lower limb power was 3/5 with sluggish deep tendon reflexes.

Electrocardiography (ECG) revealed sinus tachycardia with ST depression in leads V3-V6. Arterial Blood Gas analysis showed severe metabolic acidosis [Table 1]. 2D echocardiography on admission showed dilated Right atrium/Right ventricle (RA/RV) with mild tricuspid regurgitation and pulmonary hypertension [Table 1]. A provisional diagnosis of right heart failure with the multiorgan dysfunction possibly due to sepsis or cardiac beriberi was made and he was managed conservatively with broad spectrum antibiotics, sodium bicarbonate infusion, inotropes, and thiamine infusion. Acidosis, shock, and renal failure recovered promptly over from 24 h to 48 h. However, his paraparesis worsened over the next 2-3 days and Nerve conduction study (NCS) suggested predominantly motor, axonal type of peripheral neuropathy [Table 2]. With continued thiamine supplementation, his power in the lower limbs gradually improved and he was discharged in 2 weeks with near normal power in the lower limbs.
Table 1: Investigation reports

Click here to view
Table 2: NCV data of study subjects

Click here to view


Case 2

A 25-year-old lady who had delivered a baby 6 weeks back was admitted with generalized anasarca and paraparesis of 10 days duration. She had dyspnea and oliguria since 1 day. Deep tendon reflexes (DTR) suggested global areflexia and bilateral lower limb power was 3/5. Her thyroid function was normal and Anti-nuclear antibody (ANA) was negative. Her investigation results are tabulated in [Table 1] and NCS findings in [Table 2]. A diagnosis of heart failure with polyneuropathy was made and she was treated with IV thiamine infusion and supportive treatment. Edema disappeared over a week and she was discharged after 12 days with near normal power in lower limbs.

Case 3

A 36-year-old alcoholic male was admitted withbilateral edema of legs andbilaterallower limb weakness of 3 weeks duration. He also had oliguria and vomiting of 4 days duration. Bilateral lower limb power was 4/5 and his echocardiography as well as NCV findings are enlisted in [Table 1] and [Table 2]. Upon treatment with intravenous thiamine for 1 week followed by oral supplementation, his renal functions and acidosis recovered, edema disappeared. After 11 days he was discharged with complete recovery.


   Discussion Top


Blankenhorn [1] criteria for beriberi heart disease diagnosis include enlarged heart with normal sinus rhythm, edema and elevated venous pressure, peripheral neuritis, nonspecific ST-T changes in ECG, no other evident cause, history of dietary deficiency, and response to thiamine or autopsy evidence. All the three cases in this series had right heart failure with edema and elevated JVP. Reversible pulmonary hypertension with dilated RA/RV due to an increased pulmonary arterial blood flow and elevated LV end-diastolic pressure is the predominant presentation in Shoshin beriberi. [2]

Metabolic polyneuropathy shows the pathology of acute axonopathy, which is important for differentiating from the axonal form of GBS. In thiamine deficiency, Adenosine triphosphate (ATP) decreases because of a decrease in pyruvate dehydrogenase activity which is said to be also associated with a decrease in Na + -K + -ATPase activity. Our patients may have developed acute axonal polyneuropathy simultaneously due to a rapid thiamine deficiency caused by vomiting, as well as a state of chronic pre-existing thiamine deficiency. In thaimine deficiency neuropathy NCS shows reduced lower limb sensory and motor responses with only mild slowing of conduction velocities. Nerve biopsy shows preferential degeneration of large myelinated axons with absent segmental demyelination. Associated with endoneural edema is present. Unlike other forms of axonal polyneuropathies, recovery is relatively rapid in thiamine deficiency.

CIP is an acute or sub-acute axonal length dependent neuropathy and is viewed as motor predominant or exclusively motor disease. [3] Apart from distal axonal degeneration of motor fibers causing flaccid weakness, decreased or absent deep tendon reflexes; other features of CIP include decreased amplitudes of compound muscle and sensory action potentials with minimal conduction slowing, normal or mildly increased levels of blood Creatinine phosphokinase in a critical care setting. In CIP, nerve biopsy shows degeneration of both motor and sensory nerve fibers without evidence of inflammation, vascular injury or demyelination. Associations and risk factors include immobility, malnutrition, medications including corticosteroids, and aminoglycosides, dialysis, low albumin, and high blood glucose and underlying medical conditions. Recovery is often delayed and incomplete in CIP, and clinical deficits and electrophysiological abnormalities persist in majority even after 5 years. [4]

Other possibilities of polyneuropathy in acutely ill patients include Acute inflammatory demyelinating polyneuropathy (AIDP), uremic polyneuropathy and diphtheritic polyneuropathy. AIDP can be triggered by a critical illness and NCS shows prominent conduction block or slowing. Acute motor axonal neuropathy (AMAN) variant is clinically and electrophysiologically indistinguishable from CIP; however, nerve biopsy in the former shows endoneuiral edema and mononuclear cell infiltration, segmental demyelination and axonal loss in severe cases. Uremic polyneuropathy can occur in acute setting, especially after the initial few hemodialysis. It is predominantly a demyelinating type of sensory neuropathy and NCS shows reduced Sensory nerve action potential (SNAP) amplitudes with prolonged latencies and reduced conduction velocities. Diphtheria can be associated with cardiac failure secondary to cardiomyopathy and can present with acute mixed sensory motor neuropathy of demyelinating type in 15% of cases. Furthermore, critical illness myopathy is clinically difficult to differentiate from CIP, but may have predominant proximal muscle weakness with frequent diaphragmatic involvement. SNAP is normal on NCS, Electromyography (EMG) shows in-excitable muscle and Creatinine phospho-kinase (CK) is elevated to >20 times the normal.

The limitations of the present study included the inability to do the serum thiamine levels and nerve biopsy due to feasibility constraints. Diagnosis of Shoshin beriberi is commonly based on monitoring the therapeutic response to thiamine replacement, [5] in the appropriate clinical setting. The only definitive treatment of beriberi is the rapid intravenous administration of thiamine, which improves the adverse hemodynamic situation within minutes to hours, and is diagnostic of this rare disease. [6] To conclude, these patients had Shoshin beriberi, which is the severe form of thiamine deficiency with right heart failure and Multi-Organ Dysfunction Syndrome; coexisting with polyneuropathy. Both were reversible with thiamine replacement, the former within from 24 h to 48 h and the later over few days to weeks.

 
   References Top

1.Blankenhorn MA, Vilter CF. Occidental beriberi heart disease. J Am Med Assoc 1946;131:717-26.  Back to cited text no. 1
    
2.Park JH, Lee JH, Jeong JO, Seong IW, Choi SW. Thiamine deficiency as a rare cause of reversible severe pulmonary hypertension. Int J Cardiol 2007;121:e1-3.  Back to cited text no. 2
    
3.Zifko UA, Zipko HT, Bolton CF. Clinical and electrophysiological findings in critical illness polyneuropathy. J Neurol Sci 1998;159:186-93.  Back to cited text no. 3
    
4.Fletcher SN, Kennedy DD, Ghosh IR, Misra VP, Kiff K, Coakley JH, et al. Persistent neuromuscular and neurophysiologic abnormalities in long-term survivors of prolonged critical illness. Crit Care Med 2003;31:1012-6.  Back to cited text no. 4
    
5.Meurin P. Shoshin beriberi. A rapidly curable hemodynamic disaster. Presse Med 1996;25:1115-8.  Back to cited text no. 5
    
6.Campbell CH. The severe lacticacidosis of thiamine deficiency: Acute pernicious or fulminating beriberi. Lancet 1984;2:446-9.  Back to cited text no. 6
    



 
 
    Tables

  [Table 1], [Table 2]


This article has been cited by
1 Thiamine Deficiency in Stem Cell Transplant Patients: A Case Series With an Accompanying Review of the Literature
Amir Steinberg,Elaine Gorman,Joshua Tannenbaum
Clinical Lymphoma Myeloma and Leukemia. 2014; 14: S111
[Pubmed] | [DOI]



 

Top
Print this article  Email this article

    

 
   Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (442 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Reports
   Discussion
    References
    Article Tables

 Article Access Statistics
    Viewed2210    
    Printed60    
    Emailed0    
    PDF Downloaded59    
    Comments [Add]    
    Cited by others 1    

Recommend this journal