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CASE REPORT
Year : 2022  |  Volume : 25  |  Issue : 1  |  Page : 32-36

Coarse tremors (Kwashi shakes) occurring in children recovering from severe acute malnutrition: Case series from a tertiary hospital in Sokoto


Department of Paediatrics, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria

Date of Submission29-Mar-2021
Date of Decision16-Jun-2021
Date of Acceptance17-Jul-2021
Date of Web Publication30-May-2022

Correspondence Address:
Dr. Khadijat Omeneke Isezuo
Department of Paediatrics, Usmanu Danfodiyo University Teaching Hospital, Sokoto
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/smj.smj_28_21

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  Abstract 


Kwashi tremors or Kwashi shakes are uncommon neurological complications of unknown etiology seen in edematous forms of severe acute malnutrition (SAM) during rehabilitation phase of treatment. Four patients with SAM seen in our facility within a 30-month period who developed coarse tremors are presented. The aim was to highlight this uncommon presentation of SAM in our setting. Of the four patients, 3 were male and 1 was a female. Their age ranged from 19 months to 2 years and they weighed between 5.5 and 8.7 kg. Their common clinical presentation included body swelling, loss of appetite, fever, and tremors which developed after discharge. All the patients had increased/overzealous protein intake while at home. The tremors resolved completely in all the patients, but more slowly in two of the patients with hypocalcaemia. In conclusion, Kwashi shakes occurring during rehabilitation of edematous SAM with increased protein intake and appears to be worsened with hypocalcemia

Keywords: Hypocalcemia, Kwashi shakes, edematous malnutrition


How to cite this article:
Isezuo KO, Amodu-Sanni M, Sani UM, Garba BI, Waziri UM, Ahmad MM, Jangebe MA, Abubakar F, Mada IL. Coarse tremors (Kwashi shakes) occurring in children recovering from severe acute malnutrition: Case series from a tertiary hospital in Sokoto. Sahel Med J 2022;25:32-6

How to cite this URL:
Isezuo KO, Amodu-Sanni M, Sani UM, Garba BI, Waziri UM, Ahmad MM, Jangebe MA, Abubakar F, Mada IL. Coarse tremors (Kwashi shakes) occurring in children recovering from severe acute malnutrition: Case series from a tertiary hospital in Sokoto. Sahel Med J [serial online] 2022 [cited 2022 Jul 6];25:32-6. Available from: https://www.smjonline.org/text.asp?2022/25/1/32/346287




  Introduction Top


The World Health Organization (WHO) defined severe acute malnutrition (SAM) as combination of low weight for height index (< -3 standard deviation [SD] of z-scores of the WHO growth standards), presence of nutritional edema and a mid-arm circumference of <115 mm and covering both nonedematous and edematous forms of malnutrition.[1]

Malnutrition is still a public health problem contributing to about 60% of childhood mortality and significant morbidity in low-income nations.[2] Most of the 20 million children with SAM are found in Africa and Asia[2] with Nigeria accounting for one of the highest rates in the world.[3]

The complications of severe malnutrition could be part of the disease process or its treatment, especially aggressive feeding.[4],[5] Affected children have reduced metabolism as an adaptation to the nutrient deficiencies. Rapid increase in protein diet can therefore result to fluid retention, and rapid production of enzymes, hormones, and neurotransmitters.[5] Hence, treatment may result in complications including refeeding edema, Gomez syndrome (portal intracranial hypertension, fatty infiltration of the liver, and hypergammaglobulinemia), and benign intracranial hypertension. Other possible complications are encephalitis-like syndromes including progressive deterioration in sensorium, Kahn syndrome which occurs early in treatment, and Parkinsonian like tremors (Kwashi shakes) later during the recovery phase.[4] Kwashi shakes is a neurological complication documented to occur in edematous forms of malnutrition or kwashiorkor.[6],[7],[8] They usually occur during rehabilitation and are self-limiting. They are also called Parkinsonian-like tremors because they increase with activity and are slower at rest.[8]

In Nigeria, tremors (Kwashi shakes) complicating SAM management have hardly been documented except during the civil war about half a century ago.[6] We therefore report four cases of edematous SAM managed in our center within 30 months to highlight the peculiarities of these cases and increase awareness among physicians.

Ethical approval

Ethical approval was sought and received from the Research and Ethics Committee of Usman Danfodiyo University Teaching Hospital, Sokoto (UDUTH/HREC/2021/1023/V1).

Informed consent was obtained from the caregivers of all the patients. their identity and data was treated with utmost confidentiality.

Case 1

KS is a 21-month-old boy. He was referred from a general hospital with fever, poor appetite of 2 weeks, and body swelling of a week duration. He had suffered pertussis 4 months prior to the onset of the illness and had been losing weight since then. He had also been weaned off breast milk 6 months before his illness and was on a protein poor complementary diet comprising unfortified cereal pap and family staple foods. He weighed 5.8 kg on admission with z-score of <3SD. Other anthropometric measurements were length of 70 cm and mid arm circumference of 10 cm. He was pale, with sub normal temperature of 36.2°C and had bilateral pitting pedal edema.

He was managed for SAM for 16 days, and then discharged with a weight of 8.7 kg. He developed abnormal body movements 10 days after discharge involving the limbs and face described as continuous involuntary shakes of the limbs which reduced during sleep. They did not interfere significantly with his activity. There was no alteration of consciousness. There was no fever or worsening of diarrhea or body swelling. He had almost doubled his admission weight in 1 month as he weighed 10.4 kg at the first follow-up visit. Dietary history revealed that he was on more than 220 kcal/kg of the ready to use therapeutic food (RUTF) paste. His mother admitted she overfed him with the paste and he had regained weight rapidly. She was pleased with the improvement and insisted on getting more of the paste to feed him.

On examination, he was conscious and appeared well fed [Figure 1], with continuous rhythmic tremors of all the limbs and no abnormal abnormal posturing [Figure 1]. He was not in respiratory distress. He was neither tachypneic nor tachycardic. Muscle tone was slightly increased with hyperreflexia but no ankle clonus. His gait was normal.
Figure 1: Shows Case 1 handling an object with tremors. He also looks well fed due to rapid weight gain in one month

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Biochemical tests, including serum calcium, serum proteins, electrolyte urea and creatinine, and hemogram were normal. He received dietary supplements, Vitamin B complex, and folic acid. Mother was counseled on the danger of too much proteins in the child's diet. He was managed on the outpatient basis with frequent follow-up visits. Tremors gradually reduced and stopped in 2 weeks.

Case 2

AS, a 19-month-old boy presents with fever, poor appetite of 3 weeks, body swelling and limb jerky limb movements, and tremors for a week. He had a poor nutritional history and had been weaned off breast milk a month prior. He was managed for SAM for 2 weeks in an outpatient therapeutic center and he was on free F-100/RTUTF paste. He was referred to our center for further management.

At presentation, he was afebrile with continuous jerky movements of the limbs interspersed with continuous coarse tremors. He was also observed to have abnormal limb posturing as upper limbs were flexed on the anterior chest wall with hyperextension of the metacarpophalangeal joints and thumb adduction. He had reduced muscle tone and was unable to sit unsupported. He was however fully conscious.

He weighed 6.4 kg on admission with z-score of < - 3 SD and was discharged with a weight of 7.6 kg after 12 days.

Blood tests revealed hypokalcemia of 1.8 mmol/l and hypokalaemia of 2.2 mmol/l which were corrected. Other test results were normal. He was managed with low protein, low calorie formula then switched to a higher protein and calorie formula. He responded fairly with weight gain. However, the tremors continued despite correction of the electrolyte abnormalities (as evidenced by the results). After 3 weeks, the tremors eventually resolved. Appetite and weight had improved significantly.

Case 3

MA, a 2-year-old female toddler presents with fever, cough, diarrhea, and weight loss of 2 weeks duration. She also had a poor nutritional history and had been weaned off breast milk 2 months prior to presentation. She weighed 8 kg on admission with z-score <3SD, mid-upper arm circumference of 11 cm, and bilateral pitting pedal edema. The mother reluctantly accepted admission and the patient was managed as a case of edematous SAM with bronchopneumonia and malaria for 3 days before she eventually signed for discharge against medical advice. She was arranged for weekly follow-up and nutritional advice. At first visit after 1 week, she had worsening edema with weight of 8.3 kg. Nutrition counseling and management were reinforced.

At the second follow-up visit, 2 weeks later, his appetite was better and patient was said to have been on pap fortified with milk formulated for children aged 1–3 years, eggs and lightly fried groundnuts to chew and in paste form added to pap. The edema resolved and she weighed 6.66 kg but z-score was still <3SD. She had generalized rhythmic movement of the body which also involved the eyelids and facial muscles in a rather continuous pattern warranting stat dose of intramuscular phenobarbitone which did not control the abnormal movements. She also had sagging jaw and slight protrusion of the tongue but no drooling of saliva. She was fully conscious and could sit without support and seen struggling with trembling hand to feed self. There were no signs of meningeal irritation, cranial nerves functions were normal, slightly increased tone and reflexes in all limbs. Rhythmic movement worsened in frequency and became coarse with time.

Subsequent biochemical tests revealed hypokalcemia of 1.45 mmol/L and hyperphosphatemia of 2.03 mmol/L for which she had intravenous calcium gluconate then oral calcium supplement. Repeat serum calcium was 2.52 mmol/L and phosphate 1.66 mmol/L. Cerebrospinal fluid (CSF) analysis was normal. Tremors improved significantly from being continuous to intermittent. Protein intake was reduced and tremors resolved gradually over the following 3 weeks.

Case 4

MA, a 24-month-old boy presents with fever, diarrhea, and vomiting of 2 weeks and leg swelling of 5 days. He had a poor nutritional and vaccination history. He was weaned off breast milk at 17 months of age. He had inpatient management for SAM for 3 weeks with enriched pap. He also had broad-spectrum antibiotics. Serum calcium, phosphate, and magnesium were normal. Human immunodeficiency virus screening was negative. Serum proteins and CSF analysis were also normal. He was discharged after 3 weeks on admission on nutritional advice of enriched pap and other high protein diets.

However, he presented 3 days after discharge, with a history of gradual onset of coarse tremors which started from one limb and gradually involved other limbs and facial muscles over 2 days. There was no fever or altered consciousness. The tremors became coarser over time but reduced significantly while sleeping. Additional nutritional history revealed that his grandmother had been feeding him mainly on egg diet in different forms. She also added raw egg into his liquid diets. Average intake of eggs was about 5 per day.

He was also observed to have rapidly gained weight during the period of discharge as his weight increased from 6.0 kg to 7.7 kg within 3 days. Other anthropometric measurements were length of 73 cm, occipitofrontal circumference of 47 cm (47–51 cm) and mid arm circumference of 13 cm (normal range 13–17 cm). His z score was < - 3SD.

At presentation, he was fully conscious, afebrile with continuous coarse tremors, and intermittent jerks. There was no abnormal limb posturing. He had reduced muscle tone and could sit without being supported.

He was commenced on a low protein, low calorie formula then switched to a higher protein and calorie formula. The tremors reduced in intensity gradually over 2 weeks. He had reoccurrence of fever warranting additional antibiotics and antimalaria which prolonged his hospital stay.


  Discussion Top


A common feature of the cases in the current series is edematous SAM,with coarse tremors occurring during the rehabilitation.phase with overzealous increase in protein intake mainly introduced at home after the acute phase of malnutrition when life-threatening complications had been curtailed. This is similar to previous reports of Kwashi shakes occuring during the rehabilitation phase of SAM treatment.[6],[8],[9]

Three of the four cases in this series were males and similar to that reported by Kahn and Falcke[7] in South Africa who made similar neurological observations among 8 children (all males) being managed for SAM within a 2-year period. Furthermore, Thame et al.[8] reported a male Jamaican toddler who was the 1st to be reported in the West Indian literature according to the authors. These suggest a possible male predilection for Kwashi shakes. The age range of our patients is also similar to those seen in the previous reports who were mainly aged between 1 and 2 years.[6],[7],[8]

Prolonged intake of cereals meals poor in protein coupled with infection are identified risk factors for the development of edematous malnutrition which was seen in these four cases similar to the literature.[10] Neurologic manifestations occurring 10–14 days after commencing high protein intake has been reported in some children during the recovery phase. This is comparable to the 2 weeks observed in the current report.[7]

Tremor is the earliest and constant neurological disorder that occurs during the recovery phase of SAM.[7] All limbs can be affected as was seen in these cases similar to reports in the literature.[7] The onset and pattern of affectation were also similar to previous reports as they initially had affectation of one or two limbs before spreading over 1–2 days to affect other limbs.[9] In addition, affectation of the face, tongue, and jaw muscles was seen in one of the patients in our series. This is similar to the cases reported by Kahn which is the part of the spectrum of severity.[9]

The absence of fever and other neurological signs before the onset of tremors did not support encephalitis in these patients. Furthermore, they were all fully conscious throughout and had normal CSF analysis. Kahn reported only one case out of 8 in whom CSF findings were abnormal supporting that the etiology was predominantly noninflammatory cause.[9] Although toxicological tests were not done in our patients, none of the children had known exposure to sources of heavy metals and were not epidemiologically linked to one source of diet. Kahn had reported food contamination with toxins that can cause extrapyramidal disorders as a possible causative factor.[9] Neurological disorder of vitamin deficiency was not likely in the three cases because they were on Vitamin B-complex and folic acid long before the onset of the manifestations.

The observed hypokalcemia in cases 2 and 3 has been associated with SAM. However, symptoms of hypocalcaemia are usually abated with calcium correction unlike in these patients in whom calcium treatment did not resolve the tremors. This background hypokalcemia may have worsened the tremor that developed during the recovery phase from SAM as the onset of tremors was much earlier during the recovery phase in case 2 and 3 (2nd week) while it occurred almost at the end of the 3rd week (26th day) in case 1 and 4. The patient with later onset also had a faster resolution. Two of the cases reported by Kahn and Falcke[7] had frank features of rickets in addition to documented hypocalcemia.

The patients did well during follow-up with no reoccurrence or neurologic deficit similar to other reports.[6],[8],[9] Kahn reported low case fatality rate, but this was among patients whose clinical course had been compounded by infectious complications.[9]


  Conclusion Top


Tremors during the treatment of edematous SAM occur in children recovering from malnutrition and may be worsened with hypocalcemia. Caregivers should be taught how to prevent this complication through appropriate feeding during the recovery phase.

What is already known on this topic

  • Children with edematous malnutrition may have neurological complications such as coarse tremors// Kwashi shakes during recovery due to high protein diet.


What this study adds

  • Hypocalcemia may worsen the Parkinsonian like tremors seen in children recovering from edematous malnutrition.


Acknowledgments

We are grateful to all the nursing staff that were involved in the care of these patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Gernaat HB, Voorhoeve HW. A new classification of acute protein-energy malnutrition. J Trop Pediatr 2000;46:97-106.  Back to cited text no. 1
    
2.
WHO. Severe Acute Malnutrition; 2011. Available from: http://www.who.int/nutrition/topics/malnutrition/en/index.html. [Last accessed on 2019 Jul 16].  Back to cited text no. 2
    
3.
Severe Acute Malnurtition Management in Nigeria. Challenges, Lessons and the Road Ahead. Executive Summary March 2015. ACF International. Available from: https://ciff.org/library/severe-acute-malnutrition-northern-nigeria-challenges -lessons-road-ahead-summary/. [Last accessed on 2020 Jan 25].  Back to cited text no. 3
    
4.
Gupte S. The Short Textbook of Pediatrics. [E-Book] India: Jaypee Brothers, Medical Publishers Pvt. Limited, India; 2016. Available from: https://books.google.com.ng/books?id=6W2JDAAAQBAJ. [Last accessed on 2020 Jan 26].  Back to cited text no. 4
    
5.
Parthasarathy A. Partha's Fundamentals of Pediatrics. Jaypee Brothers, Medical Publishers Pvt. Limited, India; 2013.  Back to cited text no. 5
    
6.
Woodd-Walker RB. Kwashi shakes. Lancet 1970;1:299.  Back to cited text no. 6
    
7.
Kahn E, Falcke HC. A syndrome simulating encephalitis affecting children recovering from malnutrition (kwashiorkor). J Pediatr 1956;49:37-45.  Back to cited text no. 7
    
8.
Thame M, Gray R, Forrester T. Parkinsonian-like tremors in the recovery phase of kwashiorkor. West Indian Med J 1994;43:102-3.  Back to cited text no. 8
    
9.
Kahn E. A neurological syndrome in infants recovering from malnutrition. Arch Dis Child 1954;29:256-61.  Back to cited text no. 9
    
10.
Williams C. Kwashiorkor nutritional disease of childhood associated with maize diet. Arch Dis Childhood 1933;8:423-33.  Back to cited text no. 10
    


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