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Table of Contents    
LETTER TO EDITOR
Year : 2021  |  Volume : 69  |  Issue : 2  |  Page : 524-525

Nutritional Management of Diencephalic Syndrome: A Case Series


1 Division of Pediatric Oncology, Department of Medical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
2 Division of Pediatric Oncology, Department of Medical Oncology, Tata Memorial Hospital; Department of Medical Oncology, Homi Bhabha National Institute, Mumbai, Maharashtra, India

Date of Submission21-Jun-2020
Date of Decision04-Aug-2020
Date of Acceptance12-Dec-2020
Date of Web Publication24-Apr-2021

Correspondence Address:
Maya Prasad
Division of Pediatric Oncology, Tata Memorial Hospital, Dr. Ernest Borges Road, Parel, Mumbai - 400 012, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.314587

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How to cite this article:
Chheda E, Vora S, Prasad M. Nutritional Management of Diencephalic Syndrome: A Case Series. Neurol India 2021;69:524-5

How to cite this URL:
Chheda E, Vora S, Prasad M. Nutritional Management of Diencephalic Syndrome: A Case Series. Neurol India [serial online] 2021 [cited 2021 May 15];69:524-5. Available from: https://www.neurologyindia.com/text.asp?2021/69/2/524/314587




Sir,

Diencephalic syndrome (DS), or Russell's syndrome, is a rare cause of failure to thrive in early childhood that is associated with severe emaciation despite normal calorie intake.[1] The clinical presentation is characterized by hyper-alertness, hyperkinesia, euphoria, nystagmus, hydrocephalus, visual field defects, optic pallor, and emesis.[2],[3] Generally, successful treatment of the tumor results in normalization of growth and endocrine function.[1] The authors report four cases of DS and their experience with the nutritional management of DS in a tertiary care center. Clinical and anthropometry data were extracted from case files, electronic medical records and nutrition databases. All children underwent complete age-appropriate endocrine, ophthalmic and radiological evaluation. Complete surgical excision was done where feasible. Children with inoperable or incompletely excised tumors received institutional chemotherapy owing to young age.[4] Patients received aggressive nutritional intervention, follow-up and rehabilitation as per SIOP PODC algorithm.[5] All four children received nasogastric tube (NGT) feedings along with a balanced, nutrient-dense oral diet. Energy and protein were calculated at baseline and periodically thereafter. In spite of receiving more than 100% of the nutrient requirement, weight gain varied between patients, possibly related to decrease in tumor size or pressure effects. [Table 1] enlists the details of nutrition intervention and anthropometric measurements of the four children. At the end of treatment, there was a neurological improvement in the form of improved mood, cognition and gradual achievement of previously delayed motor milestones. Although nutrition intervention was started early on for all four children, the response was noted only after the tumor started reducing in size. Patients 1 and 2 had early and sustained clinical and nutritional improvement, whereas response in patients 3 and 4 was gradual, albeit sustained, however, the vision was static in all children due to established optic atrophy. DS is a differential diagnosis in children with failure to thrive, especially when associated with features suggestive of central nervous system tumors. Appropriate and early management of the tumor, as well as aggressive nutritional monitoring and intervention, are key to successful management of DS.
Table 1: Nutrition status of patients at baseline, and follow-up after Intervention

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  References Top

1.
Gropman AL, Packer RJ, Nicholson HS, Vezina LG, Jakacki R, Geyer R, et al. Treatment of diencephalic syndrome with chemotherapy: Growth, tumor response, and long term control. Cancer 1998;83:166–72.  Back to cited text no. 1
    
2.
Kim A, Moon JS, Yang HR, Chang JY, Ko JS, Seo JK. Diencephalic syndrome: A frequently neglected cause of failure to thrive in infants. Korean J Pediatr 2015;58:28–32.  Back to cited text no. 2
    
3.
Stival A, Lucchesi M, Farina S, Buccoliero AM, Castiglione F, Genitori L, et al. An infant with hyperalertness, hyperkinesis, and failure to thrive: A rare diencephalic syndrome due to hypothalamic anaplastic astrocytoma. BMC Cancer 2015;15:1-5.  Back to cited text no. 3
    
4.
de Blank P, Bandopadhayay P, Haas-Kogan D, Fouladi M, Fangusaro J. Management of pediatric low-grade glioma. Curr Opin Pediatr 2019;31:21–7.  Back to cited text no. 4
    
5.
Ladas EJ, Arora B, Howard SC, Rogers PC, Mosby TT, Barr RD. A framework for adapted nutritional therapy for children with cancer in low- and middle-income countries: A report from the SIOP PODC nutrition working group. Pediatr Blood Cancer 2016;63:1339–48.  Back to cited text no. 5
    



 
 
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