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|LETTER TO EDITOR
|Year : 2021 | Volume
| Issue : 2 | Page : 524-525
Nutritional Management of Diencephalic Syndrome: A Case Series
Ekta Chheda1, Samina Vora1, Maya Prasad2
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 Submission||21-Jun-2020|
|Date of Decision||04-Aug-2020|
|Date of Acceptance||12-Dec-2020|
|Date of Web Publication||24-Apr-2021|
Division of Pediatric Oncology, Tata Memorial Hospital, Dr. Ernest Borges Road, Parel, Mumbai - 400 012, Maharashtra
Source of Support: None, Conflict of Interest: None
|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
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. The clinical presentation is characterized by hyper-alertness, hyperkinesia, euphoria, nystagmus, hydrocephalus, visual field defects, optic pallor, and emesis., Generally, successful treatment of the tumor results in normalization of growth and endocrine function. 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. Patients received aggressive nutritional intervention, follow-up and rehabilitation as per SIOP PODC algorithm. 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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