Research Journal of Medical Sciences
ISSN: Online 1993-6095ISSN: Print 1815-9346
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A Study of Clinical and Laboratory Profile with Outcome and Follow up of Children with Infantile Tremor Syndrome in a Tertiary Care Center
R.Y. Kalpana, S. Venugopal, S. Sarala and Mallesh Kariyappa
Page: 84-90 | Received 31 Jul 2024, Published online: 12 Oct 2024
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Abstract
Infantile Tremor Syndrome (ITS) is characterized by anaemia, skin depigmentation, tremors and developmental delay or regression. Vitamin B12 deficiency has been found to be associated with ITS in many studies. To assess the clinical profile of infantile tremor syndrome (ITS) and to correlate serum Vitamin B12 level along with response to treatment and final outcome. It is a retrospective and prospective Observational study including total 50 patients, from 6 months to 2‐year age group, admitted to Paediatric department of Vanivilas Hospital and Bowring Hospital attached to Bangalore Medical College and Research Institute, India from March 2017 to 2020 Jan and their follow up for 3 months. 45 (90%) among 50 study subjects were between 6 and 24 months, with mean age of presentation of 12.15 months. Mothers of 45 children (90%) were vegetarian by diet. 40 children (80%) were exclusively breast fed for prolonged period and had delayed initiation of complementary feeding. 40 (80%) and 10 (20%) children had features of ITS and pre‐ITS respectively. 45 children (95%) had developmental delay, 5 children (10%) had developmental regression. Severe Acute Malnutrition was evident in 35 (70%) of children. 100 % children had anaemia and 100% children had skin hyper pigmentation. Hypotonia was predominant topographical finding observed in 35 (70%). Hyperopia in 5, dystonia in 2, seizures in 2 . Half of the children had dimorphic blood picture with predominant macrocytes. Pancytopenia was noted in 70%. All the children and their moms had evidence of anemia and low serum vitamin B12 level. Very Severe anemia was noted in 10 babies and 20 required blood transfusion, also due to cardiac failure. Other micro nutrient deficiency was noted in 30 babies (60%). Serum Homocysteine was also high in 54% . Low maternal vitamin B12 level was found in all. Two children succumbed to death due to pneumonia. 20 children (40%) required treatment for tremors with Propranolol and Clonazepam. Microcephaly was seen in 25% cases (12 cases) and MRI was abnormal in 20% cases (10) mainly showing cerebral atrophy and delayed myelination. Tremors seen were limited to hands in 10 (20%) and involving hands, feet and voice in 40 (80%). Most of the findings , clinical and laboratory, except developmental delay, fully reversed on follow up of 3 months and later lost to follow up. Most of the infants and young children with ITS will have hematological findings consistent with megaloblastic anaemia and evidence of vitamin B12 deficiency. Nutritional vitamin B12 deficiency is an easily treatable cause of pseudo‐neuroregression and long‐term neuro‐deficits can result if treatment is delayed but are easily reversed with timely B12 treatment. Mothers with Vit B12 deficient diet are the main reasons for Vit B12 deficiency in children and ensuing ITS and hence they have to be screened and supplemented with VitB12 in pregnancy and 6 months post partum making available VITB12 also along with Iron Folic Acid IFA like IFAB12. Long term follow up is required to prevent recurrences especially in those on vegetarian diet and to look for complete recovery of developmental quotient above 70%.
How to cite this article:
R.Y. Kalpana, S. Venugopal, S. Sarala and Mallesh Kariyappa. A Study of Clinical and Laboratory Profile with Outcome and Follow up of Children with Infantile Tremor Syndrome in a Tertiary Care Center.
DOI: https://doi.org/10.36478/10.36478/makrjms.2024.11.84.90
URL: https://www.makhillpublications.co/view-article/1815-9346/10.36478/makrjms.2024.11.84.90