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Indian Pediatr 2016;53: 1113 |
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Infantile Tremor Syndrome
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Robin Mukkath Thomas and *Raghavendra H Gobbur
Department of Paediatrics, BLDEA University’s, Shri B M
PATIL Medical College, Vijayapura, India.
Email: [email protected]
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A previously normal, 15-month-old boy, born at
term to nonconsanguineous parents, was hospitalized for generalized
tremors for 3 days. Child was unable to cry and feed. He was exclusively
breast fed. He was classified as grade 2 (IAP) malnutrition; he also had
hyperpigmentation over limbs, pallor and hepatosplenomegaly. Tremors
were absent during sleep, and got aggravated during activities (Web
Video 1). His hemoglobin was 5.8 g/dL with dimorphic blood
picture. EEG was normal and MRI showed diffuse cerebral atrophy.
Mother’s serum B12 levels
were low (<200 pg/mL) but child’s serum B12
levels were normal, probably because of prior treatment
with injectable B12. A
diagnosis of Infantile tremor syndrome (ITS) was made. Child received
WHO protocol of management for moderate malnutrition. The child received
blood transfusion, vitamin (A, D, B complex and C) and mineral (zinc,
magnesium, calcium) supplementation. Propranolol (0.5 mg/kg/day) was
started, and increased to 2 mg/kg/day till the tremor decreased in
intensity and general condition of child improved. Kahn’s nutritional
recovery syndrome, infections (viral encephalitis/encephalopathy),
phenothiazine toxicity, degenerative diseases, and enzyme defects in
tyrosine metabolism leading to substantia nigra depigmentation are the
differential diagnoses of ITS.
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Web Video 1 Infant with characteristic
tremors suggestive of Infantile Tremor Syndrome.
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Infantile tremors are unique in that they are present
only in awake state. Tremors are fast (7Hz) and coarse, and involve
distal part of limb, especially head face and tongue. Infants with ITS
have difficulty in speech and produce sound like bleating of goat. They
may also toss the head from side-to-side.
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