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Indian Pediatr 2015;52:
628 |
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Infantile Tremor Syndrome: Still A Mystery!
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Kanwar K Kaul
Professor Emeritus and Retired Dean, NSCB Medical
College, Jabalpur, Madhya Pradesh, India.
Email: [email protected]
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With reference to the recent publication in Indian Pediatrics
[1], infantile tremor syndrome may indeed be ‘down’ perhaps in terms of
incidence, but certainly not ‘out’ in terms of etiology. Authors found
low serum vitamin B
12 only
in 8 of 16 infants and they are justifiably cautious in stating that
vitamin B12 deficiency is
"causally associated". There was initial worsening of tremors seen in
six of their patients in spite of vitamin B12
therapy.
The quest for the cause of this syndrome of tremors,
pigmentation, anemia and regression of milestones, seen in breastfed
infants of vegetarian mothers is by no means over. We had earlier called
it Infantile ‘meninoence-phalitic’ syndrome [2]. Vitamin B 12
deficiency may be an important associated factor in the biochemical
process resulting in the syndrome [3], but is unlikely to be its cause
by itself. In one of our studies [4], we have shown associated increase
in the number of bands and heavy concentration of some amino acids and
their metabolic products on serum and urine chromatography, when we
compared it to age-matched norms. Interestingly, methyl-malonic aciduria
and homocystinuria have been demonstrated in Vitamin B12-deficient
patients [5].
A word of caution is needed about the belief that it
is an entirely self-limited, transient entity, completely responsive to
therapy. A follow-up study of 2 to 10 years carried out by us [6] showed
that as many as 18 out of 23 infants recorded low scores on IQ tests
during follow-up; twelve scored an IQ below 70, with poor scholastic
performance reported by parents. Speech defects were present in a third
of all; dyslalia was present in the majority (6 out of 7), and one had
stammering. All infants had shown initial recovery in the disappearance
of tremors, anemia and pigmentation irrespective of any specific therapy
other than improved diet and general nutritional support at the onset of
the disease. We are not aware of any other similar long-term studies.
References
1. Goraya JS, Sukhjot Kaur. Infantile tremor syndrome
– Down but not out. Indian Pedatr. 2015;52:249-50.
2. Pohowalla JN, Kaul KK, Bhandari NR, Singh SD.
Infantile "meningoencephalitic" syndrome. Indian J Pediatr.
1960;27:49-54.
3. Kaul KK, Prasan NG, Chowdhry RM. Some clinical
observations and impressions on a syndrome of tremors in infants from
India. J Pediatr. 1963;1:1158-66.
4. Kaul KK, Belapurkar KM, Parekh P. Syndrome of
mental retardation, tremors, pigmentation and anemia in infants – some
biochemical observations. Indian J Med Res. 1973;61:86-92.
5. Higginbotham MC, Sweetman L, Nyhan WL. A syndrome
of methylmalonic aciduria, homocystinuria, megaloblastic anemia and
neurologic abnormalities in a Vitamin B12 deficient breast fed infant of
a strict vegetarian mother. N Engl J Med. 1978;299:317-23.
6. Kaul KK, Belapurkar KM, Parekh P. The syndrome of
tremors in infants: A long follow up. Indian J Med Res. 1972;60:1067-72.
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