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Letters to the Editor

Indian Pediatrics 2004; 41:204-205

Mystery Behind Mysterious Disease: Far from Unraveled !  


Kudos to Indian Pediatrics for bringing upfront the issue of emergence of hitherto unknown childhood encephalopathic illnesses from different parts of the country in recent times by publishing a spate of articles in the journal(1-4). The proactive approach adopted by the journal is quite exemplary and is indeed an example of receptive and responsive piece of medical journalism. However, there are certain issues that need to be considered in some details.

Though Dr Jacob John in his viewpoint(2) did try quite earnestly to unravel the mystery of these so called ‘mysterious diseases’, but even he could not go beyond ascertaining a tag of ‘Reye syndrome’ (RS), which in itself is quite a non-specific entity to these epidemics. Hence, the exact etiology of the illness is still obscure.

Dr Jacob John is quite right in identifying RS as the major illness masquerading as ‘killer brain disease’ but the fact remains that RS is no longer a distinct clinico-pathologic entity but a descriptive term covering a group of heterogengous disorders of infectious, metabolic, or toxic etiology(5). ‘Classic’ Reye syndrome is a term used by most western experts to define a strong association between giving aspirin to children with viral illness and development of encephalopathy. However, few researchers have even started questioning its existence- if it was ever really existed?(6). A more appropriate term would be ‘Reye-like encephalopathy’ till an exact etiology of the disease is established. Even more desirable would be to coin an altogether a different name for this deadly disease as suggested by Dr Jacob John himself(2).

In his other write-up(4), Dr Jacob John has questioned the etiopathologic role of measles virus in the genesis of RS as claimed by NIV, Pune team(7). Though isolation of measles virus from CSF should provide an incontrovertible proof of causal relationship of the agent with the syndrome, there are some missing links as described in both the articles(4,7). Although measles has now acquired a ‘perennial trait’ and ceased to be a seasonal disease especially in these geographic areas, but why should it choose only early winter months to behave so unusually and so nastily is quite inexplicable and baffling!

This year again the cases of ‘Reye-like encephalopathy’ have started trickling in and so far (till last week of November 2003) we have treated 13 such children. Measles IgM were found positive in 5 out of 8 sera and 3 out of 4 CSF samples (the commercial kits used for serum IgM was ‘Anda, Italy’ and for CSF ‘Wellcogen’, manufactured by Murex Biotech, UK). This time, IgM against measles was also studied in 4 age-matched control samples (3 in serum and 1 in CSF) and all of them were found negative. One child who survived the disease, later developed IgG antibody in serum on repeat testing after 10 weeks.

All these findings do strongly favor measles role somewhere in the pathogenesis of the syndrome. Whether direct invasion of CNS was responsible for the appearance of the syndrome or the virus infection only ‘primed’ the case for a putative ‘co-factor’ to unleash cascade of events remain the major questions to be answered.

Vipin M. Vashishtha,
Consulting Pediatrician and Neonatologist,
Mangla Hospital,
Shakti Chowk, Bijnore 246 701, U.P., India
E-mail: [email protected]

References

1. Balraj V. Investigation of outbreaks in India. How good are we at it? Indian Pediatrics 2003; 40: 933-938.

2. John TJ. Outbreaks of killer brain disease in children. Mystery or missed diagnosis? Indian Pediatrics 2003; 40: 863-869.

3. Vashishtha VM. Brief profile of an epidemic of acute encephalopathy in Western Uttar Pradesh. Indian Pediatrics 2003; 40: 920-922.

4. John TJ. Encephalopathy without rash, caused by measles virus? More evidence is needed. Indian Pediatrics 2003; 40: 589-593.

5. Casteels- VanDaele M, Van Geet C, Wouters C, Eggermont E. Reye’ s syndrome revisited: a descriptive term covering a group of heterogenous disorders. Eur J Pediatrics 2000; 159: 641-648.

6. Orlowski JP. Whatever happened to Reye’s syndrome? Did it ever really exist? Crit Care Med 1999; 27: 1582-1587.

7. Wairagkar NS, Shaikh NJ, Ratho RK, Ghosh D, Mahajan RC, Singhi S, et al. Ioslation of measles virus from cerebrospinal fluid of children with acute encephalopathy without rash. Indian Pediatr 2001; 38: 589-595.

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