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NT Bureau
Chennai, Feb 18:
 

TVS Motors chairman and managing director Venu Srinivasan presenting a medallion and a scroll to Professor Stanley Prusiner from the University of California, San Francisco, for delivering the T S Srinivasan Endowment Oration in Chennai yesterday. Also seen are neurologist Dr Krishnamoorthy Srinivas and VHS secretary Dr N S Murali.

        With rising economic prosperity and extended longevity, Indians are no exception to being affected by strange sounding neurodegenerative symptoms and disorders. Well, one may be aware of Alzheimer's disease or even Parkinson's. Here is another to this growing list of brain-related diseases — Creutzfeldt-Jakob disease (CJD).

        CJD is caused by the accumulation of prions — a type of misshapen protein with no nucleic acid that differs from pathogens like bacteria and virus, but is sporadic and infectious to the core — in the brain and these agents let out no dramatic symptoms, except for mild cognitive impairment. 'All the three types of brain diseases, namely Alzheimers, Parkinson's and CJD lack a proven cure. Moreover, prion diseases are uniformly fatal with no effective therapies,' said Professor Stanley B Prusiner from the University of California, San Francisco (UCSF) and director of the Institute for Neurodegenerative Diseases. He was delivering the 27th T S Srinivasan Endowment Oration in Chennai yesterday on Viruses, Genes and Mad Cows — Lessons from Prion Disease.

        A recipient of Nobel Prize for Medicine in 1997 for establishing a new mechanism of disease formation through the incubation of prions, an infective protein that progressively leads to three major neuro-disorders in humans — CJD, Gerstmann - Straussler- Schienker (GSS) and Familial Insomnia (FI), both of which are variants of CJD, and the now extinct Kuru, prevalent in cannibals. However, it was the Mad Cow Disease — bovine spongiform encephalopathy (BSE) — that spread rapidly from Britain to rest of the Europe to create a health crisis and also threatened the safe blood transfusion in the world, Dr Prusiner said. Besides MCD, Scrapie and Chronic Wasting Disease (CWD) were other prion diseases affecting animals.

        The transmission of MCD from animals to humans across Europe in early 90s brought to focus the major problems and intrigues in deciphering the molecular layering of prion strains. Also, there were no sterilisation procedures for eliminating prions. Being highly sporadic and infective, prions multiplied by adopting at least two different conformations. Structural changes in three helices in a benign, normal protein resulted in a prion which foraged into other healthy proteins for conversion leading to fatal disorders like CJD and its variants, Dr Prusiner explained. 'But prions are not viruses despite what some would like to believe. In the spectrum of human prions, CJD is most sporadic and spontaneous affecting the age group of 60-75 years. The incidence rate is 1-2 cases for a million population. CJD is 5,000 times less common than Alzheimers and 1,000 times less common than Parkinson's disease,' Prusiner said.

        Though prions could be detected in low-density lipoprotein (LDL) enriched blood fractions, the challenge was in developing a proper blood test for identifying these infective particles, he said.

        On the therapy front, Prusiner said several approaches could be taken in search for a drug, including synthetic prions, cultured cells and transgenic mouse models. 'A combination of quinacrine and Pgp (P-glycoprotein) inhibitors such as Verapamil could be an effective therapy for CJD,' he pointed out. He informed a clinical trial for treatment of CJD patients was underway at UCSF. However, improving the candidate drug was not simple and the lead compound quinacrine was the focus of study to improve the cultured cells and synthetic prions.

        Earlier, T S Srinivasan Charitable Trust chairman Venu Srinivasan mentioned the rapid strides made by T S Srinivasan Centre for Clinical Neurosciences, the academic department at VHS, Adyar, in a short period in the fields of neurological research and study. The research centre also taught PG courses in Neuroscience and had seen 19 students graduate from its portals so far. Affiliated with the clinical programme of the VHS, the Srinivasan Centre offered innovative clinical research with integrative efforts and a utilitarian design. Under a community project, the trust has seed funded the networking of VHS with primary health centres for a low-cost, sustainable models of telemedicine, Venu Srinivasan informed.

        He recalled the idealism of his father T S Srinivasan whose actions were rooted in social responsibility and took active interest in the health and welfare of his employees. TVS Motors CMD presented a medallion and a scroll for the endowment orator Prusiner on the occasion.

        Dr N S Murali, secretary, VHS, said the Department of Neurology has built an international name in neurosciences and the setting up of T S Srinivasan Centre as a research oriented teaching institute has fulfilled the vision of Dr Sanjivi, the VHS founder. Dr Krishnamoorthy Srinivas, chairman, Institute of Neurological Sciences at the VHS presided over the function.