Sunday, September 26, 2004
Around 3000 aged Goans may be suffering from dementia: DSG
NT Staff Reporter
Panaji Sept 25: A study of only two Goan villages, Mandur and St Cruz,
has revealed a prevalence of 2.9 per cent cases of dementia among people
over the age of sixty. The study was conducted by Dr Amit Dias,
secretary of the Dementia Society of Goa (DSG). In the past two years,
the DSG has realised that degenerative diseases like Alzheimers disease
do exist in Goa contrary to the belief of some people.The above
information was given by the DSG president, Dr C J Vas, in a press
release on the commencement of the National Dementia Awareness Week
beginning on September 20. Dr Vas states that there are around 1 lakh
people over the age of 60 in Goa and DSG believes there are at least
3000 people with dementia in the state. Though Goa enjoys better health
services than most other states of the country, many patients travel to
neighbouring cities for medical care. Facilities for diagnosis and
management of the demented are meagre in Goa, but now have been initiated.
Dr Vas states that though homes have sprung up for the aged with the
means but there is still a shortage of caring people. There is no home
or respite centre for the demented anywhere in India but three day-care
centres have been established in Kerala.
Besides Alzheimers disease, Goa also has other dementias like Lewy
body, vascular, mixed, picks, CJD, progressive supranuclear palsy,
alcohol induced and the like. Dr Vas states that three CJD cases have
been reported. The first Goan patient investigated in Mumbai had
classical manifestations of CJD and the autopsy confirmed it. Diagnostic
activity in Goa has been hampered by the absence of standardised
psychological instruments such as the MMSE, the Hindi MMSE, ADAS-cog,
the CAMDEX and the like in Konkani. A beginning has now been made with a
Konkani version of the MMSE and Dr Vas hopes others will follow if time
and funds permit.
A qualitative study on ageing and mental health in Goa confirmed that
family members were more often the main carers of the old but many
elders lived alone and were subjected to abuse and neglect. They were
often cheated of their meagre financial resources and property and had
no legal protection. Dr Vas states that attempts are being made to
remedy this lacuna.
The Dementia Society in Goa, founded in 2002, aims to have full-fledged
home for those affected by Alzheimers in a 400-year old house gifted to
the DSG by the Gonsalves family of Brittona. The DSG hopes that with the
promised support and funds, a respite home and day care centre will be
set up followed by the development of structured training for
dementia-care workers and improvement in the facilities available for
The society has also completed the Dementia - Home Care Project on a
pilot basis in Bardez and Tiswadi and this has shown encouraging
results. The project was supported by the World Health Organisation and
aimed at empowering the carers. It could become the model for dementia
services throughout the country, Dr Vas states.
With the aged population of Goa growing rapidly, dementia has been a cause
of concern. Goa has around 1 lakh people aged above 60, and the six
epidemiological studies have reported that the prevalence of dementia and
Alzheimer's in India, ranged between 0 and 3.5% in the post-imaging era. A
pilot study early last year by Dr. Amit Dias in Mandur, Goa, revealed a
prevalence of 2.9% (around 3000), contrary to the belief of some. Other
dementia's like Lewy body, vascular, mixed, Picks, CJD, progressive
supranuclear palsy, alcohol induced and normal pressure hydrocephalus, have
also been noticed in Goa, says Dr. C.J. Vas of the Goa Medical College. He
stated that there was no Home or Respite Centre for the demented anywhere in
India, though three day-care centres have been established in Kerala.
(CJD) : Report of 10
Cases from North India
I read with interest the article on Creutz-Jacob
Disease (CJD) : Report of 10 cases from North
India.1 As the authors have remarked, CJD is being
increasingly reported from various regions in India.
CJD Registry at NIMHANS initiated by Shankar and
Satish Chandra2 is a step in the right direction to
know about the prevalence of CJD in India.
Singhal and Dastur,3 described 7 patients from
Western India way back in 1983. There was autopsy
confirmation in 4 patients and attention was drawn to
the fact that 2 of the 7 patients were vegetarian. They
also documented the earlier reports of CJD from India
in their article for historical purposes.
1. Mehndiratta MM, Bajaj BK, Gupta M et al : Creutzfeldt-Jacob
disease : Report of 10 cases from North India. Neurol India
2001; 49 : 338-341.
2. Shankar SK, Satish Chandra P : Creutzfeldt-Jacob disease
Cases in India in 30 years (1968-1997). CJD Registry
NIMHANS, Bangalore, India.
3. Singhal BS, Dastur DK : Creutzfeldt Jacob Disease in
Western India. Observation in 7 patients.
Neuroepidemiology 1983; 2 : 92-100.
Department of Neurology, Bombay Hospital Institute of
Medical Sciences, 12, Marine Lines, Mumbai - 400
I thank Dr. Singhal for showing interest in our article.
I am aware of the study reported by Singhal et al in
1983. I think incidence of CJD may be much more
than being reported. Many of the cases of CJD are
probably being underdiagnosed or misdiagnosed.
Many of the patients may not report to the hospital
because of rapidly progressive course and death
occuring within few weeks to months and cause of
death may get a dfferent label.
113 Neurology India, 50, March 2002
7.6 Could vaccines prepared from animal brain tissue pose a risk of
transmission of Transmissible Spongiform Encephalopathies to humans?
Over 40,000 deaths due to rabies are reported annually worldwide and
each year seven to eight million people receive antirabies vaccine
treatment following dog bites. Dog rabies poses a significant public
health problem in Asia, as 85% of the human deaths due to rabies
reported worldwide and 80% of the vaccine doses applied in
developing countries come from this part of the world.
In many Asian countries such as Bangladesh, India, Nepal and Pakistan,
sheep-brain based Semple vaccine 15 is the only vaccine available free
of cost. It represents 50 to 95% of all vaccine doses used for rabies
post-exposure treatment, depending upon the country. A complete
treatment consists of 10 subcutaneous daily injections
of 2 to 5 ml (depending mainly on patient size and nature of the
exposure) plus booster doses; that is a total of 25 to 50 ml of the 5 %
sheep brain suspension injected over a 10-day period.
According to the literature, the reported rate of neuroparalytic
complications following the use of this vaccine varies from 1:600 to
1:1575 administrations, and 20-25% of these lead to death. The exact
incidence of neuroparalytic complications throughout India or other
countries in the area is not known. However, in the State of
Karnataka, India, 112 cases of neuroparalytic accidents were admitted in
the past 20 years following Semple vaccine administration. In contrast,
the newly developed cell culture or embryonating egg vaccines are
effective and safe, with lower and less severe complication rates.
In many Asian countries, Semple type vaccine has been used for the past
90 years. In India forty million ml of this vaccine are produced in this
country to treat at least 500 000 persons each year. In Pakistan 450 000
and in Bangladesh 60 000 people receive Semple type vaccine after
possible exposure to rabies. There is a theoretical risk of TSE
transmission to humans through parenteral administration of
these products. Although there is to date no evidence of such
occurrences in human medicine, recent events in the TSE field have
demonstrated that an animal TSE agent could affect human beings.
The situation is very similar regarding rabies vaccines for animal use.
For example various Indian veterinary vaccine institutes prepare 100
million ml of Semple vaccine for use in both rabies pre-and
post-exposure prophylaxis in dogs and food production animals each year.
Scrapie could be theoretically transmitted to animal vaccine recipients,
especially ruminants, through sheep-brain based vaccines such as
Semple type vaccine. This could happen because scrapie infectivity, if
present, would not be inactivated by the manufacturing process. In this
connection, a recent 15 Ãx-propiolactone inactivated or phenolized
antirabies vaccine containing 5% suspension of sheep brain infected with
a fixed strain of rabies virus.
34 WHO Consultation on Public Health and Animal TSEs
Epidemiology, Risk and Research Requirements
publication strongly suggests that scrapie was transmitted to sheep and
goats through the administration of a veterinary vaccine whose method of
preparation is similar to the Semple type vaccine. In addition, various
Asian countries have begun to use animal tissues as feed supplement for
intensive sheep and dairy cattle production. This introduces an
additional, though still theoretical, possibility that scrapie, or even
BSE, could spread among the sheep population and enter the sheep flocks
that are used as a source of rabies vaccine production for human or
animal use. In areas where the status of animal TSE is not well
documented, this risk cannot be totally ruled out, though it may be
remote, as there is no test available at present to detect
pre-clinical cases of prion disease in sheep.
Human vaccines prepared from whole ruminant brains may carry the risk of
transmission of animal TSE agents, because the inactivation processes
usually applied to these products do not inactivate TSE agents. In
particular, considering the recent emergence of vCJD in humans related
to BSE in cattle, the consultation recommends that the use of these
vaccines should be avoided if suitable alternatives can be made
available. The Consultation strongly supported the recommendation made
by WHO Expert Committee on Rabies, which states:
"The (Expert) Committee reiterated, as stated in its 1983 report, its
support for the trend to limit or abandon completely - where
economically and technically possible - the production of
encephalitogenic brain-tissue vaccines, and strongly advocated the
production and use of inactivated cell-culture rabies vaccines in both
developed and developing countries."
The use of veterinary vaccines prepared from whole ruminant brains, for
use in ruminants, should be avoided unless the process ensures TSE
inactivation and/or removal, or the source animals have been
demonstrated to be free of any TSE.
III.3. IATROGENIC TRANSMISSION
Iatrogenic transmission of BSE has not been reported, or even suspected,
in cattle but there are some definite occurrences of scrapie in sheep
that have been reliably attributed to the use of non-commercial vaccines
containing ovine starting materials. For this reason, the issue is
discussed below. Other forms of iatrogenic transmission of TSE have been
restricted to humans and human tissues. For the sake of completeness and
convenience, these subjects are briefly discussed below.
Reference has already been made to the occurrence of at least several
hundred cases of scrapie in British sheep as a direct result of the use
of a vaccine against the tick transmitted, viral disease, louping-ill
(Gordon, Brownlee and Wilson, 1939, Gordon, 1946 and Greig, 1950). This
occurrence resulted from the accidental use of scrapie-infected source
material and processing methods that did not inactivate the scrapie
agent that was unknowingly present. A more recent possible occurrence of
possible iatrogenic scrapie has recently been reported in Etna Silver
crossbred goats in Italy by Cappucchio et al., (1998). The goats were
kept at grass and concentrate rations were not fed, thus eliminating a
source of infection from feed via mammalian proteins. Animals over two
months old were annually vaccinated against contagious agalactia caused
by Mycoplasma agalactiae. The vaccine included central nervous system
from pathogen-free sheep. The mortality rate in the goats reached
28% in 1 herd, 60% in the second and 5.5% in a third herd. About half
the 56 goats were between 2.5 - 3 years old. Only 1.15% of sheep that
were kept with the goats developed scrapie. Scrapie was confirmed by
microscopic examination of the brain and by detection of PrPSc including
by immunocytochemistry. PrPSc was widespread in the brain and beyond
sites of vacuolar change. The high mortality, severe loss of weight and
simultaneous appearance in the three herds were distinctly unusual
features in this outbreak. The source of infection remains uncertain and
unproven but iatrogenic transmission must be considered.
A larger epidemic involving 20 outbreaks of scrapie in sheep and goats,
also in Italy, has been even more recently reported by Agrimi et al.,
(1999). The annual incidence ranged from 1% to 90% with a mean incidence
for goats of 26% and for sheep of 10%. The total number of cases in
sheep and goats together was 1040. The clinical disease was confirmed by
microscopic examination of the brain and PrP immunocytochemistry or
Western blotting. The high incidence in goats, the high
within-flock/herd incidence, the temporal clustering, absence of
commercial concentrate feeding in eight flocks and association with the
use of a sub-cutaneously administered M. agalactiae vaccine, prepared
locally using brain and mammary tissue from clinically healthy sheep,
strongly suggests an iatrogenic origin. Scrapie appeared between 23 and
35 months after the vaccine was administered. A third outbreak in
southern Italy attributed also to the same vaccine has been described by
Caramelli et al, (2001) in a mixed flock of Comisana sheep and half-bred
goats in an upland area of southern Italy. High crude mortality and
scrapie incidence occurred in both species and a large proportion of
aged animals were affected. The neuropathology was similar to that in
other sheep in Italy with iatrogenic disease but different from
conventional natural scrapie. Affected sheep were all of the most
susceptible genotype (Codon 171 QQ). It is stressed that the vaccines
incriminated in the transmission of scrapie in all these incidents are
not commercially produced. They have been prepared and distributed
locally within the country. Dr Subash Arya has repeatedly drawn
attention to the possible risk of transmitting CJD to humans vaccinated
with sheep-brain derived vaccines in India, e.g. Arya, (1994). However,
neither Dr Arya nor any of his colleagues has yet found any such case.
The episodes of scrapie resulting from the use of vaccines prepared from
infected sheep tissues emphasises the need for caution and mandatory
selection of safe sources for starting materials used in the manufacture
of vaccines. Such vaccines could theoretically at least, be used in
cattle thus creating a potential risk, though it is most unlikely that
they would be licensed for this purpose in Europe. Vaccines have not
been incriminated in the transmission of BSE (Wilesmith et al., 1988,
J.W.Wilesmith, personal communication). Furthermore, large numbers of
doses of commercially produced vaccines that have used bovine starting
materials, have been inoculated by parenteral and oral routes into
cattle throughout the world and a substantial proportion have been
produced in Europe, but no incident of BSE has been attributed to their
use. This is important because, since there is no species barrier, any
chink in the armour protecting vaccines from contamination would have
been revealed, but none has.
III.3.2.OTHER MEDICINAL PRODUCTS DERIVED FROM TSE-SUSCEPTIBLE SPECIES
Animal sources of material used in medicinal products vary, but mostly
are derived from cattle. There is thus at least a possibility that
unless strict precautions are taken, disease could be transmitted in
this way. It cannot be ruled out that no case ever arose by this means,
but it is clear that the majority did not, even at the very beginning of
the BSE epidemic before publication of information on BSE, and before
any legislation was in place (Wilesmith et al., 1988). The highest risk
tissue is bovine brain from a clinically affected animal or one in the
immediate pre-clinical phase. Posterior pituitary extract (now prepared
biosynthetically), was available and used in veterinary practice mainly
in adult female cattle at the time of parturition, to assist treatment
of retained placenta or to assist in milk let down. However, no
association was found between its use and the occurrence of BSE
(Wilesmith et al., 1988).
Indian J Pediatr 1991 Sep-Oct;58(5):563-5
Centre for Logistical Research and Innovation, Greater Kailash, New Delhi.
BMJ 1996 Nov 30;313(7069):1405
* BMJ. 1996 Aug 24;313(7055):441.
Blood donated after vaccination with rabies vaccine derived from
sheep brain cells might transmit CJD.
BMJ 1996;313:1405 (30 November)
Blood donated after vaccination with rabies vaccine derived from sheep
brain cells might transmit CJD
EDITOR,--Janet Morgan reports that the National Blood Authority in
Britain has decided to tighten the donor screening programme to exclude
transmission of Creutzfeldt-Jakob disease or its variant through blood
donations.1 Prospective donors will be prevented from donating blood if
they have a history of treatment with human growth hormone or if one of
their siblings, parents, or grandparents developed the disease. I would
point out that similar care should also be taken when immigrants from
Asia and Africa offer to donate blood, in case they received rabies
vaccine derived from culture of sheep brain cells when they were living
in their country of origin.
In many countries in Asia and Africa limited supplies of imported rabies
vaccines derived from culture of human cells have been available. Many
people continue to be offered indigenously produced sheep brain vaccine
after exposure to a rabid animal. Scrapie is known to exist in sheep
around many centres where the vaccine is produced. In the mountain sheep
of the Kumaon foothills in the Himalayas, for example, scrapie was
established more than four decades ago and 1-10% of the flock was
reported to have the disease in 1961.2 In the Himalayan foothills the
Central Research Institute continues to produce four to five million
doses of sheep brain vaccine annually. Transmission of abnormal prion
protein, PrPsc, in sheep brain vaccine might have occurred in some of
the 30 documented cases of Creutzfeldt-Jakob disease in different
regions in India.3 Because Creutzfeldt-Jakob disease has a latency of
about 20 years, many recipients of sheep brain rabies vaccine could
emigrate to Britain before becoming ill.
Before accepting blood donations from immigrants it would be desirable
to ask the potential donors whether they were exposed to a rabid animal
and immunised with sheep brain rabies vaccine in their country of
origin. Furthermore, indirect assessment should be possible through, for
example, assay looking for antibodies specific to rabies.
Clinical microbiologist Centre for Logistical Research and Innovation,
M-122 (of part 2), Greater Kailash-II, New Delhi-110048, India
Subhash C Arya
: Neuroepidemiology 1991;10(1):27-32
Creutzfeldt-Jakob disease in India (1971-1990).
Satishchandra P, Shankar SK.
Department of Neurology, National Institute of Mental Health and
Neurosciences (NIMHANS), Bangalore, India.
Thirty cases including 20 definite and 10 probable cases of
Creutzfeldt-Jakob disease (CJD) seen in India between 1971 and 1990 are
reported. Demographic analysis has shown similarities to the previously
published reports from other parts of the world. Though 21 (70%) of
cases were from two centers--Bombay and Bangalore-, suggesting
clustering, this seems to be more apparent than real. One subject worked
in the medical field, where possibility of iatrogenic transmission could
not be ruled out. None of the cases had positive family history of CJD.
There is no epidemiological data of CJD from India so far and hence this
report is one such pilot study.
i recieved the 1947 report of the Louping-ill vaccine
incident and posted on www here;
Louping-ill vaccine (scrapie transmission by vaccine)
THE VETERINARY RECORD
516 No 47. Vol. 58
November 23rd, 1946
NATIONAL VETERINARY MEDICAL ASSOCIATION OF GREAT BRITAIN AND IRELAND
ANNUAL CONGRESS, 1946
The enquiry made the position clear. Scrapie was developing in
the sheep vaccinated in 1935 and it was only in a few instances
that the owner was associating the occurrence with louping-ill
vaccination. The disease was affecting all breeds and it was
confined to the animals vaccinated with batch 2. This was clearly
demonstrated on a number of farms on which batch 1 had been
used to inoculate the hoggs in 1935 and batch 2 to inoculate
the ewes. None of the hoggs, which at this time were three-
year-old ewes. At this time it was difficult to forecast whether all
of the 18,000 sheep which had received batch 2 vaccine would
develop scrapie. It was fortunate, however, that the majority of
the sheep vaccinated with batch 2 were ewes and therfore all
that were four years old and upwards at the time of vaccination
had already been disposed of and there only remained the ewes
which had been two to three years old at the time of vaccination,
consequently no accurate assessment of the incidence of scrapie
could be made. On a few farms, however, where vaccination was
confined to hoggs, the incidence ranged from 1 percent, to 35 percent,
with an average of about 5 percent. Since batch 2 vaccine
had been incriminated as a probable source of scrapie infection,
an attempt was made to trace the origin of the 112 sheep whose
tissues had been included in the vaccine. It was found that they
had been supplied by three owners and that all were of the
Blackface or Greyface breed with the exception of eight which
were Cheviot lambs born in 1935 from ewes which had been in
contact with scrapie infection. Some of these contact ewes
developed scrapie in 1936-37 and three surviving fellow lambs to
the eight included in the batch 2 vaccine of 1935 developed
scrapie, one in September, 1936, one in February, 1937, and one
in November, 1937. There was, therefore, strong presumptive
evidence that the eight Cheviot lambs included in the vaccine
althought apparently healthy were, in fact, in the incubative stage
of a scrapie infection and that in their tissues there was an
infective agent which had contaminated the batch 2 vaccine,
rendering it liable to set up scrapie. If that assumption was
correct then the evidence indicated that:-
(1) the infective agent of scrapie was present in the brain, spinal
cord and or spleen of infected sheep:
(2) it could withstand a concentration of formalin of 0-35 percent,
which inactivated the virus of louping-ill:
(3) it could be transmitted by subcutaneous inoculation;
(4) it had an incubative period of two years and longer.
Two Frenchmen, Cuille & Chelle (1939) as the result of experiments
commenced in 1932, reported the successful infection of
sheep by inoculation of emulsions of spinal cord or brain material
by the intracerebral, epidural, intraocular and subcutaneous routes
The incubation period varied according to the route employed,
being one year intracerebrally, 15 months intraocularly and 20
months subcutaneously. They failed to infect rabbits but succeeded
in infecting goats. Another important part of their work
showed that the infective agent could pass throught a chamberland
1.3 filter, thus demonstrating that the infective agent was a
filtrable virus. It was a curious coincidence that while they
were doing their transmission experiments their work was being
confirmed by the unforeseeable infectivity of a formalinized tissue
As a result of this experience a large-scale transmision experiment
involving the ue of 788 sheep was commenced in 1938 on a
farm specially taken for the purpose by the Animal Diseases
Research Association with funds provided by the Agricultural
Research Council. The experiment was designed to determine the
nature of the infective agent and the pathogenesis of the disease.
It is only possible here to give a summary of the result which
showed that (1) saline suspensions of brain and spinal cord tissue
of sheep affected with scrapie were infective to normal sheep
when inoculatted intracerebrally or subcutaneously; (2) the incubation
period after intracerebral inoculation was seven months and
upwards and only 60 percent of the inoculated sheep developed
scrapie during a period of four and a half years; (3) the incubation
period after subcutaneous inoculation was 15 months and upwards
and only about 30 percent of the inoculated sheep developed
the disease during the four and a half years: (4) the infective
agent was of small size and probably a filtrable virus.
The prolonged incubative period of the disease and the remarkable
resistance of the causal agent to formalin are features of
distinct interest. It still remains to determine if a biological test
can be devised to detect infected animals so that they can be
killed for food before they develop clinical symptoms and to
explore the possibilities of producing an immunity to the
In the 1984 census of sheep carried out in India - at least 20% of the
flock were contaminated with Scrapie. Indians, those who eat this meat,
eat the brains of the animals too - as a delicacy. Besides, in India,
the rabies vaccine is "grown" on the sheep's brain, from where it is
extracted and given to you as a vaccine.
1: J Infect Dis 1980 Aug;142(2):205-8
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.