Ronan Kelly
Retired 2020
Published Date: 2016-04-22 05:49:23
Subject: PRO/AH/EDR> Creutzfeldt-Jakob disease - India: (AP) susp, RFI
Archive Number: 20160422.4175340
CREUTZFELDT-JAKOB DISEASE - INDIA: (ANDHRA PRADESH) SUSPECTED, REQUEST FOR INFORMATION
************************************************** ************************************
A ProMED-mail post
http://www.promedmail.org
ProMED-mail is a program of the
International Society for Infectious Diseases
http://www.isid.org
Date: Wed 20 Apr 2016
Source: Times of India, Times News Network (TNN) [edited]
http://epaperbeta.timesofindia.com/A...20042016007025
In a case that spread panic in the capital region [Hyderabad, Telangana (formerly Andhra Pradesh)], a 30 year old woman from Ipurupalem village, near Chirala [Prakasam district, Andhra Pradesh state] was diagnosed with the dreaded mad cow disease [bovine spongiform encephalopathy or BSE] at government general hospital (GGH) [Guntur] on [Tue 19 Apr 2016]. The condition of the woman is said to be very critical, and the doctors said chances of her recovery are remote. The health department authorities said this could be the 1st case of mad cow disease in the state [Andhra Pradesh].
The daily wage labourer [woman] of Ipurupalem, near Chirala, is learnt to have started losing her mental balance 6 months ago [October 2015]. Worried, her family took her to noted private and government hospitals in Prakasam district. As [her] condition deteriorated, she was admitted to GGH about a fortnight ago.
GGH neurology wing chief Dr Sundarachari said the woman was 1st kept under observation, and several tests were conducted on her. Initially, the doctors suspected [she] could be suffering from a psychological disorder because of family troubles. Psychiatrists and other experts were roped in to treat her. With no response to treatment, doctors conducted more tests on the woman, which led them to suspect that she had contracted mad cow disease.
"There are no authentic labs in India which can confirm that it is a case of mad cow disease. Provisional diagnoses indicated that it could be the dreaded disease as tests have ruled out all other ailments. We strongly believe the victim has been infected by mad cow disease," Dr Sundarachari told TOI [Times of India]. He added that brain biopsies are needed for making sure it is a mad cow disease, but that has been deferred because of non-availability of equipment. He clarified that the disease may not only be contracted by eating meat or beef but even genetic reasons could propagate the disease.
Experts said people who get infected with the fatal disease might not survive for more than a year. The victim has already lost her memory, as she could not even recognise her parents who were at bedside. The doctors have kept [her] in the isolation ward, as she tried to attack other patients and staffers at GGH. "The infection, although mostly concentrated in the nerve tissue, can be found in virtually all tissues including blood. Although recovery in chronic cases is rare, we are trying our level best to bring her back to normal," said Dr Chary. After learning that the chances of her recovery are remote, family members requested the doctors to discharge her.
Mad cow disease, scientifically called bovine spongiform encephalopathy (BSE), is rarely found in India. The disease, which kills several thousand people worldwide, is contracted when people eat food contaminated by the brain, spinal cord or digestive tract of infected cattle. In humans, mad cow disease is called Creutzfeldt-Jakob disease [CJD].
--
communicated by:
ProMED-mail
<promed@promedmail.org>
[Prakasam district is an administrative district in the Coastal Andhra region of the Indian state of Andhra Pradesh. The headquarters of the district are located at Ongole. It is on the west coast of the Bay of Bengal and is bounded by Guntur district on the north, Kurnool district on the west, Kadapa and Nellore districts on the south (https://en.wikipedia.org/wiki/Prakasam_district).
Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is a fatal neurodegenerative disease (encephalopathy) in cattle that causes a spongy degeneration of the brain and spinal cord (https://en.wikipedia.org/wiki/Bovine...encephalopathy).
Genetic CJD (previously called familial CJD and sometimes referred to as inherited CJD) is an inherited form of Creutzfeldt-Jakob disease, which belongs to a group of rare, and always fatal, brain disorders called the prion diseases. These occur in both humans and animals, and include BSE and scrapie in animals.
CJD is caused by the accumulation in the brain of an abnormal form of a protein called a "prion protein". PrP can exist in 2 forms -- normal (PrPc) and abnormal (PrPsc). Most humans have the normal PrPc in our brain. The abnormal prion is different because it is folded in a different way and can cause normal prion protein to change shape and become abnormal. This leads to damage of brain cells. Genetic CJD is caused by a genetic mistake where a mutation in the PrP gene seems to make the conversion into the abnormal form more likely. Several different mutations have now been identified.
"What is Creutzfeldt-Jakob Disease?
"Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide; in the United States there are about 300 cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 per cent of individuals die within 1 year. In the early stages of disease, people may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.
"There are 3 major categories of CJD:
- In sporadic CJD, the disease appears even though the person has no known risk factors for the disease. This is by far the commonest type of CJD and accounts for at least 85 per cent of cases.
- In hereditary CJD, the person has a family history of the disease and/or tests positive for a genetic mutation associated with CJD. About 5 to 10 per cent of cases of CJD in the United States are hereditary.
- In acquired CJD, the disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures. There is no evidence that CJD is contagious through casual contact with a CJD patient. Since CJD was 1st described in 1920, fewer than 1 per cent of cases have been acquired CJD.
"CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges under a microscope. CJD is the commonest of the known human TSEs. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS). Kuru was identified in people of an isolated tribe in Papua New Guinea and has now almost disappeared. FFI and GSS are extremely rare hereditary diseases, found in just a few families around the world. Other TSEs are found in specific kinds of animals. These include bovine spongiform encephalopathy (BSE), which is found in cows and is often referred to as "mad cow" disease; scrapie, which affects sheep and goats; mink encephalopathy; and feline encephalopathy. Similar diseases have occurred in elk, deer, and exotic zoo animals.
"What are the symptoms of the disease?
"CJD is characterized by rapidly progressive dementia. Initially, individuals experience problems with muscular coordination; personality changes, including impaired memory, judgment, and thinking; and impaired vision. People with the disease also may experience insomnia, depression, or unusual sensations. CJD does not cause a fever or other flu-like symptoms. As the illness progresses, mental impairment becomes severe. Individuals often develop involuntary muscle jerks called myoclonus, and they may go blind. They eventually lose the ability to move and speak and enter a coma. Pneumonia and other infections often occur in these individuals and can lead to death.
"There are several known variants of CJD. These variants differ somewhat in the symptoms and course of the disease. For example, a variant form of the disease-called new variant or variant (nv-CJD, v-CJD), described in Great Britain and France-begins primarily with psychiatric symptoms, affects younger individuals than other types of CJD, and has a longer than usual duration from onset of symptoms to death. Another variant, called the panencephalopathic form, occurs primarily in Japan and has a relatively long course, with symptoms often progressing for several years. Scientists are trying to learn what causes these variations in the symptoms and course of the disease.
"Some symptoms of CJD can be similar to symptoms of other progressive neurological disorders, such as Alzheimer's or Huntington's disease. However, CJD causes unique changes in brain tissue which can be seen at autopsy. It also tends to cause more rapid deterioration of a person's abilities than Alzheimer's disease or most other types of dementia.
"How is CJD diagnosed?
"There is currently no single diagnostic test for CJD. When a doctor suspects CJD, the 1st concern is to rule out treatable forms of dementia such as encephalitis (inflammation of the brain) or chronic meningitis. A neurological examination will be performed and the doctor may seek consultation with other physicians. Standard diagnostic tests will include a spinal tap to rule out more common causes of dementia and an electroencephalogram (EEG) to record the brain's electrical pattern, which can be particularly valuable because it shows a specific type of abnormality in CJD. Computerized tomography of the brain can help rule out the possibility that the symptoms result from other problems such as stroke or a brain tumor. Magnetic resonance imaging (MRI) brain scans also can reveal characteristic patterns of brain degeneration that can help diagnose CJD.
"The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the patient's brain so that it can be examined by a neuropathologist. This procedure may be dangerous for the individual, and the operation does not always obtain tissue from the affected part of the brain. Because a correct diagnosis of CJD does not help the person, a brain biopsy is discouraged unless it is needed to rule out a treatable disorder. In an autopsy, the whole brain is examined after death. Both brain biopsy and autopsy pose a small, but definite, risk that the surgeon or others who handle the brain tissue may become accidentally infected by self-inoculation." [http://www.ninds.nih.gov/disorders/cjd/detail_cjd.htm]
"Genetic CJD accounts for around 15 per cent of all cases of CJD. Like the other forms of CJD, genetic CJD is characterised by dementia (mental decline with symptoms such as memory loss) and neurological problems such as unsteadiness. The brain of someone with genetic CJD will also show the spongiform change which is the hallmark of all forms of CJD -- the brain tissue has a spongy appearance when viewed under a microscope.
"There are also 2 other, even rarer, inherited brain diseases which resemble genetic CJD. These are Gerstmann Straussler Scheinker disease (GSS) and fatal familial insomnia (FFI). Like genetic CJD, they are associated with mutations of the PrP gene. The distinction between these different forms of disease (GSS, FFI and genetic CJD) is partly historical and currently many experts tend to class these diseases together under 'genetic prion diseases'" [http://www.cjdsupport.net/UserFiles/...%20cjd(1).pdf].
There is no treatment that can cure or control CJD. Researchers have tested many drugs, including amantadine, steroids, interferon, acyclovir, antiviral agents, and antibiotics. Studies of a variety of other drugs are now in progress. However, so far none of these treatments has shown any consistent benefit in humans.
Current treatment for CJD is aimed at alleviating symptoms and making the individual as comfortable as possible. Opiate drugs can help relieve pain if it occurs, and the drugs clonazepam and sodium valproate may help relieve myoclonus.
Any further information or confirmation of the CJD diagnosis will be highly appreciated. - Mod.UBA
...
http://promedmail.org/direct.php?id=20160422.4175340
GUNTUR, April 22, 2016
Concern over high incidence of mad cow disease
STAFF REPORTER
PRINT ? T T
Three patients suffering from Creutzfeldt–Jakob Disease (CJD), a rare and fatal degenerative brain disorder, have been admitted to the Department of Neurology in the Government General Hospital here.
The high incidence of the CJD, termed as Mad Cow Disease, has caused a great deal of concern. At least six cases have been reported at the GGH in the last one year.
...
“We initially believed it to be a psychiatric condition. An MRI scan, however, revealed classical CJD Cortical Ribbon Sign (CRS). Later, an EEG test confirmed our findings.
...
http://www.thehindu.com/todays-paper...cle8506589.ece
Subject: PRO/AH/EDR> Creutzfeldt-Jakob disease - India: (AP) susp, RFI
Archive Number: 20160422.4175340
CREUTZFELDT-JAKOB DISEASE - INDIA: (ANDHRA PRADESH) SUSPECTED, REQUEST FOR INFORMATION
************************************************** ************************************
A ProMED-mail post
http://www.promedmail.org
ProMED-mail is a program of the
International Society for Infectious Diseases
http://www.isid.org
Date: Wed 20 Apr 2016
Source: Times of India, Times News Network (TNN) [edited]
http://epaperbeta.timesofindia.com/A...20042016007025
In a case that spread panic in the capital region [Hyderabad, Telangana (formerly Andhra Pradesh)], a 30 year old woman from Ipurupalem village, near Chirala [Prakasam district, Andhra Pradesh state] was diagnosed with the dreaded mad cow disease [bovine spongiform encephalopathy or BSE] at government general hospital (GGH) [Guntur] on [Tue 19 Apr 2016]. The condition of the woman is said to be very critical, and the doctors said chances of her recovery are remote. The health department authorities said this could be the 1st case of mad cow disease in the state [Andhra Pradesh].
The daily wage labourer [woman] of Ipurupalem, near Chirala, is learnt to have started losing her mental balance 6 months ago [October 2015]. Worried, her family took her to noted private and government hospitals in Prakasam district. As [her] condition deteriorated, she was admitted to GGH about a fortnight ago.
GGH neurology wing chief Dr Sundarachari said the woman was 1st kept under observation, and several tests were conducted on her. Initially, the doctors suspected [she] could be suffering from a psychological disorder because of family troubles. Psychiatrists and other experts were roped in to treat her. With no response to treatment, doctors conducted more tests on the woman, which led them to suspect that she had contracted mad cow disease.
"There are no authentic labs in India which can confirm that it is a case of mad cow disease. Provisional diagnoses indicated that it could be the dreaded disease as tests have ruled out all other ailments. We strongly believe the victim has been infected by mad cow disease," Dr Sundarachari told TOI [Times of India]. He added that brain biopsies are needed for making sure it is a mad cow disease, but that has been deferred because of non-availability of equipment. He clarified that the disease may not only be contracted by eating meat or beef but even genetic reasons could propagate the disease.
Experts said people who get infected with the fatal disease might not survive for more than a year. The victim has already lost her memory, as she could not even recognise her parents who were at bedside. The doctors have kept [her] in the isolation ward, as she tried to attack other patients and staffers at GGH. "The infection, although mostly concentrated in the nerve tissue, can be found in virtually all tissues including blood. Although recovery in chronic cases is rare, we are trying our level best to bring her back to normal," said Dr Chary. After learning that the chances of her recovery are remote, family members requested the doctors to discharge her.
Mad cow disease, scientifically called bovine spongiform encephalopathy (BSE), is rarely found in India. The disease, which kills several thousand people worldwide, is contracted when people eat food contaminated by the brain, spinal cord or digestive tract of infected cattle. In humans, mad cow disease is called Creutzfeldt-Jakob disease [CJD].
--
communicated by:
ProMED-mail
<promed@promedmail.org>
[Prakasam district is an administrative district in the Coastal Andhra region of the Indian state of Andhra Pradesh. The headquarters of the district are located at Ongole. It is on the west coast of the Bay of Bengal and is bounded by Guntur district on the north, Kurnool district on the west, Kadapa and Nellore districts on the south (https://en.wikipedia.org/wiki/Prakasam_district).
Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is a fatal neurodegenerative disease (encephalopathy) in cattle that causes a spongy degeneration of the brain and spinal cord (https://en.wikipedia.org/wiki/Bovine...encephalopathy).
Genetic CJD (previously called familial CJD and sometimes referred to as inherited CJD) is an inherited form of Creutzfeldt-Jakob disease, which belongs to a group of rare, and always fatal, brain disorders called the prion diseases. These occur in both humans and animals, and include BSE and scrapie in animals.
CJD is caused by the accumulation in the brain of an abnormal form of a protein called a "prion protein". PrP can exist in 2 forms -- normal (PrPc) and abnormal (PrPsc). Most humans have the normal PrPc in our brain. The abnormal prion is different because it is folded in a different way and can cause normal prion protein to change shape and become abnormal. This leads to damage of brain cells. Genetic CJD is caused by a genetic mistake where a mutation in the PrP gene seems to make the conversion into the abnormal form more likely. Several different mutations have now been identified.
"What is Creutzfeldt-Jakob Disease?
"Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide; in the United States there are about 300 cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 per cent of individuals die within 1 year. In the early stages of disease, people may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.
"There are 3 major categories of CJD:
- In sporadic CJD, the disease appears even though the person has no known risk factors for the disease. This is by far the commonest type of CJD and accounts for at least 85 per cent of cases.
- In hereditary CJD, the person has a family history of the disease and/or tests positive for a genetic mutation associated with CJD. About 5 to 10 per cent of cases of CJD in the United States are hereditary.
- In acquired CJD, the disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures. There is no evidence that CJD is contagious through casual contact with a CJD patient. Since CJD was 1st described in 1920, fewer than 1 per cent of cases have been acquired CJD.
"CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges under a microscope. CJD is the commonest of the known human TSEs. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS). Kuru was identified in people of an isolated tribe in Papua New Guinea and has now almost disappeared. FFI and GSS are extremely rare hereditary diseases, found in just a few families around the world. Other TSEs are found in specific kinds of animals. These include bovine spongiform encephalopathy (BSE), which is found in cows and is often referred to as "mad cow" disease; scrapie, which affects sheep and goats; mink encephalopathy; and feline encephalopathy. Similar diseases have occurred in elk, deer, and exotic zoo animals.
"What are the symptoms of the disease?
"CJD is characterized by rapidly progressive dementia. Initially, individuals experience problems with muscular coordination; personality changes, including impaired memory, judgment, and thinking; and impaired vision. People with the disease also may experience insomnia, depression, or unusual sensations. CJD does not cause a fever or other flu-like symptoms. As the illness progresses, mental impairment becomes severe. Individuals often develop involuntary muscle jerks called myoclonus, and they may go blind. They eventually lose the ability to move and speak and enter a coma. Pneumonia and other infections often occur in these individuals and can lead to death.
"There are several known variants of CJD. These variants differ somewhat in the symptoms and course of the disease. For example, a variant form of the disease-called new variant or variant (nv-CJD, v-CJD), described in Great Britain and France-begins primarily with psychiatric symptoms, affects younger individuals than other types of CJD, and has a longer than usual duration from onset of symptoms to death. Another variant, called the panencephalopathic form, occurs primarily in Japan and has a relatively long course, with symptoms often progressing for several years. Scientists are trying to learn what causes these variations in the symptoms and course of the disease.
"Some symptoms of CJD can be similar to symptoms of other progressive neurological disorders, such as Alzheimer's or Huntington's disease. However, CJD causes unique changes in brain tissue which can be seen at autopsy. It also tends to cause more rapid deterioration of a person's abilities than Alzheimer's disease or most other types of dementia.
"How is CJD diagnosed?
"There is currently no single diagnostic test for CJD. When a doctor suspects CJD, the 1st concern is to rule out treatable forms of dementia such as encephalitis (inflammation of the brain) or chronic meningitis. A neurological examination will be performed and the doctor may seek consultation with other physicians. Standard diagnostic tests will include a spinal tap to rule out more common causes of dementia and an electroencephalogram (EEG) to record the brain's electrical pattern, which can be particularly valuable because it shows a specific type of abnormality in CJD. Computerized tomography of the brain can help rule out the possibility that the symptoms result from other problems such as stroke or a brain tumor. Magnetic resonance imaging (MRI) brain scans also can reveal characteristic patterns of brain degeneration that can help diagnose CJD.
"The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the patient's brain so that it can be examined by a neuropathologist. This procedure may be dangerous for the individual, and the operation does not always obtain tissue from the affected part of the brain. Because a correct diagnosis of CJD does not help the person, a brain biopsy is discouraged unless it is needed to rule out a treatable disorder. In an autopsy, the whole brain is examined after death. Both brain biopsy and autopsy pose a small, but definite, risk that the surgeon or others who handle the brain tissue may become accidentally infected by self-inoculation." [http://www.ninds.nih.gov/disorders/cjd/detail_cjd.htm]
"Genetic CJD accounts for around 15 per cent of all cases of CJD. Like the other forms of CJD, genetic CJD is characterised by dementia (mental decline with symptoms such as memory loss) and neurological problems such as unsteadiness. The brain of someone with genetic CJD will also show the spongiform change which is the hallmark of all forms of CJD -- the brain tissue has a spongy appearance when viewed under a microscope.
"There are also 2 other, even rarer, inherited brain diseases which resemble genetic CJD. These are Gerstmann Straussler Scheinker disease (GSS) and fatal familial insomnia (FFI). Like genetic CJD, they are associated with mutations of the PrP gene. The distinction between these different forms of disease (GSS, FFI and genetic CJD) is partly historical and currently many experts tend to class these diseases together under 'genetic prion diseases'" [http://www.cjdsupport.net/UserFiles/...%20cjd(1).pdf].
There is no treatment that can cure or control CJD. Researchers have tested many drugs, including amantadine, steroids, interferon, acyclovir, antiviral agents, and antibiotics. Studies of a variety of other drugs are now in progress. However, so far none of these treatments has shown any consistent benefit in humans.
Current treatment for CJD is aimed at alleviating symptoms and making the individual as comfortable as possible. Opiate drugs can help relieve pain if it occurs, and the drugs clonazepam and sodium valproate may help relieve myoclonus.
Any further information or confirmation of the CJD diagnosis will be highly appreciated. - Mod.UBA
...
http://promedmail.org/direct.php?id=20160422.4175340
GUNTUR, April 22, 2016
Concern over high incidence of mad cow disease
STAFF REPORTER
PRINT ? T T
Three patients suffering from Creutzfeldt–Jakob Disease (CJD), a rare and fatal degenerative brain disorder, have been admitted to the Department of Neurology in the Government General Hospital here.
The high incidence of the CJD, termed as Mad Cow Disease, has caused a great deal of concern. At least six cases have been reported at the GGH in the last one year.
...
“We initially believed it to be a psychiatric condition. An MRI scan, however, revealed classical CJD Cortical Ribbon Sign (CRS). Later, an EEG test confirmed our findings.
...
http://www.thehindu.com/todays-paper...cle8506589.ece
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