New Prion Disease Discovered

Via ProMED-mail: last month ScienceDaily reported the publication of a journal article on a new spontaneous prion disease:

In 2008, Pierluigi Gambetti, MD, and Wen-Quan Zou, MD, PhD, with collaborators, reported the discovery of this novel disease, which affected patients who exhibit only one of the three types of the prion protein gene. In this follow-up study, they discovered that all three genetic groups can be affected also by this novel disease which now joins sCJD in displaying this feature. However, VPSPr is associated with an abnormal prion protein that exhibits characteristics very different from those of sCJD, as well as other prion diseases, suggesting that it may be caused by a different mechanism, perhaps more akin to other neurodegenerative diseases, such as Alzheimer's disease. This finding may exemplify, for the first time, the possibility that the prion protein affects the brain with different mechanisms.

Some claim there's a smoking cow behind this new variant.

Childhood Schizophrenia

Via twitter: the LA Times reports on a tragic case of childhood schizophrenia.

Doctors and other mental health experts don't fully understand the disease, which has no cure. Jani's extreme early onset has left them almost helpless. The rate of onset in children 13 and under is about one in 30,000 to 50,000. In a national study of 110 children, only one was diagnosed as young as age 6.
"Child-onset schizophrenia is 20 to 30 times more severe than adult-onset schizophrenia," says Dr. Nitin Gogtay, a neurologist at the National Institute of Mental Health who helps direct the children's study, the largest such study in the world on the illness.
"Ninety-five percent of the time they are awake these kids are actively hallucinating," Gogtay says. "I don't think I've seen anything more devastating in all of medicine."
For Jani's parents, the most pressing issue is where Jani should live. She has been on the UCLA psych ward -- where she was placed during an emergency -- since Jan. 16. The ward is not designed for long-term care.

Abortive Rabies

Via ProMED-mail: the CDC reports on a rabies case from last year in which the victim was intermittently symptomatic ("severe frontal headache, photophobia, emesis, neck pain, dizziness, and paresthesia of face and forearms") for two and a half weeks before rabies vaccine and immune globulin were administered. She soon appeared to recover without intensive care.

This is the first reported case in which certain clinical and serologic findings indicate abortive human rabies and in which, despite an extensive medical investigation, no alternate etiology for the illness was determined. The patient's positive serologic results offer evidence of rabies virus infection; IFA and Western blot assays indicated the presence of antibodies capable of binding to rabies virus antigens before the patient received rabies PEP. Rabies virus can crossreact serologically with other members of the Lyssavirus genus, distributed in Australia, Eurasia, and Africa (5) or, theoretically, with as yet uncharacterized rhabdoviruses. However, this patient had no history of foreign travel and no evidence of infection with KCV, the only other rhabdovirus associated with bats in North America.
[...]
Certain other clinical and laboratory findings also support a diagnosis of abortive rabies in the patient described in this report. First, the onset of acute encephalopathy approximately 2 months after exposure to bats is compatible with documented incubation periods after rabies virus exposure. Second, central nervous system (CNS) findings (e.g., fever, photophobia, emesis, neck pain, dizziness, paresthesia, limitation of visual field, and altered behavior with agitation and combativeness) are compatible with clinical aspects of rabies. Although this patient did not have classic symptoms such as laryngeal spasms (manifested as hydrophobia) or autonomic instability, the lack of such symptoms has been documented in other rabies patients (1,2,6). Finally, despite an extensive medical workup, no alternate infectious etiology was identified for the patient's neurologic symptoms, increased intracranial pressure, and CSF pleocytosis.

I should note that, after her apparent recovery, the patient returned to the hospital two more times complaining of headaches, and afterwards went missing. ("The current clinical status of the patient or [sic] her boyfriend is unknown.") Judging from the reporting state, the patient was probably in the country illegally. PlagueBlog would not jump to the conclusion that she is still here, or still alive.

Mumps in NYC Update

Via ProMED-mail: the CDC reports that the mumps outbreak in New York/New Jersey has reached 1,521 reported cases, with no apparent end in sight. They also speculate about possible demographic causes of the outbreak in a relatively well-vaccinated community:

Like the mumps outbreaks that occurred in 2006 (2), much of the current outbreak is occurring in congregate settings, where prolonged, close contact among persons might be facilitating transmission. Within the affected religious community, cases have occurred predominantly among school-aged boys, who attend separate schools from girls. The higher rate among boys might be a result of the additional hours that boys in this community spend in school compared with girls, including long periods in large study halls, often face-to-face with a study partner.
In addition, transmission in the community overall might be facilitated by relatively large household sizes. According to the 2000 U.S. Census, the mean household size in one of the affected communities was 5.7, compared with a mean U.S. household size of 2.6. The limited transmission to persons outside the community might be a result of the relatively less interpersonal contact between persons inside and outside the community.
Although the school settings and large household sizes might be promoting transmission, the high vaccination coverage in the affected community likely is limiting the size of the outbreak. In addition, high vaccination coverage in surrounding communities is the most plausible reason that the few cases outside of the affected community have not caused other outbreaks.

PlagueBlog recommends letting the boys out for recess more frequently and sending them home earlier, poor things.

Mumps in NYC

Via ProMED-mail: the mumps outbreak in New York City continues, with over 900 cases identified so far. See the link for MMR clinics serving the apparently under-vaccinated population. The NYC Department of Health notes that:

Each site will have separate entrances for women and men and will have separate staff members to vaccinate them. The staff will be culturally sensitive and respectful.

At the risk of sounding culturally insensitive, PlagueBlog must state that there are worse things than getting an MMR shot from a member of the opposite sex who's just doing their job--for example, orchitis.

Mmm-mmm Melamine, Part MI

Via ProMED-mail: melamine has once again been detected in milk products in China. AsiaNews reports that the scandal has widened to six dairy companies, one of which was still in operation as of Friday.

China has also banned independent reporting of the scandal and jailed a food-safety activist:

Zhao Lianhai is a 37 year old former government employee from Beijing, who was recently arrested and charged with provoking social disorder.
His son was one of 300,000 kids poisoned by contaminated milk across China.
Mr Zhao used to work for the country's food quality and safety authority, so he started a website advising parents of affected children and campaigned to take responsible companies to court.

In other melamine news, on Friday a federal court sentenced the Chinese-American couple for importing the melamine-tainted wheat gluten from the 2007 pet-food scandal.

Mmm-mmm Cadmium, Part I

Via ProMED-mail: the Associated Press found cadmium in children's jewelry imported to the US from China. The LA Times reports:

For China's low-cost jewelry makers, it was an open trade secret: The metal cadmium is shiny, strong and malleable at low temperatures, regardless of its health hazards. And it's cheap.
Despite the risks, manufacturers in factories ringing this city on China's east coast say their top priority is profit. So offering cut-rate goods often means using lower quality materials, including cadmium, which is known to cause cancer.
"Business is business, and it's all up to our client," said He Huihua, manager of the Suiyuan Jewelry Shop at International Trade City in Yiwu[...]
Asked what he thought about the health risks associated with cadmium and other toxic metals, He said: "I can't be overly concerned about that."

PlagueBlog recommends a trade embargo.

Mmm-mmm Melamine, Part M

Via ProMED-mail: another melamine scandal has broken in China, once again belatedly, and once again involving tainted milk. The Wall Street Journal reports that the two-month investigation of Shanghai Panda Dairy Company began almost a year ago.

Shanghai Panda, one of the nation's smaller dairies, was among 22 companies originally implicated in the 2008 scandal and was briefly shut by quality inspectors.
Now, among the allegations against the company are that instead of destroying its melamine-tainted product that had been recalled in 2008, Shanghai Panda reconstituted the milk into new products.

Lovely.

Prion Evolution

Ed Yong discusses prion evolution in his blog at ScienceBlogs:

Jiali Li from the Scripps Institute in Florida has found that prions - the infectious proteins behind mad cow disease, CJD and kuru - are capable of Darwinian evolution, all without a single strand of DNA or its sister molecule RNA.
Prions are rogue version of a protein called PrP. Like all proteins, they are made up of chains of amino acids that fold into a complex three-dimensional structure. Prions are versions of PrP that have folded incorrectly and this misfolded form, called PrPSc, is social, evangelical and murderous. It converts normal prion proteins into a likeness of its abnormal self, and it rapidly gathers together in large clumps that damage and kill surrounding tissues.
Li has found that variation can creep into populations of initially identical prions. Their amino acid sequence stays the same but their already abnormal structures become increasingly twisted. These "mutant" forms have varying degrees of success in different environments. Some do well in brain tissue; others thrive in other types of cell. In each case, natural selection culls the least successful ones. The survivors pass on their structure to the "next generation", by altering the folds of normal prion proteins.

Marburg Reaches the US

Via ProMED-mail: the CDC reports on an imported case of Marburg hemorrhagic fever in Colorado last year. A bat-cave tourist came home from Uganda in January 2008 and exhibited the less dramatic symptoms of Marburg. Tests for Marburg came back negative, but she was retested six months later at her own request and Marburg was confirmed, both then and in the original sample. (It seems the CDC has a big freezer.)

On January 22, 2009, CDC notified the World Health Organization and Uganda Ministry of Health of the imported MHF case. The Python Cave had already been closed to visitors in July 2008, during the response to the Dutch MHF case. CDPHE and CDC conducted a public health investigation during January--February 2009. Interviews were conducted with the patient and her spouse, the patient's medical records were reviewed, and a retrospective contact investigation was conducted to identify possible secondary transmission. A contact was defined as a person who had physical contact with the patient, her body fluids, or contaminated materials or was in the same room as the patient during her acute illness (January 4--19, 2008). Contacts included health-care workers (including health-care providers, housekeeping staff, and hospital laboratory staff), commercial laboratory staff, and social contacts.

No explanation for the six month delay in reporting to the WHO was provided, nor for the further year it took this information to appear on the CDC site. One imagines that it may be due to the fact that the victim, though now recovered, was quite ill with Marburg, including eleven days of hospitalization in "a community hospital," and no notable precautions were taken. The story has medical thriller written all over it. Or perhaps the bird flu/H1N1 story was considered to be enough for the public to panic about at the time.

PlagueBlog recommends against bat-cave tourism of any sort, locally or in Africa.

Tis the Season

It's time again for ProMED-mail's Internet-a-thon. You can make donations at their website.

Non-Plushy Giant Virus

Via twitter: the AFP reports that French scientists have discovered a (non-plushy) giant virus.

With a genome of 368,000 basic pairs, Marseillevirus is the fifth biggest virus ever sequenced and has a diametre of 250 nanometres (around 250 millionth of a millimetre, according the a report by Raoult's for the French National Centre for Scientific Research (CNRS).
The DNA of the giant virus contains material from different sources including plant and animal matter, bacteria and other giant viruses such as the Mimivirus, the report said.
"There is a mechanism of permanent creation going on in amoeba producing a new repertoire of viruses and predisposing giant viruses to become pathogens once they specialise", Raoult said.

Some interesting statements about Darwin follow, which one hopes are merely mistranslated.

Microevolutionary Defenses against Kuru

Via Twitter: Razib at ScienceBlogs reports on cannibals, kuru, and microevolution.

127V is extremely efficacious against fatality due to kuru. Looking through the pedigrees in a region of very high kuru exposure the researchers found that of the individuals who carried 127V, only 1 out of 36 in the parent generation died of kuru. By contrast, 33 of 218 parents from those carrying 127G only (the modal allele) had died of kuru (some of these presumably would also have carried the protective variant of 129). When the researchers looked at the 127V haplotype, the nature of the variation around this mutation implied that a common ancestor existed ~10 generations ago, with a 95% confidence interval 7 to 15 generations. That means that all of the copies of 127V extant today in the Fore descend from one particular copy present on the order of 250 years in the past within the population.

Double Dipping

Via ProMED-mail: the Charleston Daily Mail reports on a pediatrician who came down with swine flu twice.

Parsons says she's spoken with CDC representatives about the results, and they said the double infection isn't all that unbelievable.
"They said this happens every year with seasonal flu, so there's no reason to expect that it wouldn't happen with swine flu," Parsons said. "Every flu strain can change a little bit."
The pediatrician says there may have been a tiny change in the virus that stopped her immune system from recognizing it or her body never built up immunity to it.

Iron and MS

Via Twitter: The Globe and Mail reports on a researcher's personal obsession that led to an unexpected treatment for multiple sclerosis.

Fighting for his wife's health, Dr. Zamboni looked for answers in the medical literature. He found repeated references, dating back a century, to excess iron as a possible cause of MS. The heavy metal can cause inflammation and cell death, hallmarks of the disease. The vascular surgeon was intrigued – coincidentally, he had been researching how iron buildup damages blood vessels in the legs, and wondered if there could be a similar problem in the blood vessels of the brain.
Using ultrasound to examine the vessels leading in and out of the brain, Dr. Zamboni made a startling find: In more than 90 per cent of people with multiple sclerosis, including his spouse, the veins draining blood from the brain were malformed or blocked. In people without MS, they were not.
He hypothesized that iron was damaging the blood vessels and allowing the heavy metal, along with other unwelcome cells, to cross the crucial brain-blood barrier. (The barrier keeps blood and cerebrospinal fluid separate. In MS, immune cells cross the blood-brain barrier, where they destroy myelin, a crucial sheathing on nerves.)
More striking still was that, when Dr. Zamboni performed a simple operation to unclog veins and get blood flowing normally again, many of the symptoms of MS disappeared.

BU's Leaky Biolabs

Via ProMED-mail: the Boston Globe reports on another BU researcher who accidentally brought his work home with him.

The genetic tests, conducted at the state laboratory in Jamaica Plain, compared a blood sample from the researcher with bacterial matter recovered from the lab where he was working on BU’s South End campus. “The bottom line,’’ said Dr. Anita Barry, top disease tracker at the Boston Public Health Commission, “is they matched.’’
The analysis erased any doubt about what caused the researcher to become sick last month and intensified investigations into precisely how he was exposed to a germ known as Neisseria meningitidis, which can cause meningitis.
The city’s biological lab safety division will review safety procedures in BU’s medical labs, to ensure that the school is doing everything possible to minimize researchers’ exposure to pathogens, Barry said.

I thought they did that last time.

PlagueBlog recommends moving to the suburbs before the new BU biolab opens downtown. And by "suburbs" I mean Maine.

Accidental Vaccinia

Via ProMED-mail: the CDC reports on a case of vaccinia infection in an immunocompromised woman who handled rabies vaccine bait, in which a genetically modified version of variola's little sister is used. There's much more on the story and the history of vaccinia at ScienceBlogs.

Atypical Scrapie

Via ProMED-mail: the New Zealand Ministry of Agriculture and Forestry reports on a case of atypical scrapie.

MAFBNZ Principal International Adviser Dr Stuart MacDiarmid says global knowledge about atypical scrapie/Nor 98 is evolving. The widely accepted mainstream scientific view is that it occurs spontaneously or naturally in very small numbers of older sheep in all sheep populations around the world.
"This positive detection of atypical scrapie/Nor 98 in a sheep from New Zealand's national flock reinforces that view. Every country that has conducted sufficient surveillance for atypical scrapie/Nor 98 has found it in their flocks. This includes most Scandinavian and EU countries, the UK, the USA and Canada," he says.
The detection does not change New Zealand's status as free from scrapie.

Mad Elk and Mad Fish

Via ProMED-mail: last month, the New York Times reported on the spread of CWD among elk via fecal-oral transmission, due to preclinical shedding of prions.

Dr. Aiken said prions tended to bind to clay in soil and to persist indefinitely. When deer graze on infected dirt, prions that are tightly bound to clay will persist for long periods in their intestinal regions. So there is no chance chronic wasting disease will be eradicated, he said. Outside the laboratory, nothing can inactivate prions bound to soil. They are also impervious to radiation.

Also, Practical Fish Keeping reported last month on a study of mad cow and scrapie transmissibility to fish:

The authors found that while the bream never displayed clinical signs of spongiform encephelopathies during the study period, the brains of TSE-fed fish sampled two years after challenge showed signs of neurodegeneration and accumulation of deposits that reacted positively with antibodies raised against sea bream PrP. The control groups, fed with brains from uninfected animals, showed no such signs.

Swine Flu Shots vs. Seasonal Flu Shots

Via ProMED-mail: temperatures are still high in Canada, where the Globe and Mail reports that two and a half provinces have rebelled against seasonal flu shots in reaction to as-yet-unpublished research showing that a seasonal flu shot makes people more susceptible to swine flu.

The hodge-podge of vaccination strategies comes after a controversial, unpublished study suggested that people under 50 are twice as likely to contract the H1N1 virus if they have received a seasonal flu shot compared to unvaccinated people.
With no data revealing the optimal way of rolling out vaccines against the looming double threat of seasonal flu and the pandemic swine flu virus, the abrupt changes by provinces and territories could create disarray in inoculation programs, with fewer people turning up for either shot, potentially resulting in more cases of severe illness.
New Brunswick's public health authorities, skeptical about the science behind the Canadian study, have moved up seasonal flu shots for all their residents to October before proceeding to the pandemic vaccine campaign. Meanwhile, Quebec and Nunavut are deferring their seasonal flu campaigns until after their H1N1 mass vaccination clinics.