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KURU CAN APPEAR DECADES AFTER INFECTION

A Fore woman with trembling hands outside her hut as a ghostly clock spins in the sky between her and a faded funeral feast decades earlier

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INCUBATION AND THE LAST CASES ⏳

Kuru Was A Fatal Prion Disease Of The Fore In Papua New Guinea

It Was Passed On At Funeral Feasts That Ended In The 1950s

Its Incidence Fell Steadily After That

Yet Kuru Kept Appearing For Decades After The Feasts Stopped

In 1996 Researchers Stepped Up Kuru Surveillance With A Larger Team

They Found 11 Kuru Patients From July 1996 To June 2004

All 11 Lived In The South Fore

Every One Was Born Before The Practice Ceased In The Late 1950s

One Of The Team's Aims Was To Investigate Possible Incubation Periods

Researchers Took Detailed Histories Of Exposure To Mortuary Feasts

Patients Also Had Repeated Neurological Examinations Where Possible

Minimum Estimated Incubation Periods Ranged From 34 To 41 Years

For Male Patients The Count Ran From Age 7 To The Onset Of Symptoms

Their Likely Periods Ranged From 39 To 56 Years

Those Could Have Been Up To 7 Years Longer

They Concluded Human Prion Infection Can Take Over 50 Years To Show

A Second Paper On The Same Patients Estimated Some Exceeded 50 Years

The Last Three Cases Were Recorded In 2003 And 2005 And 2009

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THE GENES OF KURU SURVIVORS 🧬

Some People Exposed To Kuru Survived The Epidemic

One Position In The Prion Protein Gene Is Called Codon 129

Having Two Different Versions There Gives Relative Resistance

That Applies To Both Sporadic And Acquired Prion Diseases

In Kuru It Is Linked To Older Patients And Longer Incubation

Most Patients Found From 1996 To 2004 Had Two Different Versions

Elderly Survivors Had Attended More Than One Mortuary Feast

Most Of Those Survivors Had Two Different Versions Too

A Larger Study Assessed More Than 3000 People

It Made Genetic And Family Assessments Across Eastern Highland Groups

709 Of Those People Had Taken Part In Mortuary Feasts

152 Of The Feast Participants Later Died Of Kuru

The Study Reported A New Variant In The Gene Called G127V

It Was Found Exclusively In People From Where Kuru Was Prevalent

Some Women Carried Two Copies Of The Methionine Version At Codon 129

That Pairing Left Them Otherwise Susceptible To Kuru

Half Of Those Women From The Most Exposed Region Carried G127V

The Variant Was Not Found In Patients With Kuru

Families Carrying It Had Significantly Less Kuru Than Matched Families

The Authors Call It A Resistance Factor Selected During The Epidemic

They Say It Was Not A Mutation That Could Have Triggered Kuru

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WHAT KURU TAUGHT ABOUT MAD COW 🐮

In The UK Many People Were Exposed To Mad Cow Prions Through Diet

How Many Of Them Were Infected Remained Unknown

Variant CJD Is The Human Form Of Mad Cow Disease

Its Appearance Brought Renewed Interest In Kuru

One Worry Was That Incubation In Humans Might Be Extremely Long

Researchers Say Much Can Be Learned From Kuru About Variant CJD

They Called Kuru Their Only Experience Of A Major Human Prion Epidemic

The Two Epidemics Spread In Different Ways

Kuru Probably Began With One Person Who Had Sporadic CJD

This Kind Of CJD Appears To Arise Rarely And Spontaneously Everywhere

That Person's Kinship Group Would Have Consumed The Body

Others Who Caught The Disease Then Died And Were Consumed In Turn

That Would Have Recycled Kuru Prions Within One Community

Mad Cow Prions Instead Had To Cross From Cattle Into People

That Species Barrier Is Expected To Lengthen Incubation Further

The Authors Said Kuru Data Should Inform Models Of Variant CJD

Kuru Itself Stayed Within The Fore And Nine Nearby Language Groups

Women And Children Were The Main Participants In The Feasts

Australian Authorities Banned The Feasts In The 1950s

Fieldwork On Kuru Began In 1957 And Ran For 55 Years

Surveillance Stopped In 2012 As The Epidemic Had Essentially Ended

The End

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