HUMAN MAD COW DISEASE IS INCURABLE BUT NOW RARE

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OKAY BACK FROM THE NEUROLOGY WARD 🏥
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HOW THE ILLNESS BEGINS 🕰️
Variant CJD Is A Rare And Fatal Prion Disease In Humans
It Results From The Infectious Agent Of Mad Cow Disease Or BSE
A Patient In The United States Became Ill In Late 2012
That Patient First Had Depression And Anxiety
Delusions And Hallucinations Appeared Soon After
Over The Next Months The Patient Became Withdrawn And Secretive
Aggression And Poor Judgment Were Also Reported
Doctors Later Diagnosed Probable Variant CJD
Strong Evidence Pointed To Contaminated Beef Outside The US
That Likely Exposure Came More Than A Decade Before Onset
An Earlier Likely Case Was A 22 Year Old Woman In Florida
In 2001 She Was Evaluated For Depression And Memory Loss
By December She Had Involuntary Movements And Trouble Walking
The Next Month She Was Diagnosed With A Panic Attack
Her Emergency Room Brain Scan Showed No Abnormalities
She Was Prescribed Antianxiety Medications
A BMJ Study Looked At The First 100 UK Patients With Variant CJD
Their Early Stages Were Dominated By Psychiatric Symptoms
Common Early Features Were Withdrawal And Anxiety
Insomnia And Loss Of Interest Were Also Common
63 Of The 100 Patients Saw A Psychiatrist During The Illness
Neurological Symptoms Came First In 15 Percent Of Cases
The Median Age At Onset Was 26 Years
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WHAT THE ILLNESS DOES TO THE BRAIN 🧠
Psychiatric Features May Appear Alone Or With Sensory Changes
Those Include Limb Pain And Abnormal Sensations
Neurological Signs Typically Develop Around Six Months
Worsening Unsteadiness And Visual Problems Are Among Them
So Are Cognitive Decline And Involuntary Movements
At The End Many Patients Can No Longer Move Or Speak
In The Brain The Most Striking Feature Is Florid Plaques
Numerous Plaques Lie Within The Cerebral And Cerebellar Cortex
They Are Often Surrounded By Spongy Tissue Changes
Variant CJD Lasts Much Longer Than The Sporadic Form Of CJD
Its Mean Illness Length Is About 14 Months
Sporadic CJD Cases Lasted 4 Months By Comparison
By The 16th Month The More Recent US Patient Had Left Arm Paralysis
Within A Month That Patient Was Entirely Bedridden
That Patient Died Almost 18 Months After Onset
The Florida Patient Was Given Quinacrine For 3 Months
Little Improvement Followed The Experimental Treatment
She Remained Mute And Unable To Move
Death Came In 2004 About 32 Months After Onset
CJD Is Incurable And Inevitably Fatal
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WHY NEW CASES BECAME RARE 🌱
Early Studies Suggested Variant CJD Came From Eating The BSE Agent
Experiments Mostly In Animals Later Confirmed That Link
Strong Evidence Ties Variant CJD To Eating Offal From Infected Cattle
The Evidence Points Particularly To Brain And Spinal Cord
Among Cattle Recycling Contaminated Material Expanded The Epidemic
Starting In 1988 The UK Brought In Rigorous Control Measures
Ruminant Feed Could No Longer Contain Ruminant Protein
All Cattle Suspected Of Having BSE Had To Be Slaughtered
The UK Banned Feeding Specified Cattle Offal To People Or Farm Animals
Cattle Over 30 Months Old Were Removed From The Food Chain
Annual UK Cattle Cases Rose From 446 In 1987 To 37,280 In 1992
After 2010 Fewer Than 10 Were Reported Each Year
There Were 232 Recognized Human Cases Worldwide As Of 2024
Of Those Cases 178 Were In The UK
The Human Epidemic Peaked In The Early 2000s
No New UK Cases Have Been Reported Since 2016
The Last Known UK Patient Died That Year
Italy And France Reported The Three Most Recent Cases Worldwide
Each Was Likely Due To Exposure At Work
Researchers Say More Cases In The Future Are Not Impossible
A Large Outbreak From Diet Is Now Far Less Likely
Food Chain Controls Essentially Eliminated That Risk
Apart From Milk And Blood Many Nations Ban Ruminant Protein In Feed




