Two Young Men in Michigan Die
of a Suspicious Mad Cow-Like Disease

Comment by John Stauber <>

The information below came via a recent neurology conference.

The odds of two people this young dying at the same time and place of
"sporadic" CJD are simply astronomical. British BSE (mad cow disease) was
first realized to be moving into humans in 1996 when 10 cases of what had
been thought to be "sporadic" CJD were recognized in young people under
thirty. Until then, sporadic CJD was a rare disease that occasionally
claimed people usually in their 60s. So far human mad cow disease, what is
now called variant or new variant CJD, has claimed over 100 Brits and the
death toll is now doubling every three years.

No one knows why these two young men developed CJD. But it's stunning bad
news, and simply underscores that the US has had its head buried in the sand
for a long time.

I discount the statement that these two young men, dying at the same time in
the same hospital in south east Michigan, never ate venison. I've lived
almost my entire 49 years in Wisconsin which like Michigan has a huge deer
herd and hunting culture. The venison sausage comes out, is served with
cheese and crackers, and no one even asks "what type of sausage is this,"
they just say "good sausage."

That said, perhaps these unfortunate young men used glandular nutritional
supplements, or perhaps they ran into an unrecognized TSE in cattle, like
Dick Marsh's mink (1985, Stetsonville, WI). Or perhaps they picked up
sporadic CJD from scrapie. No one knows.

But, to portray these young CJD deaths as some sort of "normal" occurrence
that has simply, to date, gone unobserved, is absurd.

We need a massive federal commitment for TSE research, monitoring and
prevention, and instead all we get is the meat-marketing mantra: "it can't
happen here, it can't happen here."


Two Cases of Early Onset Sporadic Creutzfeld-Jakob Disease in Michigan
Amanda C. Peltier, Patience H. Reading, Karen Kluin, SharinSakurai,
Ahmad Beydoun, Jonathan Edwards, Pierluigi Gambetti,
Norman L. Foster Ann Arbor , MI; Cleveland, OH

OBJECTIVE: To describe the clinical, pathological and genetic
features in two young men who developed sporadic Creutzfeldt-Jakob disease
(CJD) concurrently in Michigan.

BACKGROUND: Sporadic CJD typically occurs in the 6th and 7th
decades of life and is rarely reported in persons younger than 30, except
with exposure to bovine spongiform encephalopathy (BSE) or pituitary extract
derived human growth hormone (HGH). BSE has not been found in the US.


RESULTS: Two young men, ages 26 and 28, who were unknown to
each other and had lived their entire lives in Michigan, developed rapidly
progressive dementia and were simultaneously evaluated in our hospital.
Neither had traveled to countries with known BSE, received HGH, eaten
venison or elk, or had a family history of dementia. The first patient had a
2 month history of progressive aphasia, social withdrawal and memory
difficulties. An EEG performed on admission revealed waxing and waning
rhythmic spike and wave discharges. He was treated for nonconvulsive status
epilepticus, which became convulsive during his hospital course. His
seizures were refractory to medical therapy despite multiple anticonvulsants,
including midazolam coma. His EEG became more periodic and
he never recovered responsiveness even as medication was tapered. A brain
biopsy and subsequent postmortem examination following his death 5 months
after the onset of symptoms showed spongiform changes. The scrapie prion
protein (PrPsc) wasdistributed in a cluster pattern as revealed by
immunohistochemistry. Genetic analysis and immunoblot established that this
patient had the MM2 subtype of sporadic CJD. Treatment with quinacrine had
no benefit. The second patient had a 10 month history of progressive memory
loss, inappropriate behavior, violent outbursts, and difficulty performing
his job. He had bradykinesia and rigidity on examination. There were no
periodic discharges on EEG and CSF protein 14-3-3 was negative. Following a
brain biopsy showing spongiform changes he developed startle myoclonus. The
presence of PrPsc type 1 was confirmed with immunoblot and immunostaining.
Both patients had abnormal MRI scans with increased signal in the basal
ganglia and cerebral cortex on T2- and diffusion-weighted images.

CONCLUSIONS: These cases expand the spectrum of sporadic CJD to
include younger age of onset than previously suspected and cases
presenting as non-convulsive status epilepticus. The appearance of CJD in 2
individuals within a few months of each other in southeastern Michigan may
indicate that very early-onset CJD is more common than previously believed.
Alternatively, other unrecognized risk factors may exist. It is important to
consider CJD in young people with progressive behavioral and cognitive
disturbances, even in the absence of typical EEG or CSF abnormalities.
Supported By: This study was supported by NIH grant AG14359 and
grant CCU 515004 to the National Prion Disease Pathology Surveillance
Center and the Michigan Alzheimers Disease Research Center (NIH grant

Category - Infection/AIDS/Prion Disease SubCategory - Epidemiology

Kathy Stone
Media Relations Manager
American Academy of Neurology
1080 Montreal Avenue
St. Paul, Minnesota 55116 USA
fax: 651-695-2791

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