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From: TSS ()
Subject: 48th Annual Meeting of the Japanese Society of Neuropathology 30 May - 1 June 2007 plus PRION 2004 - 2007
Date: August 1, 2007 at 12:54 pm PST

The 48th Annual Meeting of the Japanese Society of Neuropathology 30 May–1 June 2007

The 48th Annual Meeting of the Japanese Society of
Date: 30 May–1 June 2007
Tower Hall Funabori, Tokyo
President: Kazuhiko Ikeda
Vice President: Kuniaki Tsuchiya

Running a human brain bank is a complex task which
requires infrastructure (space, security, trained staff, running
costs), often nationwide transport activity, and excellent communication
and motivation on the side of tissue bank staff.
Personal integrity is of great importance in view of increasing
pressure exerted by the financial needs of academic institutions
and their links to industry. The ethical standards of
the HapMap Project ( may represent an
example to follow. There are opportunities for neuropathologists
involved in brain banking, e.g. international standardization
of procedures, effective communication of diagnostic
results to scientists, decisions on implications for tissue uses,
and novel options such as building a histophenotypic database.
Some of these opportunities represent challenges that
call for a deeper involvement of our discipline beyond traditional
morphological needs. Finally, it is clear that a human
brain bank should have a medical neuropathologist at its



An autopsy case of Creutzfeldt-Jakob
disease after continuous intraventricular
administration of pentosan polysulfate
N Ishizu1, Y Sakiyama2,3, Y Saitoh2, L Matsumoto3, Y Tsuboi4,
T Yamada4, K Doh-ura5, A Hebisawa6, H Kurisaki1,
S Murayama2. 1Dept. of Neurology, NHOTokyo Hospital, 2Dept.
of Neuropathology, Tokyo Metroporitan insutitute of
Gerontology, 3Dept. of Neurology, Grad. School of Medicine,
Univ. of Tokyo, 4Fifth Dept. of Internal Medicine, Fukuoka Univ.
Faculty of Medicine, 5Div. of Prion Protein Biology, Center for
Translational and Advanced Animal Reserch on Human
Diseases, Tohoku Univ. Grad. School of Medicine, 6Clinical
Lab. Dept., NHO Tokyo Hosp.
A 36-year-old man underwent resection of a left acoustic neurinoma
and received LYODURA. At age 54, he began suffering
dizziness, followed by ataxia, amnesia and myoclonus within a
year. MRI (DWI) showed hyperintensity in the right caudate
nucleus and thalamus. After 15 months, he was bedridden
despite the start of intraventricular administration of pentosan
polysulfate (PPS). Brain biopsy confirmed deposit of PrPsc. After
19 months he died of pneumonia. Autopsy revealed spongiformic
changes accentuated in the entorhinal area, precentral
gyrus, anterior cingulate gyrus, anterior nuclei of the thalamus,
and putamen. 3F4 immunostaining indicated plaque-type deposits
among diffusely and lightly stained gray matter. Dark
staining around neuronal cell bodies and dendrites was noted in
the precentral gyrus, hippocampus, and globus pallidus. A rightleft
difference in PrPsc deposition was not apparent. Neuropahological
diagnosis of this case was CJD after cadaveric dural
transplant with plaque-type PrPsc deposits Indications for PPS
were considered for the case’s relatively long clinical course, but
the results were not apparent with both clinical and pathological
points of view.



An autopsy case of Creutzfeldt-Jakob
disease after continuous intraventricular
administration of pentosan polysulfate
T Terada1, T Obi1, A Sugiura1, K Yamazaki1, K Mizoguchi1,
S Murayama2, Y Saito2, Y Tsuboi3, T Yamada3. 1Shizuoka
Institute of Epilepsy and Neurological Disorders, 2Tokyo
Metropolitan Institute of Gerontology, 3Fukoka University
A 66 year-old woman experienced gait disturbance and
memory impairment. One month later, she received diagnosis
of Creutzfeldt-Jakob disease (CJD). Five months later,
she was in akinetic mutism. Ten months later, she received
continuous intraventricular infusion of pentosan polysulfate
(PP), but her clinical condition did not improve and repeated
CT examinations demonstrated progressive brain atrophy
and enlarging subdural hygloma. Twenty-seven months after
the onset, she died of pneumonia. The brain weighed
660 gm and presented with walnut-shaped severe atrophy.
Massive intraventricular hemorrhage was present. A PP infusion
tube was correctly inserted into the right lateral ventricle,
and the focus responsible for hemorrhage could not be
identified. Marked cortical atrophy was extensive and symmetrical
and relatively spared hippocampi. Histologically,
severe neuronal loss, spongiosis and gliosis with synaptic
deposition of Prscr were symmetrical in cerebral cortex,
basal ganglia and thalami. This case presented classic
pathology of CJD and the effect of PP was not evident. Fresh
intraventricular hematoma was interpreted to be agonal.



Time-course analysis of synapse-related
proteins in the transmissible spongiform
K Sasaki, H Minaki, T Iwaki. Dept. of Neuropathol., Kyushu
It has been reported that the expression of synapse-related
proteins, such as synaptophysin, are decreased in the brains
with transmissible spongiform encephalopathies (TSEs). To
examine the time course of the expression of synapserelated
proteins in association with TSEs, we performed
immunohistochemistry and western blot analysis on
NZW/Fukuoka-1 TSE mouse model. Immunoreactivity for
SNAP-25 and syntaxin was decreased greatly where the
abnormal prion protein deposits were detected. Immunoreactivity
for synaptophysin and synaptobrevin was decreased
without such association with prion protein deposition.
Western blot analysis revealed that SNAP-25 and synaptobrevin
were decreased from 3.0 months, and that synaptophysin
diminished from 3.5 months. The alteration preceded
the accumulation of protease-resistant prion protein at 3.5
months. These results suggested that impaired functions of
synaptic proteins, particularly for exocytosis of synaptic vesicles,
may play an important role on pathophysiology of TSEs
in the early stage.



An autopsy case of MV2 Creutzfeldt-
Jakob disease (CJD) presenting
cerebellar ataxia as the initial symptom
Y Saito1, M Yoshida2, I Aiba1, S Ito1, A Goto1, Y Yokokawa1,
M Kenjo1, T Katayama1, E Hayakawa1, A Inukai1,
Y Matsuoka1, R Ichihashi2, Y Hashizume2, Y Iwasaki3,
T Kitamoto4. 1Dept. of Neurol., Higashi Nagoya Natl. Hosp.,
2Institute for Medical Sci. of Aging, Aichi Medical Univ.,
3Dept. of Neurol., Nagoya Univ. Grad. Sch. of Medicine,
4Dept. of Prion Research, Tohoku Univ. Grad. Sch. of
We report an autopsied sporadic CJD case of MV2 with 21
months’ duration. In the initial stages, the patient was diagnosed
with spinocerebellar degeneration, presenting cerebellar
ataxia. Diffusion-weighted MRI showed high-intensity
lesions in the cerebral cortex and the striatum. Pathological
findings revealed mild atrophy of cerebrum and cerebellum
with 1260 g brain weight. Microscopically cortical neuronal
loss was mild, and in addition to spongiform change throughout
the cerebral cortex, large vacuoles were seen particularly
in the temporal and insular cortices. Cerebellar cortex
showed Purkinje cell loss, in contrast to the mild degeneration
of granular cell layers. Kuru-plaques were observed in
the molecular and granular cell layers. On immunohistochemical
studies (3F4), PrP deposits revealed synaptic
pattern in all cortical layers and coarse perivacuolar pattern
was seen in the areas of the cortices consistent with large
vacuoles. In the cerebellum plaque-type PrP deposits were
seen, in addition to the synaptic type PrP deposits. This
case demonstrated various pathological changes and PrP
deposits types, which indicated the characteristic of MV2
type pathology.



Neuropathologic characteristics of
cerebral neocortical lesions in sporadic
Creutzfeldt-Jakob disease
Y Iwasaki1, M Mimuro2, M Yoshida2, Y Hashizume2,
G Sobue1. 1Dept. of Neurol., Nagoya Univ. Grad. Sch. of
Med., 2Dep. of Neuropathol., Inst. for Med. Sci. of Aging,
Aichi Med. Univ.
We analyzed neuropathologic characteristics of cerebral
neocortical lesions in sporadic Creutzfeldt-Jakob disease
(sCJD). According to PrP gene polymorphism and PrP type,
19 cases were classified as MM1-type, two as MV1-type,
three as MM2-type, one as MV2-type and one as MM1+2-
type sCJD. MM1- and MV1-type cases showed various
degrees of spongiform degeneration, gliosis and neuronal
loss in the cerebral neocortex. Spongiform degeneration
showed fine vacuole-type and PrP deposition showed synaptic-
type. MM2- and MV2-type cases showed large confluent
vacuole-type spongiform change and PrP deposition showed
primitive plaque-type or perivacuolar-type. Interestingly, fine
vacuole-type and large confluent vacuole-type spongiform
changes were observed separately in same cerebral neocortex
in some cases. In these cases, synaptic-type PrP
deposition was present in the lesions showing fine vacuoletype
spongiform change, and primitiveplaque-type and/or
perivacuolar-type PrP deposition was observed in the lesions
showing large confluent vacuole-type spongiform change.



An autopsied case of Creutzfeldt-Jakob
disease with mutation in the prion protein
gene at codon 232
T Hama1, H Niwa1, Y Iwasaki3, M Yoshida2, Y Hashizume2,
N Murakami1. 1Dept. of Neurol., Kariya Toyota General
Hospital, 2Dept. of Neuropathol, Inst. for Medical Sci. of
Aging, Aichi Med. Univ., 3Dept. of Neurol, Nagoya Univ.
Grad. Sch. of Med.
We report the clinical, neuropathological, and immunohistochemical
findings in a patient with Creutzfeldt-Jakob disease
(CJD) with a substitution from methionine to arginine at
codon 232 (M232R) in the prion protein (PrP) gene and
type 1 PrP. His initial symptom was memory disturbance
and disorientation. The patient presented rapidly progressive
dementia and presence of myoclonus, and periodic synchronous
discharges in the electroencephalogram. These
findings were mostly consistent with those for sporadic CJD.
This patient reached the stage of akinetic mutism in 2
months, and died 13 months after the onset of the disease.
Neuropathologic examination revealed spongiform degeneration,
neuronal loss and severe astrocytosis in the neocortex,
basal ganglia and thalamus. Immunohistochemical
staining for PrP showed diffuse gray matter staining, socalled
synaptic-type PrP deposition. No plaque-type PrP
deposition was observed. Patients with CJD M232R mutation
have no definite familial occurrence and are very similar
to the patients with sporadic CJD.



An autopsy case of Creutzfeldt-Jakob
disease with Lewy body disease
T Haraguchi1, H Ishizu2, S Terada3, K Sakai1, T Kitamoto4,
T Nagai1, Y Tanabe1, H Takata1, K Nobukuni1, Y Ihara1,
S Kuroda3. 1Dept. of Neurol., Minami-Okayama Medical
Center, 2Zikei Hospital, 3Dept. of Neuropsy., Univ. of
Okayama, 4Dept. of Prion Biol., Univ. of Tohoku
A 77-year old man began to suffer from forgetfulness in Augst
2003. His mental deterioration progressed rapidly, and he
could neither walk nor stand by himself on October 24, 2003.
On examination, he was disoriented and confused. His limbs
were both rigid and spastic. Myoclonus is remarkable in his
face and limbs. Periodic synchronous discharges on EEG
were recorded. Head MRI (DWI) revealed high intensity area
in the cerebral cortex and caudate nucleus. He became akinetic
mutism on November 2003. He died of pneumonia on
January 2004. Post-mortem examination revealed neuronal
loss, astrocytosis and patchy spongiosis in the cerebral
cortex and lenticular nucrei. Synaptic type deposits of prion
protein were present in the temporal cortex. Additionally,
Lewy bodies were observed in the cerebral cortex. Although
the substantia nigra and locus ceruleus showed almost no
neuronal loss and gliosis, Lewy bodies observed in these
areas. Although this case was clinicopathologically diagnosed
to be sporadic CJD, this case showed the features of
both CJD and diffuse LBD by histological findings.



An autopsy case of non-plaque type
Creutzfeldt-Jakob disease (CJD)
associated with cadaveric dura mater
M Samuraki, A Morinaga, I Nozaki, M Shinohara, K Ono,
E Furui, C Ishida, M Yamada. Dept. of Neurology &
Neurobiology of Aging (Neurology), Univ. of Kanazawa
We report a 37-year-old man who developed CJD 18.5 years
after receiving a cadaveric dura mater graft. He underwent
implantation of a cadaveric dura mater graft for head injury
due to a traffic accident in 1985. In May 2004, at age 37, he
had gait unsteadiness, and insomnia. On admission to our
hospital in September 2004, he was in a bed-ridden state
with startle reflex and myoclonus. Brain MRI revealed hyperintensity
in the entire cerebral cortex and basal ganglia.
EEG showed periodic synchronous discharges. CSF 14-3-3
protein increased. Analysis of the prion protein (PrP) gene
showed no mutation and Met/Met at the polymorphic codon
129. He was diagnosed as dural graft-associated CJD
(dCJD). He died in March 2006. The brain weighted 896 g.
The grafted dura was recognized in the skull base. Neuronal
loss, gliosis, and spongiosis were severe in the entire cerebral
gray matter, while neurons in hippocampus and Purkinje
cell were relatively preserved. PrP immunostaining with an
antibody to PrP (3F4) revealed diffuse granular staining, but
no plaque formation. Western blot analysis demonstrated
type 1 protease-resistant PrP. Neuropathological diagnosis
was non-plaque type dCJD.



MM2-cortical sCJD: An autopsy case
Y Niimi1, Y Iwasaki2, Y Hashizume3, M Yoshida3,
M Hirayama4. 1Dept. of Neur., Gihu Social Ins. Hosp.,
2Dept. of Neurology, Nagoya Univ., 3Institute for Medical
Science of Aging, Aichi Med. Univ., 4Dept. of Neurology,
Kasugai Municipal Hosp.
A 67-year-old man with no medical history admitted to our
hospital in August 2005 because of rapidlly progressive
dementia. MRI (DWI) revealed HIA in both occipital and temporal
cortices. He showed no apparent paralysis, but significant
mental retardation, short step gait and myoclonus in his
right lower limb. SPECT revealed reductions of CBF in those
cortices. EEG showed PSDs. CSF examination revealed
mild increase of NSE. His symptom rapidly progressed and
he died of asphyxia in November. The postmortem examination
of the brain was performed. The gross appearance
was mild cerebral atrophy in occipital area. Histological
examination showed diffuse large-confluent-vacuole-type
spongiform change in cerebral cortices, especially in temporal
and occipital area. Immunohistochemical studies for PrP
revealed the perivacuolar pattern of PrP deposition throughout
the cerebral cortices. The genotype of PrP gene was
MM2. Our case is clinically classified as classical sCJD, but
pathologically, it is classified as MM2-cortical sCJD. We
suggest that clinically classical sCJD should lead to suspicion
of MM2-cortical sCJD.



An autopsy case with MM1 and 2-type
sporadic Creutzfeldt-Jakob disease
without myoclonus with 3 months’
S Takeuchi1, H Watanabe3, Y Iwasaki2,3, M Yoshida2,
Y Hashizume2, T Kitamoto4, M Ito5, G Sobue3. 1Dept. of
Neurol, Aichiken Saiseikai Hospital, 2Dept. of Neuropathol,
Institute for Medical Science of Aging, Aichi Medical Univ.,
3Dept. of Neurol, Nagoya Univ. Graduate School of Med.,
4Div. of CJD Science and Technolol. Tohoku Univ.
Graduate School of Med., 5Div. of Neuropathol, Japanese
Red Cross First Hosp.
A 62 y.o. woman presented with hypobulia, and subsequently
developed somnolentia, bradykinesia, gait disturbance,
dementia, apraxia and aphasia without myoclonus with 3
months duration. EEG showed PLEDs and PSD. DWI MRI
showed high intensity signal at cortex. The brain weighed
1180 g with mild frontal atrophy and well preserved brain
stem and cerebellum. Microscopically neuronal loss was not
apparent and mild gliosis was seen, and fine vacuole-type
and large confluent vacuole-type spongiform changes were
observed separately in the cerebral cortices. In the striatum,
thalamus and cerebellar molecular layer, fine vacuole-type
spongiform changes were observed. Cerebellar granule cell
and Purkinje cell layers were well preserved. PrP immunostaining
revealed synaptic-type PrP deposition in the lesions
with fine vacuole-type spongiform change, in addition to
perivacuolar-type PrP deposition in the areas showing large
confluent vacuole-type spongiform change. This case
demonstrated the combination of MM1 and MM2-type PrP
deposits in sCJD and suggested that PrP deposits types and
Western blot analysis correlated to the basic pathological
findings of cortical spongiform change patterns.


PRION 2006

PRION 2005


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.





MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

HUMAN and ANIMAL TSE Classifications i.e. mad cow
disease and the UKBSEnvCJD only theory

TSEs have been rampant in the USA for decades in many
species, and they all have been rendered and fed back
to animals for human/animal consumption. I propose that
the current diagnostic criteria for human TSEs only
enhances and helps the spreading of human TSE from the
continued belief of the UKBSEnvCJD only theory in 2005.
With all the science to date refuting it, to continue
to validate this myth, will only spread this TSE agent
through a multitude of potential routes and sources
i.e. consumption, surgical, blood, medical, cosmetics
etc. I propose as with Aguzzi, Asante, Collinge,
Caughey, Deslys, Dormont, Gibbs, Ironside, Manuelidis,
Marsh, et al and many more, that the world of TSE
Tranmissible Spongiform Encephalopathy is far from an
exact science, but there is enough proven science to
date that this myth should be put to rest once and for
all, and that we move forward with a new classification
for human and animal TSE that would properly identify
the infected species, the source species, and then the
route. This would further have to be broken down to
strain of species and then the route of transmission
would further have to be broken down. Accumulation and
Transmission are key to the threshold from subclinical
to clinical disease, and of that, I even believe that
physical and or blunt trauma may play a role of onset
of clinical symptoms in some cases, but key to all
this, is to stop the amplification and transmission of
this agent, the spreading of, no matter what strain.
BUT, to continue with this myth that the U.K. strain of
BSE one strain in cows, and the nv/v CJD, one strain in
humans, and that all the rest of human TSE is one
single strain i.e. sporadic CJD (when to date there are
6 different phenotypes of sCJD), and that no other
animal TSE transmits to humans, to continue with this
masquerade will only continue to spread, expose, and
kill, who knows how many more in the years and decades
to come. ONE was enough for me, My Mom, hvCJD, DOD
12/14/97 confirmed, which is nothing more than another
mans name added to CJD, like CJD itself, Jakob and
Creutzfeldt, or Gerstmann-Straussler-Scheinker
syndrome, just another CJD or human TSE, named after
another human. WE are only kidding ourselves with the
current diagnostic criteria for human and animal TSE,
especially differentiating between the nvCJD vs the
sporadic CJD strains and then the GSS strains and also
the FFI fatal familial insomnia strains or the ones
that mimics one or the other of those TSE? Tissue
infectivity and strain typing of the many variants of
the human and animal TSEs are paramount in all variants
of all TSE. There must be a proper classification that
will differentiate between all these human TSE in order
to do this. With the CDI and other more sensitive
testing coming about, I only hope that my proposal will
some day be taken seriously.

My name is Terry S. Singeltary Sr. and I am no
scientist, no doctor and have no PhDs, but have been
independently researching human and animal TSEs since
the death of my Mother to the Heidenhain Variant of
Creutzfeldt Jakob Disease on December 14, 1997
'confirmed'. ...TSS

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518

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