Alpers disease and apoptosis ???

Brunhilde Keijers brunhilde.keijers at ping.be
Mon Feb 2 17:31:15 EST 1998


Ron,
As I'm not medical schooled, I don't fully understand your question I'm
afraid.  I think you're talking about EEG-protocols.  The only thing I ca=
n
tell you is there were no "giant potentials" upon lightstimulation.  I've
three descriptions of EEG-protocols (in cronological way with one week
intervals, recorded one month after acute onset of the disease) :
1) Lots of delta-activity (2-3 c/s) at background-level (100-200 =B5V) an=
d
th=E9ta-activity (4-5 c/s) with amplitudo 'till 100 =B5V (symmetrical dif=
fuse
pattern).
2) Th=E9ta-activity (6 c/s) located at the "back" of the brain (100-200 =B5=
V)
with symmetrical pattern; at background-level there is diffuse
delta-activity (2-3 c/s) and th=E9ta-activity (4-6 c/s); no periodical
complexes.
3) Delta-activity (2-3 c/s) with amplitudo of 60 =B5V and th=E9ta-activit=
y (4-6
c/s) with higer voltages; general "paroxysmes" with high voltage
th=E9ta-activity and multifocal epileptic peaks. =20

I hope you can do something with this.
Kind regards

----------
> Van: Ron Blue <rcb5 at msn.com>
> Aan: Brunhilde Keijers <brunhilde.keijers at ping.be>
> Onderwerp: Re: Alpers disease and apoptosis ???
> Datum: maandag 2 februari 1998 0:27
>=20
> Any location to high frequency radio waves?
> Ron Blue
> >>>>>>>>>
> -----Original Message-----
> From: Brunhilde Keijers <brunhilde.keijers at ping.be>
> To: neur-sci at net.bio.net <neur-sci at net.bio.net>
> Date: Sunday, February 01, 1998 4:21 PM
> Subject: Alpers disease and apoptosis ???
>=20
>=20
> >Hi everyone,
> >
> >As a biochemical engineer and mother of a son who died of Alpers'
disease
> >(neurodegenerative disease of childhood, spongiform encephalopathy) at
the
> >age of 16 months, I'm looking for more information about this disease.
> >With our son it wasn't a defect in the mitochondrial DNA, nor were the=
re
> >liver-abnormalities seen at autopsy.
> >I have been searching for about one year now.  I searched in Medline a=
nd
> >visited the website of NORD and many others, but my search has come up
with
> >very little practical information.  As whe will have one chance out of
four
> >(one says) with a next pregnancy, it is very hard to decide to without
any
> >possibility of prenatal diagnosis.
> >
> >Is there anyone out there who thinks of Alpers' disease as a fatal
> >deregulation of natural (neuronal) cell death (so called apoptosis)?=20
What
> >is the chance that there would be for instance a mutation in the Bcl-2
or
> >Bcl-X-genes (see review article of D.E. Merry and S.J. Korsmeyer in
Annu.
> >Rev. Neurosci., 1997, 20:245-67)?
> >I also red the article of E.E.Manuelidis et al. (Neurology, 1989,
> >39:615-21) who induced a spongiform encephalopathy in hamsters upon
> >infection with brain-tissue from a 2,5 year old Alpers' disease patien=
t.
> >Are there others who reproduced these (or comparable) experiments ?  I=
s
> >there anyone else who sees a link with prion-diseases ?
> >As I red an interesting abstract of selective binding of cellular prio=
n
> >protein to Bcl-2 (C. Kurschner and Morgan J.I. in Brain Res. Mol. Brai=
n
> >Res., 1995, 30(1):165-168), maybe this must be the way to look at prio=
n
> >diseases and other neurodegenerative diseases, namely receptor-blockin=
g
of
> >Bcl-2 by (deformed) prion protein, followed by apoptosis (because of n=
o
> >feedback-regulation by Bcl-2).
> >
> >Is there anyone in the complicated world of neuroscience who works on
rare
> >neurodegenerative disorders (maybe specific on Alpers' disease) and wh=
o
> >also thinks it would be interesting for us to sequence one or another
gene
> >involved with apoptosis or prion-diseases ?  Please let me know !
> >
> >PS At the moment we are waiting for the results of the sequencing of t=
he
> >cellular prion protein.
> >
> >I'm looking forward to you reactions !
> >Kind regards from Brunhilde (Belgium)
> >
> >brunhilde.keijers at ping.be
> >
> >
>=20
>=20
>=20



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