The possibilities of an
infectious etiology to MS has been discussed for long and remains a
controversy. Before going further into this, the reader is asked to consider
the following alternatives:
1. Systemic infection may aggravate MS of
whatever etiology.
2. CNS-infection may directly cause MS in some
(genetically disposed) persons.
3. CNS-infection (but also other disorders) may
cause exposition of otherwise undetectable proteins behind the blood-brain
barrier, in turn leading to autoantingenic response in genetically disposed
persons.
4. Cross-reactivity (molecular mimicry) between
the antigen response towards infectious agents and myelin components triggers
an initial reaction, kept upright through continued damage of myelinshealts.
5. An infection alters the lymphocytic response
towards autoantigeneity.
Failure to distinguish between these
alternatives leads to considerable confusion among the studies cited. The
MS-patient may hope for alternative 1 (and is stimulated in this hope by
various reports), simply because it would offer another therapeutic aspect. The
second alternative is almost trivially true but may explain some of the
results. In effect, it is impossible to distinguish between alternatives 3 and
4, whether the infection would be directly or indirectly responsible and anyhow
no longer active. At best, this possibility would carry prophylactic aspects,
or it may simply explain how some clinical MS-cases develop without yielding
any possibility to alter anything. Finally, alterations induced may have the
lymphocytes as their primary target and only secondarily affect the brain.
The idea that infection might play a role was
stimulated early by the sudden appearance of MS-cases after the British
occupation of the
The present page attempts to review the newer
literature to the theme. At first, it deserves attention that some drugs, which
are interesting for treatment of MS, have both anti-infectious and
immunomodulatory effects: beta-interferon was originally tested against MS for
its general effect against viruses; tetracyclines, recommended against
chlamydia infection, are MMP-9 antagonists (see MMP-Inhibitors); and d-penicillamine has been connected
to both properties (see Mercury Clearence and d-Penicillamin).
General quotations in favor of an
infectious genesis:
Martyn [1] mentioned the possibility that not
only MS, but also Parkinson's disease and other neurological disorders may be a
consequence of childhood infection, but presently no single pathogenic virus
has been identified. Gronning et al. [2] found in a Norwegian
case-control study (among less clear differences) that MS-cases had more
frequently experienced bronchitis and/or pneumonia in the age group 11-15 years
and also tonsillectomies were reported more frequently. They judged the results
to be "consistent with the idea of MS as an age-dependent, host-immune
response to infection during childhood or adolescence." Evaluating 90 MS
paients, Korting et al. [3] found that frequent and severe upper airway
infections in childhood and youth were couples with a severe course of the
disease. They hypothesized a disturbed regulation of infection and immunity as
essential in the genesis.
Barnett et al.[4] demonstrated in an
animal study that virus infection may be a means to modulated immune
responsiveness to CNS disease. Svenninsson et al. [5] found a different
lymphocyte population among MS patients and hypothesized that activated B
lymphocytes expressing high levels of B7-1 may be of pathogenic importance in
the development and maintenance of the disease.
Restricting consideration to current disease
activity in MS patients, Giovannoni et al. [6] found infection to be a
potent inducer of symptomatic and asymptomatic disease activity in mutiple
sclerosis. Moreover, measuring urinary neopterin (a marker of IFN-gamma) this
observation was understood to provide support of a pivotal role for IFN-gamma
in the pathogenesis of mutiple sclerosis.
Special infections in the possible
ethiology
Most attention is given to various herpes
viruses [Table 1], anyhow frequently infecting humans and mostly without
causing symptoms of encephalitis.
alpha: Herpes simplex virus type 1 (aciclovir, valaciclovir, famciclovir, foscarnet)
alpha: Herpes simplex virus type 2 (aciclovir, valaciclovir, famciclovir, foscarnet)
alpha: Varicella-zoster virus (aciclovir, valaciclovir, famciclovir)
beta: Cytomegalovirus (Ganciclovir, foscarnet, cidofovir)
beta: Human herpesvirus 6 (none registered)
beta: Human herpesvirus 7 (none registered)
gamma: Epstein-Barr virus (none registered)
gamma: Human herpesvirus 8 (none registered)
Table 1: Distinction of herpes viruses and (in brackets)
current registered therapeutic agents, after Bergström [35]. Virus detection in
CSF/CNS has been reported for all subtypes.
The Epstein-Barr virus [EBV] (instantly
responsible for infectious mononucleosis) has been given particular attention
in newer studies. Opersalsky et al. [7] found in a case-control study a
strong positive association for history of this disease. In an epidemiological
study, Hernon et al. [46] found that individuals who suffered from
infectious mononucleosis, a marker of late infection with the Epstein-Barr
virus, have an increased risk of multiple sclerosis. Vaughan et al. [8]
found elevated antibodies to EBV among Norwegian MS patients. Utilizing a very
complex methodology (and perhaps not quite understood by me), Rand et al.
[9] found a similarity between the oligoclonal bands produced during active
phases in the CSF of MS patients and the antigenic response to an EBV
infection. They also wrote that "other studies have suggested a relation
between RBV infection and MS, including nearly 100% EBV seropositivity among
patients with MS and increased concentrations of antibody to EBV in CSF of
patients with multiple sclerosis."
Several new publications are dealing with herpes
virus infections. Friedman et al. [28] found 36% of the brains from
patients with MS demonstrated antibodies against herpes viruses against only
13.5% of controls. Moreover, MS patients demonstrated unusually high immune
reactivity towards these viruses. Clerica et al. [32] found immune
responses to retroviruses particular prominent among MS-patients. Using another
technique, Christensen et al. [36] found that B-lymphocytoblastoid cell
lines from MS patients produced both EBV and retrovirus-like particles. Ongradi
et al. [29] suggested that intrathecal chronic active or primary human herpes virus type 6 [HHV-6] -B infection might contribute to MS progression,
while other herpex virus types seemed to be less important. Also Xu et al. [49]
have detected the gene of HHV-6 in oligodendrocytes of MS patients. On the
contrary, Enborn et al. [33] did not find IgM response towards herpes
virus more frequent in MS patients as compared to controls but did, in the
light of previous studies, suggest a role for the virus in a subset of
patients. Enbom [48] reviewed possible mechanisms for virally induced
demyelinization and autoimmunity and summarized the conflicting evidence for
and against a role for HHV-6 in MS.
Perron et al. [10] found indications that
a novel (currently unidentified) retrovirus was
responsible - they even baptized it the "multiple sclerosis-associated
retrovirus" (MSRV). This concept was further supported by Tuke et
al. [11]. Also Christensen et al. [47] found indication for the
hypothesis that activation of normally replicatively quiescent retroviruses may
be causally involved in MS. Dybwas et al. [12] analyzed the cerebrospinal fluid
antibody specificities within one oligoclonal band. They found antibodies to
other viruses such as herpes simplex virus, human cytomegalovirus and human
papillomavirus, raising the question of the involvement of multiple pathogens
in MS. Svenningsson et al. [13] found a reduction of about 20% in the
incidence of MS in Gothenborg. They related this to the vaccination for
measles, which has been carried out on a large scale since 1971, practically
eradicating this disease in their area. Nevertheless, the children who might
profit from this had hardly reached the age where MS is seen.
Ending up the consideration of viruses, the
spectacular (though not very strong) effect of the antiviral drug acyclovir
against MS [14] deserves attention. The pro-drug valacyclovir offers a better
bioavailability of the substance, still another multicenter study [57] failed to
yield evidence for success, except for a subgroup of very active disease.
Cross-reactivity towards the autoimmune response
to herpes virus infection and oligodendrocytic products have been focused by
other authors. Espositio et al. [31] demonstrated cross reactivity of
autoantibodies binding to transaldolase, which is expressed in the brain
selectively by oligodendrocytes, with EBV and herpex simplex virus. Bronstein et
al. [34] found cross reaction of antibodies to an oligodendrocyte specific
protein and several common viral peptides. Finally, van Sechel et al.
[37] suggested that infection with common viruses such as EBV and human
herpesvirus-6 infections can trigger myelin-directed autoimmunity in a way that
is unique for humans.
Also chlamydia pneumonia has been associated with MS, but in addition
with other neuropathogenic diseases such as Mb. Alzheimer. An intracellular
form would require a prolonged (2-3 months) antibiotic therapy. Again, this
possibility is vigorously discussed [15-18,50-56].
Using an animal model of infectious
encephalitis, Burt et al. [30] found it difficult to understand any
effect of autologous hematopoietic transplantation in patients with MS if this
had a virus etiology, particularly if the graft is aggressively depleted of
lymphocytes.
Denials:
Analyzing plaque-periplaque areas from MS brain
tissue and utilizing a technique, which was otherwise successful in determining
other viruses, Gilden et al. [19] failed to detect any latent enveloped
viruses. From an epidemiological study, v. Buuren et al. [20] deduced
that perinatal infections were unlikely to be a major factor in determining MS
susceptibility. Considering only cases of parainfectious transverse myelitis,
Jeffery et al. [21] found that parainfectious cases were easily
distinguishable from MS. DeCarli et al. [22] found that that the immune
response was predominantly IgG-kappa in MS and IgG-lambda in infections. In a
limited number of MS patients, Spitler and Dau [23] found reactivity to three
viral antigens to be lower than that in normal subjects, as measured by
lymphocyte stimulation. No evidence was found for human spumaretrovirus,
oncovirus or human T-cell lymphotropic virus type 1 in three swedish
publications [24,25,26].
The methodology for analyzing this question was
investigated by Joseph et al. [27]. I am currently not aware of their
conclusion.
Molecular Mimicry
Increased serum and cerebrospinal fluid antibody
titers to numerous viruses have been reported; however, there have been no
confirmed studies detecting viral RNA or antigen in MS brain tissue. Instead,
structural similarity between viral T cell epitopes and self-peptides could
lead to an indirect induction of an autoaggressive T cell response (mimicry),
in turn aggravated by genetical and environmental factors, a possibility
mentioned by several newer publications concerning specific viruses cited
above. Myelin basic protein (MBP) and some viral proteins shows
similarities in the amino-acid sequences. Wucherpfennig & Strominger [38]
tested 129 proteins maching molecular mimicry on seven MBP-specific T cell
clones from multiple sclerosis patients. Seven viral and one bacterial peptide
efficiently activated three of these clones. A single T cell receptor can thus
recognize quite distinct but structurally related peptides from multiple
pathogens. Shaw et al. [39] found that these similarities are even
stronger what myelin proteolipid is concerned. At least what envelope proteins
of viruses suspected of inducing demyelinating processes and MBP was concerned,
Rubio and Cuesta [40] could not detect any cross-reaction. Banki et al.
[41] suggested as a result of their study molecular mimicry between viral
core proteins and the human transaldolase gene which, among others, has the
function to maintain glutathione at a reduced state and, consequently, to
protect sulfhydryl groups and cellular integrity from oxygen radicals. Talbot
et al. [42] demonstrated possible molecular mimicry between a human
coronavirus and MBP in MS-patients (29% of T-cell lines) but not in normal
subjects (1.3%). Bronstein et al. [43] found antibodies to an
oligodendrocyte-specific protein, cross-reacting to several common viral
peptides, in 80% of the samples from MS-patients but not in those from the
normal subjects; and Cortese et al. [44] found that the surface
glycoprotein gB of HSV-1 was recognized by oligoclonal immunoglbulins. In an
experimental study, Ufret-Vincenty et al. [45] incriminated both the
human papillomavirus and Epstein-Barr virus for triggering an autoimmune
disease (EAE) after transferral of the viral antigens to mice.
In Summary ...
Many viruses may induce an antigen response to
many antigens. I cannot help speculating what would be found when other
antigens, not relating to MS, were tested. Moreover, as mentioned under environmental
factors,
mimicry is not restricted to microbal antibodies, also a cow milk protein has
been identified. At the present stage, the mechanism is well possible but the
findings reviewed are not very helpful for defining a certain direction. There
may well be an infectious genesis adding to other influences on producing MS,
but it is hardly alone responsible for the majority of cases. There are not
much indications of an ongoing infection and therefore hardly any therapeutical
consequences for patients already hit by this disease, although some
prophylactic consequences might follow, of interest for the children of
MS-patients. Currently, no recommendations can be given in this direction.
Literature:
Revised May 21, 2004