Cerebral Demyelization Syndrome

Cerebral Demyelization Syndrome is extremely frustrating to research on the Internet. It's a triple threat of obscurity: they change the name frequently, it effects extremely few people, and the few groups working on it are competing against each other mostly with an eye toward a cure for Multiple Sclerosis.

Essentially CDS is the flip side of MS. While MS attacks the central nervous system, CDS works roughly the same attack on the other side of the blood/brain barrier.

The disease attacks the coating around nerve fibers which is made of myelin. Myelin allows efficient conduction of nerve impulses and when it becomes damaged or destroyed, neurons no longer transmit signals effectively and many neurological functions are diminished or lost.

Basic motor functions, hallucinations presenting along all five senses, aphasia, amnesia, memory loss and incorrect memory retrieval are common symptoms as the disease progresses.

Strokes occur in a high percentage of the afflicted at this stage as a percentage of myelin disengages from the neural walls in particles large enough cause clots in the brain vasculature. The bulk of these are temporary as increasing blood pressure behind the clog can usually dislodge it rapidly owing to the typically round structure of the myelin fragments. This often results in repeatedly random and changing symptoms presenting in the patient.

Eventually depletion of the myelin sheaths continues to a degree that the brain can no longer perform basic autonomic functions leading usually to fatality by major organ failure, suffocation, heat regulation complications, heart attack or stroke.

Originally I would point people to my definitions page on DyingNotDead.com but as I've abandoned that site, I felt I should have a page here for whatever knowledge I can accumulate on this disease. The summations will be from me, translated for the layman (myself included) and should be taken as such; loose approximations written by someone with no background in the field. The links will mostly lead to technically dense research papers, written by doctors for their peers.

CDS Cerebral Demyelization Syndrome. This is a rare disorder [which they are constantly changing the name of, six times since I was diagnosed - though this seems to have stabilized in the last three years.] which shares much in common with Balo Disease and Heubner-Schilder Syndrome. It is one of a family of Multifocal Central Demyelination Diseases which until a few years ago was often misdiagnosed as a Adrenomyeloneuropathy variant that did not attack the adrenal gland as virulently.

My understanding is that of a layman, but the way it was simplified in explanation for me, is that my white blood cells are mutated in a way that allows them to pass the blood brain barrier into the brain, where they then attack the myelin. This results in an erosion of the insulation of the "wires" of the brain resulting in short circuits that can have virtually any effect on the body and mind. Once destroyed the body does not replace myelin, so CDS is a disease of attrition.

The brain however is a triumph of redundant functions and has an amazing ability to rewire itself around problem areas. Most side effects are, for now, not long in duration.

For the most part I can't tolerate extended periods of stimulation, without a period of sensory deprivation (alone time, or TV watching works best. I'm considering exploring meditation as common sense says that would work well.) I am especially susceptible to unexpected or overwhelming auditory stimulation. Abundant sunlight can also be a problem. So of course, I live in Florida.

Much of my treatment is covered by a non-disclosure form, which in my opinion is not necessary as they don't give me enough information to endanger their research.

My treatment falls into two categories: retarding the rate of demyelination, and managing the symptoms. The first is the one that is surrounded in secrecy and I know little about and if I did I couldn't talk about. The second is an implant. A thin wire of mostly heavy metals that is inserted near the my top on my spine and slowly dissolves over a six month period. This implant slows the electrical activity of my brain, making the short circuits less severe in frequency, effect and duration.

Okay, there isn't going to be much rhyme or reason to the order, or inclusion of these various articles and links. It's mostly the posting of relevant articles as I find them.

Later, as I have more information, if I find a way to reorganize this data, I will. I've decided not to provide links to the technical terms as you can just as easily Google them if you are interested. A lot of these sites disappear regularly, so where possible I will duplicate the relevant paragraphs instead of linking. As there is no way to do that with with PDF sites (which a preponderance of scientific papers of are, links will be used for those. My comments, if any,  will be blue and in italics.

Oligodendroglial precursor cells differentiation and alteration during gliomagenesis

Adult oligodendrocyte precursor cells (OPCs) make up around 5-8 % of the glial cell population in the adult CNS. In response to demyelization disease these cells divide and are though to differentiate to provide new oligodendrocytes to replace those that have been lost (Levine et al., 2001).
OPC are characterized by phenotypic marker antigens, such as the NG2 chondroitin sulphate and transcription factors of the Olig family.

As these OPC specific antigens are also expressed by many brain gliomas, it has been proposed that OPC may be the originating cell of most oligodendro- gliomas and oligoastrocytomas (Shoshan et al., 1999; Marie et al., 2001). Characterization of the signaling pathways involved in oligodendrocyte differentiation might have high incidence for the development of therapeutics for treating demyelization diseases and for improving therapeutic strategies for glioma.

MS is very closely related to CDS at least in terms of the misfiring of nerves. MS attacks the central nervous system, while CDS starts closer to the source. Much research on MS is relevant to CDS and easier to find. It was been St. Jude's position that CDS is in the MS family. The Swiss contend it is it's own family.

Journal of Clinical and Experimental Neuropsychology
Publisher: Psychology Press, part of the Taylor & Francis Group
Issue: Volume 27, Number 1 / January 2005
Pages: 33 - 54

Cerebral Activation Patterns During Working Memory Performance in Multiple Sclerosis Using fMRI

Nancy D. Chiaravalloti A1, A2, Frank G. Hillary A1, A2, Joseph H. Ricker A1, A2, Christopher Christodoulou A1, A2, Andrew J. Kalnin A2, Wen-Ching Liu A2, Jason Steffener A2, John DeLuca A1, A2

A1 Kessler Medical Rehabilitation Research and Education Corporation, West Orange, NJ, USA
A2 University of Medicine and Dentistry of New Jersey, Newark, NJ, USA

Abstract:

Working memory deficits are common in Multiple Sclerosis (MS) and have been identified behaviorally in numerous studies. Despite recent advance in functional magnetic resonance imaging (fMRI), few published studies have examined cerebral activations associated with working memory dysfunction in MS. The present study examines brain activation patterns during performance of a working memory task in individuals with clinically definite MS, compared to healthy controls (HC). fMRI was performed using a 1.5 Tesla GE scanner during a modified Paced Auditory Serial Addition Test (mPASAT). Participants were 6 individuals with MS with working memory impairment as evidenced on neuropsychological testing, 5 individuals with MS without working memory impairment, and 5 HC. Groups were demographically equivalent. Data were analyzed using Statistical Parametric Mapping (SPM99) software, with a stringent significance level (alpha < .005, voxel extent = 8). Both MS groups and the HC group were able to perform the task, with comparable performance in terms of numbers of correct responses. Activation patterns within the HC and MS not-impaired groups were noted in similar brain regions, consistent with published observations in healthy samples. That is, activations were lateralized to the left hemisphere, involving predominantly frontal regions. In contrast, the MS impaired group showed greater right frontal and right parietal lobe activation, when compared with the HC group. Thus, it appears that working memory dysfunction in MS is associated with altered patterns of cerebral activation that are related to the presence of cognitive impairment, and not solely a function of MS.

Vascular Dementia

Disease Information

Dementia is the general term for a gradual progressive decline in a person's memory and other cognitive abilities. Vascular dementia is the second most common form of dementia after Alzheimer disease (AD) and accounts for up to a third of all dementias. This condition is not a single disease but a group of syndromes relating to different vascular mechanisms. Changes in the brain’s blood supply may result in the erosion of some brain issue (neurons). The initiating event could be a single stroke, a number of small strokes, a temporary decrease in brain blood flow (transient ischemic attack/TIA) or a traumatic brain injury (TBI). Vascular dementia can also be caused by a number of other conditions including high blood pressure (hypertension), irregular heart rhythms (arrhythmias), cardiac arrest and diseases which cause damage to the blood vessels in the brain (arteriosclerosis).

Subcortical Vascular Dementia (Binswanger's Disease) Involves vascular damage to the nerve cell fibers of the inner parts of the brain (deep white matter) by affecting the sheath which insulates nerve fibers in the brain (demyelization.)


Balo is a more common, faster acting ailment possibly related to CDS.

Balo Disease


Synonyms

Balo Disease is a rare and progressive variant of multiple sclerosis. It usually first appears in adulthood, but childhood cases have also been reported. While multiple sclerosis typically is a disease that waxes and wanes, Balo Disease is different in that it tends to be rapidly progressive. Symptoms may include headache, seizures, gradual paralysis, involuntary muscle spasms, and cognitive loss. The alternative names for Balo Disease, concentric sclerosis or Balo concentric sclerosis, refer to the fact that Balo Disease is characterized by bands of intact myelin (the sheath of fatty substances surrounding nerve fibers) alternating with rings of loss of myelin (demyelination) in various parts of the brain and brain stem. The symptoms of Balo Disease vary, according to the areas of the brain that are affected. Symptoms may progress rapidly over several weeks or more slowly over two to three years.

Multiple Sclerosis (MS) is a representative autoimmune demyelinating disease of the central nervous system (CNS) affecting mainly young adults. Clinician's interests are mainly focused on the physical impairment that MS may bring. However, during the past few decades there is an increasing awareness of cognitive dysfunction in MS patients, a symptom already described for MS cases by Charcot. It is estimated that cognitive dysfunction may be present in more than 50% of the MS patients during their lifetime and may cause a devastating impact on every day function, independently on the degree of physical disability. In several neuropsychological studies it has been reported that recent memory, attention, processing speed, visuospatial abilities and executive functions, are among the domains of cognitive function most commonly affected in MS. On the contrary, language skills and intellectual functions are generally preserved. However, patients may either underestimate their deficits due to metamemory impairment or overestimate them due to depression. In addition, one of the major problems in the every day clinical practice remains the assessment of cognitive function, since the mini – mental state examination which is included in a routine neurological evaluation, is insensitive to MS-related cognitive impairment, especially a non-severe one. It is therefore evident that a brief, cost-effective and reliable neuropsychological assessment may be of a great value and may contribute to a global evaluation of the disease progression in an individual patient.

During the last decade, an enormous progress in MS treatment has been made. However, most clinical trials of disease modifying therapies have been focused on the role of the administered agents in controlling the physical disability of MS patients. Only in a few multicenter studies cognitive function was among the outcomes studied and preliminary results indicate that currently used immunomodulatory and symptomatic agents may positively influence the cognitive outcome of the patient. There is considerable hope that by altering the cerebral demyelinating process, a slower decline in cognitive functions may be accomplished. Furthermore, combination of drug therapy with cognitive rehabilitation techniques is expected to result in the improvement of the quality of life standards of the MS patients.

Links:

Combined therapy with methylprednisolone anderythropoietin in a model of multiple sclerosis