Tumefactive Multiple Sclerosis: The Diagnosis

Tumefactive multiple sclerosis is a disease where the central nervous system for a person who has demyelinating lesions has characteristics of a normal multiple sclerosis with a 2cm edema, mass effect, or a ring enhancement. Since these characteristics can be mimicking other diseases, it takes time to diagnose this problem and it can require the brain biopsy for identification. The tests to identify this problem can include radiographic or clinical tests which will show how far the dissemination of disease has reached. Tumefactive multiple sclerosis is used for the disease which is hard to differentiate with the brain tumor but not all the cases of tumefactive are fulminant. The Marburg type is normally acute and rare and it can lead to severe or frequent relapses. It can also lead to severe disability and death within few weeks or few months.

The tumefactive demyelinating lesions have little mass and vasogenic edema, they may be showing incomplete ring enhancement. The signs may be the same as that of brain abscess, brain tumors, tuberculoma, or other inflammatory disorders. This is why it is normally hard to diagnose the tumefactive especially in its first stage. Careful clinical assessment and follow up are needed to identify if someone is really suffering the tumefactive multiple sclerosis. In case it is still hard to identify the diseases, then the biopsy can be taken.  The lesions will be characterized by massive macrophage, necrosis, acute axonal injury and infiltration. There is not yet any promising treatment or cure for this condition while the mitoxantrone and plasma exchange are the only option available. Patients who suffered the Marburg’s variant died under one year and it is the form of the tumefactive that caused a high number of mortality and morbidity. The lack of definitive diagnosis test makes hard to get the treatment for this condition.

The tumefactive features do not correlate in any way with the diagnosis, course or gender. The previous series about the lesions showed that they are unifocal when they are isolated. However their evolutions were not well defined.

The patients have to be tested before and after the biopsy magnetic resonance to know more about the mass, the size and the location of the edema, multifocality and enhancement of the lesion to fulfill the Barkhof criteria.

The women to men ratio were 1, 2:1 and the onset age was 37 years old. It can take at least 7 weeks between the onset and the currying out of the biopsy. The biopsy was conducted to 61 percent of the people who are suffering the neurogical problems for the first time, for relapsing or remitting cases were 29 percent while progressive cases were 4 percent.

External symptoms observed were sensory, cognitive and motor problems. Sometime the agnosia, visual defects, seizures or aphasia were also observed.

When the cases are followed up, at least 70 percent have a definite multiple sclelerosis while the rest have demyelinating syndrome.

Any problem known to cause the inflammation of the central nervous system is known as the CNS idiopathic inflammatory demyelinating disease. Some of these CNS can be associated by the tumefactive demyelination.

These problems include schilder disease, Balo Concentric sclerosis, Marburg acute multiple sclerosis, acute hemorrhagic leukoencephalomyelitis, multiple sclerosis and tumefactive multiple sclerosis.

Contrary to what was believed in the first place, that patients who suffer tumefactive multiple sclerosis has only one phase, it has been found out that there can be a relapse after 4 years. Since the follow up only took 5 years, any relapse after these years were not well studied. However, there is a need for long term studies to know exactly what happens with the tumefactive multiple sclerosis and the treatment needed to cure it completely.

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