Clinical History:

This 52-year-old man had presented at age 37 with blurred vision. This lasted for several weeks. Five years later he suffered an episode of right leg weakness which resolved over several months. Over the ensuing 10 years he developed dysarthria, internuclear ophthalmoplegia, and paraplegia with spasticity. He became bedridden and died of a pulmonary embolus. At the time of his initial evaluation, a spinal tap revealed a normal CSF pressure, 6 cells (all lymphocytes), an elevated protein, and a normal glucose. Protein electrophoresis revealed an elevation in IgG. An MRI was performed six years before his death and showed several T2 bright images in the white matter of the cerebral hemispheres.

Image 5.1:

This is a CT scan from another patient with the same disease. Describe the findings. What is the normal age range for this disease?

There are lucent areas around the lateral ventricles representing multiple sclerosis plaques. The normal age range for this disease is young adulthood - 20's to 40's.

Image 5.2:

This is a gross photograph of the brain from this patient. Describe the abnormalities. Are these old or new lesions?

There are sunken greyish areas around the ventricles. These represent old lesions with gliosis.

Image 5.3:

This is a microscopic section with myelin stain of one of the lesions. Describe the changes.

There is loss of myelin in the area of the plaque.

Image 5.4:

This is a microscopic section with a silver stain for axons of one of the lesions. What appears to be preserved?

Although the myelin is gone as evidenced by the myelin stain, the axons are relatively preserved.

Image 5.5:

On this H&E stained microscopic section, note the perivascular lymphocytes in the lesion. What are some of the theories regarding pathogenesis in this disease? What are some of the treatment modalities for this disease?

There are many theories for the pathogenesis of this disease, including immunological and viral. Corticosteroids have been used as treatment. Cytoxan and other immunosuppressive drugs have also been used. Beta interferon is presently being used in more acute cases. Large series need to be evaluated over long periods because of the tendency for the disease to have natural remissions.

Answers:

1. What is the most likely diagnosis?

Multiple sclerosis (MS).

2. What other test would help in confirming your diagnosis? What would you tell the patient about the prognosis?

CSF agarose gel electrophoresis to look for oligoclonal bands. Visual evoked responses and brain stem evoked responses may demonstrate abnormalities not noted on examination. The prognosis varies with each patient and many patients do not progress to severe disabilities. Many patients have periods of remission.



3. Can this patient refuse further treatment?

MS often brings up similar issues to neurodegenerative diseases such as Alzheimer disease and feeding tubes. In other words, are they still worthwhile if the patient no longer wishes to live? But the MS patient usually has capacity for the course of the disease, and so should be able to tell us whether to stop the feeding tube (or whether to start one in the first place). The important thing to remember is that dying from dehydration and malnutrition should never be painful. The symptoms are usually mild and transitory, and easily avoided or relieved by such noninvasive things as moist towels for the forehead and hands, moist swabs or hard candies or little sips of water for the mouth, and lip balm for the lips.