A 30 year old female has had several episodes, each separated by several months,

Question

A 30 year old female has had several episodes, each separated by several months, in which she experiences brief periods of physical weakness, clumsiness of the legs and hands, visual disturbances, and mood swings. Her weakness is most prominent in her lower extremities. CT scans show evidence of multiple lesions in the central white matter. During her last episode, there were increasing neurological deficits due to an increasing number of disseminated lesions. Prednisone (60 mg/day for five to seven days) was prescribed and helped her return to as normal and active a life as possible.

1.What is the disease affecting this individual?

It appears this patient is suffering from Multiple Sclerosis

2.What does disseminated mean?

3.What is myelin and what is its function?

Myelin is a substance made up of 80% lipid and 20% protein, it is the coating of the axons both in the brain and central nervous system. The main function of myelin is to relay of electrical messages, to speed them up in the nervous system. With other functions it helps protect, insulate and speed up impulses.

4.How are nerve cells ensheathed in the CNS? In the PNS?

5.List and describe some demyelinating diseases and sclerotic diseases.

Explanation / Answer

2. Disseminated mean scattered lesion. This means that lesions are scattered everywhere.

4. In CNS glial cells called oligodendrocytes make the myelin and in PNS Schwann cells make and maintain the myelin.

5.

Sclerotic Diseases

Glomerulosclerosis – Scars called sclerosis on the glomeruli is Glomerulosclerosis. Systemic disease like the diabetes and the lupus are the cause for this. The activation of glomerular cells is also the prime cause of generating materials that creates scars or marks. The growth factors of Molecules activate these and these molecular cells are passed on to the glomerulus through the flowing blood. The blood enters through a filter known as the glomerular filter.

Focal segmental glomerulosclerosis (FSGS) – This kind of disease creates marks in various sections of the kidney. These affect the glomerulus and also some part of the glomeruli. Focal segmental glomerulosclerosis (FSGS) may create a systemic problem in a person. One can be affected with an idiopathic kidney disease without knowing the underlying cause. The most usual symptom of FSGS is Proteinuria.

The aortic valve is the valve that stops blood that is ejected from the heart to the body leaking backwards. If there were no aortic valve then all the blood that was pumped out of the heart from the body would be able to fall back in! There are two main problems that can occur with the aortic valve. The aortic valve can be leaky, in a condition known as aortic regurgitation or the aortic valve can become tight in a condition known as aortic stenosis. Aortic valve sclerosis is a condition whereby the aortic valve becomes thickened but does not significantly obstruct flow, unlike aortic valve stenosis, which does obstruct flow.

There is evidence that the Guillain-Barre Syndrome (GBS) is mediated by immune attack on peripheral nerve myelin. In this condition, peripheral nerves (actually, usually at the level of the proximal nerve roots) show inflammatory infiltrates with cells involved in cell-mediated immunity. Because cell mediated immunity is involved, the condition appears to depend more on local cytokine production than the development of circulating antibodies. Nonetheless, plasmapheresis (which removes significant amounts of circulating antibodies) and human immune globulin infusions (which modulate the immune system by unclear mechanisms) are capable of limiting the acute damage in the condition (these help only if done early in the clinical course). The major pathologic changes, as would be expected with an autoimmune disease, include perivascular inflammatory infiltrate, along with demyelination in the affected nerve roots and nerves. There is usually relatively little damage to underlying axons unless the inflammatory reaction is dramatic.

Inflammatory demyelination

Inflammatory demyelination happens when the body’s immune system attacks myelin. Types of demyelination like MS, optic neuritis, and acute-disseminated encephalomyelitis are caused by inflammation in the brain and spinal cord. GBS involves inflammatory demyelination of peripheral nerves in other parts of the body.

Viral demyelination

Viral demyelination occurs with progressive multifocal leucoencephalopathy (PML). PML is caused by the JC virus. Myelin damage can also occur with alcoholism, liver damage, and electrolyte imbalances. Hypoxic-ischemic demyelination occurs due to a lack of oxygen or vascular disease in the brain.

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