CNS Disease

Kawasaki Disease (mucocutaneous lymph node syndrome)

Affecting children under the age of 5, diagnostic criteria include at least 5 days of fever, conjunctival injection without exudate, red-fissured lips, strawberry tongue, erythematous rash, redness and/or edema of palms and soles, and cervical adenopathy.
Although generally benign and self-limited, it is associated with coronary artery aneurisms in about 25% of affected children. Early treatment with high-dose IV gamma-globulin and oral aspirin have been shown to reduce the incidence of aneurisms.

Tourette's syndrome is a chronic tic disorder that is characterized by both motor and vocal tics, with onset in childhood. Table 2 lists current diagnostic criteria for Tourette's syndrome.1 This disorder usually begins with simple tics and progresses to more complex tics. Coprolalia was originally described as a pathognomonic symptom by Gilles de la Tourette, but it occurs in only 8 to 39 percent of patients, mostly males, and is not required for a diagnosis.2-

The precise etiology of Tourette's syndrome is unknown. Tics are believed to result from a tripartite dysfunction in the central nervous system. Imaging techniques have implicated the basal ganglia and frontal cortex in the pathogenesis of Tourette's syndrome.12-14 The second source of abnormality is thought to be inappropriate regulation of neurotransmitters, especially dopamine.


Echolalia (repeating others' words or statements)
Echopraxia (imitating others' actions)
Palilalia (repeating one's own statements, words or parts of words)

Hodgkin's Disease

Hodgkin's disease is a kind of lymphoma, an uncommon cancer that starts in the lymph system. The lymph system includes the lymph nodes, lymph vessels, and other parts of the body's immune and blood-forming systems, such as the spleen and bone marrow. The lymph system makes, stores, and carries infection-fighting white blood cells called lymphocytes. Lymph nodes and lymph vessels are located throughout the body.
Hodgkin's disease, also called Hodgkin's lymphoma, is one of the most curable cancers.

Hodgkin's disease can occur in children or adults. Risk factors for adult Hodgkin's disease are:
being in young or late adulthood
being male
being infected with the Epstein-Barr virus, which causes mononucleosis (also called mono)
having a first-degree relative (parent, brother, or sister) with Hodgkin's lymphoma
having acquired immunodeficiency syndrome (AIDS).

What are the symptoms?
Symptoms of Hodgkin's disease are:
painless swelling of lymph nodes, in the neck, armpits, or groin
fever
night sweats
unexplained weight loss
itching
tiredness.

How is it diagnosed?
Your health care provider will ask about your symptoms and examine you. A sample of tissue will be taken from a swollen lymph node and examined under a microscope. This removal of a sample of tissue is called a biopsy. If you have Hodgkin's disease, unusual cells called Reed-Sternberg cells will be found in the tissue.

If Hodgkin's disease is found, more tests will be done to learn the stage or the extent of the disease. These tests may include:
blood tests
chest x-rays
CT scans of the chest and abdomen
biopsy of your bone marrow to see if the disease has spread there.

What is the treatment?
The diagnosis of Hodgkin's disease is classified into stages, depending on the areas of lymph nodes and other organs affected. There are several types of Hodgkin's disease. Treatment will depend on the type and stage of disease, as well as your general health.
Radiation therapy or anticancer drugs (chemotherapy) will be used to shrink tumors and kill cancer cells. If Hodgkin's is not cured initially with chemotherapy, stem cells (immature blood cells) may be transplanted from your blood or bone marrow or from a donor's blood or marrow.
You may be prone to serious infections during and after your treatment.

What causes Huntington's disease?

Huntington's Disease is an inherited degenerative neuropsychiatric disorder which affects both body and mind. Symptoms most commonly begin between the ages of 35 and 50, although onset may occur any time from childhood to old age. Research is progressing rapidly, but there is currently no cure.
Huntington's disease is inherited in an autosommal dominant fashion. Each child of an affected parent has a 50 % chance of inheriting the disease and is said to be at risk. The discovery of the HD gene in 1993 has made it possible to test at-risk individuals for Huntington's disease before symptoms occur. In the absence of a cure, however, the decision to be tested or not remains a difficult one, and there are many important legal, financial and personal considerations. For more information about the genetic basis of Huntington's disease, you may wish to visit the Online Mendellian Inheritance in Man (OMIN) to learn about the genetic basis or the DNA library at the National Library of Medicine to see the actual gene sequence.
The clinical features of Huntington's disease can be thought of as a triad of emotional, cognitive and motor disturbances. Symptoms include chorea (dance-like involuntary movements), clumsiness, slurred speech, depression, irritability and apathy. Cognitive losses include intellectual speed, attention and short-term memory.
Huntington's disease affects people in different ways. One member of a family may have more trouble with clumsiness while another may have emotional outbursts. Moreover, symptoms of Huntington's disease in the same individual change over time.
Although there is currently no overall remedy for this disease, professionals are in agreement that a symptom-specific, interdisciplinary approach is the best way to care, maintain and improve the quality of life for the affected individual or family.

Huntington's disease is caused by a faulty gene on chromosome 4. The gene, which produces a protein called Huntingtin, was discovered in 1993.
In some way - which is not yet understood - the faulty gene leads to a damage of the nerve cells in areas of the brain, including the the basal ganglia and cerebral cortex.
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