LCH-CNS


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Almost all parts of the central nervous system (CNS) can be affected by LCH. The areas of Hanoch's (CNS) involvement are the meninges, cerebellum, hypothalamus, optic chiasm, pons and medulla. All areas of the brain are very important for our everyday functioning. We tend to take most things that we do for granted, but we shouldn't.

The cerebellum is mainly responsible for balance, muscle tension and fine motor control. It contains as many neurons (nerve cells) as all the rest of the brain. The hypothalamus controls body temperature, regulates appetite and is part of our alerting mechanism and perception (mind-body integration, emotions). It is the source of 8 hormones and controls the pituitary gland. The pons and the medulla are often referred to as the brain stem. This area controls the most basic life functions, such as breathing, heart rate, and blood pressure and is also a reflex center with the cranial nerves situated in it. The cranial nerves control eye movement, the muscles of facial expression, smell, taste, saliva, chewing, hearing and balance, swallowing and tongue movements. The cranial nerve known as the vagus reaches all the way to the digestive organs. Lesions in any of those vital areas can cause great damage.

One of the problems when it comes to treatment of CNS lesions is determining what the cause of these lesions is. Even when there is no evidence of active LCH, it is believed there is a secondary phenomenon occurring and causing damage to the nerve cells in the brain. This could be an autoimmune phenomenon where the body's immune system attacks itself or it could be what is known as a paraneoplastic neurological syndrome. A paraneoplastic neurological syndrome signifies that there is a tumor elsewhere in the body and the immune system, in an attempt to fight the tumor, also attacks the central and peripheral nervous system.

Treatment for these lesions is highly experimental. Active LCH can be treated with chemotherapy which, in the case of the CNS, has to be able to cross the blood/brain barrier. If the lesions are thought to be immune-mediated, then other drugs that target the immune system and that have been used in autoimmune disease and paraneoplastic syndromes may be tried. The problem in the case of paraneoplastic syndrome is that very often, unless the underlying tumor is found and treated, immune therapy drugs are often not effective on their own.

The proposed treatment for Hanoch is intravenous immunoglobulin which is an immune therapy that works by regulating the immune system. The treatment has had variable success in certain autoimmune diseases and some forms of paraneoplastic syndromes. Let's hope it's going to work for Hanoch.