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(1)The Langherans cell histiocytosis (LCH) is a rare disease which may affect almost any of the body organs

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The Langherans cell histiocytosis (LCH) is a rare disease which may affect almost any of the body organs. The involvement of the Central Nervous System (CNS) may be present in 25% of the patients both as tumor lesions and as

“neurodegenerative form”.

This hypothesis is based on a study on the neurodegenerative complication associated with clinics which are variable for severity and progression time. A recognised risk factor seems to be the diabetes insipidus.

At the present it’s not possible to discriminate which patients, among those with diabetes insipidus, may have a higher risk to have neurodegenerative lesions and, most of all, which patients, among those with positive neuroimaging, will develop more critical clinics.

The subjects of this study have undergone both neuroradiology and neurophysiology investigations with the aim to define if the latter may help the diagnose of a damage already happened and predictive of a damage still not happened.

The early diagnosis of the neurodegenerative form of the LCH could allow to identify the subjects at risk in order to choose the better time to start the treatment.

This could limit the neurogenic damage and could increase life quality of the patients.

In addition, this study highlights the important role of the Neurophysiology Technician in all of these processes.

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