Building a World Wide Database

Given the number of at least 200 accessible cases, a structured collection of clinical observations and systematic analysis of data will define the natural course of the NA syndromes and may provide a basis for treatment studies. The data registry should be internet-based and easily accessible so that physicians worldwide can share information on their patients. Data entry into the case record forms must be standardized to maintain quality and comparability across centers. To achieve this, an NA submodule has been established within the European Huntington's Disease Network (EHDN), where procedures to maintain data confidentiality are already established (Fig. 3).

In addition to the instruments used for Huntington's disease, special rating scales are needed in NA, e.g. to document dysphagia or involvement of the peripheral nervous system.

Since the movement disorders of NA defy easy classification, it is of great interest to build a video collection so that the motor abnormalities may then be categorized by a panel of experts. A standard protocol for filming has recently been agreed upon; the sample video can be downloaded from the submodule webpage. Further developments may include a standardized neuropsychology battery [11] as well as neuroimaging protocols for MRI volumetric studies (see chapter in this book by Henkel et al.) or PET transmitter imaging (see chapter by Leenders and Jung) to make data comparable among the patients scattered throughout various countries.

The most important aspect, however, from a clinical point of view, is the gathering of detailed information about past treatments. Experiences with botulinum toxin injections were recently collected among the patients (via [email protected] yahoogroups.com) but this approach must be extended using a systematic protocol. Similarly, the effects of deep brain stimulation ought to be compared between the few patients who so far have undergone the procedure. Drug treatments such as sulpiride, tetrabenazine, tiapride, levetiracetam [21] or other substances should be documented, as should be approaches such as physiotherapy, dysphagia treatment, and psychotherapy. Accumulation of an extensive set of therapeutic experiences may then be followed by the design of observational treatment studies that in such uncommon medical conditions are only feasible if close international collaboration is achieved. Collectively, these activities effectively constitute a "Virtual Neuroacanthocytosis Institute."

www.euro-hd.net/html/na/submodule) within the webpages of the European Huntington's Disease Network"/>
Fig. 3 Screenshot of the NA submodule portal (www.euro-hd.net/html/na/submodule) within the webpages of the European Huntington's Disease Network
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