European Academy of Neurology guidance for developing and reporting clinical practice guidelines on rare neurological diseases.

Aleksovska, Katina; Kobulashvili, Teia; Costa, Joao; Zimmermann, Georg; Ritchie, Karen; Reinhard, Carola; Vignatelli, Luca; Fanciulli, Alessandra; Damian, Maxwel; Pavlakova, Lucia; Burgunder, Jean-Marc; Kopishinskaya, Svetlana; Rakusa, Martin; Kovacs, Norbert; Erdogan, Fusun Ferda; Linton, Lori Renna; Copetti, Massimiliano; Lamperti, Costanza; Servidei, Serenella; Evangelista, Theresina; ... (2022). European Academy of Neurology guidance for developing and reporting clinical practice guidelines on rare neurological diseases. European journal of neurology, 29(6), pp. 1571-1586. Wiley 10.1111/ene.15267

[img] Text
European_Academy_of_Neurology_guidance_for_developing_and_reporting_clinical.pdf - Published Version
Restricted to registered users only
Available under License Publisher holds Copyright.

Download (1MB) | Request a copy


Rare diseases affect up to 29 million people in the European Union, and almost 50% of them affect the nervous system or muscles. Delays in diagnosis and treatment onset and insufficient treatment choices are common. Clinical practice guidelines (CPGs) may improve the diagnosis and treatment of patients and optimize care pathways, delivering the best scientific evidence to all clinicians treating these patients. Recommendations are set for developing and reporting high-quality CPGs on rare neurological diseases (RNDs) within the European Academy of Neurology (EAN), through a consensus procedure.


A group of 27 experts generated an initial list of items that were evaluated through a two-step Delphi consensus procedure and a face-to-face meeting. The final list of items was reviewed by an external review group of 58 members.


The consensus procedure yielded 63 final items. Items are listed according to the domains of the AGREE instruments and concern scope and purpose, stakeholder involvement, rigour of development, and applicability. Additional items consider reporting and ethical issues. Recommendations are supported by practical examples derived from published guidelines and are presented in two tables: (1) items specific to RND CPGs, and general guideline items of special importance for RNDs, or often neglected; (2) items for guideline development within the EAN.


This guidance aims to provide solutions to the issues specific to RNDs. This consensus document, produced by many experts in various fields, is considered to serve as a starting point for further harmonization and for increasing the quality of CPGs in the field of RNDs.

Item Type:

Journal Article (Review Article)


04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology

UniBE Contributor:

Burgunder, Jean-Marc, Bassetti, Claudio L.A.


600 Technology > 610 Medicine & health








Pubmed Import

Date Deposited:

24 Mar 2022 11:22

Last Modified:

02 Mar 2023 23:36

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

AGREE Delphi consensus guideline neurology rare diseases recommendation




Actions (login required)

Edit item Edit item
Provide Feedback