Background: Updated, robust estimates of the incidence and prevalence of rare long-term neurological conditions in the UK are not available. Global estimates may be misrepresentative as disease aetiology may vary by location. Objectives: To systematically review the incidence and prevalence of long-term neurological conditions in the UK since 1988. Search Strategy: Medline (January 1988 to January 2009), Embase (January 1988 to January 2009), CINAHL (January 1988 to January 2009) and Cochrane CENTRAL databases. Selection Criteria: UK population-based incidence/prevalence studies of long-term neurological conditions since 1988. Exclusion criteria included inappropriate diagnoses and incomprehensive case ascertainment. Data Collection and Analysis: Articles were included based on the selection criteria. Data were extracted from articles with ranges of inReceived: February 12, 2010 Accepted: September 30, 2010 Published online: November 17, 2010 Dr. Thomas Hoppitt Primary Care Clinical Sciences University of Birmingham Edgbaston, Birmingham B15 2TT (UK) Tel. +44 121 414 8544, Fax +44 121 414 6571, E-Mail t.j.hoppitt @ bham.ac.uk © 2010 S. Karger AG, Basel Accessible online at: www.karger.com/ned Hoppitt/Pall/Calvert/Gill/Yao/Ramsay/ James/Conduit/Sackley Neuroepidemiology 2011;36:19–28 20 the commission to encompass a range of aetiologies, symptoms, diagnoses and prognoses. Rationale and Aim of Review The aim was to systematically identify and update the incidence and prevalence of the following long-term neurological conditions: • Motor neurone disease • Huntington’s disease • Progressive supranuclear palsy • Multiple system atrophy • Postpolio syndrome • Charcot-Marie-Tooth disease • Dominantly inherited ataxias Knowledge of these data is valuable in informing future research and health service policies. Methods The review protocol is accessible at http://www.ltnc.org.uk/ research_files/RESULT_study.html. Population-based studies of incidence and prevalence were sought. Scoping Search A scoping search identified existing reviews of incidence and prevalence. Existing reviews would be updated, not repeated. Medline (Ovid; 1950 to week 2 of 2009], Embase (Ovid; 1980 to week 46 of 2008], CINAHL (Ovid; 1982 to week 1 of 2009], the Science Citation Index, Cochrane Library (CENTRAL) and Centre for Reviews and Dissemination databases were searched. Main Search Strategy The search strategy (Appendix 1) identified articles from Medline, Embase and CINAHL. The Cochrane Library (CENTRAL) and databases of ongoing research and unpublished literature were also searched. Reference lists of included articles were assessed to capture further articles omitted from the search strategy. Inclusion/Exclusion Criteria Articles were included based on the criteria outlined in table 1 . Comprehensive case ascertainment was required in order to ensure maximum patient capture. Studies before 1988 were excluded as the review aimed to present up-to-date statistics. Strict diagnostic criteria were set to minimise bias from misdiagnoses. Inclusion was based on agreement between 2 of the independent reviewers (T.H., J.C., G.J., J.R.). In cases of non-consensus, a third independent review was obtained. In cases of incomprehensive study methodology, authors were approached to determine a study’s potential inclusion. Data Extraction The following data were extracted into tables: • Source: authors and journal published • Study design: e.g. cross-sectional, cohort, etc. • Population denominator • Timescale: incidence time frame and prevalence date • Case ascertainment method • Diagnostic method • Outcome: incidence per 100,000 person-years; prevalence per 100,000 of population • Methodological limitations • Potential bias Data Analyses Incidences were reported as ranges. Pooling statistics was not possible due to methodological heterogeneity and shared population denominations between certain studies.