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Validation of MATCH score: a predictive tool for identification of patients with kelch-like protein-11 autoantibodies
  1. M Bakri Hammami1,2,
  2. Mohamed Rezk1,3,
  3. Divyanshu Dubey1,3
  1. 1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA
  2. 2Department of Medicine, Jacobi Medical Center, Bronx, New York, USA
  3. 3Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr Divyanshu Dubey, Department of Laboratory Medicine and Pathology, Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA; Dubey.Divyanshu{at}mayo.edu

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Introduction

Kelch-like protein 11 (KLHL11)-IgG is a high-risk paraneoplastic antibody associated with rhombencephalitis.1–3 Since its initial description, the clinical spectrum associated with KLHL11-IgG paraneoplastic autoimmunity has widened.2 However, some core neurological features are present in the majority of KLHL11-IgG seropositive cases. Using some of these core features, Vogrig et al proposed a MATCH score, which can be used to identify patients harbouring KLHL11-IgG.4 The MATCH score is composed of five elements: M (male sex, 1 point), A (ataxia or other cerebellar signs, 1 point), T and C (testicular tumour 2 points or other types of tumours 1 point), H (hearing impairment, 1 point). The authors further clarified that ataxia can also comprise other forms of cerebellar dysfunction including nystagmus, intention tremor, impaired pursuit and dysarthria. Testicular tumours also included spontaneously regressed germ cell tumours and metastatic testicular germ cell tumours detected in mediastinal lymph nodes without a primary source. Hearing impairment included sensorineural hearing loss or tinnitus. The score was tested in a cohort of patients that included 138 patients suspected to have paraneoplastic neurological syndrome (PNS). With MATCH score cut-off ≥4 points, the sensitivity and specificity for KLHL11-IgG seropositivity were 78% and 99%, respectively. In this study, we aimed to independently validate the MATCH score utilising our PNS database. …

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Footnotes

  • Contributors MBH contributed to acquisition and analysis of data, conception and design of the study, drafting the manuscript and figures. MR contributed to acquisition and analysis of data, and critical revision of the manuscript. DD contributed to conception and design of the study, acquisition and analysis of data and study supervision.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests DD has received research support from from the DOD (CA210208) for germ cell tumor immunoprofiling. He has consulted for UCB, Immunovant and Astellas Pharmaceuticals. All compensation for consulting activities is paid directly to Mayo Clinic. He has patents pending for Kelch-like protein 11 (KLHL11) IgG, Leucine Zipper 4 (LUZP4) IgG and Caveolae Associated Protein-4 (cavin-4) IgG as markers of neurological autoimmunity.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.