The yield of genetic workup for middle-aged and elderly patients with neurological disorders in a real-world setting

J Neurol Sci. 2024 Aug 15:463:123074. doi: 10.1016/j.jns.2024.123074. Epub 2024 May 31.

Abstract

Genetic workup is becoming increasingly common in the clinical assessment of neurological disorders. We evaluated its yield among middle-aged and elderly neurological patients, in a real-world context. This retrospective study included 368 consecutive Israeli patients aged 50 years and older (202 [54.9%] males), who were referred to a single neurogenetics clinic between 2017 and mid-2023. All had neurological disorders, without a previous molecular diagnosis. Demographic, clinical and genetic data were collected from medical records. The mean age at first genetic counseling at the clinic was 62.3 ± 7.8 years (range 50-85 years), and the main indications for referral were neuromuscular, movement and cerebrovascular disorders, as well as cognitive impairment and dementia. Out of the 368 patients, 245 (66.6%) underwent genetic testing that included exome sequencing (ES), analysis of nucleotide repeat expansions, detection of specific mutations, targeted gene panel sequencing or chromosomal microarray analysis. Overall, 80 patients (21.7%) received a molecular diagnosis due to 36 conditions, accounting for 32.7% of the patients who performed genetic testing. The diagnostic rates were highest for neuromuscular (58/186 patients [31.2%] in this group, 39.2% of 148 tested individuals) and movement disorders (14/79 [17.7%] patients, 29.2% of 48 tested), but lower for other disorders. Testing of nucleotide repeat expansions and ES provided a diagnosis to 28/73 (38.4%) and 19/132 (14.4%) individuals, respectively. Based on our findings, genetic workup and testing are useful in the diagnostic process of neurological patients aged ≥50 years, in particular for those with neuromuscular and movement disorders.

Keywords: Diagnostic yield; Exome sequencing; Genetic testing; Neurogenetics; Neurological disorders; Older population.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Female
  • Genetic Testing* / methods
  • Humans
  • Israel / epidemiology
  • Male
  • Middle Aged
  • Nervous System Diseases* / diagnosis
  • Nervous System Diseases* / epidemiology
  • Nervous System Diseases* / genetics
  • Retrospective Studies