Motor nerve biopsy for early diagnosis of lower motor neuron syndromes

In a retrospective evaluation of 90 indicated patients, the diagnostic performance of motor nerve biopsy in lower motor neuron syndromes (LMNS) was informative [1]. Motor nerve biopsy was conclusive in 76.4% of cases and correctly classified 95.4% of pathologic biopsies as ALS or MN.

Of 90 patients, 49 were classified as MND (57%), 17 as MN (19.8%), 20 as non-diagnostic (23.2%), and 4 as technically non-evaluable (4.4%). At follow-up, the pathological diagnosis showed a sensitivity and specificity of 78% and 88.9% for ALS, and 85% and 100% for MN, respectively.

The outcomes were also used to retrospectively validate the new-revised El Escorial Criteria (NR-EEC) in ALS and ALS-mimics. Compared to the older version of these criteria, NR-EEC showed a significant increase in sensitivity: 45.9% versus 11%. The degree of axonal degeneration was associated with shortened survival in ALS. TAR DNA-binding protein-43 (TDP-43)-immunoreactivity in motor nerves correlated with the risk of developing ALS and could be a potential new biomarker for ALS.

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   1. Riva N, et al. EAN 2019, O4121.

Posted on 27 August, 201922 February, 2024