{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T19:06:41Z","timestamp":1793041601024,"version":"4.5.0"},"reference-count":34,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,10,23]],"date-time":"2024-10-23T00:00:00Z","timestamp":1729641600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:p>Biallelic variants in phosphatidylinositol glycan anchor biosynthesis, class G (<jats:italic>PIGG<\/jats:italic>) cause hypotonia, intellectual disability, seizures, and cerebellar features. We present 8 patients from 6 families with a childhood\u2010onset motor neuropathy and neurophysiology demonstrating variable motor conduction block and temporal dispersion. All individuals had a childhood onset tremor, 5 of 8 had cerebellar involvement, and 6 of 8 had childhood febrile seizures. All individuals have biallelic <jats:italic>PIGG<\/jats:italic> variants, including the previously reported pathogenic variant Trp505*, plus 6 novel variants. Null enzyme activity is demonstrated via <jats:italic>PIGO<\/jats:italic>\/<jats:italic>PIGG<\/jats:italic> double knockout system for Val339Gly and Gly19Glu, and residual activity for Trp505* due to read\u2010through. Emm negative blood group status was confirmed in 1 family. <jats:italic>PIGG<\/jats:italic> should be considered in unsolved motor neuropathy. 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