{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T03:50:50Z","timestamp":1784173850381,"version":"3.55.0"},"reference-count":23,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2021,3,5]],"date-time":"2021-03-05T00:00:00Z","timestamp":1614902400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["asbmr.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JBMR Plus"],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Treatment with asfotase alfa has transformed the prognosis of hypophosphatasia in children and improves the bone and muscle signs in adults. The doses used in adults are the same as in children, whereas bone remodeling is different between them. We report on the cases of two patients treated with 1\u2009mg\/kg\/day of asfotase alfa who developed spinal cord compression from spinal ossifications during treatment. The first patient, 50\u2009years old, presented after 2\u2009years of treatment with quadraparesis secondary to an increase in ossifications of the cervical vertebral ligaments. The neurological damage was resolved after laminectomy, and the patient was then treated for 18\u2009months with doses of 80\u2009mg per week, without recurrence of the bone and muscle signs. The second patient, 26\u2009years old, 78\u2009kg, developed pain and cervical stiffness with pyramidal tract irritation secondary to ossifications of the vertebral ligaments. This improved with a reduction of doses to 80\u2009mg\/week, which then, after 6\u2009months of follow\u2010up, enabled maintained improvement of the bone and muscle pain that was initially obtained. To our knowledge, these are the first reported cases of increased spinal ligamentous ossifications with neurological complications. Biological monitoring in adults does not seem to enable asfotase alfa doses to be adjusted. The levels of serum alkaline phosphatase (ALP) while on the recommended treatment of 1\u2009mg\/kg\/day are significantly supraphysiological (5000 to 20,000\u2009IU) and the assays of pyrophosphate and pyridoxal phosphate are not correlated with clinical efficacy. In both of our patients, the treatment with 80\u2009mg of asfotase alfa per week, which was proposed after the occurrence of spinal complications, seemed as effective, after a follow\u2010up of 18\u2009months and 6\u2009months, as the initial treatment for improving the bone and muscle signs, and could be provided as \u201cattack\u201d doses after healing of the pseudoarthroses. \u00a9 2021 American Society for Bone and Mineral Research \u00a9 2020 The Authors.\n                    <jats:italic>JBMR Plus<\/jats:italic>\n                    published by Wiley Periodicals LLC. on behalf of American Society for Bone and Mineral Research.\n                  <\/jats:p>","DOI":"10.1002\/jbm4.10449","type":"journal-article","created":{"date-parts":[[2020,12,10]],"date-time":"2020-12-10T15:28:44Z","timestamp":1607614124000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Hypophosphatasia: A Case of Two Patients With Spinal Cord Compression From Increase in Ligamentous Ossifications During Treatment"],"prefix":"10.1093","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2319-6686","authenticated-orcid":false,"given":"Michel","family":"Laroche","sequence":"first","affiliation":[{"name":"Centre de Rhumatologie du Centre Hospitalo\u2010Universitaire de Toulouse [Rheumatology Center of Toulouse University Hospital]  Toulouse France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Couture","sequence":"additional","affiliation":[{"name":"Centre de Rhumatologie du Centre Hospitalo\u2010Universitaire de Toulouse [Rheumatology Center of Toulouse University Hospital]  Toulouse France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Faruch","sequence":"additional","affiliation":[{"name":"Service de Radiologie du Centre Hospitalo\u2010Universitaire de Toulouse [Department of Radiology of Toulouse University Hospital]  Toulouse France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adeline","family":"Ruyssen\u2010Witrand","sequence":"additional","affiliation":[{"name":"Centre de Rhumatologie du Centre Hospitalo\u2010Universitaire de Toulouse [Rheumatology Center of Toulouse University Hospital]  Toulouse France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Val\u00e9rie","family":"Porquet\u2010Bordes","sequence":"additional","affiliation":[{"name":"Endocrinologie, Maladies Osseuses, H\u00f4pital des Enfants, Centre de R\u00e9f\u00e9rence des Maladies Rares du M\u00e9tabolisme du Calcium et Phosphate, European Reference Network on rare bone diseases, Centre Hospitalo\u2010Universitaire de Toulouse  Toulouse France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean Pierre","family":"Salles","sequence":"additional","affiliation":[{"name":"Endocrinologie, Maladies Osseuses, H\u00f4pital des Enfants, Centre de R\u00e9f\u00e9rence des Maladies Rares du M\u00e9tabolisme du Calcium et Phosphate, European Reference Network on rare bone diseases, Centre Hospitalo\u2010Universitaire de Toulouse  Toulouse France"},{"name":"INSERM UMR 1043 CNRS5825, Centre de Physiopathologie de Toulouse Purpan, Centre de Physiopathology Toulouse Purpan, Universit\u00e9 de Toulouse  Toulouse France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yannick","family":"Degboe","sequence":"additional","affiliation":[{"name":"Centre de Rhumatologie du Centre Hospitalo\u2010Universitaire de Toulouse [Rheumatology Center of Toulouse University Hospital]  Toulouse France"},{"name":"INSERM UMR 1043 CNRS5825, Centre de Physiopathologie de Toulouse Purpan, Centre de Physiopathology Toulouse Purpan, Universit\u00e9 de Toulouse  Toulouse 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