{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T02:04:42Z","timestamp":1760148282822,"version":"build-2065373602"},"reference-count":56,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2023,4,18]],"date-time":"2023-04-18T00:00:00Z","timestamp":1681776000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Chiari 1 Malformation (CM1) is classically defined as a caudal displacement of the cerebellar tonsils through the foramen magnum into the spinal cord. Modern imaging techniques and experimental studies disclose a different etiology for the development of CM1, but the main etiology factor is a structural defect in the skull as a deformity or partial reduction, which push down the lower part of the brain and cause the cerebellum to compress into the spinal canal. CM1 is classified as a rare disease. CM1 can present with a wide variety of symptoms, also non-specific, with consequent controversies on diagnosis and surgical decision-making, particularly in asymptomatic or minimally symptomatic. Other disorders, such as syringomyelia (Syr), hydrocephalus, and craniocervical instability can be associated at the time of the diagnosis or appear secondarily. Therefore, CM1-related Syr is defined as a single or multiple fluid-filled cavities within the spinal cord and\/or the bulb. A rare CM1-related disorder is syndrome of lateral amyotrophic sclerosis (ALS mimic syndrome). We present a unique clinical case of ALS mimic syndrome in a young man with CM1 and a huge singular syringomyelic cyst with a length from segment C2 to Th12. At the same time, the clinical picture showed upper hypotonic-atrophic paraparesis in the absence of motor disorders in the lower extremities. Interestingly, this patient did not have a disorder of superficial and deep types of sensitivity. This made it difficult to diagnose CM1. For a long time, the patient\u2019s symptoms were regarded as a manifestation of ALS, as an independent neurological disease, and not as a related disorder of CM1. Surgical treatment for CM1 was not effective, but it allowed to stabilize the course of CM1-related ALS mimic syndrome over the next two years.<\/jats:p>","DOI":"10.3390\/jcm12082932","type":"journal-article","created":{"date-parts":[[2023,4,18]],"date-time":"2023-04-18T05:18:13Z","timestamp":1681795093000},"page":"2932","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Amyotrophic Lateral Sclerosis Mimic Syndrome in a 24-Year-Old Man with Chiari 1 Malformation and Syringomyelia: A Clinical Case"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3643-982X","authenticated-orcid":false,"given":"Mustafa","family":"Al-Zamil","sequence":"first","affiliation":[{"name":"Department of Physiotherapy, Faculty of Continuing Medical Education, Peoples\u2019 Friendship University of Russia, 117198 Moscow, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2840-837X","authenticated-orcid":false,"given":"Natalia A.","family":"Shnayder","sequence":"additional","affiliation":[{"name":"Institute of Personalized Psychiatry and Neurology, V.M. Bekhterev National Medical Research Centre for Psychiatry and Neurology, 192019 Saint Petersburg, Russia"},{"name":"Shared Core Facilities \u201cMolecular and Cell Technologies\u201d, V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, 660022 Krasnoyarsk, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatiana K.","family":"Davydova","sequence":"additional","affiliation":[{"name":"Department of Neurogenerative Disorders, Yakut Science Centre of Complex Medical Problems, 677000 Yakutsk, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1874-9434","authenticated-orcid":false,"given":"Regina F.","family":"Nasyrova","sequence":"additional","affiliation":[{"name":"Institute of Personalized Psychiatry and Neurology, V.M. Bekhterev National Medical Research Centre for Psychiatry and Neurology, 192019 Saint Petersburg, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vera V.","family":"Trefilova","sequence":"additional","affiliation":[{"name":"Hospital for War Veterans, 193079 Saint Petersburg, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6184-9206","authenticated-orcid":false,"given":"Ekaterina A.","family":"Narodova","sequence":"additional","affiliation":[{"name":"Shared Core Facilities \u201cMolecular and Cell Technologies\u201d, V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, 660022 Krasnoyarsk, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marina M.","family":"Petrova","sequence":"additional","affiliation":[{"name":"Shared Core Facilities \u201cMolecular and Cell Technologies\u201d, V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, 660022 Krasnoyarsk, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Irina V.","family":"Romanova","sequence":"additional","affiliation":[{"name":"Shared Core Facilities \u201cMolecular and Cell Technologies\u201d, V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, 660022 Krasnoyarsk, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Galina A.","family":"Chumakova","sequence":"additional","affiliation":[{"name":"Department of Therapy and General Medical Practice with a Course of Postgraduate Professional Education, Altai State Medical University, 656038 Barnaul, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,4,18]]},"reference":[{"key":"ref_1","unstructured":"Kular, S., and Cascella, M. (2022). StatPearls, StatPearls Publishing."},{"key":"ref_2","unstructured":"Hidalgo, J.A., Tork, C.A., and Varacallo, M. 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