دورية أكاديمية
Amyotrophic Lateral Sclerosis Mimic Syndrome in a 24-Year-Old Man with Chiari 1 Malformation and Syringomyelia: A Clinical Case
العنوان: | Amyotrophic Lateral Sclerosis Mimic Syndrome in a 24-Year-Old Man with Chiari 1 Malformation and Syringomyelia: A Clinical Case |
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المؤلفون: | Al-Zamil M.Kh., Shnayder N.A., Davydova Tatiana K., Nasyrova Regina F., Trefilova Vera V., Narodova Ekaterina A., Petrova Marina M., Romanova Irina V., Chumakova Galina A. |
المصدر: | Journal of Clinical Medicine |
بيانات النشر: | MDPI |
المجموعة: | RUDN University Open Repository (RUOR) |
مصطلحات موضوعية: | amyotrophic lateral sclerosis, syringomyelia, Chiari 1 malformation, cerebrospinal fluid, related disorder, differential diagnosis, adult, ALS mimic syndrome |
الوصف: | 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’s 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. |
نوع الوثيقة: | article in journal/newspaper |
اللغة: | English |
العلاقة: | https://doi.org/10.3390/jcm12082932Test; https://repository.rudn.ru/records/article/record/94469Test/ |
الإتاحة: | https://doi.org/10.3390/jcm12082932Test https://repository.rudn.ru/records/article/record/94469Test/ |
رقم الانضمام: | edsbas.2BB4F40E |
قاعدة البيانات: | BASE |
الوصف غير متاح. |