التفاصيل البيبلوغرافية
| العنوان: |
Amyotrophic Lateral Sclerosis Mimic Syndrome in a 24-Year-Old Man with Chiari 1 Malformation and Syringomyelia: A Clinical Case. |
| المؤلفون: |
Al-Zamil, Mustafa, Shnayder, Natalia 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; Apr2023, Vol. 12 Issue 8, p2932, 20p |
| مصطلحات موضوعية: |
AMYOTROPHIC lateral sclerosis, ARNOLD-Chiari deformity, SYRINGOMYELIA, NEUROLOGICAL disorders, MOVEMENT disorders, SPINAL canal |
| مستخلص: |
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. [ABSTRACT FROM AUTHOR] |
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