Epidemiology of Amyotrophic Lateral Sclerosis

Goncharova, P. S.; Davidova, T. K.; Shnayder, N. А.; Novitsky, M. A.; Nasyrova, R. F.

doi:10.52667/2712-9179-2022-2-1-57-66

Cited by 3 publications

(2 citation statements)

References 27 publications

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“…The spinal cord cavities are filled with cerebrospinal fluid (CSF) or a fluid similar in composition to it [19]. Syr is a rare disease (ORPHA3280); the prevalence varies from 1.9 to 8.4 per 100,000 population [20,21]. The widespread use of MRI has increased the number of cases diagnosed with Syr [22][23][24].…”

Section: Introductionmentioning

confidence: 99%

Amyotrophic Lateral Sclerosis Mimic Syndrome in a 24-Year-Old Man with Chiari 1 Malformation and Syringomyelia: A Clinical Case

Al-Zamil

Shnayder

Davydova³

et al. 2023

JCM

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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.

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Section: Introductionmentioning

confidence: 99%

Amyotrophic Lateral Sclerosis Mimic Syndrome in a 24-Year-Old Man with Chiari 1 Malformation and Syringomyelia: A Clinical Case

Al-Zamil

Shnayder

Davydova³

et al. 2023

JCM

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“…The spinal cord cavities filled with cerebrospinal fluid (CSF) or a fluid similar in composition to it [19]. Syr classified as a neurological rare disease (ORPHA3280), with a prevalence of 1.9-8.4 per 100,000 population [20,21]. Widespread MRI use in medicine increase prevalence of Syr several times [22,23,24].…”

Section: Introductionmentioning

confidence: 99%

Phenocopy of Amyotrophic Lateral Sclerosis in Patients with Chiari 1 Malformation Associated Syringomyelia: Brief Literature Review

Al-Zamil,

Kulikova

2023

jour

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Arnold-Chiari malformation or Chiari malformation (CM1) is the name of a group of deformities of the posterior fossa and hindbrain (cerebellum, pons and medulla oblongata). The pathogenetic basis of this disease is associated with herniation of the cerebellar tonsils through the foramen magnum. 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. Syringomyelia (Syr), hydrocephalus, craniocervical instability, encephalocele, scoliosis, spina bifida and spinal dysraphism are the most common comorbidities that may present at the time of diagnosis or develop secondarily. Most attention has been paid to syringomyelia complicated by CM1 (CM1-related Syr). Formation of single or multiple fluid-filled cavities in the spinal cord and/or bulb as a result of pulse changes in intracranial pressure associated with disruption of normal cerebrospinal fluid circulation due to morphological abnormalities of the brain at the magnum level. This condition can be complicated by a rarer disease caused by the development of damage to the anterior horns of the spinal cord - amyotrophic sclerosis (ALS syndrome). In this brief literature review we are trying to demonstrate the mean pathogenic basis of amyotrophic lateral sclerosis in patients with chiari 1 malformation associated syringomyelia.

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From Bench to Bedside: Unveiling the Effects of Phloroglucinol as a Promising Neuroprotective Agent in the Battle Against Neurodegenerative Disorders

Bhuyan,

Ghose,

Biswas

et al. 2024

Curr Behav Neurosci Rep

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Epidemiology of Amyotrophic Lateral Sclerosis

Cited by 3 publications

References 27 publications

Amyotrophic Lateral Sclerosis Mimic Syndrome in a 24-Year-Old Man with Chiari 1 Malformation and Syringomyelia: A Clinical Case

Amyotrophic Lateral Sclerosis Mimic Syndrome in a 24-Year-Old Man with Chiari 1 Malformation and Syringomyelia: A Clinical Case

Phenocopy of Amyotrophic Lateral Sclerosis in Patients with Chiari 1 Malformation Associated Syringomyelia: Brief Literature Review

From Bench to Bedside: Unveiling the Effects of Phloroglucinol as a Promising Neuroprotective Agent in the Battle Against Neurodegenerative Disorders

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