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Thoracic spinal variant amyotrophic lateral sclerosis (T-ALS) is a rare subtype that affects only 3% of patients with amyotrophic lateral sclerosis (ALS)
This article aims to describe the typical characteristics of T-ALS, including clinical manifestations, distribution patterns, and prognosis
A retrospective chart analysis was performed on 59 T-ALS patients from the clinical database of 3549 ALS patients in the Department of Neurology of Ulm University Hospital (Ulm, Germany) from 2002 to 2018, including deaths in 2020 Record query
The control group included 517 non-thoracic amyotrophic lateral sclerosis (NT-ALS) patients from the epidemiological database of the ALS registry Swabia2 from 2010 to 2018, excluding primary lateral sclerosis and family/heredity The form control group included 517 non-thoracic amyotrophic lateral sclerosis (NT-ALS) patients from the epidemiological database of the ALS registry Swabia2 from 2010 to 2018, excluding primary lateral sclerosis and family/ Inherited forms This article was published in the Journal of Neurology, Neurosurgery & Psychiatry ( Journal of Neurology, Neurosurgery & Psychiatry ) This article was published in the Journal of Neurology, Neurosurgery & Psychiatry ( Journal of Neurology, Neurosurgery & Psychiatry )
The clinical parameters of the two cohorts were compared, including gender, age of onset, delay in diagnosis, survival (time from symptom onset to death), weight, use of non-invasive ventilation (NIV) and invasive ventilation (IV), and mode of transmission
The clinical parameters of the two cohorts were compared, including gender, age of onset, delay in diagnosis, survival (time from symptom onset to death), weight, use of non-invasive ventilation (NIV) and invasive ventilation (IV), and mode of transmission
From 2002 to 2018, of the 3549 ALS patients treated in the Department of Neurology at Ulm University Hospital (Ulm, Germany), 59 (1.
Statistics of clinical results
Based on the clinical presentation, we identified two different T-ALS subgroups, characterized by variations in the respiratory muscles (n=37) or paraspinal muscles (n=22)
According to the clinical manifestations, we identified two different T-ALS alkylene group, wherein the respiratory muscles (n = 37) or the paraspinal muscles (n = 22) variation based on clinical manifestations, we identified two different T- The ALS subgroup, which is characterized by variation in
respiratory muscles (n=37) or paraspinal muscles (n=22) within 3 months, showed no difference in weight loss between the two groups .
In most T-ALS patients (n=32, 54.
2%), the second area affected is the neck .
The lumbar area was followed by 25.
4% (n=15), and the medulla oblongata area was 20.
3% (n=12) .
The results show that T-ALS is rare in women.
Compared with NT-ALS patients, T-ALS female patients have an earlier age of onset .
Considering the rarity of thoracic spine disease, it is expected that the delay in diagnosis of T-ALS patients will be significantly prolonged .
The results show that T-ALS is rare in women.
Compared with NT-ALS patients, T-ALS female patients have an earlier age of onset
.
Considering the rarity of thoracic spine disease, it is expected that the delay in diagnosis of T-ALS patients will be significantly prolonged
.
The results show that T-ALS is rare in women.
Compared with NT-ALS patients, T-ALS female patients have an earlier age of onset
.
Considering the rarity of thoracic spine disease, it is expected that the delay in diagnosis of T-ALS patients will be significantly prolonged
.
In short, compared with NT-ALS, the main characteristics of T-ALS are male predominance, females with an earlier age of onset, weight loss, early reliance on mechanical ventilation, but a longer survival period
.
Kandler K , Witzel S , Eder K Kandler K Kandler Witzel S Witzel Eder K Eder , et al Phenotyping of the thoracic-onset variant of amyotrophic lateral sclerosis Journal of Neurology, Neurosurgery & Psychiatry Published Online First Published: 19 October 2021.
Online First: doi: 10.
1136/jnnp-2021-326712 doi:
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