Increased iron level in motor cortex of amyotrophic lateral sclerosis patients: An in vivo MR study

Abstract

Amyotrophic lateral sclerosis (ALS) is one of the most common neurodegenerative disorders, but no definite mechanism has been defined on the loss of motor neurons in ALS and currently no therapy can block its progression. Many lines of evidence indicate that there is a disorder of iron homeostasis in ALS, and thus we sought to test the iron level in ALS patients by susceptibility weighted imaging (SWI). Sixteen ALS patients and 16 healthy persons underwent brain scans using SWI with a 3T Siemens MR scanner. The red nucleus, substantia nigra, globus pallidus, putamen, the head of caudate nucleus, and motor cortex were measured in the filtered phase images and analysed for their SWI phase values as relative marker for iron content. We found that phase shift values were significantly higher in the motor cortex of ALS patients by SWI, indicating increased iron level in this area. In contrast, we found that there were no differences of phase shift values between ALS patients and healthy controls in the other nuclei including the red nucleus, substantia nigra, globus pallidus, putamen and the head of the caudate nucleus. Furthermore, we found that there were no relationships between SWI signal and some clinical features of ALS. In conclusion, these results demonstrate that iron level increases in the motor cortex of ALS and that SWI is a reliable method to test iron in the brain.

Serum ferritin is a candidate biomarker of disease aggravation in amyotrophic lateral sclerosis

“…By contrast, the Caucasian patients assessed previously lived in developed countries (26), and likely had greater access to treatments and adequate nutrition compared with patients in the present study, which may explain the significant increase in serum ferritin reported (26). Our previous study indicated that iron levels are increased in the motor cortex of patients with ALS by using susceptibility weighted imaging (48). The majority of the patients with ALS in the present study also participated in our aforementioned previous study.…”

The value of magnetic resonance imaging as a biomarker for amyotrophic lateral sclerosis: a systematic review

“…Later studies found a correlation being T2* shortening and iron deposition in the microglia of the motor cortex (according to a post-mortem examination) [7, 106, 108]. Iron overload in the motor cortex was also described in SWI studies [109] and qualitative studies [100]; in the later study, there were no differences between ALS patients and controls in terms of T2 or T2*. A few quantitative studies have looked at white matter or deep grey matter [109, 110].…”

Effects of epigallocatechin-3-gallate on iron metabolism in spinal cord motor neurons

“…Langkammer et al (6) identified increased iron deposition in localized regions along the corticospinal tract in ALS patients (6). Corroborating these findings, it was previously reported that phase shift values were significantly higher in the motor cortex of patients with ALS using susceptibility weighted imaging, indicating increased iron levels in this area (7). Additionally, pathological studies have demonstrated increased iron accumulation in the motor cortex of patients with ALS (8).…”