Microvascular disturbance with decreased pericyte coverage is prominent in the ventral horn of patients with amyotrophic lateral sclerosis.
In amyotrophic lateral sclerosis (ALS) there is emerging evidence for vasculature disturbance. The aim of this study was to investigate the area of predominant vasculature disturbance in ALS. We used immunohistochemistry to quantitatively evaluate the microvascular density (MVD) and pericyte coverage (PC) in the lumbar spinal cord of 25 ALS patients and six controls. In controls, MVD was almost equal in the ventral horn (VH) and dorsal horn (DH). In the VH of ALS, MVD was significantly increased, and PC was significantly decreased compared with the DH in ALS and the VH in controls (p < 0.001), possibly reflecting that PC is an essential requirement for the vasculature in the VH. We then found a significant relationship between the severity of aberrant angiogenesis and the use of artificial respiratory support (ARS). In conclusion, vasculature disturbance is observed in all ALS patients including patients with ARS. Therefore, breakdown of the blood-spinal cord barrier due to aberrant angiogenesis with decreased PC may be responsible for the predominant neuronal death in the VH in ALS.
a Department of Neurology , National Hospital Organization, Toneyama Hospital , Toneyama , Osaka , Japan.
Misaki
M
Yamadera
a Department of Neurology , National Hospital Organization, Toneyama Hospital , Toneyama , Osaka , Japan.
Harutoshi
H
Fujimura
a Department of Neurology , National Hospital Organization, Toneyama Hospital , Toneyama , Osaka , Japan.
Kimiko
K
Inoue
a Department of Neurology , National Hospital Organization, Toneyama Hospital , Toneyama , Osaka , Japan.
Keiko
K
Toyooka
a Department of Neurology , National Hospital Organization, Toneyama Hospital , Toneyama , Osaka , Japan.
Chiaki
C
Mori
b Department of Pathology , National Hospital Organization, Toneyama Hospital , Toneyama , Osaka , Japan.
Hiroshi
H
Hirano
a Department of Neurology , National Hospital Organization, Toneyama Hospital , Toneyama , Osaka , Japan.
Saburo
S
Sakoda
D000199:Actins / Q000378:metabolism; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications*; D018952:Antigens, CD34 / Q000378:metabolism; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009389:Neovascularization, Pathologic / Q000209:etiology; D020286:Pericytes / Q000473:pathology*; D013116:Spinal Cord / Q000098:blood supply / Q000378:metabolism / Q000473:pathology*; D018709:Statistics, Nonparametric; D057772:Vascular System Injuries / Q000209:etiology*
C541116:ACTA2 protein, human; D000199:Actins; D018952:Antigens, CD34
Amyotrophic lateral sclerosis; angiogenesis; artificial respiratory support; microvascular density; pericyte