Motor neuron diseases and amyotrophic lateral Sclerosis

Sanders, Katherine A.; Rowland, Lewis P.; Murphy, Peregrine L.; Younger, David S.; Latov, N.; Sherman, William H.; Pesce, Michael A.; Lange, Dale J.

doi:10.1212/wnl.43.2.418

Cited by 35 publications

(9 citation statements)

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“…Younes-Chennoufi et al described most cases with significant anti-SGPG antibodies as having lower limb site of onset of the disease, although they did not discuss the implications of the findings.We did not find a correlation between the site of onset and the presence of anti-SGPG antibodies. IgM antibodies against gangliosides and SGPG were detected in ALS (Pestronk et al, 1988;Sanders et al, 1993;Younes-Chennoufi et al, 1995). In contrast to previous reports, our findings indicate that IgG antibodies were more common in our patients.…”

Section: Discussioncontrasting

confidence: 99%

Elevation of serum soluble E-selectin and antisulfoglucuronyl paragloboside antibodies in amyotrophic lateral sclerosis

2000

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Immunological abnormality is often found in amyotrophic lateral sclerosis (ALS). Antibodies to sulfoglucuronyl paragloboside (SGPG) were reported in ALS, although the pathogenetic significance of the antibodies is still unknown. We have already demonstrated that SGPG, a unique glycolipid, is present in both peripheral nerve and vascular endothelial cells. To investigate whether serum anti-SGPG antibodies would participate in activation and/or injury of endothelial cells in ALS, we examined serum anti-SGPG antibodies in association with serum soluble E-and P-selectins, which are markers of activated endothelial cells, in 25 patients with ALS and 14 age-matched patients with other neurological diseases (ONDs) using the microtiter-ELISA method. Seven out of 25 ALS patients had anti-SGPG antibodies. Levels of sE-selectin were significantly higher in patients with ALS (48.5 6 23.4 ng/ml) compared with ONDs (24.0 6 11.8 ng/ml) (P , 0.005). Four out of seven ALS patients with anti-SGPG antibodies had concomitantly high sE-selectin levels. The mean sE-selectin levels were higher in patients with anti-SGPG antibodies (61.9 6 25.2 ng/ml) than in those without anti-SGPG antibodies (43.3 6 21.1 ng/ml). Anti-SGPG antibodies may take part in the activation and/or injury of endothelial cells.The increased expression of E-selectin may be related to an immunological process in some ALS patients. Materials and methods PatientsWe studied 25 patients with ALS (16 men and nine women; mean age 6 standard deviation: 62.1 6 8.4 years) and 14 age-matched other neurological diseases (ONDs) patients without inflammatory disorders or motor neuron disorder (seven men and seven women; mean age,

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Section: Discussioncontrasting

confidence: 99%

Elevation of serum soluble E-selectin and antisulfoglucuronyl paragloboside antibodies in amyotrophic lateral sclerosis

2000

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“…The association between lower MND and anti‐GM1 IgM antibodies is well known, but the pathogenetic role of this finding is poorly understood ( Sanders et al, 1993 ; Tan et al, 1994 ; Van den Berg et al, 1997 ) . In this case, the morphologic characteristics of motor nerve biopsy were consistent with a diagnosis of chronic motor axonal neuropathy, probably immune‐mediated.…”

Section: Discussionmentioning

confidence: 99%

Chronic motor axonal neuropathy

Riva¹,

Gallia²,

Iannaccone³

et al. 2011

Journal of the Peripheral Nervous System

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The identification of a distinct subgroup of patients within the spectrum of lower motor neuron syndromes (LMNS) is crucial as some are potentially treatable. We describe the clinical and neuropathological characteristics of a patient presenting with a rapidly progressive LMNS associated with high titers of anti-GM1 antibodies, leading to respiratory failure within 10 months. Histopathological study of a biopsy of a obturator nerve motor branch demonstrated a predominantly axonal motor neuropathy, while electron microscopy analysis localized macrophages located within the periaxonal space. Immunohistochemistry demonstrated deposits of complement activation products (C3i) and immunoglobulins (IgM) on nerve fibers. The patient's clinical, immunological and pathological findings are consistent with a diagnosis of a chronic motor axonal neuropathy (CMAN), likely of immune-mediated origin.

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“…We did not find a correlation between the site of onset and the presence of anti‐SGPG antibodies. IgM antibodies against gangliosides and SGPG were detected in ALS (Pestronk et al ., 1988; Sanders et al ., 1993; Younes‐Chennoufi et al ., 1995). In contrast to previous reports, our findings indicate that IgG antibodies were more common in our patients.…”

Section: Discussionmentioning

confidence: 99%

Elevation of serum soluble E‐selectin and antisulfoglucuronyl paragloboside antibodies in amyotrophic lateral sclerosis

Ikeda

Kohriyama

Nakamura

2000

Euro J of Neurology

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Immunological abnormality is often found in amyotrophic lateral sclerosis (ALS). Antibodies to sulfoglucuronyl paragloboside (SGPG) were reported in ALS, although the pathogenetic significance of the antibodies is still unknown. We have already demonstrated that SGPG, a unique glycolipid, is present in both peripheral nerve and vascular endothelial cells. To investigate whether serum anti-SGPG antibodies would participate in activation and/or injury of endothelial cells in ALS, we examined serum anti-SGPG antibodies in association with serum soluble E- and P-selectins, which are markers of activated endothelial cells, in 25 patients with ALS and 14 age-matched patients with other neurological diseases (ONDs) using the microtiter-ELISA method. Seven out of 25 ALS patients had anti-SGPG antibodies. Levels of sE-selectin were significantly higher in patients with ALS (48.5 +/- 23.4 ng/ml) compared with ONDs (24.0 +/- 11.8 ng/ml) (P < 0.005). Four out of seven ALS patients with anti-SGPG antibodies had concomitantly high sE-selectin levels. The mean sE-selectin levels were higher in patients with anti-SGPG antibodies (61.9 +/- 25.2 ng/ml) than in those without anti-SGPG antibodies (43.3 +/- 21.1 ng/ml). Anti-SGPG antibodies may take part in the activation and/or injury of endothelial cells. The increased expression of E-selectin may be related to an immunological process in some ALS patients.

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Motor neuron diseases and amyotrophic lateral Sclerosis

Cited by 35 publications

References 0 publications

Elevation of serum soluble E-selectin and antisulfoglucuronyl paragloboside antibodies in amyotrophic lateral sclerosis

Elevation of serum soluble E-selectin and antisulfoglucuronyl paragloboside antibodies in amyotrophic lateral sclerosis

Chronic motor axonal neuropathy

Elevation of serum soluble E‐selectin and antisulfoglucuronyl paragloboside antibodies in amyotrophic lateral sclerosis

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