Amyotrophic Lateral Sclerosis

A motor point biopsy of deltoid muscle will be carried out at the time of inclusion using a standardized procedure, as routinely performed. Muscle samples will be removed from the deltoid muscle by open biopsy under local anaesthesia. The region containing NMJs will be determined by the small twitch provoked by the tip of the scalpel on the surface of the muscle fascicles.

Muscle biopsy

Pitié-Salpêtrière Hospital

Presymptomatic Neuromuscular Junction Defects and Compensatory Mechanisms in ALS

Presymptomatic Neuromuscular Junction Defects and Compensatory Mechanisms in Amyotrophic Lateral Sclerosis (ALS)

Quantification of innervation by confocal microscopy will be performed by classifying neuromuscular junctions observed in each biopsy specimen according to the relationship between the intrasynaptic axonal branches and the postsynaptic membrane in four categories: "normal", "denervated", "partially innervated", or "reinnervated"

Assistance Publique - Hôpitaux de Paris

Frontotemporal Lobar Degeneration

characterization

Groupe Hospitalier Pitié-Salpêtrière - Charles Foix

Predict to Prevent Frontotemporal Lobar Degeneration (FDT) and Amyotrophic Lateral Sclerosis (ALS)

Predict to Prevent Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

frontotemporal dementia

asthenia

frontotemporal dementia/asthenia

Presymptomatic Neuromuscular Junction Defects and Compensatory Mechanisms in ALS

Predict to Prevent Frontotemporal Lobar Degeneration (FDT) and Amyotrophic Lateral Sclerosis (ALS)

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