What is a typical early hallmark of LAMA2-related congenital muscular dystrophy?

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Multiple Choice

What is a typical early hallmark of LAMA2-related congenital muscular dystrophy?

Explanation:
LAMA2-related congenital muscular dystrophy stems from merosin (laminin-α2) deficiency in the muscle basement membrane, causing weakened muscles from birth. The most characteristic early sign is generalized hypotonia in infancy—the baby feels floppy and has poor spontaneous movement and head control. This low muscle tone reflects the fundamental muscle weakness rather than increased neural drive. Signs like hypertonia, hyperreflexia, or early spasticity suggest upper motor neuron involvement and tend to appear later or in other conditions, not as the initial hallmark of this disorder. So, hypotonia in infancy is the best clue to this condition’s early presentation.

LAMA2-related congenital muscular dystrophy stems from merosin (laminin-α2) deficiency in the muscle basement membrane, causing weakened muscles from birth. The most characteristic early sign is generalized hypotonia in infancy—the baby feels floppy and has poor spontaneous movement and head control. This low muscle tone reflects the fundamental muscle weakness rather than increased neural drive. Signs like hypertonia, hyperreflexia, or early spasticity suggest upper motor neuron involvement and tend to appear later or in other conditions, not as the initial hallmark of this disorder. So, hypotonia in infancy is the best clue to this condition’s early presentation.

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