Myotonic_Muscular_Dystrophy_Flashcards

1
Q

What is the main approach to managing muscle involvement in Myotonic Muscular Dystrophy?

A

Physiotherapy (strength and flexibility training), occupational therapy (specially designed utensils for hand weakness, wrist braces), orthopaedic (ankle–foot arthroses for foot-drop), and monitoring for deformities.

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2
Q

How is muscle pain managed in Myotonic Muscular Dystrophy?

A

Muscle pain is managed with NSAIDs and Gabapentin.

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3
Q

What medication is used for treating myotonia in Myotonic Muscular Dystrophy?

A

Mexiletine with careful supervision, previously treated with quinine or procainamide.

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4
Q

How are difficulties swallowing and dysarthria due to muscle weakness managed in Myotonic Muscular Dystrophy?

A

These are managed with Speech and Language Therapy (SALT).

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5
Q

What is the recommended management for cardiac disturbances in Myotonic Muscular Dystrophy?

A

Referral to cardiologists.

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6
Q

What interventions are recommended for respiratory function and sleep issues in Myotonic Muscular Dystrophy?

A

Noninvasive positive airway pressure ventilation (NIPPV) may be useful in correcting apnea, and infants with congenital muscular dystrophy may require continuous ventilatory support.

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7
Q

How are cataracts managed in Myotonic Muscular Dystrophy?

A

Cataracts are managed with surgery.

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8
Q

What additional support is recommended for families affected by Myotonic Muscular Dystrophy?

A

Genetic counselling for antenatal diagnosis and psychological support for parents and children.

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