Dr. Liz Househam Consultant Neurologist Derriford Hospital
Dr. Liz Househam Consultant Neurologist Derriford Hospital
Dr. Liz Househam Consultant Neurologist Derriford Hospital
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<strong>Dr</strong>. <strong>Liz</strong> <strong>Househam</strong><br />
<strong>Consultant</strong> <strong>Neurologist</strong><br />
<strong>Derriford</strong> <strong>Hospital</strong>
An overview of:<br />
� How muscle disease affects breathing<br />
� Which diseases<br />
� What do I assess in my muscle clinic
How?<br />
� Weakness of breathing muscles.<br />
� Difference between lung disease and ventilatory<br />
problems (See later)<br />
�� Kyphosis and posture<br />
� Deconditioning<br />
� Obstructive sleep apneoa
Which muscle diseases?<br />
� As a consequence of global progression<br />
� Disease specific involvement
Global progression<br />
� Duchenne<br />
� Becker<br />
� SMA<br />
� Myasthenia Gravis<br />
� Gullain Barre Syndrome (GBS)<br />
� Chronic inflammatory Demyelinating polyneuropathy<br />
(CIDP)
Localised muscle involvement<br />
� Myotonic Dystrophy<br />
� LGMD 2I<br />
� Emery <strong>Dr</strong>eifuss MD<br />
� SMARD<br />
� Nemeline myopathy<br />
� Carnitine palmatoyl transferase deficiency<br />
� Acid maltase deficiency (Pompe’s disease)
How do I assess for a problem<br />
� History<br />
� Examination<br />
� Tests
History<br />
� Disturbed sleep, nightmares, restless<br />
� Waking with groggy feeling or frank headache<br />
� Usually improves during morning<br />
�� Daytime sleepiness.<br />
� Fatigue<br />
� Fearfullness of going to sleep
Examination<br />
� General progression in weakness<br />
� Postural problems- kyphosis<br />
� Beevor’s sign
Tests<br />
� Lying and standing/sitting FVC
Over to the respiratory team!
Any questions?
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