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Information about MED Cns
Health & Medicine

Published on May 31, 2014

Author: ooijianmin



Bifacial weakness • Guillaine barre syndrome (peripheral nerve) • Myasthenia gravis (neuromuscular junction) • Dystrophia myotonica (muscle) • Fascioscapulohumeral dystrophy (muscle)

• Adult onset • Autosomal dominant disorder • Characterized by progrssive weaness and degeneration of skeletal muscle that control movement Dystrophia myotonica

• History taking & PE – Facial features: 1. Frontal balding 2. Wasting of temporalis 3. Bilateral proptosis 4. Wasting of masseter 5. Elongated face 6. Bifacial weakness 7. Pouting of lips 8. Wasting of sternocleidomastoid-swan neck – Muscle • wasting • Weakness : site, onset, progression, severity/function affected (SOPS) • Myotonia : grip object, difficult to release/ hard to get up from seated position – Complications • Eyes : cataract • CVS : arrhythmia (heart block), hear failure • Respi : lung infection • Endocrine : DM • Hypogonadism : gynaecomastia, loss of axillary and pubic hair – Family history – RULE OUT GUILLAIN BARRE, MYASTHENIA GRAVIS, FASCIOSCAPULOHUMERAL DYSTROPHY

*myotonia – neuromuscular disorder where the relaxation of muscle is impaired. Repeated effort will be needed to relax the muscle *hypogonadism – reduced functional activity of the gonads, thus reduced the sex hormone biosynthesis *gynaecomastia is results of imbalance between estrogen: testosterone ratio, incr estrogen will stimulate breast glandular tissue hypertrophy.

PE (express pg 4) 1. SHAKE HAND-slow relaxation of muscle 2. General inspection- face features & muscle wasting 3. Hands – small muscle wasting – percussion of thenar eminence show abduction of thumb and slow relaxation – RADIAL PULSE – tone, power, reflex, sensory, proprioception, coordination 4. Eyes – cataract 5. Neck – wasting of sternocleidomastoid (swan neck) 6. CHEST – Axillary hair- loss dt hypogonadism – breast-gynaecomastia – CVS- irregular rhythm and intensity of s1 s2 (atrial fibrillation), gallop rhythm & basal lung crackles(heart failure) 7. End by doing fundoscopy (diabetic retinopathy), pubic hair (hypogonadism), blood glucose level

• IX- serum creatinine kinase, electromyography (EMG) • TX – There is no specific treatment to stop or reverse any form of MD. – Treatment may include physical therapy, respiratory therapy, speech therapy, orthopedic appliances used for support, and corrective orthopedic surgery. – Drug therapy includes corticosteroids to slow muscle degeneration – Anticonvulsants(phenytoin) to control seizures and some muscle activity, – immunosuppressants to delay some damage to dying muscle cells – antibiotics to fight respiratory infections. – Some individuals may benefit from occupational therapy and assistive technology. – Some patients may need assisted ventilation to treat respiratory muscle weakness – pacemaker for cardiac abnormalities.

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