MED Cns

CNS

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 balding2. Wasting of temporalis3. Bilateral proptosis4. Wasting of masseter5. Elongated face6. Bifacial weakness7. Pouting of lips8. 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 muscle2. General inspection- face features & muscle wasting3. 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 – cataract5. 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.