Week 10 - CPEO/Myositis

Question 1

What is CPEO and its associated syndrome?

Answer 1

• Chronic progressive external ophthalmoplegia
• Mitochondrial disorder
• Associated with Kearns sayre syndrome

Question 2

What are the clinical features of CPEO, and its progression?

Answer 2

• Progressive symmetrical loss of motility
• Usually upgaze is the first to be affected
• Ptosis and Obicularis weakness
• Normal pupil and accommodation
• Diplopia is not commonly complained of as symmetrical and very slowly
• Final stages have virtually no eye movements, with secondary fibrosis

Question 3

What is Kearns Sayre syndrome and it’s signs?

Answer 3

• CPEO (in childhood)
• Fine pigmentary retinopathy
• Heart conduction block

Question 4

Differential diagnosis of CPEO?

Answer 4

• Myasthenia
• Graves
• Supranucler Gaze Palsy
• Multiple nerve Palsies

Question 5

Management of CPEO?

Answer 5

• Fundoscopy
• ECG
• Orthoptic assessment to include UFOF
• Ptosis Props/Fresnel

Question 6

What is ocular myositis?

Answer 6

• Inflammatory swelling of one or more of the Extraocular Muscles
• Pseudo tumour
• Patients presents with painful diplopia

Question 7

Signs and symptoms of Myositis?

Answer 7

• Acute pain
• Proptosis
• Diplopia
• Oedema - periorbital/chemosis
• Underlying Inflammatory disease/ Autoimmune disorder/sinusitis/cellulitis

Question 8

Diagnosis of myositis?

Answer 8

• Acute onset but can become chronic
• CT scan will show marked inflammation/swelling of one or more muscles, including the tendon
•Ensure this is not Rhabdomyosarcoma, a highly malignant tumour that presents in childhood.
Usually associated with acute proposis and strabsimus

Question 9

What is the comparison of graves vs myositis?

Answer 9

• Dull pain vs severe pain
• Bilateral/asymmetrical vs unilateral typically
• Gradual onset vs acute onset
• Lid retraction/lid lag vs None
• Abnormal TFT vs Normal TFT
• Swelling of more than one EOM, with tendon sparing vs swelling of one muscles WITH tendon involvement

Question 10

Treatment of myositis?

Answer 10

• Corticosteroids are highly effective
• Prisms as required
• Botox if patients

Question 11

What are the 2 types of nystagmus an