Week 10 - CPEO/Myositis Flashcards
What is CPEO and its associated syndrome?
• Chronic progressive external ophthalmoplegia
• Mitochondrial disorder
• Associated with Kearns sayre syndrome
What are the clinical features of CPEO, and its progression?
• 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
What is Kearns Sayre syndrome and it’s signs?
• CPEO (in childhood)
• Fine pigmentary retinopathy
• Heart conduction block
Differential diagnosis of CPEO?
• Myasthenia
• Graves
• Supranucler Gaze Palsy
• Multiple nerve Palsies
Management of CPEO?
• Fundoscopy
• ECG
• Orthoptic assessment to include UFOF
• Ptosis Props/Fresnel
What is ocular myositis?
• Inflammatory swelling of one or more of the Extraocular Muscles
• Pseudo tumour
• Patients presents with painful diplopia
Signs and symptoms of Myositis?
• Acute pain
• Proptosis
• Diplopia
• Oedema - periorbital/chemosis
• Underlying Inflammatory disease/ Autoimmune disorder/sinusitis/cellulitis
Diagnosis of myositis?
• 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
What is the comparison of graves vs myositis?
• 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
Treatment of myositis?
• Corticosteroids are highly effective
• Prisms as required
• Botox if patients
What are the 2 types of nystagmus an
