Diagnostic approach to unilateral ophthalmoplegia with or without visual impairment. Addressing the cavernous sinus and orbital apex syndromes.
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Added: Dec 01, 2019
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Opthalmoplegia +/- II Yasser Alzainy Neurology Resident AL-AZHAR UNIVERSITY Faculty of Medicine Neurology Department
Clinical Approach What is the presentation? History Examination Binocular diplopia +/- deminution of vision III, IV, VI, V1 +/- II +/- V2
Clinical Approach Where is the Lesion? (diplopia) Supranuclear Nuclear / Internuclear Infranuclear Neuronal Neuromuscular junction Orbital pathology What is the Lesion? History Onset, course, duration Asccoiated symptoms Investigation Lab Imaging
Introduction
Differential Cavernous sinus syndrome III, IV, VI, V1 V2 +/- II Orbital Apex syndrome III, IV, VI, V1 II
Causes Cavernous sinus syndrome Cavernous sinus thrombosis Caroti d -cavernous fistula / aneurysm Pituitary disorders Pituitary adenoma Craniopharyngioma Pituitary Apoplexy Orbital Apex syndrome Orbital infection Orbital Neoplasm (metastases, lymphoma) Thyroid eye disea s e Orbital pseudo tuomor / Idiopathic orbital myo s itis Tolosa Hunst syndrome + Ocular myesthenia + Miller Fischer syndrome
General Work-up Routine Lab. Brain & orbital MRI . S ystemic search for malignancy . M yasthenia & thyroid testing: AChR-Ab, TSH, T4, thyroid-stimulating Ab, antithyroid Abs. some selected cases: LP + CSF cytology
Cavernous sinus thrombosis Suggestive Findings sinusitis ; facial, dental or orbital infection severe retrobulbar pain, isolated VI, ptosis: early sx progress to become bilateral ocular bruits papilloedema and retinal hemorrhages > deminution of vision Work-Up MRI brain and Orbit Coagulopathy profile (aseptic CST) Leukocytosis in CBC
Caroti d -cavernous fistula / Aneurysm Suggestive Findings throbbing retro-orbital pain orbital bruits severe conjunctival injection pulsating exophtahlmos Work-Up Angiography
Pituitary disorders (pituitary adenoma, craniopharyngioma) Suggestive Findings endocrine dysfunction bitemporal hemanopia earlier than ophthalmoplegia monocular deminution of vision may occur personlaity changes / seizures (frontal lobe) Work-Up MRI brain + C hormonal work-up
Pituitary adenoma
Craniopharyngioma
Orbital infection Suggestive Findings similar to CST prominent optic affection purulent discharge Work-Up MRI braind and orbit exclude CST CBC Blood culture
Orbiral Neoblasm Suggestive Findings prominent proptosis early optic affection Work-Up MRI brand and orbit + C Metastatic work-up
Thyroid eye diseae Suggestive Findings associated hyper > hypo- thyroidism may precede / co-exist / follow Work-Up MRI brain and Orbit regular enlargment of extraocular muscles with tendon sparing
Orbital pseudo tuomor ( idiopathic inflammatory orbital Dz) Suggestive Findings Dx of exclusion almost strictly unilateral associated other autoimmune / inflammatory condition dramatic response to steroids Work-Up MRI brain and orbit + C enlargement of extraocular muscle (one or more) with tendon involvement avid enhancement
Tolosa Hunst syndrome Suggestive Findings Diagnosis of exclusion duration: days to weeks II involvement in 25% Highly responsive to steroids Work-Up MRI brain and orbit +C enhancing lesion Biopsy