Disease Information for Tolosa-Hunt syndrome

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Clinical Manifestations
Signs & Symptoms
Face Pain
Ipsilateral absent corneal reflex/contralateral intact
Supraorbital hypesthesia/numbness, unilateral
Trigeminal nerve distribution pain
Vertigo
Arthralgias Polyarthralgias
Joint Pains
Joint pains Arthralgias in Elderly
Chronic Fatigue
Fatigue
Fatigue Tiredness Exhaustion
Absent corneal reflex/unilateral
Difficulty Focusing Eyes
Dimming vision/acute
Diplopia Double vision
Double Image Vision Confirmed
Eye Pain, Unilateral
Eye Pains, Bilateral
Eye symptoms/signs
Ptosis, unilateral (blepharoptosis)
Pupillary reaction/sluggish
Squint/sign
Vision Loss
Clinical Presentation & Variations
Presentation/Dramatic response corticosteroid
Presentation/Steroid responsive disorder Sensitive
Disease Progression
Course/Chronic disease crisis/flare/attacks
Course/Chronic disorder
Course/Chronic only
Course/Episodes last 1 to 3 weeks/remit recur months later
Course/Episodic clusters of attacks
Onset/adult
Onset/fifth decade
Demographics & Risk Factors
Sex & Age Groups
Population/Adult Aged Only
Population/Adult/all
Laboratory Tests
Abnormal Lab Findings - Increased
Highly sensitive CRP (LAB)
Sed Rate/ESR/Sedimentation rate (Lab)
Diagnostic Test Results
Pathology
PATH/Granulomatous vasculitis
PATH/Granulomatous tissue/specimen
CT Scan
MRI/MRA Carotid irregular narrowing/cavernous sinus area
Associated Diseases & Rule outs
Rule Outs
Ophthalmic migraine
Palsy, trochlear nerve/IV th cranial nerve
Trigeminal neuralgia
Associated Disease & Complications
Cavernous sinus thrombosis
Granulomatous Vasculitis
Ophthalmoplegia
Ophthalmoplegia/progressive/ext/chronic
Orbital apex syndrome
Palsy, sixth nerve
Palsy, third nerve
Palsy, trochlear nerve/IV th cranial nerve
Sixth nerve palsy
Supranuclear ophthalmoplegia
Tolosa-Hunt syndrome
Trigeminal neuralgia
Visual acuity loss
Disease Mechanism & Classification
Class
CLASS/Ophthalmology Specialty Population
CLASS/Primary organ/system disorder (ex)
CLASS/Ophthalmology disorders
CLASS/Eye involvement/disorder (ex)
CLASS/Eyes/accessory (category)
CLASS/Orbit involvement/disorder (ex)
Pathophysiology
Pathophysiology/Carotid artery/cavernous sinus
Pathophysiology/Granulomatous inflam cavernous sinus
Pathophysiology/Regional vasculitis/Arteritis
Pathophysiology/Granulomatous arteritis
Pathophysiology/Unilateral effects
Pathophysiology/Decreased eye sympathetic enervation
Pathophysiology/Eye movement problems
Pathophysiology/Superior orbital fissure/lesion/involved
Process
PROCESS/Eponymic (category)
PROCESS/Idiopathic/unclassified/unknown (category)
PROCESS/INCIDENCE/Esoteric disease (example)
PROCESS/Inflammatory/Granulomatous disease (ex)
PROCESS/Inflammatory/Granulomatous disorder (category)
PROCESS/Pathophysiology/organ-system (category)
PROCESS/Granulomatous Disease
PROCESS/Orbital inflammatory disease
Synonyms
Synonym
Tolosa Hunt Syndrome, Tolosa Hunt syndrome (disorder), Synonym/Cavernous Sinus granulomatosis, Synonym/Chronic cavernositis, Synonym/Ophthalmoplegia Dolorosa, Synonym/Ophthalmoplegia painful recurrent, Synonym/Orbital pain/Apicitis, Synonym/Painful Ophthalmoplegia, Synonym/Recurrent Algetic Ophthalmoplegia, Synonym/Recurrent ophthalmoplegia syndrome
Treatment
Drug Therapy - Indication
RX/Corticosteroid (Cortisone)
RX/Prednisone (Deltasone)
Definition

An idiopathic syndrome characterized by the formation of granulation tissue in the anterior cavernous sinus or superior orbital fissure, producing a painful ophthalmoplegia;--------(Adams et al, Principles of Neurology, 6th ed, p271)-------------Tolosa-Hunt syndrome (William Edward Hunt); Also known as: Tolosa-Hunt ophthalmoplegia; Cavernous sinus syndrome, cavernous sinus granulomatosis, ophthalmoplegia dolorosa, ophthalmoplegia pain, orbital apicitis, orbital pain, painful ophthalmoplegia, recurrent algetic ophthalmoplegia, recurrent ophthalmoplegia; Description: A syndrome characterized by recurrent, sharp unilateral retro-orbital pain with extraocular palsies, usually involving the third, fourth, fifth, and sixth cranial nerves; Other features include proptosis and sensory loss over the forehead, sluggish pupil reaction to light, and diminished corneal sensitivity with blurred vision up to complete blindness; It is attributed to a granulomatous infiltration or an invasive tumour at the apex of the orbit or in the region of the cavernous sinus; Males and females equally affected; The disease usually lasts several months to several years; Onset usually in the fifth decade------------[whonamedit-com website 2005]-------------Tolosa-Hunt Syndrome is a painful unilateral ophthalmoplegia caused by a granulomatous infiltration of nerves and muscles in the region of the superior orbital fissue; It is accompanied by partial or complete lesions of the third, fourth, fifth - ophthalmic division - and sixth cranial nerves; The ESR is usually raised; Treatment with steroids eg prednisolone is usually [quickly] effective

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External Links Related to Tolosa-Hunt syndrome
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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