I.
II.
III.
IV.
Case History
a. Patient Demographics: 52 y.o. Caucasian female
b. Chief Complaint: Patient presents to ER with c/o severe, aching, constant eye
pain (7/10) OS that radiates to the V1 area with associated ptosis, photophobia,
temporal visual field loss and cranial nerve VI palsy OS.
c. OHx: simple hyperopia / presbyopia OU; MHx: adrenal insufficiency, allergic
rhinitis, anxiety disorder, chronic thyroiditis, hypothyroidism, h/o thyroid nodule,
s/p rt lobectomy, depression, GERD, lumbago, torticollis, s/p botox, venous
insufficiency, s/p appendectomy, s/p cervical vertebral fusion & laminectomy,
s/p TAH-BSO, s/p tonsillectomy.
d. Medications: calcium, hydrocortisone, diazepam, lidoderm 5% patch, morphine,
MVI, synthroid.
e. Other salient information: The patient reported no history of alcohol or
recreational substance abuse, but admitted to nicotine dependence. She was
employed as a county clerk. She was oriented to time, person, & place; her
mood was appropriate, but she appeared distressed.
Pertinent Findings
a. Clinical: Best corrected distance visual acuity at presentation OD: 20/20-, OS:
20/25; pupils 4/6, ¾ RRL, no APD; EOMS: FROM OD, abduction deficit OS; CF:
FTFC OD, constricted temporal hemifield OS; CT: 4∆ constant Lt ET; Ishihara CV:
12/12 OD, OS; MRD1: OD: 1 mm, OS: 0 mm, MRD2: OD: 4 mm, OS: 2 mm; slit
lamp: adnexa: +hydrosis OD, OS, lids/lashes: ptosis & reverse ptosis OS,
conjunctiva: trace diffuse injection OD, OS, cornea: clear OD, OS, iris: flat / blue
OD, OS, A/C: narrow & quiet OD, OS; tonometry: 12 mmHg OD, OS; DFE: lens: 1+
NS OD, OS, optic nerve 0.60 healthy rims, OD, OS; unremarkable macula,
vitreous, vessels, & periphery OD, OS.
b. Physical: BP: 102/64, Temperature 97◦ F, CN exam: Lt V1 hypoesthesia
c. Laboratory studies: CBC w/ diff, CMP, toxic screen, ESR, CRP, ANA w/ reflex titer,
TFT, RPR w/ MHA-TP reflex, ACE
d. Radiology studies: CXR, CT head w/o contrast, CTA head & neck, MRI brain &
orbits w/ & w/o contrast w/ attention to the cavernous sinus using 1.5 mm cuts,
MRV.
e. Others: UA, EKG, Humphrey visual fields, gonioscopy, lumbar puncture w/
opening pressure, CSF analysis & cytology
Differential diagnosis
a. Primary / leading: Tolosa-Hunt Syndrome
b. Others: Tumor / mass, meningitis, neurosyphilis, SLE, leukemia / lymphoma,
paraneoplastic disease, Raeder paratrigeminal neuralgia, CNS vasculitis,
neurosarcoid, tuberculosis, carotid artery aneurysm, carotid artery dissection,
carotid-cavernous fistula, cavernous sinus thrombosis, Wegener granulomatosis,
polyarteritis nodosa, multiple sclerosis, and CVA.
Diagnosis & Discussion
V.
a. Elaborate on the condition: Tolosa-Hunt Syndrome is a painful ophthalmoplegia
caused by nonspecific inflammation of the cavernous sinus or superior orbital
fissure. It is a rare condition that is considered a diagnosis of exclusion.
b. Expound on unique features: In this case, all testing including neuroimaging
studies were normal. Upon initiation of oral prednisone, the expected exquisite
response within 72 hours was not achieved. While mild improvement occurred,
the patient began to experience painful attacks with associated rhinorrhea,
unilateral ptosis, lacrimation, and conjunctival hyperemia OS leading to the
diagnosis of Raeder Paratrigeminal Neuralgia. Of note, it is extremely rare that
cranial nerve II is involved as manifested in this case with transient visual field
loss and mild blur OS. After the initial attack, the patient later developed
hemicrania continua; there is only one report in the literature of this occurrence.
Treatment, management
a. Treatment & response to treatment: This patient was co-managed with a
neurologist for treatment. She experienced an adverse reaction to indomethacin
but responded well to propranolol. She made a full ocular recovery; however,
her headache pain later transitioned into hemicrania continua. Her pain is being
maintained with 0.5% bupivacaine injections of the left occipital and supraorbital
nerves.
b. Research: There is one report published in 2008 in which benign Raeder
Syndrome evolved into hemicrania continua.
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VI.
Conclusion
a. Clinical pearls: When the preliminary diagnosis and treatment fail to resolve
signs and symptoms, this should alert the examiner to search for an alternative
diagnosis. The listener should gain knowledge of the work-up of a painful
ophthalmoplegia as well as greater understanding of the trigeminal autonomic
cephalgias.