Tolosa Hunt syndrome is a rare disorder caused by nonspecific
inflammation in the cavernous sinus/superior orbital fissure and/or orbital apex. It is clinically characterized by alternating remissions and exacerbations, and manifested as
diplopia associated with unilateral periorbital hemicranial
headache. The symptoms include
blepharoptosis, which is usually mild if present, bulbomotor
paresis involving the pupil, and loss of sensation in the area supplied by the first division of the trigeminal nerve.
Therapy for
Tolosa-Hunt syndrome are systemic
steroids. The course of disease in a 25-year-old man hospitalized for
painful ophthalmoplegia and
diplopia is presented. The history included severe
pain on rightward eye movement and parabulbarly on the right, considerable defect in the area supplied by the first division of the trigeminal nerve, right
hemicrania, and
diplopia on looking to the left, right, upward and downward that developed after four days. A month before, the patient was observed at neurology department for severe right
hemicrania. Current status included severe
pain parabulbarly on the right, discrete
proptosis with mild ptosis on the right eye, restricted right eye bulbar motoricity on looking nasally, nasally upward and downward, and loss of sensation in the area supplied by the first division of the trigeminal nerve. Pupilar motoricity was normal. Upon admission, neuroradiologic examination (orbit CT) and brain MR were performed, and
therapy with systemic
corticosteroids was initiated ex iuvantibus, in consultation with a neurologist. At 24 hours of
corticosteroid therapy, the
pain subsided, whereas
diplopia disappeared almost completely after 5 days, now being only occasionally recorded on looking to the left and upward.