A 17-year-old female with history of obesity, pre-diabetes, and marijuana use presented for care in the setting of progressive left-sided ophthalmoparesis and worsening headache. Prior to admission, she had a 1-month history of left frontotemporal and retro-orbital headaches accompanied by photophobia, nausea, vomiting, and localized numbness over the left temple. The headaches often woke her up from sleep, and while previously intermittent, they had been constant for the 3 weeks prior to presentation. Over the preceding 2 weeks, the patient developed persistent, binocular diplopia, which eventually progressed to complete ophthalmoplegia. She also reported 1 week of intermittent left-sided mandibular pain and finally developed sensory changes over the maxillary (V2) division of the left trigeminal nerve. On examination, she was found to have CN III and VI palsies despite an unremarkable head computed tomography (CT). The patient was transferred to our hospital for inpatient evaluation and management.
At admission, she was afebrile, with a blood pressure of 156/83 mmHg; vital signs were otherwise normal for age. She was mildly ill-appearing and obese, weighing 112 kg. Pupils were 5 mm and briskly reactive to direct light, with sluggish restriction of the left pupil to indirect light. Funduscopic examination revealed sharp optic margins without pallor. Extraocular movements were markedly abnormal in the left eye with near-complete ophthalmoplegia and minimal upward and downward gaze. She had marked ptosis of the left eye, most consistent with severe oculomotor and abducens nerve palsies. Visual fields were full bilaterally. No facial asymmetry was noted. Trigeminal sensory examination revealed left-sided hyperesthesia in the V1 and V2 distributions.
Following consultation with Pediatric Neurology and Ophthalmology, the patient underwent an extensive workup revealing a normal complete blood count (CBC) and comprehensive metabolic panel (CMP) (except for glucose of 170), HbA1C of 6.1, angiotensin-converting enzyme (ACE) of 14 U/L (adult range, 16-85 U/L), negative serum autoimmune studies (antinuclear antibody (ANA), cyclic citrullinated peptide (CCP), anti-SSA/Ro (Sjogren's syndrome-related antigen A) Ab, anti-SSB/Ro (Sjogren's syndrome-related antigen B) Ab, Smith antibody (Sm Ab), ribonucleoprotein antibody (RNP Ab), topoisomerase I antibody (Scl 70 Ab), topoisomerase I antibody (Jo1 Ab), rheumatoid factor (RF), myeloperoxidase antibody (MPO Ab), proteinase 3 Ab), normal lactate dehydrogenase (LDH) and uric acid, and negative serum electrophoresis for monoclonal proteins. Infectious workup was negative for Borrelia burgdorferi, syphilis IgG Ab, CSF-VDRL (Venereal Disease Research Laboratory), and QFT-TB (QuantiFERON-TB) Gold. Bacterial, fungal, and mycobacterial cultures and acid-fast smear were negative. A lumbar puncture demonstrated normal CSF studies other than lymphocytosis (92%). CSF ACE was negative, and IgG, albumin, and flow cytometry were normal.
MRI of the brain and orbits demonstrated an extra-axial enhancing, ill-defined lesion centered in the left cavernous sinus (CS), including involvement of Meckel's cave, superior orbital fissure, orbital apex, foramen rotundum, foramen ovale, and middle cranial fossa (Figure 1). Mild narrowing of the patent right cavernous internal carotid artery was also described. The lesion was noted to approach but not involve the optic nerve.
Findings were consistent with THS and the patient was initiated on high-dose oral steroids. She demonstrated improvement in severity of headache within 12 hours of steroid initiation and complete resolution of headache by 24 hours. CN deficits remained unchanged at discharge (24 hours after initiation of steroids).
One month following discharge, the patient described resolution of headache and improved extraocular movements and diplopia. Repeat imaging at this time noted a "slight decrease in the size of the lesion." Unfortunately, the patient discontinued her 10-week steroid course after approximately 1 month. Shortly after discontinuing treatment, she began to experience new-onset, left-sided facial droop, consistent with a CN VII palsy, as well as recurrence of ptosis, diplopia, ophthalmoplegia, and hyperalgesia in V2. Follow-up imaging revealed mildly expanded asymmetric enhancement in the left cavernous sinus and Meckel's cave with extension along V3 through foramen ovale and V2 through foramen rotundum to the pterygopalatine fossa. New enhancement visualized in the distal left internal auditory canal, extending along the facial nerve inferiorly to the visualized upper parotid gland.
High-dose steroids were initiated once more. The patient was followed by Pediatric Neurology at 1 and 2 years since completion of treatment and noted to have complete resolution of symptoms.