A rare case of TOLOSA HUNT SYNDROME

1. An Interesting Case
Dr. Panner.A Unit
Presenter: Dr. Ramesh Babu.M

2. Brief History
• A 53yrs old female from Westbengal, Diabetic -
10yrs
• C/o Headache - Lt. periorbital - 4 months
• Parasthesia over left frontal area
• H/o loss of Weight ~5kg in last 4 months
• Evening rise of temparature - (99-100○f) on &off

3. • Headache U/L Lt. periorbital spreads to frontal &
rest of the cranium, started on 10/2/2017 at 9.30pm
at bedtime which was sudden in onset very severe
unbearable, throbbing type, continuous lasts for 2-3
days and episodic in nature, not subsiding with
painkillers, no pain with eye movements.
• No H/O nausea, vomitings, giddiness, double vision,
LOC, seizures, sweating, lacrimation, flushing,
redness of the face and the eye, photophobia,
phonophobia.
• No h/o similar episodes in the past

4. • with the above complaints approached to local
physician(20/2/17) - treated with NSAIDS and antidepressants,
asked for MRI- showed left 1cm mass lesion at the base of the
skull and suggested to see a Neurosurgeon.
• He referred to Neurologist INS,kolkata (1/3/17)- they asked
complete tuberculosis work - all reports against T.B except
mantoux test - 18mm and started on ATT and tapering dose of
steroids over 1 month (8/3/17), referred to NIMHANS for CSF
evaluation.
• In NIMHANS (15/3/17)- LP - dry tap and suggested to continue
on ATT. Patient couldn't tolerate ATT, hence she stopped ATT.

5. • On 20/3/17 in INS they repeated CSF analysis
which was normal.
• On 31/3/17 she was started on antibiotic course
(T. Septran and Cipro) for 5 days ,NSAIDS with
steroids. Patient improved her symptoms by
90%.
• Presently she had mild periorbital pain.

6. • On Examination :
• Patient conscious well oriented
• No Neurological deficits
• Opthalmology- Unremarkable/ No ocular or
cranial bruits/ Fundus - normal
• Other systems - normal

7. Investigations
• CBC- Normal limits
• ESR- 34mm/hr
• CRP- negative
• FBS- 152mg/dl
• HbA1c- 6.6%
• Mantoux test- strong + 18mm
• CSF- TC- 8 cells all are lymphocytes, no cogweb, sugar- 76mg/dl (RBS-153md/dl),
protein- 75mg/dl, Grams stain, Indian ink - negative,
• CSF XPERT MTB/RIF- Negative
• S. ACE levels- normal
• Chest xray- normal

8. MRI

9. MRI

10. MRI Report

11. Summary
• A 53 yrs old lady with acute retroorbital headache and
Lt.frontal parasthesia of 4 month duration, not
associated eye movements, without any associated
symptoms of migraine/ raised ICT features / autonomic
symptoms/ with constitutional symptoms/with no
neurological deficits/ with normal investigations except
positive Mantoux test & MRI - showing contrast
enhancing lesion of cavernous sinus ? inflammatory
peudotumor with good response to short antibiotic
course and to steroids, the likely differentials will be
Cavernous sinus syndrome

12. Discussion
• Causes for retroorbital pain - orbital, superior
orbital fissure, cavernous sinus, or intracranial
infiltrative, neoplastic, or inflammatory disease
processes with normal ophthalmologic and
neuro-ophthalmologic exam.
• In some patients, no etiology for the pain
syndrome is idiopathic eye pain, eye strain, or
atypical facial pain.

13. Cavernous Sinus

14. Cavernous sinus mass
has a wide differential including:
• Meningioma
• Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)
• Infection / non - infectious inflammatory
• Schwannoma -trigeminal schwannoma is the most common
• Cavernous haemangioma
• Lymphoma
• Metastatic disease (i.e. perineural spread of tumour through neural foramina)
• Aneurysm
• Haemangiopericytoma
• Neurosarcoidosis: rarely involves the cavernous sinus
• Base of skull tumour
◦ chondrosarcoma
◦ osteosarcoma
◦ chordoma (usually midline)
• Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)

15. • Cavernous sinus tumors are the most common
cause of cavernous sinus syndrome.
• Tumors may be primary or may arise from either
local spread or as metastases.
• Cavernous sinus syndrome is defined by its
resultant signs and symptoms: ophthalmoplegia,
chemosis, proptosis, Horner syndrome, or
trigeminal sensory loss. Infectious or
noninfectious inflammatory, vascular, traumatic,
and neoplastic processes are the principal
causes.

16. Cavernous sinus lesions are characterized by the following signs:
• Unilateral and isolated third, fourth, or sixth cranial nerve palsy
• Combination patterns of ophthalmoplegia
• Painful ophthalmoplegia
• Proptosis (pulsating exophthalmos suggests a direct C-C fistula)
• Ocular and cranial bruits
• Conjunctival congestion; arterialization of conjunctival veins
• Ocular hypertension
• Optic disc edema or pallor; retinal hemorrhages
• Anesthesia in the ophthalmic division of the trigeminal nerve (V1)
and/or decreased or absent corneal reflex and possibly anesthesia in the
maxillary or V2 branch
• Pupil in midposition and nonreactive if both sympathetics and
parasympathetics from the third nerve are affected

17. •The Tolosa-Hunt syndrome was first described in 1954, in the case report of a patient
with painful ophthalmoplegia caused by nonspecific granulomatous inflammation of the
cavernous sinus and the cavernous portion of the internal carotid artery.
•The criteria to diagnose the syndrome, as follows:
•1 - acute retro-orbital pain;
•2 - alterations on the third, fourth, sixth, or first branch of the fifth cranial nerve and, less
commonly, involvement of the optic nerve or sympathetic fibers around the cavernous
portion of the carotid;
•3 - symptoms persisting for days or weeks;
•4 - spontaneous pain remission;
•5 - recurrent episodes;
•6 - prompt response to steroidsPathology
•The constant pain that characterises the disorder is due to infiltration of lymphocytes
and plasma cells, along with thickening of dura mater within the cavernous sinus.

18. • The exact cause of Tolosa-Hunt syndrome is unknown,
one theory is an abnormal autoimmune response
linked with an inflammation.
• In some cases, inflammation may be due to a clumping
of a certain type of cell (granulomatous inflammation).
• Some authorities suggest that resolution of imaging
abnormalities after a course of systemic corticosteroids
should be considered “diagnostic” of Tolosa-Hunt
syndrome.
• Steroids are generally tapered over weeks to months,
in some cases prolonged therapy may be necessary.

19. Post Treatment

21. Repeat MRI Report

22. Treatment
• The three most common treatments of cavernous sinus tumors are:
• 1.Observation,
• 2.Non-surgical radiation therapy (SRS), and
• 3.Microsurgical resection (corrective surgery).
• Observation is chosen when a patient - asymptomatic or with mild symptoms
because meningiomas typically grow very slowly and can remain dormant for
extended periods of time.
• Regular scans should be performed with careful monitoring for change.
• If tests indicate the tumor has spread, SRS is an option and can provide temporary
relief of symptoms.
• Surgery to completely remove the tumor is only performed when the patient has
become symptomatically disabled, such as when the optic nerves have become
compressed.
•

23. • Thank You