A Case of TOLOSA HUNT SYNDROME

An Interesting Case

Dr PannerA Unit

Presenter Dr Ramesh BabuM

Brief History

bull A 53yrs old female from Westbengal Diabetic -

10yrs

bull Co Headache - Lt periorbital - 4 months

bull Parasthesia over left frontal area

bull Ho loss of Weight ~5kg in last 4 months

bull Evening rise of temparature - (99-100f) on ampoff

bull Headache UL Lt periorbital spreads to frontal amp

rest of the cranium started on 1022017 at 930pm

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

bull No HO nausea vomitings giddiness double vision

LOC seizures sweating lacrimation flushing

redness of the face and the eye photophobia

phonophobia

bull No ho similar episodes in the past

bull with the above complaints approached to local

physician(20217) - 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

bull He referred to Neurologist INSkolkata (1317)- they asked

complete tuberculosis work - all reports against TB except

mantoux test - 18mm and started on ATT and tapering dose of

steroids over 1 month (8317) referred to NIMHANS for CSF

evaluation

bull In NIMHANS (15317)- LP - dry tap and suggested to continue

on ATT Patient couldnt tolerate ATT hence she stopped ATT

bull On 20317 in INS they repeated CSF analysis

which was normal

bull On 31317 she was started on antibiotic course

(T Septran and Cipro) for 5 days NSAIDS with

steroids Patient improved her symptoms by

90

bull Presently she had mild periorbital pain

bull On Examination

bull Patient conscious well oriented

bull No Neurological deficits

bull Opthalmology- Unremarkable No ocular or

cranial bruits Fundus - normal

bull Other systems - normal

Investigationsbull CBC- Normal limits

bull ESR- 34mmhr

bull CRP- negative

bull FBS- 152mgdl

bull HbA1c- 66

bull Mantoux test- strong + 18mm

bull CSF- TC- 8 cells all are lymphocytes no cogweb sugar- 76mgdl (RBS-153mddl)

protein- 75mgdl Grams stain Indian ink - negative

bull CSF XPERT MTBRIF- Negative

bull S ACE levels- normal

bull Chest xray- normal

MRI

MRI

MRI Report

Summary

bull A 53 yrs old lady with acute retroorbital headache and

Ltfrontal parasthesia of 4 month duration not

associated eye movements without any associated

symptoms of migraine raised ICT features autonomic

symptoms with constitutional symptomswith no

neurological deficits with normal investigations except

positive Mantoux test amp 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

Discussion

bull 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

bull In some patients no etiology for the pain

syndrome is idiopathic eye pain eye strain or

atypical facial pain

Cavernous Sinus

Cavernous sinus mass

has a wide differential including

bull Meningioma

bull Orbital apical inflammation with cavernous sinus involvement (Tolosa-Hunt syndrome)

bull Infection non - infectious inflammatory

bull Schwannoma -trigeminal schwannoma is the most common

bull Cavernous haemangioma

bull Lymphoma

bull Metastatic disease (ie perineural spread of tumour through neural foramina)

bull Aneurysm

bull Haemangiopericytoma

bull Neurosarcoidosis rarely involves the cavernous sinus

bull Base of skull tumour

chondrosarcoma

osteosarcoma

chordoma (usually midline)

bull Nasopharyngeal carcinoma with intracranial extension (especially in Southeast Asia)

bull Cavernous sinus tumors are the most common

cause of cavernous sinus syndrome

bull Tumors may be primary or may arise from either

local spread or as metastases

bull 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

Cavernous sinus lesions are characterized by the following signs

bull Unilateral and isolated third fourth or sixth cranial nerve palsy

bull Combination patterns of ophthalmoplegia

bull Painful ophthalmoplegia

bull Proptosis (pulsating exophthalmos suggests a direct C-C fistula)

bull Ocular and cranial bruits

bull Conjunctival congestion arterialization of conjunctival veins

bull Ocular hypertension

bull Optic disc edema or pallor retinal hemorrhages

bull Anesthesia in the ophthalmic division of the trigeminal nerve (V1)

andor decreased or absent corneal reflex and possibly anesthesia in the

maxillary or V2 branch

bull Pupil in midposition and nonreactive if both sympathetics and

parasympathetics from the third nerve are affected

bullThe 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

bullThe criteria to diagnose the syndrome as follows

bull1 - acute retro-orbital pain

bull2 - 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

bull3 - symptoms persisting for days or weeks

bull4 - spontaneous pain remission

bull5 - recurrent episodes

bull6 - prompt response to steroidsPathology

bullThe 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

bull The exact cause of Tolosa-Hunt syndrome is unknown

one theory is an abnormal autoimmune response

linked with an inflammation

bull In some cases inflammation may be due to a clumping

of a certain type of cell (granulomatous inflammation)

bull Some authorities suggest that resolution of imaging

abnormalities after a course of systemic corticosteroids

should be considered ldquodiagnosticrdquo of Tolosa-Hunt

syndrome

bull Steroids are generally tapered over weeks to months

in some cases prolonged therapy may be necessary

Post Treatment

Repeat MRI Report

Treatmentbull The three most common treatments of cavernous sinus tumors are

bull 1Observation

bull 2Non-surgical radiation therapy (SRS) and

bull 3Microsurgical resection (corrective surgery)

bull 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

bull Regular scans should be performed with careful monitoring for change

bull If tests indicate the tumor has spread SRS is an option and can provide temporary

relief of symptoms

bull 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

bull

bull Thank You