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TOLOSA HUNT SYNDROME

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A rare case of TOLOSA HUNT SYNDROME

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TOLOSA HUNT SYNDROME

  1. 1. An Interesting Case Dr. Panner.A Unit Presenter: Dr. Ramesh Babu.M
  2. 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. 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. 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. 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. 6. • On Examination : • Patient conscious well oriented • No Neurological deficits • Opthalmology- Unremarkable/ No ocular or cranial bruits/ Fundus - normal • Other systems - normal
  7. 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. 8. MRI
  9. 9. MRI
  10. 10. MRI Report
  11. 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. 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. 13. Cavernous Sinus
  14. 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. 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. 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. 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. 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. 19. Post Treatment
  20. 20. Repeat MRI Report
  21. 21. 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. •
  22. 22. • Thank You

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