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International Journal of Trend in Scientific Research and Development (IJTSRD)
Volume 4 Issue 5, July-August 2020 Available Online: www.ijtsrd.com e-ISSN: 2456 – 6470
@ IJTSRD | Unique Paper ID – IJTSRD32961 | Volume – 4 | Issue – 5 | July-August 2020 Page 649
Tolosa Hunt Syndrome: A Case Report
Vicky Mary Johnson, Vinty Mary Johnson, Aksa Simon
Pharm-D Interns, Pushpagiri Medical College Hospital, Tiruvalla, Kerala, India
ABSTRACT
Tolosa Hunt Synsdrome is a rare disorder characterized by severe and
unilateral headache associated with painful and restricted eye movements. It
is mainly due to paresis of one or more of the oculomotor (3rd), trochlear
(4th), abducent(6th) cranial nerves caused by a granulomatous inflammation
in the cavernous sinus, superior orbital fissure or orbit. In this case, a 61year
old male patient with complaints of headache, right complete ptosis with
throbbing pain around right eye. Thiscase report studyhasbeenpresentedfor
the consideration of steroid therapy in tolosa hunt syndrome.
How to cite this paper: Vicky Mary
Johnson | Vinty Mary Johnson | Aksa
Simon "Tolosa Hunt Syndrome: A Case
Report" Published in
International Journal
of Trend in Scientific
Research and
Development(ijtsrd),
ISSN: 2456-6470,
Volume-4 | Issue-5,
August 2020, pp.649-
650, URL:
www.ijtsrd.com/papers/ijtsrd32961.pdf
Copyright © 2020 by author(s) and
International Journal of TrendinScientific
Research and Development Journal. This
is an Open Access article distributed
under the terms of
the Creative
Commons Attribution
License (CC BY 4.0)
(http://creativecommons.org/licenses/by
/4.0)
INTRODUCTION
Tolosa Hunt Syndrome is a rare disorder, defined as painful
ophthalmoplegia consists of periorbital/hemicranial pain
associated with ipsilateral ocular motor nerve palsies,
oculosympathetic paralysis and sensory loss in the
distribution of the ophthalmic branch of trigeminal nerve
and occasionally the maxillary division of the trigeminal
nerve. Various combinations of these cranial nerve palsies
may occur. There is a specific subset of patients whodevelop
painful ophthalmoplegia due to a non- specific inflammatory
process in the region of the cavernous sinus/ superior
orbital fissure.
It is a steroid -responsive painful ophthalmoplegia which is
secondary to idiopathic granulomatous inflammation. It has
been categorized as a diagnosis of exclusion because of its
non-specific radiologic presentation. The orbital apex
pseudotumor and granulomatous inflammation of the
cavernous sinus have same clinical features and should be
considered as a part of the spectrum of Tolosa Hunt
Syndrome. Symptomatic improvement after steroid therapy
is an essential but is not a exact proof of the syndrome,
because lesions such as lymphomas may also respond to
steroids.
DIAGNOSIS
It is based on;
 Eye pain on one side of the head that persists for atleast
eight weeks if untreated.
 Associated damage to third-fourth/ sixthcranialnerves.
 Relief of pain within 48hrs after the administration of
steroids.
Diagnosis may be confirmed by a thorough clinical
evaluation
 Detailed patient history
 Radiologic tests like computed tomography (CT) scan
and magnetic imaging (MRI)
 These examinations may show characteristic
enlargement/ inflammation of the areas (cavernous
sinus and superior orbital fissure).
CASE REPORT
A 61 year old male patient came with complaints of
headache, right ptosis, pain over right eye for 2 days. He had
woken up in the middle of the night with severe headache
bifrontally whichis throbbing in nature, and also hadwaken
up in the next morning with partial ptosis of right eye. By
evening it progressed to right complete ptosis with
throbbing pain around right eye.
On examination 3 rd cranial nerve ophthalmic nerve (V1)
impairment noticed but there was no facial palsy.MRI
showed normal cavernous sinus region, rightsuperiorrectus
and levator palpebre superioris complex was thickened and
had minimal contrast uptake. CSF tapping was done under
aseptic precaution. In which, CSF protein and glucose was
found to be elevated. Patient showed positive bitot’s spot
and left conjuctival congestion. He had k/c/o type II DM for
10 year, hemorrhoids for 10 years, 4 years back a blood
transfusion was done for severe anemia and also had a
history of acute bronchitis 2 weeks ago. Intially patient was
given T. Deflazacort 6 mg but patient didn’t show any
improvement. The selective involvements of muscles
IJTSRD32961
International Journal of Trend in Scientific Research and Development (IJTSRD) @ www.ijtsrd.com eISSN: 2456-6470
@ IJTSRD | Unique Paper ID – IJTSRD32961 | Volume – 4 | Issue – 5 | July-August 2020 Page 650
generated discussion towards a possible neuromuscular
junction disorder. Repetitive nerve stimulation and
neostigmine test were performed and results were negative.
After that methylprednisolone pulse therapywasgiven,then
patient showed symptomatic improvement. For pain T.
ultracet was given .On discharge inj. Methylprednisolone
was changed to T. wysolone 40 mg for 2 weeks.
DISCUSSION
It is usually affects only one eye (unilateral). Affected
individuals experience intense sharp painanddecreasedeye
movements. Symptoms often will subside without
spontaneous remission and may reoccur randomly. These
individuals may exhibit signs of paralysis of certain cranial
nerves such as drooping of the upper eyelid (ptosis), double
vision (diplopia), large pupil and facial numbness. The
affected eye may also exhibit protrusion of the eye
(proptosis). The main cause of Tolosa Hunt Syndrome is
unknown, but the disorder is thought to be associated with
inflammation of specific areas behind the eye like cavernous
sinus and superior orbitalfissure. Theaverageageofonsetis
41 yrs, but it had been reported among people younger than
30yrs. Methylprednisolone and prednisolonearefoundtobe
effective in Tolosa Hunt Syndrome. MRI findings before and
after corticosteroid therapy are the important diagnostic
criteria to differentiate itfrom other cavernous sinuslesions
that stimulate THS both clinically and radiologically.
CONCLUSION
The pain associated with Tolosa Hunt Syndrome subsides
with the use of short term use of steroid drugs. Pain is
usually reduced in untreated cases within fifteen to twenty
days.With steroid treatment, pain typically briskly subsides
within twenty four to seventy two hours. The diagnosis is
based on the brisk steroid response. Although steroids are
generally tapered over weeks to months, in some cases
prolonged therapy may be necessary. The affected
individuals may be vulnerable to recurrent future attacks.
Steroids like prednisone and methylprednisolone are
effective in tolossa hunt syndrome than other steroids.
Immunosuppresive agents like azathioprine and
methotrexate can be beneficialif patients show no response
to steroids.
REFERENCE
[1] Kline LB, WF Hoyt “the tolosa hunt syndrome” journal
of neurology, neurosurgery and psychiatry. 2001
november, vol 71, ,issue 5;577-582,
[2] Eddie S. Kwan, Samuel M. Wolpestetal, tolosa hunt
syndrome revisited, American journal of neurology 8;
1067-1072. november/December 1987.
[3] Maxuel nogeueria dos santos etal,”tolosa hunt
syndrome, a painful ophthalmolplegia” revisita
brasileria de ophtalmologia.vol.78 (4), july/august
2019, 271-273.
[4] Smith JL etal.Painful ophthalmoplegia-Tolosa Hunt
Syndrome, American journal of ophthalmoplegy, 1966
june; vol 61, issue 6, 466-1472.
[5] Lanning B. Kline M. D, The Tolosa Hunt Syndrome,
survey of ophthalmology, volume 27,issue 2,
September-october 1982, 79-95.
[6] Cakirer S, “MRI findings in Tolosa Hunt Syndrome
before and after systemic corticosteroid therapy”,
European journal of radiology.2003 Feb;45 (2);83-90

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tolosa hunt syndrome a case report

  • 1. International Journal of Trend in Scientific Research and Development (IJTSRD) Volume 4 Issue 5, July-August 2020 Available Online: www.ijtsrd.com e-ISSN: 2456 – 6470 @ IJTSRD | Unique Paper ID – IJTSRD32961 | Volume – 4 | Issue – 5 | July-August 2020 Page 649 Tolosa Hunt Syndrome: A Case Report Vicky Mary Johnson, Vinty Mary Johnson, Aksa Simon Pharm-D Interns, Pushpagiri Medical College Hospital, Tiruvalla, Kerala, India ABSTRACT Tolosa Hunt Synsdrome is a rare disorder characterized by severe and unilateral headache associated with painful and restricted eye movements. It is mainly due to paresis of one or more of the oculomotor (3rd), trochlear (4th), abducent(6th) cranial nerves caused by a granulomatous inflammation in the cavernous sinus, superior orbital fissure or orbit. In this case, a 61year old male patient with complaints of headache, right complete ptosis with throbbing pain around right eye. Thiscase report studyhasbeenpresentedfor the consideration of steroid therapy in tolosa hunt syndrome. How to cite this paper: Vicky Mary Johnson | Vinty Mary Johnson | Aksa Simon "Tolosa Hunt Syndrome: A Case Report" Published in International Journal of Trend in Scientific Research and Development(ijtsrd), ISSN: 2456-6470, Volume-4 | Issue-5, August 2020, pp.649- 650, URL: www.ijtsrd.com/papers/ijtsrd32961.pdf Copyright © 2020 by author(s) and International Journal of TrendinScientific Research and Development Journal. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0) (http://creativecommons.org/licenses/by /4.0) INTRODUCTION Tolosa Hunt Syndrome is a rare disorder, defined as painful ophthalmoplegia consists of periorbital/hemicranial pain associated with ipsilateral ocular motor nerve palsies, oculosympathetic paralysis and sensory loss in the distribution of the ophthalmic branch of trigeminal nerve and occasionally the maxillary division of the trigeminal nerve. Various combinations of these cranial nerve palsies may occur. There is a specific subset of patients whodevelop painful ophthalmoplegia due to a non- specific inflammatory process in the region of the cavernous sinus/ superior orbital fissure. It is a steroid -responsive painful ophthalmoplegia which is secondary to idiopathic granulomatous inflammation. It has been categorized as a diagnosis of exclusion because of its non-specific radiologic presentation. The orbital apex pseudotumor and granulomatous inflammation of the cavernous sinus have same clinical features and should be considered as a part of the spectrum of Tolosa Hunt Syndrome. Symptomatic improvement after steroid therapy is an essential but is not a exact proof of the syndrome, because lesions such as lymphomas may also respond to steroids. DIAGNOSIS It is based on;  Eye pain on one side of the head that persists for atleast eight weeks if untreated.  Associated damage to third-fourth/ sixthcranialnerves.  Relief of pain within 48hrs after the administration of steroids. Diagnosis may be confirmed by a thorough clinical evaluation  Detailed patient history  Radiologic tests like computed tomography (CT) scan and magnetic imaging (MRI)  These examinations may show characteristic enlargement/ inflammation of the areas (cavernous sinus and superior orbital fissure). CASE REPORT A 61 year old male patient came with complaints of headache, right ptosis, pain over right eye for 2 days. He had woken up in the middle of the night with severe headache bifrontally whichis throbbing in nature, and also hadwaken up in the next morning with partial ptosis of right eye. By evening it progressed to right complete ptosis with throbbing pain around right eye. On examination 3 rd cranial nerve ophthalmic nerve (V1) impairment noticed but there was no facial palsy.MRI showed normal cavernous sinus region, rightsuperiorrectus and levator palpebre superioris complex was thickened and had minimal contrast uptake. CSF tapping was done under aseptic precaution. In which, CSF protein and glucose was found to be elevated. Patient showed positive bitot’s spot and left conjuctival congestion. He had k/c/o type II DM for 10 year, hemorrhoids for 10 years, 4 years back a blood transfusion was done for severe anemia and also had a history of acute bronchitis 2 weeks ago. Intially patient was given T. Deflazacort 6 mg but patient didn’t show any improvement. The selective involvements of muscles IJTSRD32961
  • 2. International Journal of Trend in Scientific Research and Development (IJTSRD) @ www.ijtsrd.com eISSN: 2456-6470 @ IJTSRD | Unique Paper ID – IJTSRD32961 | Volume – 4 | Issue – 5 | July-August 2020 Page 650 generated discussion towards a possible neuromuscular junction disorder. Repetitive nerve stimulation and neostigmine test were performed and results were negative. After that methylprednisolone pulse therapywasgiven,then patient showed symptomatic improvement. For pain T. ultracet was given .On discharge inj. Methylprednisolone was changed to T. wysolone 40 mg for 2 weeks. DISCUSSION It is usually affects only one eye (unilateral). Affected individuals experience intense sharp painanddecreasedeye movements. Symptoms often will subside without spontaneous remission and may reoccur randomly. These individuals may exhibit signs of paralysis of certain cranial nerves such as drooping of the upper eyelid (ptosis), double vision (diplopia), large pupil and facial numbness. The affected eye may also exhibit protrusion of the eye (proptosis). The main cause of Tolosa Hunt Syndrome is unknown, but the disorder is thought to be associated with inflammation of specific areas behind the eye like cavernous sinus and superior orbitalfissure. Theaverageageofonsetis 41 yrs, but it had been reported among people younger than 30yrs. Methylprednisolone and prednisolonearefoundtobe effective in Tolosa Hunt Syndrome. MRI findings before and after corticosteroid therapy are the important diagnostic criteria to differentiate itfrom other cavernous sinuslesions that stimulate THS both clinically and radiologically. CONCLUSION The pain associated with Tolosa Hunt Syndrome subsides with the use of short term use of steroid drugs. Pain is usually reduced in untreated cases within fifteen to twenty days.With steroid treatment, pain typically briskly subsides within twenty four to seventy two hours. The diagnosis is based on the brisk steroid response. Although steroids are generally tapered over weeks to months, in some cases prolonged therapy may be necessary. The affected individuals may be vulnerable to recurrent future attacks. Steroids like prednisone and methylprednisolone are effective in tolossa hunt syndrome than other steroids. Immunosuppresive agents like azathioprine and methotrexate can be beneficialif patients show no response to steroids. REFERENCE [1] Kline LB, WF Hoyt “the tolosa hunt syndrome” journal of neurology, neurosurgery and psychiatry. 2001 november, vol 71, ,issue 5;577-582, [2] Eddie S. Kwan, Samuel M. Wolpestetal, tolosa hunt syndrome revisited, American journal of neurology 8; 1067-1072. november/December 1987. [3] Maxuel nogeueria dos santos etal,”tolosa hunt syndrome, a painful ophthalmolplegia” revisita brasileria de ophtalmologia.vol.78 (4), july/august 2019, 271-273. [4] Smith JL etal.Painful ophthalmoplegia-Tolosa Hunt Syndrome, American journal of ophthalmoplegy, 1966 june; vol 61, issue 6, 466-1472. [5] Lanning B. Kline M. D, The Tolosa Hunt Syndrome, survey of ophthalmology, volume 27,issue 2, September-october 1982, 79-95. [6] Cakirer S, “MRI findings in Tolosa Hunt Syndrome before and after systemic corticosteroid therapy”, European journal of radiology.2003 Feb;45 (2);83-90