Transcript Tolosa-Hunt Syndrome

Tolosa-Hunt
Syndrome
Intern 趙若雁
2005.11.29.
Supervisor: Dr. 俞芹英
Outline
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Introduction
Clinical presentation
Laboratory and Image studies
Diagnostic criteria
Treatment
Tolosa-Hunt syndrome
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Painful ophthalmoplegia caused by
nonspecific inflammation of the cavernous
sinus or superior orbital fissure.
Pathophysiology
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Nonspecific inflammation (noncaseating
granulomatous or nongranulomatous) within
the cavernous sinus or superior orbital fissure
Pain
Ophthalmoplegia (Cranial nerve III, IV, VI)
Pupillary dysfunction
Trigeminal nerve involvement (V1)
Epidemiology
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Incidence: uncommon
No gender predilection
Age: rare during the first 2 decades of life
Clinical Presentation
History~
 Severe retro-orbital or periorbital pain of
acute onset, boring and constant in nature
 Diplopia follows the onset of pain
 Patient may have visual loss
 Paresthesia along the forehead
 May be self-limited, relapsing-remitting
 Most often unilateral
Clinical Presentation
Physical exam~
 Painful ophthalmoparesis or ophthalmoplegia
 Evidence of ocular motor nerve palsies (III, IV,
VI)
 Loss of corneal reflex (V1 involvement).
 Ptosis
 Horner syndrome
International Headache Society
criteria for THS
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Episode(s) of unilateral orbital pain for an
average of 8 weeks if untreated
Associated paresis of the 3rd, 4th or 6th
cranial nerves, which may coincide the
onset of pain or follow it by a period of up to
2 weeks
Pain is relieved within 48 hours of steroid
therapy initiation. Exclusion of other
conditions by neuroimaging and (not
compulsory) angiography.
Differential Diagnosis
Laboratory
Studies
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a diagnosis of exclusion
requiring careful patient
evaluation
(J. Neurol. Neurosurg. Psychiatry
2001; 71; 577-582)
Image studies
Contrast-enhanced MR imaging
 HRCT
 Cerebral angiography
- narrowing of the intracavernous portion of
internal carotid artery
 Phlebography
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MR Imaging
(Headache 1999; 39: 321-325)
MR Imaging
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Convex enlargement of the symptomatic
cavernous sinus by an abnormal tissue
isointense with gray matter on short TR/TE
images and iso-hypointense on long TR/TE
images
This abnormal tissues markedly increase in
signal intensity after contrast injection and
extend into contiguous regions, mainly the
orbital apex and subtemporal fossa
ipsilaterally
Conventional and Dynamic MR
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5 patients with THS and 12 control subjects
Dynamic MRI in coronal planes
Dynamic images: Spine-echo (SE)
sequences in 3 patients and fast spine-echo
(FSE) in 2 patients
Conventional MR images: T1W pre- and
post-contrast SE, T2W FSE sequences
European Journal of Radiology 51(2004) 209-217
Dynamic and Conventional MR
Conventional MRI
A 38-year-old patient with left Tolosa–Hunt syndrome (THS), T1-weighted
coronal (a) pre-contrast (400/20) and (b) post-contrast (500/30) images show
enlarged left cavernous sinus with outer bulging of the lateral wall.
The signal intensity and contrast enhancement of the left affected
cavernous sinus are similar to that of the right unaffected cavernous sinus.
Dynamic MR Imaging
Comparing with the right unaffected
cavernous sinus, (c) the dynamic coronal
(500/14.2) images show an area along the
inferior-lateral wall (arrowheads) of the left
cavernous sinus, which enhance very
slowly and gradually from the early
(obtained at 15 s) to the late (obtained at
75 s) images.
Follow-up MR images after steroid
therapy, the left cavernous sinus appears
normal on T1W coronal (d) pre-contrast
(400/14) and (e) post-contrast (400/20)
images.
(f) The dynamic coronal images
(500/14.2) show no residual gradually
enhancing area along the lateral wall of the
left previously affected cavernous sinus.
Conventional and Dynamic MR
Conventional MRI:
 Enlarged cavernous sinus
(CS) with abnormal soft
tissue
 Post-contrast images:
 marked enhancement of
the enlarged CS
 But no exact demarcation
depicted between normal
and abnormal tissues
Dynamic MRI:
 Better demarcation of normal
structures within the venous
spaces of the CS
 Small slowly and gradually
enhancing areas from the early
to the subsequent dynamic
images adjacent to the dark
filling defects of the cranial
nerves
 Consistent with the pathologic
processes ”granulomatous
inflammation”
Medical Treatment
Corticosteroid (treatment of choice)
 60-120 mg/day for 7-10 days, then taper…
 Significant pain relief: within 24-72 hr of
therapy initiation
 Ophthalmoparesis: weeks to months for
resolution
 For refractory cases:
- Azathioprine, methotrexate, radiation therapy
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Follow-up
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Positive MRI or CT remain suspect until a
malignant tumor or inflammation other than
THS is excluded by biopsy or follow-up
examination(s).
Clinical and radiological follow-up
examination performed for at least 2 years.
J Neurol (1999) 246; 371-377
Thanks for Your
Attention
References
1.
2.
www.emedicine.com/neuro/topic373.htm
Nosological entities? The Tolosa-Hunt syndrome.
J. Neurol. Neurosurg. Psychiatry. 2001; 71; 577-582
3.
The criteria of the International Headache Society
for Tolosa-Hunt syndrome need to be revised. J.
Neurol. (1999) 246; 371-377
4.
5.
Neuroimaging diagnosis of Tolosa-Hunt syndrome:
MRI contribution. Headache 1999; 39; 321-325
Dynamic MR imaging in Tolosa-Hunt syndrome.
Eur. J. Radiol. 51(2004) 209-217