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Identifier |
946-2 |
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Title |
Bilateral Sixth Nerve Palsy |
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Ocular Movements |
Bilateral Sixth Nerve Palsy;
Bilateral Esotropia; |
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Creator |
Wray, Shirley H. |
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Contributor Primary |
Shirley H. Wray, MD, PhD, FRCP, Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital |
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Subject |
Bilateral Sixth Nerve Palsy;
Bilateral Esotropia;
CNS Non-Hodgkin’s Lymphoma;
Cavernous Sinus Syndrome;
Lymphoma;
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Video Instructions |
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Get Media |
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Supplementary Materials |
PowerPoint Presentation:
CNS Lymphoma: http://library.med.utah.edu/NOVEL/Wray/PPT/CNS_Lymphoma.ppt
Shirley H. Wray, M.D., Ph.D., FRCP, Harvard Medical School |
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Presenting Symptom |
Diplopia |
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History |
The patient is a 70 year old Italian man with atrial fibrillation on long-term coumadin therapy.
In October 1995, he developed generalized headache, horizontal double vision and his left eye deviated inwards (esotropia).
A diagnosis of left sixth nerve palsy was made and attributed to microvascular disease.
In November 1995, a brain MRI showed “multiple lacunes”.
In December 1995, in addition to the left esotropia, the right eye deviated in and his double vision was worse.
MRI Brain reviewed by his PCP showed no intracranial mass lesion(s).
He was referred for a neuro-ophthalmic opinion.
Ocular motility examination:
Bilateral esotropia OS > OD
With the right eye fixing alone, partial right sixth nerve palsy.
With the left eye fixing alone, complete left sixth nerve palsy.
Right 3rd and 4th cranial nerve intact
Left 3rd and 4th cranial nerve intact
Corneal reflexes normal
Mild bilateral age related ptosis
The remainder of the cranial nerves were intact
Brain CT with contrast:
1. Fullness of the cavernous sinus bilaterally, especially on the right
2. Fullness of the sella tursica and abnormal soft-tissue along the dorsum of the upper clivus
3. Opacification of the right sphenoid sinus and thinning and irregularity of its posterior-superior wall
Impression: In the setting of bilateral sixth nerve palsies these findings are consistent with invasive aspergillosis or metastatic disease.
Chest CT:
1 cm. paratracheal, precarinal and AP window lymph nodes
Small (5 mm) right retrocrural and left hilar nodes also present.
Abdominal CT:
4 cm. heterogeneous left adrenal mass suspicious for neoplasm
Biopsy of soft tissue in the sella and parasellar region was performed by a right ethmoid approach.
Pathology:
Non-Hodgkin’s lymphoma
Additional studies:
Left adrenal enlargement secondary to hemorrhage
Oncology therapy:
Intensive CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy for six cycles.
Radiation therapy:
Radiation therapy to the skull base.
April 1996 four months post biopsy:
Complete recovery of the right sixth nerve palsy
Mild esotropia OS with a partial left sixth nerve palsy and diplopia at distance.
No headache
By August 1996:
Symptom free with no diplopia
Complete recovery of the left sixth nerve palsy
In September 1998 he was in good health and had a normal examination.
Comment:
Aggressive non-Hodgkin’s lymphomas (NHLs) are characterized by effacement of lymph node architecture with a diffuse infiltration of large lymphocytes and include the diffuse large B-cell lymphoma (31% of NHL), and anaplastic large cell lymphoma (2% of NHL).
The outcome and likelihood of cure in patients with diffuse aggressive NHL is directly related to the total number of adverse prognostic features that are present at presentation.
Adverse prognostic features include:
1. Age older than 60
2. Advance stage (III or IV),
3. Elevated LDH levels and
4. The presence of 2 or more extranodal sites of disease.
The likelihood of cure and long-term disease-free survival ranges from more than 75% in patients with one or fewer adverse factors (as in this patient) to less than 30% in patients with 4 or more adverse factors. |
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Clinical |
This patient with non-Hodgkin’s lymphoma infiltrating the cavernous sinus presented with a bilateral sixth nerve palsy with:
• Bilateral esotropia OS > OD
• Horizontal diplopia at distance, left > right
• Limitation of full abduction of the left eye
• Limitation of full abduction of the right eye
• Full vertical gaze |
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Neuroimaging |
The neuroimaging from two cases of B-cell lymphoma are shown here
Figure 1: Sagittal T-1 W1 shows infiltration of the clivus, sphenoid sinus, upper cervical spine and pituitary gland with cellular material that is isointense with gray matter. The fatty marrow of the sphenoid and cervical spine are almost completely replaced with tumor. Only a small area of normal bone persists at the bottom of the clivus and the anterior ring of C1.
Figure small area of normal bone persists at the bottom of
Figure 2: Axial T2 WI shows the infiltrate in both sides of the cavernous sinus as well as the pituitary gland. the tumor is rather hypointense of this sequence, characteristic of neoplasms that have a high nuclear to cytoplasm ratio.
Case 2 is a 24 year old man with primary CNS lymphoma and multiple cranial nerve palsies. Brain MRI findings are illustrated by a series of 4 axial fat-saturated post contrast T1 weighted MR scans in a patient with known B-cell lymphoma and multiple cranial nerve palsies. The images show enhancement in both internal auditory canals, enlargement and enhancement of both trigeminal nerves, and tumor infiltrating the left cavernous sinus.
Courtesy of Anne Osborn, M.D. |
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Pathology |
Review (1) |
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Disease/Diagnosis |
CNS non-Hodgkin’s lymphoma; Cavernous sinus syndrome; Bilateral sixth nerve palsy; |
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Treatment |
Review (1) |
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References |
1. Bachelor T. Lymphoma of the Nervous System. Butterworth-Heinemann 2004
2. Delpassand ES, Kirkpatrick JB. Cavernous sinus syndrome as the presentation of malignant lymphoma: case report and review of the literature. Neurosurgery. 1988 Oct;23(4):501-504. http://www.ncbi.nlm.nih.gov/pubmed/3059213
3. Kambham N, Chang Y, Matsushima AY. Primary low-grade B-cell lymphoma of mucosa-associated lymphoma tissue (MALT) arising in dura. Clin Neuropthol 1998:17:311-317. http://www.ncbi.nlm.nih.gov/pubmed/9832258
4. Keane JR. Arch Neurol. Bilateral sixth nerve palsy. Analysis of 125 cases. 1976 Oct;33(10):681-683. http://www.ncbi.nlm.nih.gov/pubmed/184766
5. Kumar S, Kumar D, Kaldjian EP, Bauserman S, Raffield M, Jaffe ES. Primary low-grade B-cell lymphoma of the dura: a mucosa associated lymphoid tissue-type lymphoma. Am J Surg Pathol 1997;21:81-87. http://www.ncbi.nlm.nih.gov/pubmed/8990144
6. Lam S, Margo CE, Beck R, Pusateri TJ, Pascucci S. Cavernous sinus syndrome as the initial manifestation of multiple myeloma. J Clin Neuro-ophthalmol. 1987 Sep;7(3):135-138. http://www.ncbi.nlm.nih.gov/pubmed/2958504
7. Lehman NL, Horoupian DS, Warnke RA, Sundram UN, Peterson K, Harsh GR 4th. Dural marginal zone lymphoma with massive amyloid deposition: rare low-grade primary central nervous system B-cell lymphoma. J Neurosurg 2002;6:368-372. http://www.ncbi.nlm.nih.gov/pubmed/11838814
8. Leigh RJ, Zee DS. Diagnosis of Peripheral Ocular Motor Palsies and Strabismus. Ch 9:385-474. In: The Neurology of Eye Movements 4th Edition. Oxford University Press, New York 2006.
9. Miranda RN, Glantz LK, Myint MA, Levy N, Jackson CL, Rhodes CH, Glantz MJ, Medeiros LJ. Stage IE non-Hodgkin’s lymphoma involving the dura: a clinicopathologic study of five cases. Arch Pathol Lab Med 1996:120:254-260. http://www.ncbi.nlm.nih.gov/pubmed/8629900
10. Supler ML, Friedman WA. Acute bilateral ophthalmoplegia secondary to cavernous sinus metastasis: a case report. Neurosurgery. 1992 Oct;31(4):783-786. http://www.ncbi.nlm.nih.gov/pubmed/1407469 |
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Relation-Is Part Of |
163-3; 169-34; 939-8, 944-5 |
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Contributor Secondary |
Steve Smith, Videographer; Ray Balhorn, Digital Video Compressionist; |
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Publisher |
Spencer S. Eccles Health Sciences Library, University of Utah |
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Date-Original |
1996 |
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Context URL |
http://library.med.utah.edu/NOVEL/Wray/ |
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Resource Type |
Video |
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Source |
3/4"" Umatic master videotape |
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Rights Management |
Shirley H. Wray, MD, PhD, Copyright 2002. For further information regarding the rights to this collection, please visit http://library.med.utah.edu/NOVEL/about/copyright |
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Holding.Institution |
Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E, SLC, UT 84112-5890 |
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Annotation |
No |
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Collection |
Neurology / Neuro-ophthalmology |
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Catalogue |
http://library.med.utah.edu/NOVEL/Wray |
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Language |
eng |
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Completion Date |
2009-06-01 |
