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This patient has CNS B-cell lymphoma involving the dura mater.
She presented with a chief complaint of “double vision and a squint is his right eye”.
On examination she was found to have bilateral sixth nerve palsy and paralytic esotropia.
Lymphoma involving the dura is uncommon. Most patients are middle-aged and older adults. There is no apparent association with immunosuppression.
The presenting symptoms include:
Cranial nerve abnormalities
A combination of these disorders
Dural lymphomas are usually localized tumors. Therapy has varied from case to case, but almost all patients fair well.
This patient with CNS B-cell lymphoma of the dura presented with diplopia due to bilateral sixth nerve palsy. She has:
• Esotropia of the right eye
• Marked abduction weakness of the right eye
• Limitation of full abduction of the left eye
• Full upgaze, both eyes converge
Testing each eye fixing alone on a target, as illustrated in this case, is important in order to distinguish a paralytic sixth nerve palsy from a non-paralytic strabismus when the esotropic eye fixing alone moves fully.
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 Anne Osborn, M.D.
(See ref 1)
CNS B-cell lymphoma of the dura mater; Bilateral sixth nerve palsy;
(See ref 1)
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. Keane JR. Bilateral sixth nerve palsy. Analysis of 125 cases. Arch Neurol 1976 Oct;33(10):681-683. http://www.ncbi.nlm.nih.gov/pubmed/184766
4. 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
5. 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;7(3):135-138. http://www.ncbi.nlm.nih.gov/pubmed/2958504
6. 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
7. Leigh RJ, Zee DS. Diagnosis of Peripheral Ocular Motor Palsies and Strabismus. Chp 9:385-474. In: The Neurology of Eye Movements 4th Edition. Oxford University Press, New York 2006.
8. 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
9. 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
Relation-Is Part Of
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Spencer S. Eccles Health Sciences Library, University of Utah