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Show / ou", al of Clillical Nruro-( ll'hthall1lot".~ 1/ 12( 3); 203- 206, 1992. Transient Oculomotor Nerve Synkinesis in Non- Hodgkin's Lymphoma Sze Haur Lee, M. Med., Yew Kim Yeow, M. Med., Chai Beng Tan, M. Med., and Helen Tjia, M. Med. « , 1992 Raven Press, Ltd., New York A patient with large cell malignant lymphoma presented with transient left oculomotor nerve synkinesis, left trigeminal and abducens nerve palsies. Magnetic resonance imaging showed thickening of the oculomotor and trigeminal nerves characteristic of central nervous system lymphoma. To our knowledge, this is the first reported case of transient oculomotor nerve synkinesis in non- Hodgkin's lymphoma. The rapid onset and quick recovery of the synkinesis following 2 weeks of chemotherapy support the ephatic transmission theory. Key Words: Lymphoma- Transient oculomotor nerve synkinesis-- Ephatic transmission- Magnetic resonance imaging. From the Department of Neurology, Tan Tock Seng Hospital, Singapore. This paper was presented at the Sixth Annual Scientific Meeting of the Neuro- Ophthalmology Society of Australia, October 18 and 19, 1991. Address correspondence and reprint requests to Dr. Sze Haur Lee, Department of Neurology, Tan Tock Seng Hospital. Moulmein Road, Singapore 1130, Republic of Singapore. 203 Unilateral and bilateral oculomotor nerve dysfunction has been reported in lymphoma ( 1,2). Oculomotor nerve synkinesis is known to occur in slow- growing intracavernous lesions like meningioma and aneurysm ( 3,4). We report the first case of transient oculomotor nerve synkinesis in nonHodgkin's lymphoma. CASE REPORT A 44- year- old Chinese lady was admitted with a I- month history of giddiness, left facial numbness, and frequent generalized headache. Three days prior to admission she had fever with chills and rigors as well as diplopia on looking downward. There was no diplopia on looking sideways. There was no facial asymmetry, hearing impairment, weakness, or numbness of the limbs. She did not have joint pain or loss of appetite or weight. There was no history of hypertension, diabetes mellitus, or other significant medical conditions. Clinical examination revealed a well- nourished lady who was febrile with a temperature of 38° C. The blood pressure was 130/ 80 mmHg and pulse rate was 90/ min. She was conscious and rational. The neck was supple. The right pupil was 2 mm in diameter and reactive to light, whereas the left pupil measured 4 mm and reacted sluggishly. There was partial ptosis and impairment of elevation, depression, and adduction of the left eye. Visual acuity was normal. There was hypoesthesia over the area supplied by the maxillary and mandibular branches of the left trigeminal nerve. The left corneal reflex was present. There were no cerebellar or long tract signs. Examination of the heart, lungs, and abdomen revealed no abnormalities and there was no significant lymphadenopathy. 204 S. H. LEE ET AL. She continued to have a remittent fever while in the ward. Two weeks after admission she developed partial left abducens nerve palsy, and paradoxical retraction of the left upper eyelid on downward gaze ( Fig. 1). There was no pupillary synkinesis associated with ocular motility. The left trochlear nerve function was intact. The hemogram showed pancytopenia ( hemoglobin: 10.7 g/ dl, white blood cells: 2,100/ f. L1, and platelets: 20,000/ f. Ll). Bone marrow biopsy showed large cell malignant lymphoma. Cerebrospinal fluid ( CSF) examination was normal, but cytospins of the CSF were not done. Septic workup and immune markers were negative. Chest x- ray was normal. Magnetic resonance imaging ( MRI) showed a mass over the medial aspect of the left temporal lobe abutting on the cavernous sinus and thickening of the left trigeminal ( Fig. 2) and oculomotor nerves ( Fig. 3). All the lesions enhanced well with gadolinium. Chemotherapy was started about 4 weeks after admission using the MACOP- B regimen ( methotrexate, adriamycin, cyclophosphamide, vincristine, prednisolone, and bleomycin). The fever subsided rapidly after the first dose of chemotherapy. Two weeks after treatment, all extraocular movements recovered without any trace of synkinesis ( Fig. 4). However, the left pupil, though reactive to light, was still larger than the right. In summary, this patient had non- Hodgkin's lymphoma and possibly central nervous system involvement as evidenced by the dramatic response of the neurological deficits to chemotherapy. FIG. 2. Gadolinium magnetic resonance imaging ( TR = 700 msec. TE = 12 msec) showing a left medial temporal mass abutting the cavernous sinus ( big arrow) and thickened left trigeminal nerve ( small arrow). DISCUSSION Lymphoma can affect the nervous system in several ways. Extradural spinal cord compression, cranial bony and dural involvement, cranial and peripheral nerve infiltration ( 5), infiltration of brain parenchyma ( 6) and leptomeninges ( 7) have been reported. The overall incidence of central nervous system ( CNS) involvement in lymphoma is around 10' 7c ( 8- 10). The pathogenesis is unknown. t FIG. 1. Left oculomotor and abducens nerve palsies. Paradoxical lid retraction of the I.? ft eye ') n ,: 10wnward gaze. :,.,', OCULOMOTOR NERVE SYNKINESIS 205 FIG. 3. Gadolinium magnetic resonance imaging ( TR = 700 msec, TE = 12 msec) showing thickened left oculomotor nerve ( arrow). Hematogenous spread is the most likely mechanism, as there is a relative lack of lymphatics in the CNS ( 8). Autopsy studies have shown that lymphomatous cells can separate individual nerve fascicles, as well as individual fibers within the fascicles. Demyelination is also a frequent finding ( 7). In our patient, thickening of the oculomotor and trigeminal nerves was demonstrated on the MRI scan. This is likely to be due to lymphomatous infiltration of the leptomeninges or nerve fibers. Thus magnetic resonance imaging is a useful tool in the diagnosis of CNS lymphoma. t The commonest presentation of CNS lymphoma is cranial nerve palsies. The facial nerve is most commonly involved. Occasionally two or more nerves may be involved ( 7,8). Despite the predilection for cranial nerves, this is the first reported case of transient oculomotor nerve synkinesis in non- Hodgkin's lymphoma. These paradoxical movements are usually seen at least 6 weeks after acute third nerve paralyses caused by trauma, intracranial tumors, intracranial aneurysms, syphilis, or septic cavernous thrombosis ( 11,12). Three mechanisms have been postulated for these paradoxical movements, namely, aberrant regeneration ( 13), ephatic transmission ( 14,15) and chromatolysis- induced reorganization of nuclear synapses ( 14,16). Our case is unique in that the synkinetic movement appeared about 21/ 2 weeks after the acute 3rd cranial nerve injury and disappeared 2 weeks after starting chemotherapy. The rapid onset of the synkinesis and its transient nature would strongly support the ephatic transmission theory. This is an electrotonic, as opposed to chemical, spread of impulses between cells ( 15). The ephapse between adjacent cells could have been created by demyelination ( 17), which is not uncommon in CNS lymphoma. In aberrant regeneration there is misdirection of regenerated axons so that they innervate muscles to which they do not belong ( 13). Misdirection is not the likely cause in our patient because a much longer time than 6 weeks would be needed for regression of misdirected axons followed by regrowth of properly oriented oculomotor nerve axons ( 14). In conclusion, this is the first reported case of transient oculomotor nerve synkinesis in non- FIG. 4. Complete recovery of ptOSis. extraocular movements and synkinesis following 2 weeks' of chemotherapy. I Clin Nellro · ophlhalrnol. Vol. 12. No. 3. 1992 206 S. H. LEE ET AL. Hodgkin's lymphoma. It illustrates the fact that the MRI scan can aid in the diagnosis of CNS lymphoma by demonstrating cranial nerves thickening. The transient synkinesis supports the ephatic transmission theory. REFERENCES 1. Miller NR. Solitary oculomotor nerve palsy in childhood. Am / Opllt/ wlmol 1977; 83: 10&- 11. 2. Wilkins DE, Samhouri AM. Isolated bilateral oculomotor paresis due to lymphoma. Neurology 1979; 29: 1425- 8. 3. Schatz Nj, Savino Pj, Corbett JJ. 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