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Show Journal of Clinical Neuro- ol, llthalmology 11( 4): 300- 305, 1991. Fascicular Oculomotor Nerve Palsy in Neuro- Beh~ et's Disease Okay Saribas, M. D., Pinar Aydin- Kirkali, M. D., Enis Erdern, M. D., and Meral Calgiiner, M. D. © 1991 Raven Press, Ltd., New York A 43- year- old man with Behc; et's disease developed a left oculomotor palsy and a right elevation paresis. His clinical picture was consistent with an isolated fascicular third cranial nerve palsy, considering the mass effect of a large left capsulothalamic lesion extending down into the mesencephalon, which was visualized by CT and ( more precisely) by MR!. The patient's clinical status was improved with steroid and immunosuppressive therapy, and radiologic abnormalities resolved markedly in a month. Key Words: Behc; et's syndrome- Computerized tomography- Eye motility disorders- Oculomotor nerveMagnetic resonance imaging. From the Departments of Neurology ( 0.5., E. E.) and Internal Medicine ( M. e); Institute of Neurological Sciences and Psychiatry, Neuro- Ophthalmology Unit ( P. A- K.), Hacettepe University School of Medicine, Ankara, Turkey. Address correspondence and reprint requests to Dr. Okay Sanbas at Department of Neurology, Hacettepe University H( l< pit~ J< Ankara 06100 Turkey. 300 Fascicular syndrome of the third cranial nerve is usually associated with other neurological findings because of the involvement of neighboring structures ( 1). Isolated fascicular oculomotor palsies are rare, and all of the reported cases have been of vascular origin ( 2- 6). We report a clinical case of fascicular oculomotor palsy with a few associated minor signs, secondary to neuro- Behc; et's disease with the magnetic resonance imaging ( MRI) and computerized tomography ( CT) findings. CASE REPORT A 43- year- old man was seen because of double vision and drowsiness. For 5 years he had recurrent aphthous lesions on his tongue, painful ulcerous lesions on the scrotum, and arthralgias. Two months before referral, he began dragging his right leg and became confused. A week prior to referral, he developed malaise. Physical examination revealed erythema nodosum on the lower extremities and swollen knee joints. On the neurological examination he was lethargic. He had a minor right hemiparesis and a mild intention tremor in both hands. Sensory systems were intact. On neuro- ophthalmological examination, his best corrected visual acuity was 20/ 30 in both eyes. Visual fields and both fundi were normal. His left pupil was dilated and unreactive. There was a moderate left ptosis. Upward gaze by voluntary, pursuit efforts, and Bell's maneuver was limited 50% bilaterally. There was a 40% limitation on adduction and depression in the left eye. Intorsion of the left eye could be elicited on attempted downgaze and right gaze. Ocular motility in the right eye was full except for elevation ( Fig. 1). OCULOMOTOR NERVE PALSY IN NEURO- BEH~ ET'S DISEASE A, 301 B ., c FIG. 1. A: Left ptosis and mydriasis with mild right hypotropia. B: Bilateral elevation paresis with left ptosis and left dilated pupil, note also the mild exotropia in the left eye. C: Depression paresis in the left eye; mild left exotropia is evident. J Clin Neuro- ophthalmol, Vol. 11, No. 4, 1991 302 O. SARIBAS ET AL. FIG. 2. Initial CT ( without contrast) demonstrating a hypodense abnormality with mass effect in the left thalamus, compressing the third ventricle. Left cerebral peduncle is also involved. The patient's erythrocyte sedimentation rate was 50 mrn/ h ( Westergren method) and C- reactive protein was 16900 ILglL ( Normal range: 68- 8200). His complete blood cell count, urinalysis, and serum biochemical studies were normal. Rheumatoid factor and antinuclear antibodies were also normal. Cranial computed tomography ( CT) showed a large enhancing capsulothalamic hypodensity extending down into the mesencephalon ( Fig. 2). Magnetic resonance imaging ( MRI) ( 1.0 T magnet, Siemens Magnetom) demonstrated a lesion in the same location, hyperintense on T2weighted images ( Fig. 3A), hypointense on Tlweighted images. There was an extensive high signal in the left mesencephalon ( Fig. 3B). A diagnosis of neuro- Beh~ et's disease was made, and prednisone 40 mg/ d and chlorambucil 4 mg/ d were initiated. One month later there was a mild left ptosis. The left eye was depressed 30% and adducted 20% of normal. Bilaterally there was a 30% limitation in elevation. The pupils were isocoric; both reacted equally to the light. The remainder of the neurological examination and physical examination were normal. Repeat cranial CT showed resolution of the lesion ( Fig. 4). The MRI abnormalities were resolved partially. High signal abnormalities were still present in the left internal capsule ( Fig. SA) and in the mesencephalon ( Fig. 5B), but in markedly reduced sizes. COMMENT Beh~ et's disease is a multisystem disease with the features of mucocutaneous, ocular, intestinal, A B FiG. 3. fnitiat a~ lat T2- weighted MR images ( TR 2.500 ms; TE 90 ms), shows a hyperIntense lesion In the left Internal capsule and basal ganglia ( A), and in left cerebral c · ", · -!" nr; e> extending posteriorly ( B). 1Clin Neuro-<> phthalmol. Vol. 11, No. 4, 1991 OCULOMOTOR NERVE PALSY IN NEURO- BEH( TT'S DISEASE 303 FIG. 4. Follow- up CT: the hypodense abnormality resolved completely. The faint left capsulothalamic enhancement represents a normal choroidal enhancement. articular, vascular, and neurologic involvement ( 7). The subclassification neuro- Beh~ et's is applied if there are nervous system complications ( 7). The clinical picture is characterized by signs of either a meningoencephalitis or a sinus thrombosis. Our patient was determined as having neuro- Beh<; et's disease with oral aphthae, genital ulcers, erythema nodosum, arthralgias, and neurologic involvement ( 8). Neuro- Beh<; et lesions are most consistent with a vasculitis affecting predominantly the small ve-nules of the central nervous system ( 7,9). These lesions have a predilection for the diencephalic structures and the brain stern ( 7,9), and they are radiologically demonstrable ( 10- 20). Resolution of the abnormalities on the CT and MR scans correlate with the clinical improvement brought about by steroid and immunosuppressive therapy ( 1016). Our patient has a unilateral oculomotor nerve palsy combined with an upgaze palsy. Considering on CT and MRI the size and the mass effect of the lesion, which extends from mesencephalon to capsulothalamic area without involvement of the posterior commissure, we believe that this upgaze palsy is due to the mass effect of the lesion, particularly on the rostral mesencephalon, and that the oculomotor nerve palsy is the result of the fascicular involvement ( 1). A month later, the upgaze palsy and left third nerve palsy improved considerably with the resolution of the radiologic abnormalities. Fascicular syndrome of the third cranial nerve is usually associated with other neurological findings resulting from the involvement of neighboring structures [ as in Weber's, Benedikt's, or Claude- Nothnagel's syndrome ( 1)]; isolated fascicular syndrome of the third cranial nerve is rare ( 1- 6). Our patient had a few other minor neurological signs, supporting an involvement in the intra- axial trajectory of the fascicle. However the possibility of a partial nuclear third nerve palsy is not fully eliminated ( 1,21,22). Involvement of both crossed and uncrossed fibers to the superior rectus muscles and sparing of the central caudal nucleus may cause a partial nuclear third nerve palsy ( leva- B FIG. 5. Follow- up axial T2- weighted MR ( TR 2.500 ms; TE 90 ms). Marked resolution of the signal abnormality: There are residual signal abnormalities in the left internal capsule ( arrow) ( A) and in the mesencephalon, along the medial border of the left crus cerebri ( arrow) ( 8). 1Clin Neuro- ophtlullmol, Vol. 11, No. 4, 1991 304 O. SARIBAS ET AL. tor- sparing oculomotor palsy) as documented anatomically in two previous cases ( 23,24), but it is less likely in our case, considering the extent of the lesion and the presence of the mass effect. We found three isolated fascicular syndrome cases ( 2- 4). One case originated from circumscribed infarction of the brain stem ( 2), two from brainstem hematoma ( 3,4). Three additional cases with partial involvement of the intra- axial fascicle, two due to mesencephalic hemorrhage ( 3,5), and one due to mesencephalic infarction ( 6) were also reported. Intra- axial fibers of the third cranial nerve are supplied by the branches from the posterior cerebral artery ( 25). An extensive lesion of vascular origin/ seen on CT and MRl, which suggests unilateral occlusion of the branches of posterior communicating artery, is less likely for our case. Clinically/ the fascicular oculomotor nerve palsy combined with an upgaze palsy with a few mild findings, despite the extent of the lesion on CT and MRl and further resolution of the radiologic abnormalities, are against an ischemic infarction and support our diagnosis of neuro- Beh<; et's disease ( 14). Computed tomography disclosed various focal and nonfocal abnormalities in neuro- Beh<; et's disease ( 13). Focal abnormalities appeared as areas of low density with homogeneous or patchy enhancement in the majority of the cases, occasionally with mass effect. In some cases there were demonstrable lesions only on contrast- enhanced scans. Contrast enhancement was thought to be related to the breakdown of the blood- brain barrier. Perhaps it resolved with clinical improvement, because inflammatory changes subsided and the blood- brain barrier was restored. As with most of the previous neuro- Beh<; et cases, enhancing hypodensity on the CT of our patient resolved markedly after a month with clinical improvement. Magnetic resonance imaging is superior to CT in detection and delineation of the neuro- Beh<; et lesions ( 11- 12,14- 20), as confirmed by postmortem examination in one case ( 20). The abnormalities are hyperintense on T2- weighted scans. Despite the detectable abnormalities on T2- weighted images, T1- weighted images were normal in some cases, and there were abnormalities ( usually with low signal) in some. Magnetic resonance abnormalities in the brain stem and diencephalic region had perplexing sizes in two previous cases, despite the few clinical signs ( 15- 16). These unexpected extensions of the abnormalities were attributed to the present edema since they resolved partially or rnmpletelv on repeat scans. Fascicular syndrome 1Clift Neuro- ophtluJlmDl. Vol. 11, No. 4, 1931 of the third cranial nerve in our case, with a few associated neurological signs in regard to the extensive hyperintensity on MRI and its late resolution, also verifies this observation. The MRI abnormality usually persists longer, in reduced sizes, than the corresponding CT abnormality. Occasionally/ a hypointense area that is attributed to the liquefying necrosis with an adjacent hyperintense residual area suggesting gliosis on T2- weighted images remains ( 14- 15). This finding is also true for our case. We present a case of neuro- Beh<; et's disease with CT and MR images. The MRl findings correlated with the clinical findings of a fascicular oculomotor nerve palsy with associated paresis of upgaze in contralateral eye. As clinical improvement occurred MRl abnormalities resolved. REFERENCES 1. Bogousslavsky J. Syndromes oculomoteurs resultant de lesions mesencephaliques chez l'homme. Rev Neurol 1989; 145: 546- 59. 2. Achard C. Levi L. Paralysie totale et isolee du moteur oculaire commune par foyer de ramollissement pedonculaire. Rev NeuroI1901; 9: 646- 8. 3. Shuaib E, Murphy W. Mesencephalic hemorrhage and third nerve palsy. J Comput Tomog 1987; 11: 385-- 8. 4. Keane JR. Isolated brain- stem third nerve palsy. Arch Neurol 1988; 45: 813- 4. 5. 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