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Show Journal of CliniCJll Neuro-ophthalmology 7(2): 98-103, 1987. Oculomotor Palsy and Papilledema with Pial-Dural Arteriovenous Malformation Sarkis M. Nazarian, M.D., Abdorasool Janati, M.D., Edgardo J. Angtuaco, M.D., and Walter M. Jay, M.D. © 1987 Raven Press, New York A 62-year-old man presented with papilledema, a cranial bruit, and a partial left oculomotor nerve palsy. Arteriography revealed a large mixed pial-dural arteriovenous malformation involving the superior sagittal and both transverse sinuses. After the superior part of the malformation was embolized, the patient's papilledema and ocular motility disturbance resolved. The oculomotor disturbance may have been a nonspecific sign of increased intracranial pressure. Cranial auscultation should be performed in all cases of papilledema and cranial nerve palsy. Key Words: Dural arteriovenous malformation-Oculomotor palsy- Papilledema. From the Neurology (S.M.N., A.J.) and Radiology (E.J.A.) Services, John 1. McClellan Memorial Veterans Hospital, Little Rock, Arkansas, and the Departments of Neurology (S.M.N., A.J+ Radi<110~ (E.J A.}.'.a~d ()P.hthalm~logy (W.M.J.), Uni-versity of Arkansas for·M~.~ Little Rock, Arkansas. .!\ciin'sB .~orTespondenceattdrep!llbfhequeststo Dr. Sarkis M. N~;':,,,],!:,, ;,jc·".t:·doljY Service 127-LR,iM~lan Memorial Veterans Hosp.. U';i, l.d;~ R,xk.. AR 72205 U.S.A, 98 Dural arteriovenous malformations are vascular anomalies clustered around intracranial venous sinuses, and these malformations derive their blood supply from arteries supplying the meninges. Their feeding vessels arise from end branches of the external carotid artery and meningeal branches of the internal carotid, ophthalmic, and vertebral arteries. These make up the dural arterial system, as opposed to the pial system, which is made up of those arteries which supply the brain itself. Dural arteriovenous malformations are estimated to make up 6-15% of all cerebral vascular malformations (1). They most commonly affect the transverse sigmoid sinus. Occasionally, they have a feeding vessel derived from the pial circulation and are more accurately called mixed pial-dural arteriovenous malformations. We report a patient with a partial left oculomotor nerve palsy, associated with intracranial hypertension and an extensive pial-dural arteriovenous malformation involving the posterior-superior group of sinuses. Following embolization of the superior sagittal portion of the malforma~ and decrease in the intracranial pressure, the papilledema and partial oculomotor palsy resolved. CASE REPORT A 62-year-old white man was admitted to the McClellan Memorial Veterans Hospital in Little Rock on November 20, 1985, with a history of frequent, bilateral temporal and occipital headaches for 1 year. He had been evaluated in May 1985 and found to have a normal computed tomographic scan of the head. Three months prior to this admission, he developed left retroorbital headaches which were. duJ):, throbbing, and almost continuous. They increasel PIAL-DURAL ARTERIOVENOUS MALFORMATION 99 with exertion, coughing, and upon assuming the supine position. One month before admission, he developed blurred vision on right gaze and while reading. The blurring was described as due to images being slightly offset. His wife had noticed that his eyes appeared crossed, and that his left eyelid seemed to droop at times. The patient also complained of a pulsatile humming noise in both ears. Past medical history was significant for chronic atrial fibrillation, and for a right brachial embolectomy in November 1985. His only current medication was quinidine sulfate 300 mg three times a day. Examination of the head was significant for prominent pulses in the superficial temporal and occipital arteries. Bruits were heard over both mastoid regions; these dampened with compression of the ipsilateral occipital artery. On ocular exam, visual acuity was 20/25 in both eyes, with normal color vision by Ishihara plates. Goldmann visual fields revealed slightly enlarged blind spots, greater in the right eye. There was a 1 mm anisocoria, with the larger left pupil somewhat sluggish to light and accommodation. Oph-thalmoscopic exam revealed moderate swelling of the optic discs, greater in the right eye. Extraocular movements were full in the right eye. In the left eye, there was decreased adduction and infraduction (Fig. 1). Eye closure was normal, and no fatiguable weakness was present. Ocular and neurologic exams were otherwise normal. Contrast-enhanced computed tomographic scans of the head, orbits, and cavernous sinuses were normal. Tensilon test was normal. On November 26, a lumbar puncture revealed an opening pressure of 32 cm H20, and closing pressure of 21 em H20. Protein was 33 mg/dl, and glucose was 87 mgldl. There were ten red blood cells and two lymphocytes per mm3 . VORL, cryptococcal antigen, and cytologic exam of the cerebrospinal fluid were negative. The patient's headaches improved moderately after the lumbar puncture but returned in a few days. A repeat lumbar puncture done on December 3 revealed an opening pressure of 40 cm H20. Routine examination of the cerebrospinal fluid was again normal. Cerebral angiography on December 4 revealed a FIG. 1. Composite photograph of eye movements in December. 1985. Note adduction and infraduction deficits in left eye. JClin Neuro-ophthalmol, Vol. 7, No.2, 1987 100 S. M. NAZARIAN ET AL. large arteriovenous malformation involving the superior sagittal sinus and both transverse sinuses. Supraselective arteriography revealed that supply to the malformation was from both superficial temporal arteries (Fig. 2) and both occipital arteries (Fig. 3), and also from a small branch of the right superior cerebellar artery (Fig. 4). The decision was made to avoid craniotomy and attempt to obliterate part of the malformation by supraselective embolization. On December 20, the patient had successful embolization of both superficial temporal arteries, with obliteration of the superior sagittal sinus component of the malformation. Immediately following the procedure, he had complete resolution of his headache and marked improvement of his tinnitus. His eye movements were unchanged. He was discharged the following day. Upon readmission on January 14, 1986, the patient reported that his diplopia had cleared 3-4 days after discharge, and that he had remained free of headaches. There was no longer any limitation of adduction and infraduction of the left eye, but the left pupil remained sluggish (Fig. 5). The papilledema had resolved. The cranial bruit was still present. A lumbar puncture revealeda. opening pressure of 26 em H20, but was othello'< wise normal. During a clinic visit on March 13, 1986, he coR plained of bilateral eyelid puffiness and red eyesil and was found to have mild chemosis and dilated; conjunctival veins. Ocular motility and optic diso& were normal. No proptosis was present. The tentporal artery pulse was bounding on the right ana absent on the left. The clinical impression Wil:$ ~ canalization of the thrombosed right superficial temporal artery, with increased blood flow into the arteriovenous malformation and developmem; of a dural-cavernous shunt. On August 14, 1986, he complained of incre~ in his pulsatile tinnitus, but was otherwise ~ His vision, optic discs, and eye movements w~ normal. His conjuctival. veins were mOde.'-:r,a:t, congested. The left pupil was now smaller .'. the right in the dark, but slightly larger in bri~ light. It was sluggishly reactive to light. 1Gin Neuro-ophthalmol, Vol, 7, No, 2, 1987 PIAL-DURAL ARTERIOVENOUS MALFORMATION 101 FIG. 3. Left straight sinus component of the malformation, visualized by contrast injection into the left occipital artery (arrow). DISCUSSION The case presented illustrates a transient ocular motility disturbance associated with intracranial hypertension due to a dural arteriovenous malformation of the posterior-superior group of sinuses. The pattern of extraocular muscle and pupillary involvement suggests a dysfunction of the inferior division of the oculomotor nerve. Tinnitus is the most common symptom of dural arteriovenous malformations of the transversesigmoid sinus, and the most common findings are cranial bruit and papilledema (2-4). In two series compiled from the literature, hemiparesis was found in 10-12% of cases, hemianopia in 5-7%, and cranial nerve palsies in 7% (2,3). The only previous report of oculomotor dysfunction was a complete palsy following subarachnoid hemorrhage (5). The inferior division of the oculomotor nerve usually branches off at the apex of the orbit and innervates the pupil, as well as the medial and inferior rectus and inferior oblique muscles. In the present case, the pupil and both the medial and inferior recti were clinically involved. Two possible mechanisms of oculomotor nerve dysfunction are compression and ischemia. The compression could be due to an anomalous vascular structure or to herniating cerebral tissue. Ischemia could be due to arterial spasm as a result of subarachnoid hemorrhage or represent a distant effect of the malformation on the local circulation. Dural arteriovenous malformations, because of their physical separation from brain tissue, rarely cause direct compression of cerebral structures (6). In the present case, none of the anomalous vascular structures were in close proximity to the left oculomotor nerve. Because the partial oculomotor palsy had cleared two months before signs of increased venous pressure within the cavernous sinus appeared in our patient, compression within the cavernous sinus is unlikely. There was no evidence of transtentorial herniation as a cause of oculomotor palsy in our patient. Subarachnoid hemorrhage, common in pial arteriovenous malformations, is seen in up to 20% of dural malformations (2). No evidence of a bleed was found in our case to explain the neurologic findings. The mechanism of focal cerebral ischemia in patients with dural arteriovenous malformations is an unsettled topic. Aminoff (7) believes a "steal" phenomenon, or shunting of blood away from brain tissue and to the malformation, is respon- I Clin Neuro-ophthalmol, Vol. 7, No.2, 1987 102 FIG. 4. Straight sinus components of the malformation, visualized by contrast injection into the right vertebral artery. The right superior cerebellar artery in indicated by an arrow. S. M. NAZARIAN ET AL. FIG. 5. Composite photograph of eye movements in January, 1986. TClin Neuro-uphthalmol, Vol. 7, No.2, 1987 PIAL-DURAL ARTERIOVENOUS MALFORMATION 103 sible. Another proposed mechanism is focal venous stagnation and chronic passive congestion of brain tissue (1,6). Intracranial hypertension, manifested as papilledema and headaches, was a major feature in our patient. Increased intracranial pressure due to superior sagittal sinus pathology is a well-known phenomenon (8). It was, therefore, decided to treat the patient initially by simply embolizing the superior sagittal component of the malformation. This was accomplished by supraselective embolization of both superficial temporal arteries and resulted in dramatic relief of symptoms, making further invasive procedures unnecessary. It is not surprising that selective embolization of the superior sagittal sinus component of the malformation provided relief of the headaches and papilledema in our patient. What is surprising is that the oculomotor disturbance simultaneously disappeared. This raises the possibility that the oculomotor disturbance in our patient was a nonspecific consequence of the intracranial hypertension. Although isolated reports of transient, unilateral, oculomotor palsy (9) and bilateral total (internal and external) ophthalmoplegia (10) in association with idiopathic intracranial hypertension have appeared in the literature, oculomotor palsy is generally not accepted as a false localizing sign in increased intracranial pressure. In the present case, it is impossible to know whether the oculomotor dysfunction was due to intracranial hypertension, to focal ischemia, to passive congestion, or to a combination of these factors. The purpose of this paper is to alert the clinician to a rather infrequent cause of papilledema, orbital congestion, and extraocular palsies. In particular, we recommend cranial auscultation in all cases of papilledema and cranial nerve palsies. Acknowledgment: The authors wish to thank Dr. Peter J. Savino of Philadelphia, Pennsylvania, for analysis and advice. REFERENCES 1. Houser OW, Campbell JK, Campbell Rl, Sundt TM. Arteriovenous malformation affecting the transverse dural venous sinus-An acquired lesion. Mayo Clin Proc 1979;54:651-61. 2. Obrador 5, Soto M, Silvela J. Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus. I Neur Neurosurg Psychiatry 1975;38:436-51. 3. Kuhner A, Krastel A, Stoll W. Arteriovenous malformations of the transverse dural sinus. I Neurosurg 1976;45: 12-9. 4. Newton TH, Weidner W, Greitz T. Dural arteriovenous malformation in the posterior fossa. Radiology 1968;90:2735. 5. Laine E, Galibert P, Lopez C, Delahousse J, Delandtsheer 1M, Christiaens JL. Anevrysmes arterio-veineux intraduraux (developpes dans I'epaisseur de la dure-mere) de la fosse posterieure. Neurochirurgie 1963;9:147-58. 6. Kosnik El, Hunt WE, Miller CA. Dural arteriovenous malformations. I Neurosurg 1974;40:322-9. 7. Aminoff MJ. Vascular anomalies in the intracranial dura mater. Brain 1973;96:601-12. 8. D'Avella 0, Greenberg RP, Mingrino 5, Scanarini M, Pardatscher K. Alterations in ventricular size and intracranial pressure caused by sagittal sinus pathology in man. I Neurosurg 1980;53:656-61. 9. McCammon A, Kaufman HK, Sears ES. Transient oculomotor paralysis in pseudotumor cerebri. Neurology 1981;31:182-4. 10. Snyder OA, Frenkel M. An unusual presentation of pseudotumor cerebri. Ann OphthaI1979;11:1823-7. 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