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Show ! oumal of Clinical Nrun>- ophthalmology 9( 41: 249- 253. 1989 Dolichoectasia and Cranial Nerve Palsies A Case Report Julie R. Durand, M. D., and John R. Samples, M. D. < j) 1989 Raven Press. Ltd.. New York An elderly man with glaucoma and acute onset of leftsided cranial nerve III, V, and VII palsies was found to have associated marked intracranial artery dolichoectasia. Dolichoectasia ( arterial elongation and distension) affects the intracranial arteries, producing various neurological and ophthalmological findings. The patients are usually men who are more than 40 years old and have a history of hypertension. Diagnosis is made by characteristic radiologic findings. In patients with neurologic symptoms and signs suggesting a spaceoccupying mass, intracranial dolichoectasia should be considered. Key Words: Dolichoectasia- Cranial nerve palsiesGlaucoma. From the Department of Ophthalmology, The Oregon Health Sciences University, Portland, Oregon, U. s. A. Address correspondence and reprint requests to Dr. J. R. Samples at Department of Ophthalmology, The Oregon Health Sciences University, 3181 S. W. Sam Jackson Park Road, Portland, OR, 97201, U. S. A. 249 Dolichoectasia ( dolichos, long; ectatic, distended) ( 1) is a distinct disease entity affecting the arteries which may present clinically as an intracranial space- occupying lesion ( 1). Multiple reports of cranial nerve palsies ( III- XII) associated with dolichoectatic intracranial arteries are found in the nonophthalmological literature ( 1- 10), but a recent search revealed only one such report ( using different nomenclature) noted in the ophthalmology literature ( 11). We report a case in which a patient experienced an acute onset of left- sided third, fifth, and seventh cranial nerve palsies associated with marked intracranial dolichoectasia. CASE REPORT A 74- year- old white man with a long- standing history of hypertension, alcoholism, and primary open angle glaucoma was being followed in the eye clinic for glaucoma, when a rise in intraocular pressure associated with progression of visual field loss was noted in both eyes. The patient was scheduled for a laser trabeculoplasty, but in the interim he presented to an otolaryngologist with left- sided otalgia and otorrhea, which were diagnosed as symptoms of otitis extema. At that visit ( which was 11 days after his last eye exam) the patient complained of diplopia and instability when walking. The patient denied any past history of diabetes or symptoms that would suggest giant cell arteritis. Re- examination of the patient revealed a marked limitation of the left eye in supraduction and adduction and a slight decrease in infraduction ( Fig. 1 top). Abduction was normal, as was incycloduction in downgaze. Marked ptosis on the left was also apparent. Pupillary involvement was indeterminate because the patient was using 4% pilocarpine drops. Decreased corneal sensitivity was also noted on the left and hyperesthesia was found over the left brow and cheek. No 250 ]. R. DURAND AND]. R. SAMPLES FIG. 1. ( Top row) Note limitation of the left eye in supraduction, adduction, and infraduction, which suggests a third cranial nerve palsy. ( Bottom row) Note the normal extraocular muscle movements in up, right, and down gazes observed 6 weeks later. definite seventh nerve involvement was noted by voluntary muscle contraction, although the patient had a slightly asymmetric facial appearance with some loss of skin lines on the left. No proptosis was noted. Because of the apparent involvement of cranial nerves III, V, and VII, a computed tomographic ( CT) scan was obtained to rule out an inflammatory or infectious process of the petrous area, orbit, and/ or cavernous sinus versus an aneurysm. The CT scan showed clear sinuses, a probable old right medial orbital wall fracture, but no evidence of a mass lesion in the region of the left caverous sinus or orbital apex. However, there was dolichoectasia of the great cerebral vessels, including the right vertebral artery, basilar artery, and both carotid arteries. The basilar artery bifurcation was noted to be markedly elevated, lying at the level of the mid portion of the third ventricle on the left. There was marked leftward displacement of the medulla by the enlarged tortuous right vertebral artery ( Fig. 2). No evidence of a focal aneurysm was noted and the lesion was felt to be inoperable. Magnetic resonance imaging ( MRI) was also obtained, which again showed marked tortuosity of the basilar artery, with its tip indenting the hypothalmus ( Fig. 3). The cerebral peduncles were displaced superiorly and dorsally, and the third nerves appeared to be stretched, particularly on the left ( Fig. 4). Enlargement of the carotid arteries was also noted. JClin Ncurv- opllltwi",,, I, Vol. ' i. No.. 1. J989 One week later the patient was again seen with a marked seventh nerve palsy on the left, in addition to a persistent third and fifth nerve weakness ( Fig. 5). The Department of Neurosurgery at The Oregon Health Sciences University was consulted, but no treatment or further imaging ( including an- FIG. 2. This axial CT image of the midposterior fossa at the junction of the pons with the brainstem shows enhancement in the right vertebral and carotid artery, and the dilated basilar artery. DOLICHOECTASIA AND CRANIAL NERVE PALSIES 251 FIG. 3. This coronal T- 1 weighted MRI image shows marked tortuosity of the vertebrobasilar vessels ( arrows) with indentation of the hypothalamus ( star). giography) was advised. Therefore, no cerebral angiogram was obtained. Six weeks after the onset of his diplopia, much less ptosis was evident and marked improvement of the extra- ocular muscle movement was noted. Approximately 3 weeks later, the patient returned for followup with no further complaints of diplopia. Extra- ocular muscle movements were full ( Fig. 1 bottom), and only a slight ptosis and facial droop remained ( Fig. 6). Also, no paresthesia or hyperesthesia was noted over the distribution of cranial nerve V. Followup since that time has been sporadic, with poor patient compliance and refusal of the patient to see a neurologist for further workup. However, blood glucose was found to be within normal limits ( 98). Blood pressure was last noted to be elevated at 160/ 104, and the Department of General Medicine is continuing to follow the patient for hypertension. DISCUSSION Dolichoectasia ( elongation and distension) of the arteries is an uncommon condition ( 3,7,12,13) that may affect the intracranial arteries. Yu et al. ( 7) noted the rarity of this condition when reviewing - 50,000 carotid and vertebral angiograms performed in Queen Square, London, England from 1959 to 1980. Of these, only 29 cases demonstrated unequivocal arterial ectasia. In another report, Nijensohn et al. ( 3) found 23 patients diagnosed with basilar artery ectasia among all autopsies performed from 1952 to 1972 at the Mayo Clinic, Rochester, MN, U. s. A. First termed dolichoectasia in 1969 ( 1), this condition is considered by some to be a distinct disease entity. Sacks and Lindenburg ( 1) studied the histopathology of the affected arteries and found gaps in the internal elastic membrane and thinning of the media, which suggested a degenerative process. The same authors noted that dolichoectasia can occur without evidence of arteriosclerosis and they proposed that the disease may actually be present for many years without clinical symptoms. They speculate that stress, such as high blood pressure, may further enlarge the arteries and produce symptoms that suggest an intracranial mass. Other authors ( 2,7) describe the anomaly as being most common in males > 40 years old and as being found in association with hypertension ( 7,13). The clinical picture of dolichoectasia of the intracranial arteries can be quite varied. Boeri and Passerini ( 2) noted the frequent presence of an extensive third cranial nerve paresis on the side corresponding to the convexity of the curve of the artery. In addition to multiple cranial nerve palsies ( 1- 10), symptoms and signs associated with dolichoectasia of intracranial arteries include retinal ischemia ( 7), hydrocephalus ( 7,10,14), chiasmaI compression ( 7), headache ( 6), tinnitus ( 6), hemiparesis ( 6), paresthesias ( 6), trigeminal neuralgia ( 9,15), seizure ( 12), senile dementia ( 14), papilledema ( 13), nystagmus ( 13), ataxia ( 13), isolated envi- , Clill Neuro- ophthalmol, Vol. 9, No. 4, 1989 252 f. R. DURAND AND f. R. SAMPLES FIG. 4. In this axial proton density MRt image of the midposterior fossa, the bright signal evident is slow- flowing blood in the right vertebral artery ( arrow) with marked indentation of the brainstem. Note the convexity of the curve is on the side of the clinical findings. ronmental tilt ( 16), and hemifacial spasm ( 10,17). A finding in the evaluation by Smoker et al. ( 10) of 22 patients diagnosed with isolated hemifacial spasm deserves special emphasis: vertebrobasilar dolichoectasia was demonstrated by CT in all of their 22 cases. The diagnosis of dolichoectasia of the intracranial arteries is determined by radiologic findings ( 2,10,13,17- 22). Numerous measurements can be made using angiography that correspond closely AG. 5. Note marked left- sided ptosis and facial droop f () f" sistt> nt with cranial nerve III and VII palsies. ] Clin Neuro- ophthalmol, Vol. 9, No. 4, 1989 to autopsy data ( 2). However, characteristic CT findings are considered diagnostic, making invasive procedures unnecessary in many cases ( 10,13). MRI has also been used ( 16). The prognosis of dolichoectasia varies. In one report ( 7) mortality ( defined as death in followup period ranging from 1 month to 17 years) for ca- FIG. 6. Six weeks after onset, much less ptosis and facial asymmetry are evident. DOLICHOECTASIA AND CRANIAL NERVE PALSIES 253 rotid ectasia was 45% and for vertebrobasilar ectasia was 63%. In some cases, surgical correction of the anomalous vessel can be followed by relief of some symptoms ( 23,24), and still many others are found to be inoperable ( 5,10). Others may respond to antihypertensive drugs or anticoagulants ( 7). SUMMARY An elderly man with glaucoma and hypertension presented with acute onset of left- sided cranial nerve III, V, and VII palsies and was found to have marked intracranial artery dolichoectasia. The brainstem was displaced and the convexity of the curve of the artery was shown to correspond to the side of the clinical findings. Dolichoectasia is a rare disease process that can affect the intracranial arteries, producing various neurological and ophthalmological findings. The patients are usually males > 40 years old with a history of hypertension. In most instances, diagnosis can be made by CT scan or MR!. However, although dolichoectasia has a persistent effect on the intracranial arteries, our patient had symptoms for < 9 weeks. Transient cranial neuropathies may occur in the setting of diabetes, hypertension, or other forms of ischemia. Diabetic ophthalmoplegia clears within 3 months and often within 6 weeks. However, our patient had no history of diabetes or abnormal blood sugar. Also, there were no constitutional symptoms consistent with giant cell arteritis. ( However, a sedimentation rate was not obtained.) Our patient has a long history of high blood pressure, but hypertension is often found in patients with dolichoectasia. We cannot explain the transient nature of nerve palsies in the presence of dolichoectasia. However, we also note that hemifacial spasm may be intermittent, yet it is associated with dolichoectasia ( 10,17). 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