Arteriovenous malformation (AVM) in the occipital lobe has been known to cause visual symptoms and headaches.

Clinical differentiation of migraine from arteriovenous malformation is often regarded as difficult, since both conditions may manifest similar features of visual aura, with or without headaches. Early diagnosis and treatment of AVM may reduce the risk of serious complications. When a patient manifests alarming headaches with or without visual aura, neuro-imaging workup is warranted to rule out sinister causes ¹⁾.

A 51-year-old woman with daily attacks of migraine with visual aura is described. The aura always occurred on the right and the headache always on the left side of the head, suggesting a structural lesion in the left occipital lobe. The lesion appeared to be an arteriovenous malformation of which almost full obliteration resulted in a decrease in frequency of the aura and in intensity of the headache. Subsequent treatment of borderline hypothyroidism with levothyroxine brought about a dramatic improvement in frequency of both the aura and the headache. The case is discussed in the light of our present understanding of the pathogenesis of the migraine attack ²⁾.

Resolution of migraine with aura caused by an occipital arteriovenous malformation ³⁾.

Pure alexia and prosopagnosia are two separate and uncommon disorders of visual recognition in neuro-ophthalmology. An extremely rare case of pure alexia coincident with prosopagnosia secondary to occipital arteriovenous malformation. The manifestations of these two visual recognition disorders are also described ⁴⁾.

A 43-year-old patient presenting with a highly congruous homonymous hemianopia was shown by neuro-imaging to have a very large arteriovenous malformation of the brain. The significance of finding this visual field defect, its an unusual cause with absence of symptoms other than longstanding migraine with aura ⁵⁾.

A 23-year-old woman presented with ipsilateral hemiparesia due to rupture of a left occipital arteriovenous malformation (AVM). Emergency decompression (the onset-operation interval was 46 minutes,) was carried out and the patient could leave the hospital upon recovery without neurological deficits. In general, Kernohan's phenomenon is caused by the gradual displacement of the cerebral peduncle against the tentorial edge caused by compression by the contralateral mass. This phenomenon is very rare among the cases with spontaneous intracranial hemorrhage and only three cases including the present one have been reported in the literature. In all cases the onset-operation intervals of were very short. Kernohan's phenomenon associated with a ruptured AVM is a rare condition and emergency decompression is required ⁶⁾.

Visual intraoperative monitoring of occipital arteriovenous malformation surgery ⁷⁾.

The authors report their experience on one patient with hypersexuality from resection of left occipital arteriovenous malformation. To the best of our knowledge, this is the first case reported in the literature. A 35-year-old right-handed female farmer suffered a sudden left occipital hemorrhage with subarachnoid and subdural hemorrhages of the left hemisphere. Transient left uncal herniation occurred at the onset and was released by conservative treatment. Digital subtraction angiography showed a brain left occipital arteriovenous malformation. After microsurgical resection of the arteriovenous malformation, the patient developed hypersexual behavior. Positron emission tomography showed hypermetabolism in the left frontal region and left posterior hippocampal gyrus and hypometabolism in the left anterior hippocampal gyrus and the left occipital surgical area. Theories concerning normal pressure perfusion breakthrough and specific areas in the brain responsible for the human sexual response are discussed ⁸⁾.

A 32-year-old right-handed female tour guide developed a transient prosopagnosia after the total removal of an arteriovenous malformation in the right occipital lobe. Neurological examination revealed only a left homonymous hemianopsia. The ablation on the right side involved total occipital lobe, the posterior region of the parahippocampal gyrus, and a part of the precuneus and angular gyrus. Neuropsychologically she was alert and oriented in time and place. No aphasia or apraxia was observed. Visual recognition of objects, colors and letters was normal. She could match faces and insects easily, despite a close resemblance among them. Her prosopagnosia resolved in one month, while her impairment in topographical orientation and nonverbal memory continued more than eight months. These findings exclude the possibility that her prosopagnosia was derived from visual agnosia, poor discrimination of details, or nonverbal memory deficit. Excluding cases with malignant brain tumor which may affect more diffuse area of the brain, there was only one reported case who developed persistent prosopagnosia after the right occipital lobectomy. Given the rarity of prosopagnosics with only right-sided lesions, it seems that in most of humans posterior parts of both hemispheres participate in the identification of faces ⁹⁾.

A 31-year-old healthy male presented with a rare case of cerebral arteriovenous malformation (AVM) manifesting as repeated ischemic attacks and cerebral infarction causing left sensori-motor disturbance. Neuroimaging revealed cerebral infarction in the right thalamus as well as right occipital AVM without bleeding. The AVM was mainly fed by the right angular artery, and the right posterior cerebral artery (PCA) showed mild stenosis and segmental dilation at the P(2)-P(3) portion. After referral to the hospital, transient ischemic attacks causing left homonymous hemianopsia, and left arm and leg numbness were frequently recognized. Additional imaging revealed a new ischemic lesion in the occipital lobe, and repeated cerebral angiography showed right PCA occlusion at the P(2)-P(3) segment. Cerebral AVM presenting with cerebral infarction due to occlusion of feeding arteries is rare. In this case, intimal injury due to increased blood flow or spontaneous dissection of the artery were possible causes. We should monitor any changes in the architecture and rheology of the feeding vessels during the clinical course to prevent ischemic complications ¹⁰⁾.

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10: Oran I, Parildar M, Memis A, Yunten N. Catheter and MR angiography of persistent trigeminal artery associated with occipital arteriovenous malformation. Comput Med Imaging Graph. 2000 Jan-Feb;24(1):33-5. PubMed PMID: 10739320.

11: Suzuki K, Yamadori A, Takase S, Nagamine Y, Itoyama Y. [Transient prosopagnosia and lasting topographical disorientation after the total removal of a right occipital arteriovenous malformation]. Rinsho Shinkeigaku. 1996 Sep;36(9):1114-7. Japanese. PubMed PMID: 8976140.