Two cases of idiopathic carotid-cavernous fistula with headache and ophthalmoplegia]

We report two cases of idiopathic carotid-cavernous fistula (CCF) with primary symptoms of headache and diplopia. A 47-year-old woman presented with throbbing headache in her right frontal region followed by right trochlear nerve palsy. Brain magnetic resonance imaging (MRI) was normal but magnetic resonance angiography (MRA) and computed tomographic angiography (CTA) revealed abnormal signals around the right cavernous sinus. CCF was diagnosed by conventional angiography. The symptoms improved naturally but after about 1 year she suddenly exhibited conjunctival congestion. A 41-year-old man complained of fluctuating headache with sudden left abducens nerve palsy. MRI and MRA were normal but CTA showed abnormal signals around the left cavernous sinus. A final diagnosis of CCF was made by conventional angiography and he was transferred to another hospital for stereotactic radiosurgery. Diagnosis of CCF tends to be delayed in cases presenting with only headache and external ophthalmoplegia. However, CCF with cortical vein drainage can lead to cerebral hemorrhage and early correct diagnosis is needed. Our cases showed a dilated superior ophthalmic vein in enhanced CT and an abnormal signal around the cavernous sinus in CTA. Therefore, CTA may be useful as a relatively non-invasive method that can provide diagnostic clues for CCF.     

A case of venous dural sinus thrombosis presenting dementia syndrome. An autopsy case

We report clinical and pathological features of a case of dementia syndrome due to dural sinus thrombosis. In three years before admission, the patient, a 64-year old man, had had four convulsive attacks; scattered calcification shadows were disclosed by plain CT. Ten months before the admission, he complained of progressive memory disturbance and dyscalculia. General physical examination showed no remarkable abnormalities except for bruit at the left mastoid process. Although his time orientation was poor, he was otherwise fully awake. Neurologically, memory disturbance, finger agnosia, and dyscalculia were observed. Right Barré's sign, and exaggerated right patellar tendon reflex were observed. Plain X-ray CT revealed calcification-like, scattered high-density areas in the floor of the cerebral cortical sulci. Enhanced CT showed abnormal vessel high-density areas in both the cerebrum and brainstem. Cerebral angiography showed thrombosis of bilateral transverse sinuses, arterio-venous fistula in the left transverse sinus, and remarkably dilated cortical veins over both cerebral hemispheres. Positron emission tomography revealed misery perfusion areas in bilateral cerebral hemispheres. We presume that the mass effect of venous engorgement and chronic ischemia due to the arterio-venous fistula caused the dementia syndrome. These clinical manifestations were improved by amantadine and the patient was discharged. During follow-up, he died of subarachnoid hemorrhage, and an autopsy was performed. Pathological findings were as follows: 1) cortical and subcortical multiple-infarction in the cerebrum, 2) hematoma in the subarachnoid space, 3) venous dilatation of the cortical veins and pseudocalcification of their walls, and 4) thrombus in the transverse dural sinus.     

A case of convexity subarachnoid haemorrhage from intracranial hypotension treated with epidural blood patch

The majority of subarachnoid haemorrhages (SAH) are of aneurysmal origin, and nontraumatic convexity SAH is rare. We describe one patient who had a convexity SAH caused by cortical veins thrombosis (CVT) secondary to intracranial hypotension (IH). We discuss possible pathophysiologic mechanisms and its treatment. A 52-year-old female presented with left hemiparesis and tonic–clonic seizures, following lumbar epidural injections of local anaesthetics and steroids because of chronic low back. Brain CT showed right fronto-parietal convexity SAH. Brain MRI revealed diffuse pachymeningeal enhancement and confirmed the SAH. Cerebral angiography showed no/low flow signal in few frontal and parietal cortical veins in the correspondence of the SAH. A restoration of flow signal in this area was revealed after 2?months. The patient was treated with lumbar epidural blood patch with recovery. Convexity SAH caused by CVT from IH may be treated only with lumbar epidural blood patch which eliminates by itself the determinants of CVT (vein dilatation and slow vein drainage).     

硬脑膜动-静脉瘘合并髓静脉扩张的临床及影像学表现(附1例报告及文献复习)

目的报道1例硬脑膜动-静脉瘘(DAVF)合并髓静脉扩张病例的临床资料,探讨DAVF合并髓静脉扩张的临床及影像学表现。方法回顾性收集1例DAVF合并髓静脉扩张患者的临床及影像学资料,并结合文献复习进行分析。结果患者以症状性癫痫起病。头颅MRI表现为脑白质内迂曲、扩张血管影,并继发性脑梗死和脑水肿;全脑数字减影血管造影(DSA)提示右侧颈内动脉造影静脉期上矢状窦前1/3不显影,静脉晚期颅内多发髓静脉迂曲增粗,经室管膜下静脉-大脑内静脉回流,右侧颈外动脉造影示DAVF形成,大脑上静脉逆向回流,左侧颈内动脉造影示左侧发育性静脉畸形。经血管内介入栓塞DAVF治疗后患者病情改善。结论DAVF合并髓静脉扩张少见,可继发脑实质病变进而出现神经功能缺损症状与体征;影像学表现为头颅MRI提示髓静脉扩张涉及的疾病谱较多,需仔细鉴别;MRI和DSA联合检查能更好评价脑实质和血管情况。     

Diagnosis of acute phase of venous infarction by diffusion-weighted image: case report and review of the literature]

Diffusion-weighted imaging (DWI) can diagnose early stage not only of the arterial infarction but also of venous infarction. We successfully diagnosed a case as acute venous infarction by DWI. The patient, an infant of one year and ten months, presented disturbance of consciousness and left hemiparesis two weeks after dehydration and infections. Computed tomographic scan revealed a cerebral hemorrhage in the right parietal lobe. Cerebral angiography revealed no contrast filling of the posterior side of superior sagittal sinus, straight sinus and transverse sinus. DWI demonstrated a large hyperintensity lesion around the hematoma, suggesting venous infarction in the early stage. We thought that venous infarction was caused by secondary extension of thrombus to cerebral cortical veins and deep cerebral veins. External decompression and postoperative hypothermia therapy were performed because of rapidly deteriorating intracranial hypertension. Intracranial hypertension was, however, uncontrollable. The patient died four days after the onset. Diagnosis of the venous infarction by DWI was discussed along with other recent reports. Diffusion hyperintensity was displayed in almost all subjects with acute venous infarction. DWI pattern of venous infarction is more heterogeneous than that of arterial one because pathway from venous obstruction to infarction is complicated.     

A case of chronic hypertrophic cranial pachymeningitis causing cranial polyneuropathy and unilateral central retinal vein occlusion]

We presented a 70-year-old woman who developed unilateral visual loss due to central retinal vein occlusion caused by chronic hypertrophic cranial pachymeningitis. She had developed right blindness with optic atrophy due to chronic intracranial pachymeningitis one year before admission. In June 1999, she noticed visual loss of the left eye. On admission, neurological examination revealed left visual loss and sensory impairment in the first branch area of bilateral trigeminal nerves. Ophthalmological examination revealed central retinal vein occlusion of the left eye. Brain MRI showed dural thickening with gadolinium enhancement of the cavernous sinus near the left optic nerve and dilatation of the left supraorbital vein. There was no compression of the left optic nerve. We suggest that central retinal vein occlusion may be caused by compression of the supraorbital vein by dural thickening. This is the first case report of central retinal vein occlusion associated with chronic hypertrophic cranial pachymeningitis.     

Thrombosis of the superior cerebral vein with hemorrhagic cerebral infarction--serial MRI and pathological study of a case

We reported an autopsy case of thrombotic occlusion of the superior cerebral vein with hemorrhagic laminar necrosis of the right parietal cortex. A 68-year-old woman was admitted to our hospital because of a severe headache and left hemiplegia of acute onset. There was a past history of hypertension, fever of unknown origin, leukocytopenia and nasal dermatitis. Magnetic resonance images (MRI) disclosed thrombosis of the superior sagittal sinus and of the right parietal cortical vein as well as right parieto-occipital cerebral infarction. Although she improved with mild sequelae, the subsequent MRI showed a recurrent thrombosis of the superior sagittal sinus. Ten months after the onset she died suddenly, presumably due to acute myocardial infarction. Pathologically, thrombotic occlusion of the right parietal cortical vein, recurrent thrombosis of the superior sagittal sinus and old hemorrhagic cortical laminar necrosis of the right parietal region were revealed. Moreover, intracranial arteritis and phlebitis were observed, as well as arteriolitis in the peripheral nerves. In our case, MRI was useful for the diagnosis and following the course of cerebral venous thrombosis. Cerebral noninfective vasculitis may well have caused the venous thrombosis.     

A case of leptomeningeal angiomatosis clinically improved by flunarizine

A 3-year-old boy was reported, who suffered from attacks of hemiconvulsion and hemiparesis. Carotid angiography showed anomalous cerebral venous drainage with a decrease in the number of superficial cortical veins, and dilatation of medullary veins and deep veins. Brain scanning demonstrated abnormality of regional cerebral blood flow and delay of both uptake and drainage of radionuclide. These are similar to the findings known in Sturge-Weber disease, so that a leptomeningeal angiomatosis was strongly suggested. Flunarizine which has a cerebral vasodilating effect improved his clinical symptoms significantly, and his attack disappeared completely.     

A case of brainstem vascular malformation with isolated trochlear nerve palsy as the initial symptom]

We report a 46-year-old, non-hypertensive man who suddenly developed isolated right trochlear nerve palsy. His diplopia was most prominent in the left lower gaze, and partially alleviated by head tilt to the left or by anteflexion of the neck. His CT scans showed a small high density area consistent with a hemorrhage in the lateral side of the right mesencephalic tectum. His MRI (T2-weighted images) showed a lesion consisting of mixed high- and iso-intensity areas with linear low intensity areas. The margin of the lesion was irregular and nodular. Cerebral angiography (prolonged injection) showed small feeding arteries (or capillaries) in the late arterial phase and dilated draining veins in the venous phase. No tumor stain, early draining veins, or capillary brushes were present. We thought he had an angioma (vascular malformation). AVM seemed unlikely. Review of the literature revealed that trochlear nerve palsy caused by a mesencephalic angioma is extremely rare. MRI and cerebral angiography (prolonged injection) seemed useful for the diagnosis of angiomas (Vascular malformations).     

Circulatory disturbance of deep cerebral veins in eclampsia

A case of eclampsia with interesting angiographic findings is reported. A 19-year-old woman in the 35th week of gestation by date was admitted due to a sudden onset of generalized clonic convulsion and disturbance of consciousness. The diagnosis was eclampsia. On the second hospital day, extraction of a stillborn female was performed by laminaria. Thereafter, the consciousness improved rapidly and she became alert on the following day. She was discharged without neurological deficit on the 18th hospital day. A CT scan on the day of admission showed narrow lateral ventricles and symmetrical low-density lesions in and around the basal ganglia. These had almost disappeared by the 10th hospital day. Carotid angiography on admission revealed no abnormality in the arterial phase including the lenticulostriate arteries, but, early appearance of deep cerebral veins and some cortical veins was noted. These deep veins, however, were not distinct even in the venous phase. These angiographic findings suggested medullary dilatation caused by circulatory disturbance of the deep cerebral veins. Most authors have stressed the contribution of diffuse arterial vasospasm in the pathogenesis of eclampsia in relation to low-density lesions on CT scans. In the present case, we could not find vasospasm but found circulatory disturbance of the deep cerebral veins. These angiographic findings suggested that the appearance of the low-density lesions on the CT scan was most likely due to venous congestion caused by circulatory disturbance of the deep cerebral veins, since most of the deep medullary veins in the low-density lesions flowed into the deep cerebral veins.(ABSTRACT TRUNCATED AT 250 WORDS)     

Two cases of brain infarction associated with dissection of the thoracic aorta]

Case 1: a 57-year-old woman, who suddenly developed disturbance of consciousness, left spatial neglect, and left hemiparesis, was admitted to our hospital on the day 1. Brain CT scan on the day 2 revealed broad edematous infarction in her middle cerebral artery territory. The dissection of the thoracic aorta was observed with transesophageal echocardiography and brain embolism due to mural thrombus of the dissecting thoracic aorta was suspected. Case 2: a 67-year-old woman, who developed disturbance of consciousness, left spatial neglect, and left hemiparesis, was admitted to our hospital on the day 15. Brain CT scan on the day 15 showed non-edematous infarction in her middle cerebral artery territory. Contrast thoracic CT scan on the day 31 revealed the dissection of the thoracic aorta. No clinical exacerbation was observed, but brain CT scan on the day 94 showed broad infarction in her middle and anterior cerebral artery territories. It is considered that pseudolumen of the dissected thoracic aorta caused occlusion of her right internal carotid artery. Transesophageal echocardiography, or contrast thoracic CT scan should be considered for detection of the dissection of the thoracic aorta in the cases of cryptogenic stroke.     

A case of primary subarachnoid hemorrhage due to cerebral amyloid angiopathy]

A 77-year-old man who was suffering from an intracerebral hemorrhage of the left subcortex without hypertension was admitted to our hospital. The only neurological symptom was right arm monoparesis. Brain MRI demonstrated a subarachnoid hemorrhage (SAH) in the left frontal lobe. On the day of admission, conventional cerebral angiography revealed no abnormalities in brain arteries. His symptom was disappeared immediately after admission. He was discharged without neurological deficit on day 25. However, he was rehospitalized in our hospital on the same day because he experienced a right subcortical hemorrhage. The neurological symptoms were consciousness disturbance, aphasia and right hemiparesis. Brain CT disclosed a subcortical hemorrhage in the left temporal lobe. CT stereo-guided drainage was performed. Then, we examined tissue removed from the brain's surface. Histologically, beta-amyloid protein was deposited on the walls of the meningeal and cortical vessels, and it replaced all the layers of those walls. Therefore, a diagnosis of cerebral amyloid angiopathy (CAA) was made. His condition gradually improved, but CT showed an asymptomatic ICH in the right parietal lobe on day 36. On day 47, he had a symptomatic ICH in the left caudate nuclei and right frontal lobe. He died on day 66 because of pneumonia. Intracranial hemorrhages due to CAA have been reported and the majority of the lesions have been lober hemorrhage. To the best of our knowledge, few reports have been published regarding primary SAH caused by CAA. The cause of SAH should be considered as CAA when SAH appears without hypertension or in elderly patients.     

A case of anterior cerebral artery dissection causing hemorrhagic infarction]

A 56-year-old man presented with weakness in his right arm and leg. Throbbing headache occurred several hours prior to his weakness. Brain CT obtained on day 3 demonstrated low density areas in the medial part of the left frontal lobe. Cerebral angiography on day 14 demonstrated dilatation and narrowing of the left anterior cerebral artery (ACA) corresponding to "pearl and string sign". The diagnosis of spontaneous ACA dissection was established with clinical features, laboratory findings, and angiographic findings. Antiplatelet therapy was undertaken for the prevention of ischemic events. Serial Brain CT demonstrated hemorrhagic change in the area of infarction. However, there was no definite clinical deterioration. Antiplatelet therapy was withdrawn after hemorrhagic change was noted. Cerebral angiography on day 35 showed improvement of both dilatation and narrowing. Possible reperfusion injury caused by absorption of intramural hematoma seems to be responsible for hemorrhagic change. In patients with cerebral infarction due to dissection of intracranial arteries, antithrombotic therapy is controversial as hemorrhagic complications including hemorrhagic infarction as well as subarachnoidal hemorrhage can occur. Further accumulation of cases is required.     

Locked-in syndrome due to bilateral cerebral peduncular infarctions with occlusion of persistent primitive trigeminal artery]

A 61-year-old woman with diabetes mellitus was admitted to our hospital with right hemiparesis and dysarthria. Brain MRI showed bilateral cerebral peduncular infarctions. Three days after admission, she was unable to generate any voluntary movements, except for those of the eye, suggesting locked-in syndrome (LIS). She could not speak, but showed good comprehension by blinking in response to verbal commands. Brain CT 5 days later revealed subarachnoid hemorrhage (SAH) around quadrigeminal and ambient cistern. Cerebral angiogram on the following day revealed no aneurysm, occlusion of right persistent primitive trigeminal artery (PPTA) and a little flow of the bilateral vertebral arteries. Eye movements were impossible in all directions on the 11th day and MRI showed new infarctions of the midbrain and the ventral portion of the pons. However, an EEG on the 20th day was almost normal. We speculated that low blood flow in the basilar artery from the PPTA caused bilateral cerebral peduncular infarctions, and that weakness of the PPTA caused SAH.     

Dyke-Davidoff-Masson syndrome manifested by seizure in late childhood: a case report.

The patient was a 19-year-old woman who presented with hemiatrophy and diminished superficial sensation on the left side of her body including her face. She had a past history of tonic-clonic seizures accompanied by left hemiparesis in late childhood. Brain CT demonstrated dilatation of the frontal sinus, calvarial thickening, cerebral hemiatrophy and dilatation of the lateral ventricle on the right side. Brain MRI showed atrophy of the right cerebrum and midbrain and dilatation of the lateral ventricle on T1-weighted images, as well as a high signal intensity area from the parietal to the occipital lobe on T2-weighted images. These findings are suggestive of an episode that may have caused a transient ischemia through the right cerebral hemisphere after the intrauterine period.     

Systemic lupus erythematosus manifesting as subarachnoid hemorrhage induced by cortical venous thrombosis and followed by medial medullary infarction]

A 43-year-old female, who had been treated for systemic lupus erythematosus (SLE), presented with a subarachnoid hemorrhage (SAH) induced by rupture of cortical venous thrombosis, and be followed by medial medullary infarction during the acute stage of the SAH. The patient initially manifested a SAH. Angiography demonstrated no evidence of any aneurysms or arteriovenous malformations, but revealed cortical venous thrombosis. She suddenly developed left hemiplegia caused by medial medullary infarction on the 6th day. An active anticoagulant therapy was thought to be inappropriate because of initial symptoms as a hemorrhage. Since she had been accompanied by the medullary infarction, then, initially started by antiplatelet therapy. After the confirmation of no saccular or dissecting aneurysms with 2nd angiography, her treatment could be changed to anticoagulant therapy. Because of the sustained negative reactions of anti-cardiolipin beta 2 glycoprotein I antibody and lupus anticoagulant during the course of SLE, the definite diagnosis of antiphospholipid syndrome (APS) could not be made. However, this case is pathogenically thought to be cerebrovascular disease based upon APS, considering that this syndrome may be related to various antigen/antibody systems.     

Slowly progressive dementia and multiple cerebral cortical infarctions following mitral valve replacement.

We describe the clinicopathological findings of a woman, 83 years of age at the time of death, who demonstrated dementia and numerous cerebral infarctions. She had a history of repeated mitral valve replacements 15 and 13 years prior to death and showed a dull responsive state 1 month after the second operation. Thereafter, dementia manifested and slowly progressed. Brain computed tomography revealed cortical atrophy and ventricular dilatation. Histological examination revealed a large number of minute foci of infarction in the cerebral cortex. Such lesions may have developed in association with the valve replacement and resulted in progressive dementia.     

Cerebrovascular symptomatic involvement in sarcoidosis

Clinical reports of cerebrovascular system involvement in sarcoidosis are extremely rare though pathological studies frequently describe granulomatous cerebral arterial and venous lesions. We report the case of a 47-year-old man with a history of pulmonary sarcoidosis at age 32 and abducens palsy at age 40, who presented cerebral pseudotumoral histologically proven sarcoidosis. He was admitted for acute left hemiplegia. Brain CT scan and MRI demonstrated a right posterior parietal haematoma associated with a superior sagittal sinus occlusion. He received intravenous corticosteroids and anticoagulant therapy. Six months later, he presented a right motor status epilepticus. MRI revealed new parenchymal haematomas. Cerebral angiography demonstrated cerebral vasculitis.     

Simultaneously Occurring Ischemic Stroke,Leg Artery Occlusion,and Pulmonary Embolism Induced by a Uterine Myoma

ObjectivesParadoxical embolism from venous thrombosis through the patent foramen ovale is a rare but well-known cause of stroke in young adults. Here, we report a case of simultaneous middle cerebral artery infarction, multiple occlusions of the leg arteries, and pulmonary thromboembolism from the venous thrombus, all due to compression of the external iliac vein by a uterine leiomyoma.Materials and Methods (Case presentation)A 44-year-old woman presented with left hemiparesis and central-type left facial palsy. She denied a history of hypertension, diabetes mellitus, previous cerebral infarction, myocardial infarction, smoking, or oral contraceptive use. The patient recovered completely after injection of tissue plasminogen activator. Brain diffusion-weighted imaging showed an acute right middle cerebral artery infarction. Transcranial Doppler with saline agitation test revealed a right-to-left shunt, suggesting a patent foramen ovale. Chest computed tomography revealed multiple pulmonary thromboembolisms. Lower extremity sonography and lower extremity computed tomography revealed a multifocal thrombus in the major veins and arteries of the left leg. Moreover, a large uterine myoma compressing the left external iliac vein was noted on lower extremity computed tomography.ResultsAfter the treatment of pulmonary thromboembolism and venous thrombosis with rivaroxaban, surgical thrombectomy of the left popliteal artery, patent foramen ovale closure, and total hysterectomy were performed. Subsequently, she had no recurrent paradoxical embolism or pulmonary thromboembolism.ConclusionStructural abnormalities in the pelvic cavity are not commonly suspected as stroke etiology. However, examination of the pelvic cavity is advisable in young female stroke patients with pulmonary thromboembolism or other paradoxical embolisms.     

Overlooked early CT signs of cerebral venous thrombosis with lethal outcome

The aim of this report is to emphasize the consequences of overlooked initial CT signs of cerebral venous thrombosis. Brain CT was ordered in an afebrile patient with neck pain and occipital headache. Since no abnormalities were noted on non-contrast CT study, the patient was discharged with recommendation for routine laboratory tests and plain X-ray of the cervical spine. Right hemiparesis developed the next day with persistent headache and the patient was sent back to the Neurology Clinic where he developed myoclonic seizures compatible with focal motor status epilepticus. Neuroimaging, performed two days later, revealed a huge hemorrhagic venous infarcts in the left posterior cerebral hemisphere associated with typical signs of dural sinus thrombosis. Subtle curvilinear hyperdensities were detected within the left parietal cortico-subcortical border zone on reevaluation of the initial brain CT. A posteriori these were thought to be compatible with a developing venous infarct, associated with subtle signs most consistent with combined cortical vein and sinus thrombosis. No improvement was noted after administration of anticoagulant treatment and the patient died 11 days after the initial CT scan. Detection of early CT signs of cerebral venous thrombosis is extremely important, since delaying adequate treatment may have catastrophic consequences.