Alexia without agraphia following cerebral venous thrombosis associated with protein C and protein S deficiency

A 26-year-old right handed female was admitted to hospital with right homonymous hemianopia associated with alexia without agraphia. Her cranial magnetic resonance imaging and magnetic resonance angiography revealed a left occipital venous infarction due to thrombosis of the left transverse, sigmoid sinuses and the left internal jugulary vein. The underlying conditions were protein C and protein S deficiency associated with the use of oral contraceptives. To our knowledge, alexia without agraphia has never been described due to a venous infarction associated with hereditary thrombophilia in the literature.     

Alexia without agraphia following cerebral venous thrombosis associated with protein C and protein S deficiency

A 26-year-old right handed female was admitted to hospital with right homonymous hemianopia associated with alexia without agraphia. Her cranial magnetic resonance imaging and magnetic resonance angiography revealed a left occipital venous infarction due to thrombosis of the left transverse, sigmoid sinuses and the left internal jugulary vein. The underlying conditions were protein C and protein S deficiency associated with the use of oral contraceptives. To our knowledge, alexia without agraphia has never been described due to a venous infarction associated with hereditary thrombophilia in the literature.     

Cerebral venous thrombosis and homocystinuria: case report

Homocystinuria presenting as cerebral venous thrombosis is not usual. We report on a 13-year-old boy who was admitted to the hospital due to severe headache, nausea, vomiting and fever (38 degrees C). The patient was Marfan like and presented left hemiparesis and meningeal irritation sings. He was mentally retarded, had severe myopia, and had right lens dislocation one month before. Cranial CT scan was suggestive of cerebral venous infarct. MRI and magnetic resonance angiography showed venous infarcts more prominent in the right thalamic projection with hemorrhagic transformation and multiple foci of cortical (occipital and parietal bilaterally) deep parietal and left capsular bleeding, secondary of thrombosis of the transverse and sigmoid venous sinuses. High levels of homocysteine were detected in the blood and urine. Homocystinuria is an autosomal recessive inborn error of methionine metabolism caused by cystathionine-beta-synthase defect in most cases. We discuss the clinical and radiological findings in this patient, analyzing the pathophysiology of the thrombotic events related to homocystinuria.     

A case showing Wallerian degeneration of the bilateral middle cerebellar peduncles on MRI followed by the right pontine infarction]

We report a 63-year-old man who presented with the left facial palsy, the left hemiparesis, the left limb ataxia, and the bilateral truncal ataxia. On admission, magnetic resonance imaging (MRI) showed an abnormal high intensity lesion at the right paramedian region of the upper to middle pons on T2-weighted images (T2WI). He was diagnosed as having a pontine lacunar infarction. The contralateral cerebellar lesions were caused by involvement of the pontocerebellar fibers. On the 29th day from the onset, MRI showed the new abnormal high intensity lesions at the bilateral middle cerebellar peduncles on T2WI. These lesions were supposed to be Wallerian degeneration caused by involvement of the pontocerebellar fibers. This case suggests that Wallerian degeneration occurs followed by a unilateral infarction involving pontocerebellar fibers.     

A case of deep cerebral venous thrombosis with sudden convulsion after delivery]

A 25-year-old woman was admitted to our hospital because of convulsions after delivering a baby. Her neurological examination revealed a disturbance of consciousness and weakness in both arms. A brain magnetic resonance imaging scan (MRI) showed thrombosis of the left transverse sinus and many patchy high signals at bilateral basal ganglia and subcortical areas. A digital subtraction angiogram (DSA) of the brain revealed a defect at the left transverse sinus, congestion of the cerebral venous flow at the vein of Galen and vasospastic changes at both posterior cerebral arteries. After treating the patient with a venous infusion of heparin, nicardipine and phenytoin, her neurological deficits improved within a day. When MRI and DSA were repeated 2 weeks later, the abnormal lesions had disappeared. These findings suggest that venous thrombosis and vasospasm had the pathogenesis of eclampsia in this case.     

Babinski-Nageotte syndrome on magnetic resonance imaging

A 70-year-old woman developed left hypoglossal nerve palsy, a right hemiparesis sparing the face, and a typical left Wallenberg's syndrome. These symptoms resulted from a lesion in the left half of the medulla oblongata, suggesting Babinski-Nageotte syndrome, a rare cerebrovascular disease. This is the first case of ischemic infarction in the territory of the left vertebral artery and posterior inferior cerebellar artery demonstrated on magnetic resonance imaging. Severe bilateral lesions of the distal vertebral arteries demonstrated on digital subtraction angiography may have contributed to the development of this syndrome.     

磁敏感加权成像在出血性脑梗死诊断中的初步应用

目的 探讨磁敏感加权成像(SWI)诊断出血性脑梗死的临床价值.方法 20例亚急性期出血性脑梗死患者分别行常规MRI、扩散加权成像(DWI)和SWI检查,根据图像分析结果比较不同扫描序列所显示的梗死灶内出血灶数目及其阳性检出率;测量SWI序列出血最大层面出血灶面积和T2WI序列梗死灶最大层面的梗死灶面积,并行相关分析;观...     

磁敏感加权成像在出血性脑梗死诊断中的初步应用

目的探讨磁敏感加权成像（SWI）诊断出血性脑梗死的临床价值。方法20例亚急性期出血性脑梗死患者分别行常规MRI、扩散加权成像（DWI）和SWI检查,根据图像分析结果比较不同扫描序列所显示的梗死灶内出血灶数目及其阳性检出率;测量SWI序列出血最大层面出血灶面积和T2WI序列梗死灶最大层面的梗死灶面积,并行相关分析;观察SWI序列对梗死灶内静脉血管的显示程度,以及梗死灶以外区域微出血灶的诊断敏感性。结果20例患者SWI序列均显示梗死灶内出血,两名医师共诊断43个出血灶,其中SWI序列显示42个、T1WI序列25个、DWI序列15个、T2WI序列12个;SWI序列阳性检出率与T1WI、T2WI、DWI序列相比,差异具有统计学意义（X^2=51．516,P=0．000）。T2WI序列梗死灶最大层面的梗死灶面积为（18．08±12．47）cm^2,SWI为（5．02±6．27）cm^2,梗死灶面积与出血灶范围之间呈明显正相关（r=0．562,P=0．010）。其中,13例患者SWI序列检出梗死灶以外区域的微出血灶;12例显示梗死灶内小静脉血管分支减少和（或）变细,6例血管增多、增粗和（或）扭曲。结论SWI序列对出血性脑梗死病灶内出血的显示优于常规MRI和DWI序列,并能显示梗死灶内静脉血管的变化及梗死灶以外区域的微出血灶,可作为诊断出血性脑梗死的MRI常规扫描序列。     

小脑后下动脉解剖变异致双侧小脑梗死2例报道并文献复习

目的 探讨小脑后下动脉(posterior inferior cerebellar artery, PICA)解剖变异致双侧小脑梗死的临床特征及发病机制。方法 对2例经颅脑MRI确诊的双侧小脑梗死患者采用CT血管造影(CTA)、磁共振血管成像(MRA)或数字减影血管造影(DSA)显示其头颈部血管,从而了解后循环血管的形态特征并复习相关文献。结果 病例1经DSA证实左侧椎动脉较细,远端管腔闭塞,通过右椎动脉代偿供血原左侧PICA供血区但欠充分,双侧PICA共同起源于右侧椎动脉。病例2经CTA证实右侧椎动脉较左侧明显细且远端显示欠清,MRA示双侧PICA共同起源于左侧椎动脉。结论 2例双侧小脑梗死患者均存在一侧椎动脉优势供血,且双侧PICA共同起源于该侧椎动脉。在该解剖变异基础上一侧椎动脉发生病变时可出现双侧小脑梗死。因此,在临床中出现双侧小脑梗死时临床医师不能忽略这一解剖变异基础。     

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 bilateral cerebellar infarction in the distribution of the bilateral superior cerebellar artery]

We reported a case of bilateral cerebellar hemorrhagic infarction in the distribution of the bilateral superior cerebellar artery. A 58-year-old man suddenly developed dizziness and transient loss of consciousness. The neurological examination revealed left hearing disturbance, left sensory disturbance involving face, dysarthria and bilateral ataxia. This patient was considered to be classic clinical syndrome of right superior cerebellar artery. CT and MRI revealed hemorrhagic infarction corresponding to the full territory of the bilateral superior cerebellar artery. The right posterior cerebral artery was filling through the right posterior communicating artery on the right carotid angiography taken 2 hours after the onset. Bilateral vertebral angiography on the 18th day demonstrated no occlusions in the basilar artery and the bilateral superior cerebellar artery. Hemorrhagic infarction corresponding to the full territory of the bilateral superior cerebellar artery, sparing other territories as the present case, is extremely rare. In this case, cerebral embolism (top of the basilar syndrome) was suggested because of existence of atrial fibrillation and sudden onset.     

Acute cerebellar ataxia of a patient with SLE

We described a 28-year-old woman with systemic lupus erythematosus (SLE) presented with digestive tract, skin and renal symptoms and afterwards developed acute cerebellar ataxia, a paresis of the right inferior rectus muscle, left abducens paralysis and left facial palsy which seemed to be consistent with a brainstem lesion visible on magnetic resonance imaging (MRI). This lesion disappeared within 9 days of corticosteroid treatment. It is suggested that this lesion is focal edema induced by acute changes in the blood brain barrier secondary to a vasculopathy. Other causes, including local infarction, are unlikely.     

Ataxic hemiparesis and simultaneous supra- and subtentorial hematomas]

In a chronically hypertensive woman with a rapidly developing right ataxic hemiparesis syndrome, computed tomography and magnetic resonance imaging (MRI) revealed two small unrelated hemorrhages: one in the posterior limb of the left internal capsule, the other in the right cerebellar hemisphere. In this patient, the ataxic hemiparesis syndrome might have resulted either from the association of the two lesions or from the capsular hemorrhage alone. The rarity of simultaneous hypertensive hematomas and the value of MRI in the diagnosis are underlined.     

A case of cerebellar infarction presenting as thunderclap headache

Thunderclap headache (TCH) refers to a sudden-onset, severe headache that features in subarachnoid hemorrhage, unruptured intracranial aneurysm, cerebral venous thrombosis, pituitary apoplexy, cervical artery dissection, and hypertensive reversible posterior leukoencephalopathy. TCH is a rare manifestation in cerebral or cerebellar infarctions. Herein, we report on a 60-year-old woman with a thunderclap headache as the first symptom of cerebellar infarction, in the absence of abnormal findings in the brain computed tomography (CT), CT angiography, and lumbar puncture. An urgent brain MRI showed an acute infarction of the right cerebellar hemisphere. The next day, the patient presented with right side ataxia. In emergency cases presenting with thunderclap headache, one should consider an expanded evaluation and/or close observation, with frequent neurological examinations, even though the findings are normal on the initial neurological examination, cerebrospinal fluid analysis, and brain CT.     

An Unusual Case of a Thrombosed Giant Distal PICA Aneurysm Simulating a Large Cavernous Angioma

A 64-year-old woman was referred to our hospital with a one-month history of progressive headache. Magnetic resonance imaging (MRI) showed a hemorrhagic mass adjacent to the left inferior cerebellar hemisphere associated with a peripheral rim of signal void. Angiography demonstrated an avascular mass and the provisional diagnosis was a large cavernous angioma in the cerebellum. Intraoperative findings revealed a thrombosed giant aneurysm of the left distal posterior inferior cerebellar artery (PICA). We report an unusual case of a completely thrombosed giant aneurysm simulating a large cavernous angioma in the cerebellum. The cerebellar cisternal location of the mass may be a clue for the pre-operative diagnosis of an aneurysm.     

Venous infarction associated with carotid-cavernous fistula]

We report an 88-year-old woman who developed a hemorrhagic venous infarction in the left cerebral hemisphere and brainstem, in association with left carotid-cavernous fistula (CCF). Without aura the patient noticed diplopia due to left abducens palsy, and exophthal mos and congestion of the left eye. Brain CT revealed extrusion of the left eye, and dilatation of left superior orbital vein and cerebral cortical veins. She received diagnosis of CCF. Brain CT also revealed a small mass in the left ethmoidal sinus, which was not attached to the CCF. Biopsy of the mass was done under local anesthesia. On the following she had high fever. Her consciousness level deteriorated and she developed right hemiparesis FLAIR images of MRI showed, extensive high signal lesions in the left frontal and temporal cortices, basal ganglia, thalamus, midbrain and pons. These findings were consisted with venous infarction, possibly associated with peri-operative infection and hypovolemia. Intracranial hemorrhage occur in 3% of cases with CCF, but venous infarction was much rarer. The patients with CCF, who show dilatation of cortical veins in CT or MRI, have a higher risk of cerebral hemorrhage or infarction, and should be carefully observed.     

Spontaneous Hyperacute Postischemic Hemorrhage Leading to Death

Hyperacute, spontaneous, and severe parenchymal postischemic hemorrhage is considered rare but might be frequently misdiagnosed as primary intracerebral hemorrhage. The authors report 2 patients with catastrophic postischemic hemorrhage unrelated to anticoagulation, thrombolytics, or coagulopathy. Patient 1 was a 73-year-old woman with left posterior frontal lobe infarction, followed at 5.5 hours by massive postischemic hemorrhage leading to death. Patient 2 was a 52-year-old man with recent brain irradiation who developed left middle cerebral artery occlusion and basal ganglia infarction. Initial gradient-echo magnetic resonance imaging (MRI) showed petechial hemorrhage within areas of infarction and widespread old microbleeds. He developed massive postischemic hemorrhage at 12 hours, leading to death. The authors conclude that early brain imaging is essential to distinguish postischemic from primary brain hemorrhage. In patients with ischemic stroke, MRI findings such as hyperacute petechial hemorrhage or chronic cerebral microbleeds might predict subsequent parenchymal hemorrhage and thus have implications for tissue plasminogen activator use.     

Fibromuscular dysplasia of the vertebral artery presenting with lateral medullary syndrome: a case report

We have recently encountered a rare case of fibromuscular dysplasia (FMD) of the vertebral artery (VA) presenting as lateral medullary syndrome. A 39-year-old male was admitted to our hospital due to vertigo, dysarthria and numbness of the left face and the right limbs. A magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of brain revealed lateral medullary infarction in the territory of the left posterior inferior cerebellar artery (PICA). The angiography of the VA revealed tubular stenosis of the left extracranial VA and a focal vascular kinking as well as web in the right extracranial VA, confirming the diagnosis of FMD. We present this rare case to emphasize that FMD could be one of the risk factors causing lateral medullary syndrome in young people.     

Hemianopic ataxic hemiparesis. A case report.

Ataxic hemiparesis and homonimous hemianopia developed in a hypertensive 60-year-old woman. Magnetic resonance imaging detected multiple lesions in the territory of distribution of the posterior cerebral artery and in the inferior region of the left cerebellar hemisphere. At computed tomography only a right hypodense area involving the posterior right thalamus, the postero-medial region of the temporal lobe and the occipital lobe was evident.     

Magnetic resonance studies of brain lesions in patients with Kawasaki disease

We evaluated brain lesions in patients with coronary arterial lesions (CAL) as a complication of Kawasaki disease (KD) by magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). Among 47 patients who underwent coronary angiography for the evaluation of CAL due to KD at Kyushu University Hospital from April 1996 to September 2004, 24 patients were evaluated prospectively by brain MRI and MRA 0.1-21.2 years after the onset of KD. Although most patients had irritability or lethargy, none of them had significant neurological symptoms or signs during the acute phase, except one who showed neck stiffness. In one patient with no apparent neurological symptoms out of the 24 patients, brain MRI and MRA revealed right cerebellar infarction and obliteration of the right posterior inferior cerebellar artery, respectively. These results revealed the presence of cerebrovascular lesion in one of the 24 KD patients with CAL and suggested the need to consider the possibility of brain lesions in severe cases of KD with or without neurological symptoms.