Progression of stenosis into occlusion of the distal posterior cerebral artery supplying an occipital arteriovenous malformation manifesting as multiple ischemic attacks: case report.

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 our 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 our 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.

Delayed parenchymal hemorrhage following successful embolization of brainstem arteriovenous malformation. Case report.

A 64-year-old man presented with subarachnoid hemorrhage from a small brainstem arteriovenous malformation (AVM). Cerebral angiography showed a small AVM in the lateral midbrain, which was fed by a basilar perforating artery, and drained into the right transverse pontine vein and superior petrous vein. Endovascular embolization in the acute stage was selected to occlude the arteriovenous shunt and provide additional intensive treatment for cerebral spasm with lower risk of rebleeding. The AVM was occluded by embolization using n-butyl cyanoacrylate. Intraparenchymal hemorrhage in the ipsilateral pons was detected 1 month after treatment. The causes of the hemorrhage remain unclear.

Elevated concentrations of sphingosylphosphorylcholine in cerebrospinal fluid after subarachnoid hemorrhage: a possible role as a spasmogen.

This study investigates the role of sphingosylphosphorylcholine (SPC) in the mechanisms underlying cerebral vasospasm after subarachnoid hemorrhage (SAH). The levels of SPC were measured in cerebrospinal fluid (CSF) of patients with SAH and also in an experimental canine model. CSF samples were collected from 11 patients with SAH, and from dogs that had received an injection of SPC into the cisterna magna to examine SPC kinetics in the CSF. SPC was assayed using solid-phase extraction and triple quadrupole mass spectrometry. The SPC concentrations in SAH patients on days 3, 8, and 14 after the onset of SAH were significantly higher than those in normal CSF. In the canine model, rapid dilution of SPC in CSF was observed. In combination with data from previous studies, these results suggest that SPC is involved in the development of cerebral vasospasm. Rapid dilution of SPC in CSF suggests that SPC is released into CSF at higher concentrations than those measured in the present study.

Cause of hematic cysts of the orbit: increased fibrinolysis and immunohistologic expression of tissue plasminogen activator.

PURPOSE: Hematic cysts of the orbit are relatively uncommon. These cysts expand gradually, leading to progressive orbital symptoms. To clarify the cause of hematic cyst, especially the mechanisms leading to its gradual expansion, we investigated the immunohistologic expression of tissue-type plasminogen activator (tPA), a key enzyme of fibrinolysis, in cases of hematic cysts. DESIGN: Retrospective small case series. PARTICIPANTS: Three patients with hematic cyst of the orbit were studied. METHODS: Three surgically removed hematic cysts were studied from a histologic perspective, including immunohistochemistry for tPA. The cyst content was also analyzed chemically. MAIN OUTCOME MEASURES: The pathologic features of hematic cyst of the orbit, expression of tPA in the cyst wall, and coagulation-fibrinolytic factors in the content of the cyst. RESULTS: The cyst wall was composed of dense collagen fibers lacking an epithelial lining and contained many fine capillary- or venule-like vessels. Hemosiderin-laden macrophages were observed among the collagen fibers. Strong immunoreactivity for tPA was revealed in the endothelial cells of the vessels in the cyst wall. Chemical analysis of the cyst content revealed a marked delay in the activated partial thromboplastin time and prothrombin time, a low fibrinogen concentration, and high concentrations of the D-dimer and tPA-plasminogen activator inhibitor-1 complex. CONCLUSIONS: Our findings indicate that blood coagulation and hemostasis occur first and that fibrinolysis occurs second in hematic cysts. Gradual expansion of the cyst may be due to hyperfibrinolysis resulting from oversecretion of tPA from the endothelial cells in the cyst wall, thus impairing normal hemostasis. Hemorrhage may then recur, resulting in enlargement of the hematic cyst. These mechanisms are similar to those occurring in chronic subdural hematomas. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

[Surgical treatment for intractable epilepsy caused by tuberous sclerosis complex with multiple tubers: case report].

We report a case of intractable epilepsy caused by tuberous sclerosis with multiple tubers that was treated successfully by single resection of a tuber A 2-year-old boy developed tonic spasm-like seizures at the age of 4 months and the frequency of the seizures increased despite therapy with various anticonvulsants. He had no neurological deficits, and his intelligence was normal. MRI showed lesions in the left frontal and the right parietal lobe, and EEG (electroencephalogram) demonstrated sharp waves and spike-and-wave complex bursts over the right parietal region. Subdural electrodes were implanted over these lesions, and ictal low-voltage fast waves were demonstrated on the right parietal lesion. Therefore the right cortical lesion and the epileptogenic cortex were resected. Multiple subpial transaction was also added in the irritative cortex. Histological examination proved that the lesion was a tuberous sclerosis complex (TSC) tuber. Since surgery, the patient has remained seizure-free for 2 years. In patients with multiple TSC tubers in the cortex, we suggest that seizures can be suppressed by single resection of a tuber if the tuber can be identified as epileptogenic before surgery.

Angiogram-negative subarachnoid hemorrhage in the era of three dimensional rotational angiography.

BACKGROUND: Three dimensional rotational angiography (3DRA) is a powerful method for depicting intracranial vascular lesions because of its 3D imaging capability. The purpose of this study was to analyse if 3DRA had reduced the incidence of angiogram-negative subarchnoid haemorrhage (SAH) and which type of aneurysm tended to be overlooked with conventional digital subtraction angiography. METHOD: Angiogram-negative SAH was defined as present in those patients with SAH who had no demonstrable lesion revealed by more than two adequate cerebral angiograms. From January 1, 1992, to December 31, 2004, angiography was performed on 247 patients at the Yamaguchi University Hospital. Digital subtraction angiography (DSA) alone was used for 105 patients (DSA group) from 1992 to 2000. After the technology of 3DRA was introduced to our hospital in 2000, 142 patients were evaluated by 3DRA and DSA together (3DRA group). FINDINGS: The incidence of angiogram-negative SAH was 9/105 (8.6%) in the DSA group and 6/142 (4.2%) in the 3DRA group. 3DRA revealed six aneurysms not depicted by the conventional DSA, including a basilar tip aneurysm, anterior cerebral artery aneurysm and a basilar tip aneurysm originating from a previously clipped aneurysm. CONCLUSION: Three dimensional RA is more sensitive in detecting aneurysms, but in our study still produced a 4.2% rate of angiogram-negative SAH. Three dimensional RA has some advantages for evaluation, especially of complicated sites and previously clipped aneurysms because of its three dimensional imaging capability.

Surgery for spontaneous intracerebral hemorrhage has greater remedial value than conservative therapy.

BACKGROUND: The aim of this study was to compare the efficacy of surgery for spontaneous intracerebral hemorrhage with that of medical treatment, based on data from the Japan Stroke Registry Study. METHODS: From 1999 to 2001, 1010 patients with spontaneous intracerebral hemorrhage were registered in the Japan Standard Stroke Registry Study from 45 stroke center hospitals in Japan. The National Institutes of Health Stroke Scale (NIHSS), Japan Stroke Scale (JSS), and modified Rankin Scale scores were used to compare severity and improvement in patients given surgical and medical treatment. CONCLUSIONS: Surgically treated patients, especially those with cerebellar hemorrhage, had significantly greater improvement in NIHSS or JSS score compared with medically treated patients. Our findings indicated that the patients who underwent surgery appeared to have better outcomes. But, because the study was not randomized, this observation cannot be interpreted as indicating that surgery is advantageous.

Management of patients with cavernous angiomas presenting epileptic seizures.

BACKGROUND: The aim of this study was to establish a treatment strategy for patients with cavernous angiomas and have seizures. METHODS: We analyzed retrospectively 31 patients with cavernous angiomas presenting with seizures. Of the 31 patients, 15 (11 men, 4 women) had been treated medically (the medical group) and 16 (11 men, 5 women) had been treated surgically (the surgical group). The percentage of patients who became seizure-free and the number of anticonvulsants used were compared between these 2 groups. The mean follow-up period was 5.3 years (range, 7 months-10 years). There was no statistically significant difference between the 2 groups with regard to the number of patients, sex, age, and follow-up period. RESULTS: The percentage of patients who became seizure-free was higher in the surgical group than in the medical group (80% vs 19%, P < .01). The number of anticonvulsants required was reduced in the surgical group after surgery (from 1.5 +/- 0.7 to 1.1 +/- 0.8, P < .01) and was not reduced after in the medical group. CONCLUSIONS: Surgical intervention may have greater benefits than medical intervention for patients with cavernous angiomas presenting with epileptic seizures.

Multiple aneurysms arising from the posterior inferior cerebellar artery with unknown etiology--case report--.

A 67-year-old man presented with multiple aneurysms arising from the caudal loop of the posterior inferior cerebellar artery (PICA), possibly as a result of blunt trauma. Computed tomography of the head revealed subarachnoid hemorrhage in the posterior fossa and sylvian fissure. Repeated angiography demonstrated an aneurysmal dilatation and an irregular wall on the caudal loop of the PICA. Under the operating microscope, two lesions were observed 10 mm distal to the apex of the caudal loop, both consisting of a tiny hole on the vessel wall with a fragile fringe of connective tissue and covered with a firm clot. The height of the lesions corresponded to the C-l lamina, so the lesions were probably traumatic rather than saccular.

Chlamydia pneumoniae infection as a risk factor for subarachnoid hemorrhage.

Many risk factors for subarachnoid hemorrhage (SAH), such as hypertension and recent infection, have already been established. Chlamydia pneumoniae is a common respiratory pathogen that has been implicated as a potential risk factor for hypertension and atherosclerotic diseases. The purpose of the case-control study presented here was to examine the causative relationship between C. pneumoniae infection and SAH. Serum C. pneumoniae IgG and IgA antibodies were measured using an enzyme-linked immunosorbent assay in 52 patients with SAH and in 104 healthy age-matched control subjects. Multivariate analysis revealed a significant association between SAH and both a strong seropositivity for IgG and/or IgA antibodies against C. pneumoniae (odds ratio, OR, 3.62; 95% confidence intervals, CI, 1.06-12.39; p = 0.040), which indicates the presence of acute C. pneumoniae infection, and hypertension (OR, 2.91; 95% CI, 1.42-5.96; p = 0.0035). These results provide evidence that infection with C. pneumoniae may be a risk factor for SAH from a ruptured intracranial aneurysm.

A marked exophthalmos and corneal ulceration caused by delayed massive expansion of a subgaleal hematoma.

CASE REPORT: A 12-year-old girl had the hair on the right side of her head pulled during a quarrel, after which a subgaleal hematoma (SGH) developed over her right cranium. The subcutaneous swelling progressed to the forehead, and a marked exophthalmos then developed on the left side. The bilateral, liquefied SGH was removed surgically, and two drainage catheters connected to a vacuum-drain pump were introduced. After the surgery, the SGH disappeared. The liquefied hematoma was aspirated by puncturing the superolateral portion of the left orbit. Thereafter, the left exophthalmos rapidly disappeared. A chemical analysis of the SGH revealed that it contained extremely low levels of fibrinogen and platelets, and high levels of fibrinogen and fibrin degradation products, suggesting that secondary fibrinolysis had occurred in the subgaleal space. DISCUSSION AND CONCLUSION: Subgaleal hematomas are usually treated conservatively. However, closed observation is necessary, and if increased expansion is seen, aspiration with a closed drainage system should be considered.

[A case of intractable epilepsy presenting epileptic spasm treated with callosotomy in childhood].

We present a case of intractable epilepsy presenting epileptic spasm successfully treated with anterior callosotomy in childhood. A 12-year-old boy had seizures frequently since the age of 11 months which were diagnosed as an infantile spasm. He underwent various treatments including ACTH therapy, but the seizures were intractable. Characteristics of his seizures were an abrupt axial movement leading to sustained posturing, most often truncal, and neck flexion with pronounced arm extension and abduction. He lost consciousness transiently and these seizures frequently occurred in clusters and the duration of seizure was 1 to 3 seconds. Neurologically he had no deficit, but his intelligence was below the standard level. Magnetic resonance image (MRI) showed no abnormalities in the brain. Electroencephalogram (EEG) demonstrated secondary bilateral synchrony (SBS) with left frontal dominance in amplitude. Subdural strip electrodes were implanted bilaterally over the frontal lobes to detect an epileptogenic region. However intracranial ictal-EEG demonstrated an abrupt bilateral desynchronization for 1-3 seconds. As the epileptogenic zone was not clearly identified and an abrupt bilateral desynchronization was identified, anterior callosotomy was performed. Since surgery, he has remained seizure-free for two years while taking anticonvulsants and the SBS has disappeared. This case may indicate that the corpus callosum influences the occurrence of epileptic spasm and SBS on EEG.

[A case of Rathke's cleft cyst in association with bilateral unruptured aneurysms of internal carotid artery].

Recent progress in diagnostic imaging techniques, such as MRI and CT, has shown that Rathke's cleft cyst is more common than once thought. However, few cases have been reported in which a cerebral arterial aneurysm was merged with a symptomatic Rathke's cleft cyst. We present a case of bilateral internal carotid aneurysms associated with a symptomatic giant Rathke's cleft cyst. A 66-year-old-woman with a visual disturbance was admitted to our hospital. CT and MRI showed a large mass in the frontal base to the suprasellar region. The mass showed isosignal intensity in T1-weighted images (WI), a ringed enhancement with Gd-DTPA, and hyperintensity in T2WI. An angiography showed bilateral A1 elevation but no tumor stain. In addition, unruptured aneurysms appeared in the inside back C2 portion of the bilateral internal carotid arteries. For these lesions, a bifrontal craniotomy was performed. In a single operative approach, the mass lesion was removed and the aneurysms were successfully clipped. The aneurysms adhered strongly to the wall of the mass. The histological diagnosis of the mass was Rathke's cleft cyst. A precise pre-operative understanding of the anatomical structure and a careful operative procedure are important for the treatment of these complex lesions.

Increased matrix metalloproteinase-9 in blood in association with activation of interleukin-6 after traumatic brain injury: influence of hypothermic therapy.

Recent experimental data have shown that levels of matrix metalloproteinase-9 (MMP-9) increase after traumatic brain injury (TBI), degrading components of the basal lamina disrupting the blood-brain barrier. However, the post-traumatic secretion patterns of MMP-9 in humans are unknown. We measured the concentration of MMP-9 in plasma after TBI at the same time as the concentration of interleukin-6 (IL-6) in serum. Levels of MMP-9 and IL-6 in systemic arterial and jugular venous blood from seven patients with TBI were measured on days 0 and 1 post-injury. All patients underwent hypothermia at 32-35 degrees C as soon as possible after admission. Before induction of hypothermia, levels of MMP-9 in arterial and internal jugular venous blood exceeded the normal range. Higher MMP-9 levels were detected in internal jugular venous blood than in arterial blood. After hypothermia had been induced, MMP-9 levels in arterial blood and internal jugular venous blood decreased significantly, to within the normal range. In addition to these changes, a significant correlation was seen between levels of MMP-9 and IL-6 in internal jugular venous blood during the investigation period. These results indicate that MMP-9 is elevated in patients with acute TBI, and may play an important role in traumatic brain damage. The elevation of MMP-9 is associated with inflammatory events following TBI. Hypothermic intervention may suppress the elevation of MMP-9 with suppression of the inflammatory response, affording neuroprotection in TBI.

[Surgical reconstruction of the common carotid artery by an ePTFE graft for invasive thyroid cancer: a case report].

Surgical reconstruction of the carotid artery using a synthetic graft is rarely performed. A 71-year-old woman developed hoarseness and was diagnosed as having thyroid cancer. Computed tomography (CT) and angiography revealed an irregular thyroid tumor invading the left common carotid artery, and encasement of the artery by the tumor. Left common carotid angiography showed an irregularity of the common carotid artery wall and a dense tumor stain fed by the superior thyroid artery. Dissection of the thyroid cancer from the common carotid artery was judged to be impossible during surgery, so resection of the whole tumor together with the common carotid artery was carried out. The left common carotid artery was reconstructed using an expanded polytetrafluoroethylene (ePTFE) graft. No cerebral ischemia was evident after surgery. Postoperative CT and magnetic resonance angiography demonstrated patency of the carotid artery, and no tumor recurrence was confirmed at a 27-month follow up. The ePTFE graft appears to be a suitable synthetic graft for reconstruction of the carotid artery.

Improvement in cognitive function after radical excision of an anterior skull base meningioma--a report of 2 cases.

It has been well documented that memory difficulties in patients with anterior skull base meningioma may improve after tumour resection. However, there have been few reports on studies where precise testing of cognitive function has been conducted before and after surgery. Here we report 2 cases of anterior skull base meningioma where 2 tests of cognitive function, the Wechsler adult intelligence scale-revised (WAIS-R) and the Wechsler memory scale-revised (WMS-R), were performed by the patient before and after surgical treatment. After the operation there was a dramatic increase in the performance IQ as measured by the WAIS-R. In addition, the scores for both the "Attention/Concentration" and the "Delayed Recall" subsets of the WMS-R scale improved. There were differences between the 2 patients in the length of time required for functional recovery and this was thought to be due to the tumour size and the age of the patient. The use of these scales would enable physicians who are treating patients with anterior skull base meningioma to assess the recovery time that is likely to be needed before full return of cognitive function after surgery.

Ictal onset and spreading of seizures of parietal lobe origin.

The characteristics of seizures associated with parietal lobe epilepsy were investigated in six consecutive patients with medically intractable epilepsy due to parietal lobe lesions. Intracranial electrode recordings were retrospectively reviewed to investigate the seizure onset symptoms and spreading patterns associated with the disorder. All six patients underwent implantation of subdural or depth electrodes and subsequent video/electroencephalography monitoring. Common symptoms included motionless stare, contralateral eye deviation, and head turn. Tonic posturing, contralateral sensory disturbance, and motor weakness were also seen. Asymmetrical generalized tonic and clonic seizures were seen in all six patients. All seizure activities began in the parietal lobe harboring the lesions, and then spread immediately to the adjacent lobes in most seizures, where the clinical symptoms were produced. The parietal lobe is a pure generator of seizures, whereas most clinical symptoms originate from adjacent lobes following seizure onset. No apparent specific symptoms other than sensory disturbance arising from the parietal lobe proper were recognized. Regardless of clinical symptoms, the seizure onset occurred in the parietal lobe harboring the lesion.

[A case of a ruptured dermoid cyst in the sylvian fissure].

A 22-year-old woman was admitted to our department after developing a headache. The neurological findings were unremarkable on her first visit, but CT demonstrated a lot of low-density masses in the subarachnoid space. The largest mass was in the right sylvian fissure. These lesions appeared hyper-intense in T1, T2 and diffusion-weighted MR images. A right frontotemporal craniotomy was performed to remove the main mass lesion in the right sylvian fissure. During surgery, thickening of the arachnoid membrane and floating oily globules were seen in the subarachnoid space. The histopathological examination revealed that the tumor was a dermoid cyst. Follow-up MRI revealed that some of the small lesions had moved since the operation. These findings suggested that the tumor was a ruptured dermoid cyst. The patient's postoperative course was uneventful and her headache disappeared completely.

Endoscopic fenestration of posterior fossa arachnoid cyst for the treatment of presyrinx myelopathy--case report.

A 32-year-old man presented with an arachnoid cyst of the posterior fossa manifesting as cervical syringomyelic myelopathy. Magnetic resonance (MR) imaging demonstrated edematous enlargement and T2 prolongation of the cervical spinal cord, indicating a "presyrinx" state. MR imaging showed the inferior wall of the cyst, which disturbed cerebrospinal fluid (CSF) pulsatile movement between the intraspinal and intracranial subarachnoid spaces. The cyst wall was fenestrated with a neuroendoscope. The presyrinx state and the CSF movement improved. Posterior fossa arachnoid cyst, as well as Chiari malformation, can cause CSF flow disturbance at the craniocervical junction and syringomyelia. Endoscopic fenestration is less invasive than foramen magnum decompression and should be the procedure of choice.

Neuro-Behçet's disease manifesting as a neoplasm-like lesion--case report.

A 50-year-old man presented with neuro-Behçet's disease (NBD) manifesting as a large neoplasm-like lesion affecting the brainstem, basal ganglia, and white matter of the cerebral hemisphere. He had no history of disease except for psychoneurosis. On admission, neurological examination found left hemiparesis and dysarthria. Magnetic resonance (MR) imaging showed multiple small ring-like enhancement in the basal ganglia, brainstem, and deep white matter. Biopsy of the mass was performed. Histological examination revealed invasion of inflammatory cells in the white matter, especially around the blood vessels. After the brain biopsy, the patient developed oral aphthae, genital ulcers, and skin eruptions, which are indicative of Behçet's disease. MR imaging after three courses of steroid pulse therapy revealed that the edematous lesion had become smaller with minimum midline shift. NBD should be considered in the differential diagnosis of lesions with multiple ring-like enhancement extending from the basal ganglia to the brainstem, because dermatological manifestations are sometimes obscured during periods of remission.