Acute progression of cerebral amyloid angiopathy-related inflammation diagnosed by biopsy in an elderly patient: A case report.

Background: Cerebral amyloid angiopathy-related inflammation (CAA-I) presents with slowly progressive nonspecific neurological symptoms, such as headache, cognitive function disorder, and seizures. Pathologically, the deposition of amyloid-ß proteins at the cortical vascular wall is a characteristic and definitive finding. Differential diagnoses include infectious encephalitis, neurosarcoidosis, primary central nervous system lymphoma, and glioma. Here, we report a case of CAA-I showing acute progression, suggesting a glioma without enhancement, in which a radiological diagnosis was difficult using standard magnetic resonance imaging. Case Description: An 80-year-old woman was admitted due to transient abnormal behavior. Her initial imaging findings were similar to those of a glioma. She presented with rapid progression of the left hemiplegia and disturbance of consciousness for 6 days after admission and underwent emergent biopsy with a targeted small craniotomy under general anesthesia despite her old age. Intraoperative macroscopic findings followed by a pathological study revealed CAA-I as the definitive diagnosis. Steroid pulse therapy with methylprednisolone followed by oral prednisolone markedly improved both the clinical symptoms and imaging findings. Conclusion: Differential diagnosis between CAA-I and nonenhancing gliomas may be difficult using standard imaging studies in cases presenting with acute progression. A pathological diagnosis under minimally invasive small craniotomy may be an option, even for elderly patients.

Transvenous Embolization of Cavernous Sinus Dural Arteriovenous Fistula through the Angiographically Occlusive Superior Ophthalmic Vein.

Transvenous embolization (TVE) through the superior ophthalmic vein (SOV) is a useful approach for the treatment of cavernous sinus (CS) dural arteriovenous fistulae (DAVFs). This venous route is usually confirmed by angiography. Herein, we present a case of favorable embolization of the CS DAVF through the angiographically occlusive SOV. A 61-year-old man presented with progressive exophthalmos and hypertonia. The patient was diagnosed with a CS DAVF, and TVE was planned. The first approach through the inferior petrosal sinus was infeasible; therefore, we attempted to approach the fistula through the left facial vein. The microcatheter was easily advanced to the shunt point through the angiographically occlusive SOV. We performed coil embolization, and the CS DAVF was completely obstructed.

Factors Associated with Prolonged Impairment of Consciousness in Adult Patients Admitted for Seizures: A Comprehensive Single-center Study.

Seizures are common neurological emergencies that occasionally cause prolonged impairment of consciousness. The aim of this retrospective single-center study is to clarify factors associated with prolonged impairment of consciousness for admitted adult patients investigating patient backgrounds, blood tests, electroencephalographic patterns, and MRI findings. The patients who were admitted to the hospital due to epileptic seizures were classified into two groups: (1) early recovery group, in which patients recovered their consciousness within 6 hr, and (2) delayed recovery group, in which patients showed impairment of consciousness more than 6 hr. Factors associated with prolonged impairment of consciousness were compared between these groups. In this study, 42 cases (33 patients), with a mean age of 67.8 years, were included. Fifteen cases (13 patients) and 27 cases (20 patients) were classified into the early and delayed recovery groups, respectively. The populations of older patients and patients from a nursing home were significantly higher in the delayed recovery group. With regard to radiological analyses, a high grade of periventricular hyperintensity (PVH), high Evans index score, and enlarged bilateral atrial widths were significantly associated with prolonged impairment of consciousness. Multivariable analyses showed that a high grade of PVH was significantly associated with delayed recovery of consciousness independent of age and status epilepticus. In conclusion, we proposed that diffuse white matter degeneration around the lateral ventricles contributes to prolonged impairment of consciousness.

Efficacy of cabergoline and triptans for cluster-like headache caused by prolactin-secreting pituitary adenoma: A literature review and case report.

OBJECTIVE: Cluster-like headache (CLH) associated with pituitary adenoma (PA) is rare. Although numerous cases have been reported, no summary of the literature has been published. Furthermore, the mechanism and efficacy of medication in CLH associated with PA remains unknown. METHODS: We reviewed 14 cases of CLHs associated with PA published in the English and Japanese literature. We have also included and presented our experience with such a case. RESULTS: The median age of patients with CLHs associated with PA was 46 years (range, 17-58 years). The ratio of men to women was 14:1. Headache duration ranged from 15-480 min, with left fronto-orbital pain being common. The most common autonomic nervous symptoms were eye-related in 13 patients (86.6 %), followed by nasal symptoms in 12 (80.0 %). Thirteen patients (86.6 %) had functional adenomas; the remaining two were nonfunctional. Twelve of the functional adenomas were lactotroph adenomas (80.0 %), and one was a somatotroph adenoma (6.6 %). CLHs significantly improved after cabergoline administration in 7/9 patients with a lactotroph adenoma (77.7 % response rate). In 5/11 patients with either a functional or nonfunctional PA who received a triptan, CLHs improved (45.4 % response rate). CONCLUSION: Based on the efficacies of cabergoline and triptans, two different mechanisms may coexist in the pathogenesis of CLHs associated with PA: endocrinological and physical effects of the tumor itself. Cabergoline is the first-line treatment for headaches caused by lactotroph adenomas. Triptans can be effective as an acute drug for headaches associated with nonfunctional PAs and persistent headaches that remain after cabergoline administration.

A retrospective study of bevacizumab for treatment of brainstem glioma with malignant features.

Brainstem glioma is impossible to resect completely, and patients with this type of glioma show a poor prognosis. Therefore, a more effective adjuvant therapy is required to prolong survival. Bevacizumab is an endothelial growth factor monoclonal antibody with strong anti-vascular effects, which may suppress tumor progression. We performed a retrospective study of data from 6 patients with brainstem glioma showing malignant features who were treated with bevacizumab. Tumor-associated lesions, as evaluated by T2 weighted or fluid-attenuated inversion-recovery magnetic resonance imaging, were reduced in all patients, although the timing of the start of bevacizumab administration and pretreatment were not uniform. Clinical symptoms improved in 4 patients and progression was inhibited in 2 patients. The Karnofsky performance status improved from 56.7 to 71.7 on average. The median reduction ratio of tumor-associated lesions was 76.3%, but tumor suppression did not last in any of the cases. Furthermore, 5 patients died of tumor progression, and 1 patient died of a complication of necrotizing colitis. The median progression-free survival after bevacizumab administration was 7 months. The median overall survival after diagnosis was 16.5 months. Bevacizumab might be a potential therapeutic option for progressive brainstem gliomas with malignant features.

Predictive Value of Motor Evoked Potential Monitoring during Surgery of Unruptured Anterior Circulation Cerebral Aneurysms.

OBJECTIVE AND BACKGROUND: Surgery of unruptured aneurysms is always a great challenge to neurovascular surgeons because no postoperative neurological deficits should be expected postoperatively as the patients are fully asymptomatic before the surgery. Here, we present our experience with selective motor evoked potential (MEP) monitoring of our patients in a 2-year time window. PATIENTS AND METHODS: From 2012 to 2014, 27 patients with unruptured intracranial aneurysms were operated in our institute with the help of MEP monitoring. All patients underwent endoscope-assisted microsurgery with pre- and post-clipping indocyanine green angiography. RESULTS: In this period, no mortality was observed, but 18.5% of the patients developed postoperative deficits which showed good recovery in all cases. Overall, MEP showed about 90% accuracy in predicting postoperative deficits. CONCLUSIONS: MEP as a part of multimodality monitoring of aneurysm surgeries is a valuable tool to improve the outcome. However, we should know its limitations as its results are not always consistent with the outcome.

Neuroprotective effects of pregabalin in a rat model of intracisternal facial nerve avulsion.

BACKGROUND: Pregabalin (PGB), a drug used for treating neuropathic pain, has immune-modulating property that may have therapeutic implications. Suppression of microglial activation and improvement in functional recovery was observed in experimental spinal cord injury after PGB administration. An experimental study was conducted to evaluate whether PGB could afford neuroprotection in a rat model of intracisternal facial nerve avulsion. METHODS: Twenty-eight male Wistar rats (250-300 g) were dichotomized into two groups: a PGB group (N.=14) and a control group (N.=14). The PGB group received a total of 4 intraperitoneal PGB injections (30 mg/kg, 15 minutes preoperatively and 4, 24, and 48 hours postoperatively), and the control group underwent intraperitoneal saline injection. Intracisternal facial nerve avulsion was created by tangential pull-out of the nerve surgically exposed at the stylomastoid foramen. In both groups, the brainstem containing the facial motor nuclei neurons was thin-sliced and stained with cresyl violet, and the number of viable neurons in the facial motor nuclei on days 14 and 28 was counted under microscope. RESULTS: The total viable neuron count was significantly greater in the PGB group than in the Control group both on day 14 (271.4±14.9 vs. 196.2±22.2, P<0.01) and day 28 (160.2±21.6 vs. 102.6±13.4, P<0.01). Furthermore, CD11b/c immunostaining on days 3 and 8 showed that CD11b/c-positive cells, suggestive of activated microglia, were observed only in the control group. CONCLUSIONS: Better neuronal survival by PGB administration may be beneficial and clinically relevant when surgical reconstruction of the facial nerve, such as hypoglossal-facial nerve anastomosis, is considered.

Neurogenic stunned myocardium associated with pediatric brain tumor may not be catecholamine-induced.

A rare case of pediatric neurogenic stunned myocardium (NSM) associated with a brain tumor is reported. A previously healthy 6-year-old boy presented with coma, and imaging studies revealed a brain tumor. On hospitalization day 3, he developed NSM and neurogenic pulmonary edema necessitating intensive cardiopulmonary support. Although blood marker levels of cardiac injury were elevated, his plasma and urinary norepinephrine levels were within normal limits. His cardiorespiratory functions markedly improved by hospitalization day 8. This case report may be one of the first to document plasma and urinary catecholamine levels in pediatric NSM. While solid conclusion cannot be drawn based on experience from a single case, these results suggest that pediatric NSM may not be catecholamine-induced.

Primary Intraventricular Brain Abscess Resulting in Isolated Dilation of the Inferior Horn and Unilateral Hydrocephalus.

Primary intraventricular brain abscesses are rare, and there are no established treatment guidelines for this condition. We report a case in which isolated ventricular dilatation and unilateral hydrocephalus developed after seemingly successful conservative management and which required surgical diversion of the cerebrospinal fluid. A 59-year-old woman presented to our emergency department with high-grade fever and headache. Brain magnetic resonance imaging (MRI) revealed abscesses in the bilateral posterior horn. Although surgical evacuation of the abscesses was considered, conservative management with antibiotics was selected because of the paucity of severe neurological deficits and the concern that an attempt to evacuate the intraventricular abscess might lead to inadvertent rupture of the abscess capsule and acute ventriculitis. Despite reduction in the abscess volume, the patient developed an altered mental status 4 weeks after admission. Follow-up MRI revealed isolated dilation of the left inferior horn, compressing the brainstem. Emergency fenestration of the dilated inferior horn was performed, and endoscopic observation revealed an encapsulated abscess with adhesion to the ventricular wall which was thought responsible for the ventricular dilation and unilateral hydrocephalus. Two weeks after the initial surgery, the unilateral hydrocephalus was treated by placement of a ventriculoperitoneal shunt. Eradication of the intraventricular brain abscesses without surgical evacuation may justify the conservative management of this patient. However, the possibility that earlier surgical evacuation might have prevented development of the isolated ventricular dilation cannot be denied. Additional clinical experience is required to determine which treatment (surgical vs. conservative) is more appropriate in patients with primary intraventricular brain abscesses.

Early rebleeding in patients with subarachnoid haemorrhage under intensive blood pressure management.

The objective of this study was to report the frequency and clinical characteristics of early rebleeding in subarachnoid haemorrhage (SAH) patients who underwent intensive blood pressure (BP) management. Patients with aneurysmal SAH frequently present to the emergency department (ED) with elevated BP. Intensive BP management has been recommended to lower the risk of early rebleeding. However, few studies have reported the frequency of early rebleeding in SAH patients undergoing BP management. In our institution, SAH patients with systolic BP (SBP)>140 mmHg received continuous intravenous nicardipine to maintain their SBP within 120±20 mmHg after diagnosis. An attempt to implement intensive BP management was made on 309 consecutive SAH patients who presented to our ED within 48 hours of SAH onset. Overall, 24 (7.8%) of the 309 patients sustained early rebleeding. Fifteen patients sustained early rebleeding before the implementation of BP management, and the other nine sustained early rebleeding after the implementation of BP management. Therefore, the frequency of early rebleeding under BP management was 3.1% (9/294). When the 309 patients were dichotomised using ED SBP of 140 mmHg as a cut off (SBP>140 mmHg; n=239 versus SBP⩽140 mmHg; n=70), the latter counter-intuitively exhibited a significantly higher frequency of early rebleeding (5.9% versus 14.2%; p=0.04). This relatively low frequency of early rebleeding under BP management may be acceptable. However, early rebleeding is not eradicated even with strict BP control as factors other than elevated BP are involved. ED SBP within the target range (SBP⩽140 mmHg) does not negate the risk of early rebleeding. Other treatment options that reduce the risk should also be explored.

Spontaneous intracranial hypotension manifesting as a unilateral subdural hematoma with a marked midline shift.

Spontaneous intracranial hypotension (SIH) is a syndrome in which hypovolemia of the cerebrospinal fluid (CSF) results in various symptoms. Although its prognosis is usually benign, cases with a rapid neurologic deterioration resulting in an altered mental status have been reported. One of the characteristic radiographic findings in such cases is the presence of bilateral accumulation of subdural fluid (hematoma/hygroma). When SIH-related subdural hematoma is present only unilaterally with a concomitant midline shift, making an accurate diagnosis may be challenging, and inadvertent hematoma evacuation may result in further neurologic deterioration. We report a 58-year-old woman with an altered mental status who had visited a local hospital and in whom a brain CT showed a unilateral subdural hematoma with a marked midline shift. She was referred to our department because of her neurologic deterioration after hematoma evacuation. A CT myelography revealed a massive CSF leakage in the entire thoracic epidural space. She made a full neurologic recovery following blood patch therapy. Our case is unique and educational because the suspicion for SIH as an underlying cause of subdural hematoma is warranted in nongeriatric patients not only with bilateral but also unilateral lesions. An immediate search for CSF leakage may be important in cases with failed hematoma evacuation surgery.

Role of catecholamines in acute hypertensive response: subarachnoid hemorrhage versus spontaneous intracerebral hemorrhage.

BACKGROUND AND OBJECTIVE: Acute hypertensive response, defined as systolic blood pressure (SBP) 140 mmHg or more within 24 h of onset, is frequently observed in hemorrhagic stroke patients. Although catecholamine surge is pivotal in its pathogenesis, few studies have evaluated the relationship between admission SBP and plasma catecholamine levels. PATIENTS AND METHODS: A prospective observational study was carried out to investigate potential differences in the acute hypertensive reaction between subarachnoid hemorrhage (SAH) and spontaneous intracerebral hemorrhage (SICH) by analyzing 200 SAH and 200 SICH patients. In each category, patients were quadrichotomized on the basis of their SBPs in emergency department: less than 140 mmHg, 140-184 mmHg, 185-219 mmHg, and 220 mmHg or more. The plasma catecholamine levels were compared among the four groups. Furthermore, multivariate regression analyses were carried out to identify variables correlated with hypertensive emergency (SBP≥185 mmHg). RESULTS: In SAH patients, there was a proportional increase in norepinephrine levels relative to the graded SBPs, and norepinephrine levels in the 220 mmHg or more group were significantly higher than those in the less than 140 mmHg group (1596±264 vs. 853±124 pg/ml, P=0.03). By contrast, no proportional increase in norepinephrine levels to the graded SBPs was observed in SICH patients. Multivariate regression analyses showed that the initial Glasgow Coma Scale scores of 8 or less (odds ratio 2.251, 95% confidence interval 1.002-5.117) and plasma norepinephrine levels (odds ratio 1.002, 95% confidence interval 1.001-1.003) were correlated with hypertensive emergency in SAH patients. By contrast, none of the variables evaluated were correlated with hypertensive emergency in SICH patients. CONCLUSION: An acute hypertensive response may be more complex, multifactorial, and less catecholamine dependent in SICH patients compared with SAH patients.

Subdural hematoma caused by epithelioid angiosarcoma originating from the skull.

Cancers metastatic to the skull or dura may cause subdural hematoma (SDH). However, the frequency is low, and the presence of underlying cancers has almost always been known in such situations. We report a case of skull angiosarcoma manifesting as SDH, posing a diagnostic challenge to physicians. A 75-year-old man visited our clinic with sensorimotor disturbance of gradual onset approximately 1 month after a minor head trauma. He was diagnosed with SDH after imaging studies, and underwent surgery to evacuate the hematoma. Because the hematoma was organized, surgery was switched from burr-hole drainage to craniotomy. The bone flap as well as the dura over the hematoma had grossly normal appearance, and only the hematoma itself was submitted for histological examination. Although postoperative recovery was uneventful, the patient experienced recurrence of the SDH 2 months after surgery. At the second surgery, the bone flap and dura were intermingled with tumor tissue, and histological examination revealed that an epithelioid angiosarcoma originating from the skull was responsible for the SDH. Timely diagnosis of angiosarcoma manifesting as SDH is difficult because of its rarity. In retrospect, however, the diagnosis might have been established earlier if the bone flap and/or the dura had been biopsied at the time of the first surgery. The present case gives us a lesson that SDH may be an unusual manifestation of malignant tumors of skull or dural origin, and histological examination of not only hematoma capsule but also of the surrounding tissues may provide important diagnostic clues.

Transvenous embolization in spontaneous direct carotid-cavernous fistula in childhood.

Carotid cavernous fistula (CCF) is an abnormal arteriovenous communication in the cavernous sinus. Direct CCF results from a tear in the intracavernous carotid artery. Typically, it has a high flow and usually presents with oculo-orbital venous congestive features such as exophthalmos, chemosis, and sometimes oculomotor or abducens cranial nerve palsy. Indirect CCF generally occurs spontaneously with subtle signs. We report a rare case of spontaneous direct CCF in childhood who did not have the usual history of craniofacial trauma or connective tissue disorder but presented with progressive chemosis and exophthalmos of the right eye. This report aims also to describe the safety and success of transvenous embolization with coils of the superior ophthalmic vein and cavernous sinus through the inferior petrosal sinus.

Detection of pulsation in ruptured and unruptured cerebral aneurysms by electrocardiographically gated 3-dimensional computed tomographic angiography with a 320-row area detector computed tomography and evaluation of its clinical usefulness.

BACKGROUND: In ruptured cerebral aneurysms (RCAs), identification of the rupture point of a cerebral aneurysm is useful for treatment planning. In unruptured cerebral aneurysms (URCAs), detection of the risk of aneurysmal rupture is also useful for patient management. OBJECTIVE: Electrocardiographic (ECG)-gated 3D-CT angiography was performed for patients with RCAs and URCAs using 320-row area detector CT (ADCT) to detect pulsation of the cerebral aneurysms. The clinical usefulness of this method was then evaluated. METHODS: Twelve patients had 12 RCAs, and 39 patients had 53 URCAs. A 320-row ADCT system was used to scan. ECG-gated reconstruction was then performed with the R-R interval divided into 20 phases. RESULTS: Pulsation was observed in 10 of the 12 RCAs. The bleeding site was considered to correspond to the area of pulsation. Pulsation was observed in 14 of 53 URCAs. Thirteen patients with 18 URCAs were followed. Of the 11 URCAs in which pulsation was not observed, 1 showed a change in shape. Of the 7 URCAs in which pulsation was observed, 3 showed a change in shape. URCAs in which pulsation was observed were more likely to show a change in shape (P = .082). CONCLUSION: The area of pulsation was found to correspond to the bleeding site in many RCAs. This information would be extremely useful for treatment planning. The detection of pulsation in an URCA is therefore considered to provide useful information for patient management.