Persistent Primitive Hypoglossal Artery with Ipsilateral Symptomatic Carotid Artery Stenosis and Cerebral Aneurysm.

OBJECTIVE: Persistent primitive hypoglossal artery (PPHA) is a rare type of persistent carotid-basilar anastomosis sometimes associated with other vascular lesions. We treated an extremely rare case of PPHA with concomitant ipsilateral symptomatic cervical internal carotid artery (ICA) stenosis and unruptured aneurysm. CASE PRESENTATION: A 67-year-old woman visited our institution with acute onset of diplopia. Magnetic resonance imaging revealed multiple acute infarctions in the right anterior and posterior circulations. Digital subtraction angiography demonstrated the right PPHA concomitant with ipsilateral cervical ICA stenosis and an unruptured ICA aneurysm with maximum diameter of 8 mm. The multiple infarctions were considered to result from artery-to-artery embolism due to microthrombi from the ICA plaque passed along the PPHA, so carotid endarterectomy was performed as the first step with preoperative modified Rankin Scale (mRS) grade 1. During the operation, the patient had impaired ICA perfusion due to internal shunt catheter migration into the PPHA followed by acute infarction in the right hemisphere causing mild left hemiparesis. The patient was transferred to the rehabilitation hospital with mRS grade 3. After 3 months of rehabilitation, the patient recovered to mRS grade 1 and clipping surgery for the unruptured right ICA aneurysm was performed as the second step with uneventful postoperative course. CONCLUSION: The treatment strategy should be carefully considered depending on the specific blood circulation for such cases of PPHA with unique vasculature.

[Analysis of a Spontaneous Spinal Epidural Hematoma Mimicking Cerebral Infarction:A Case Report and Review of the Literatures].

Spontaneous spinal epidural hematoma is a rare disease, and the critical form may mimic cerebral infarction in the acute stage. Consequently, misdiagnosis of a cerebral infarction may result in unnecessary antithrombotic therapy. The present study investigated 19 cases of spontaneous spinal epidural hematoma first diagnosed as cerebral infarctions and treated with antithrombotic therapy. Of these, 16 cases(84.2%)presented with pain in the neck, shoulder, and back on admission, 19 cases(100%)with hemiplegia not including the face, 7 cases(36.8%)with limb sensory disturbance, and all 19 cases(100%)underwent MRI findings for definite diagnosis. After diagnosis, 6 of the 19 cases(31.6%)were treated with recombinant tissue-type plasminogen activator(rt-PA)administration, 13 cases(68.4%)with surgical treatment, and 9 cases(47.4%)were without after effect and showed good progress. MRI is effective in detecting spontaneous spinal epidural hematoma, however, CT is also used for this diagnosis. The most common site of spontaneous spinal epidural hematoma is the cervicothoracic spine junction or thoracolumbar spine junction, but occurrence in the upper cervical vertebra may present with hemiplegia similar to cerebral infarction. Pathognomonic symptoms include pain in the neck, shoulder, and back, however, symptoms such as hemiplegia not including the face, cervical spine, and cervical cord lesion may be important indicators. Although, some cases may not present with pain and sensory disturbance symptoms, therefore an initial misdiagnosis of cerebral infarction may be made and subsequent antithrombotic therapy can result in increased bleeding and serious after-effects. In particular, 4 of the 6 cases(66.7%)treated with hyperacute phase thrombolytic therapy(rt-PA treatment)in this study required surgical treatment, and the time until definite diagnosis was shorter compared with other antithrombotic agents, presumably due to the rapid increase in hematoma. Therefore, the possibility of spinal cord epidural hematoma should be considered before beginning rt-PA therapy with careful examination to confirm the presence of lesions in the cervical spine and cervical cord.

[Ruptured Aneurysms on the Cortical Segment of the Distal Posterior Inferior Cerebellar Artery:A Report of Two Cases and a Literature Review].

Two unusual cases of ruptured distal posterior inferior cerebellar artery(PICA)aneurysm on the cortical segment were successfully treated with open surgery. A 76-year-old woman presented with a Hunt and Kosnik(H&K)grade II Subarachnoid hemorrhage(SAH). CT showed a slight SAH in the cisterna magna and around the vermis, and fourth intraventricular hematoma. Cerebral angiography revealed an aneurysm on the cortical segment of the distal PICA. Intraoperative findings identified the aneurysm as fusiform. Trapping of the aneurysm was performed, and the indocyanine green angiography fiuding confirmed aneurysmal flow disappearance and good circulation in the cerebellar cortex. An 89-year-old woman presented with H&K grade II SAH. CT revealed a thick SAH in the posterior cranial fossa, and third to fourth intraventricular hematoma with hydrocephalus. Cerebral angiography revealed an aneurysm on the cortical segment of the distal PICA. Intraoperative findings showed that the aneurysm was fusiform. Trapping and resection of the aneurysm were performed. Thirteen previous cases of aneurysms on the cortical segment of the distal PICA have been reported. Distal PICA aneurysms apparently show rebleeding more frequently than do aneurysms at other locations, so immediate direct surgery is necessary to avoid rebleeding. After proximal ligation or trapping of the aneurysm, indocyanine green angiography is useful to determine the need for revascularization.

[Frontal Base Penetrating Brain Injury by a Gardening Scissors:A Case Report].

A 43-year-old woman accidentally fell from a 1.5-m height with gardening scissors, and the cutting edge of the scissors pierced into her skull base through the left nasal cavity. After she pulled the scissors out by herself, her consciousness immediately deteriorated. She was transferred to Juntendo University Shizuoka Hospital by an ambulance helicopter. Intracerebral hematoma in the right frontal lobe caused by the middle cerebral artery injury was detected using brain computed tomography. Emergent hematoma evacuation with decompressive craniectomy was performed. In this procedure, cerebrospinal fluid(CSF)leakage was repaired using a multi-layered flap technique, which placed both the free autologous dura mater and pedicled temporalis muscle flaps into the damaged skull base space. After the surgery, CSF drainage was managed to control both intracranial pressure and CSF leakage. Upon discussion, several clinical issues can be highlighted. First, the gardening scissors were pulled out by the patient herself. Second, the foreign material penetrating the intracranial space might have conferred a high infection risk. Finally, it might be difficult to repair the severe damage inflicted upon the skull base by the large gardening scissors. In conclusion, it was considered that favorable results were obtained by the emergent surgical intervention and by repairing the skull base through forming a multi-layered flap with a combination of free and pedicled tissues.

[Dissecting Aneurysm of the Distal Middle Cerebral Artery Formed the Out Pouch during Long-term Follow-up:A Case Report].

A 40-year-old man with a rare case of dissecting aneurysm of the distal middle cerebral artery(MCA, M2 segment)presented with ischemic stroke, manifesting as headache, nausea, and visual disorder. Computed tomography(CT)scan revealed cerebral infarction, and three-dimensional CT angiogram revealed stenotic change at the M2 segment. The patient was conservatively treated and remained neurologically stable. Ten months later, CT scan and magnetic resonance imaging(MRI)revealed outpouching of the M2 segment at the location of the initial stenosis. Angiogram revealed pearl and string sign at the M2 segment of the inferior trunk. The preoperative diagnosis was spontaneous dissecting aneurysm of the right distal MCA. The patient was treated with direct surgery for aneurysm resection and revascularization using the superficial temporal artery. The brain surface in contact with the dissecting aneurysm was colored xanthochromic, so minor bleeding from the dissecting aneurysm was highly suspected. Previously, 19 cases of dissecting aneurysm of the distal MCA have been reported. Distal dissecting aneurysms seem to rupture more frequently in the MCA than in the proximal segment. Long-term follow-up MRI and angiogram might be important, not only in the acute stage in patients with atypical caliber changes in the distal MCA. Immediate direct surgery is indicated to avoid hemorrhagic stroke if dilatation of the major trunk at the dissection is suspected.

Expression analysis of high mobility group box-1 protein (HMGB-1) in the cerebral cortex, hippocampus, and cerebellum of the congenital hydrocephalus (H-Tx) rat.

High mobility group box-1 protein (HMGB-1), a protein expressed highly in developing neurons, is involved in the development and differentiation of neurons. At the same time, it functions as a transcriptional regulator of particular genes and as a cytokine: HMGB-1 released from a defective cell has been reported to induce damage to the adjacent cells.With a view to examine the relationship between neuronal damage caused by hydrocephalus and HMGB-1, we analyzed the expression of HMGB-1 in the cerebellum, cerebrum, and hippocampus of 1-day-old congenitally hydrocephalic H-Tx rats.As opposed to nonhydrocephalic H-Tx rats, the hydrocephalic H-Tx rats were observed to show stronger expression of HMGB-1 in the cerebellum, cerebrum, and hippocampus. Consequently, the protein was presumed to influence the development of neurons from an early postnatal stage not only in the cerebral cortex and hippocampus but also in the cerebellum, which is less susceptible to the direct effects of hydrocephalus. We expect that, in the future, regulating the expression or functions of HMGB-1 will lead to the possibility of impeding the progress of neuronal damage caused by hydrocephalus.

Significance of endoscopic hematoma evacuation in elderly patients with spontaneous putaminal hemorrhage.

BACKGROUND: The efficacy of endoscopic surgery for spontaneous intracerebral hemorrhages (ICH) has been previously reported, but differences in the effect between early and late elderlies remain unclear. METHODS: Ninety-seven patients diagnosed with putaminal hemorrhage (age, ≥65 years; hematoma volume, ≥30 mL) were included in this retrospective analysis and separated into three treatment groups: craniotomy surgery (CR), endoscopic surgery (EN), and non-surgical (NS) groups. The patients were additionally subdivided into two groups according to age: patients aged 65-74 years ("early elderlies") and patients aged ≥75 years ("late elderlies"). Their clinical data and outcomes at discharge were compared using statistical analyses. RESULTS: The CR and the EN groups were associated with lower mortality rates (P < 0.001), lower modified Rankin Scale (mRS; P = 0.007), and lower National Institutes of Health Stroke Scale (NIHSS; P = 0.029) compared to the NS group. Early elderlies in the CR and EN groups with ICH scores of 3 also had significantly better outcomes (P = 0.001). The proportion of patients with mRS ≤ 4 was highest in the early elderlies of the EN group (P = 0.553). Although significant differences in the change of NIHSS scores between the early and late elderlies was not observed, significantly improved NIHSS scores were observed in the EN group compared to the NS group, even in the late elderlies (P = 0.037). CONCLUSION: The evacuation of deep-seated intracranial hematomas using the endoscope might improve functional outcomes and mortality, regardless of age.

Novel Anatomic Classification of Spontaneous Thalamic Hemorrhage Classified by Vascular Territory of Thalamus.

BACKGROUND: Spontaneous thalamic hemorrhage has increased in incidence in recent years. Analysis of the characteristics of thalamic hemorrhage was based on the vascular territories of the thalamus. METHODS: Retrospective analysis included 303 consecutive patients with spontaneous thalamic hemorrhage. Thalamic hemorrhage was classified into 4 types: anterior type (supplied mainly by the tuberothalamic artery), medial (mainly paramedian thalamic-subthalamic artery), lateral (mainly thalamogeniculate artery), and posterior (mainly posterior choroidal artery). The baseline characteristics, complications, and functional outcomes were assessed. RESULTS: The anterior type was found in 10 patients (3.3%), the medial type in 47 (15.5%), the lateral type in 230 (75.9%), and the posterior type in 16 (5.3%). Intracerebral hemorrhage volume was smallest in the anterior type, and significantly smaller than in the medial (P = 0.002) and lateral types (P < 0.001). Intraventricular hemorrhage (IVH) or acute hydrocephalus was significantly associated with the medial type (P < 0.01 or P < 0.01, respectively). Non-IVH or non-acute hydrocephalus was significantly associated with the anterior (P < 0.05 or P < 0.05, respectively) and lateral (P < 0.05 or P < 0.05, respectively) types. Emergency surgery was correlated only with the medial type (P < 0.01). The independent predictors of poor outcome were age (odds ratio [OR], 1.07; P = 0.002), admission National Institutes of Health Stroke Scale score (OR, 1.32; P < 0.001), and type of thalamic hemorrhage (OR, 2.08; P = 0.038). CONCLUSIONS: The present study proposed a novel anatomic classification of thalamic hemorrhage according to the major thalamic vascular territories.

Preventive effect of continuous cisternal irrigation with magnesium sulfate solution on angiographic cerebral vasospasms associated with aneurysmal subarachnoid hemorrhages: a randomized controlled trial.

OBJECT Although cerebral vasospasm (CV) is one of the most important predictors for the outcome in patients with subarachnoid hemorrhage (SAH), no treatment has yet been established for this condition. This study investigated the efficacy of continuous direct infusion of magnesium sulfate (MgSO4) solution into the intrathecal cistern in patients with an aneurysmal SAH. METHODS An SAH caused by a ruptured aneurysm was identified on CT scans within 72 hours after SAH onset. All patients were treated by surgical clipping and randomized into 2 groups: a control group of patients undergoing a standard treatment and a magnesium (Mg) group of patients additionally undergoing continuous infusion of 5 mmol/L MgSO4 solution for 14 days. The Mg(2+) concentrations in serum and CSF were recorded daily. Neurological examinations were performed by intensive care clinicians. Delayed cerebral ischemia was monitored by CT or MRI. To assess the effect of the Mg treatment on CV, the CVs were graded on the basis of the relative degree of constriction visible on cerebral angiograms taken on Day 10 after the SAH, and transcranial Doppler ultrasonography was performed daily to measure blood flow velocity in the middle cerebral artery (MCA). Neurological outcomes and mortality rates were evaluated with the Glasgow Outcome Scale and modified Rankin Scale at 3 months after SAH onset. RESULTS Seventy-three patients admitted during the period of April 2008 to March 2013 were eligible and enrolled in this study. Three patients were excluded because of violation of protocol requirements. The 2 groups did not significantly differ in age, sex, World Federation of Neurosurgical Societies grade, or Fisher grade. In the Mg group, the Mg(2+) concentration in CSF gradually increased from Day 4 after initiation of the continuous MgSO4 intrathecal administration. No such increase was observed in the control group. No significant changes in the serum Mg(2+) levels were observed for 14 days, and no cardiovascular complications such as bradycardia or hypotension were observed in any of the patients. However, bradypnea was noted among patients in the Mg group. The Mg group had a significantly better CV grade than the control group (p < 0.05). Compared with the patients in the Mg group, those in the control group had a significantly elevated blood flow velocity in the MCA. Both groups were similar in the incidences of cerebral infarction, and the 2 groups also did not significantly differ in clinical outcomes. CONCLUSIONS Continuous cisternal irrigation with MgSO4 solution starting on Day 4 and continuing to Day 14 significantly inhibited CV in patients with aneurysmal SAH without severe cardiovascular complications. However, this improvement in CV neither reduced the incidence of delayed cerebral ischemia nor improved the functional outcomes in patients with SAH.

Multifocal primary intracerebral malignant fibrous histiocytoma--case report.

A 58-year-old female presented with a unique case of multifocal primary intracerebral malignant fibrous histiocytoma (MFH) manifesting as partial seizure. Neuroimaging showed a mass lesion in the right frontal lobe, which was totally removed. The histological diagnosis was MFH. Follow-up neuroimaging one month after surgery showed another lesion rapidly growing in the left frontal lobe. This lesion was totally removed, and identified as MFH. Her condition gradually worsened. Neuroimaging performed 3 months after first operation revealed bilateral recurrence. She died of respiratory failure 7 months after the initial diagnosis of MFH. Primary intracranial MFH is an extremely rare entity with only 31 cases of solitary tumor previously reported.

Cerebellar Purkinje cells exhibit increased expression of HMGB-1 and apoptosis in congenital hydrocephalic H-Tx rats.

BACKGROUND: Highly integrated anatomic and functional interactions between the cerebrum and the cerebellum during development have been reported. In our previous study, we conducted a proteome analysis to identify the proteins present in the congenital noncommunicating hydrocephalus in the cerebellum. We found higher expression of high-mobility group box-1 protein (HMGB-1) in hydrocephalic H-Tx rats. OBJECTIVE: We studied the expression pattern of HMGB-1 in the cerebellum. METHODS: We studied congenital hydrocephalic H-Tx rats aged 1 day and 7 days along with age-matched nonhydrocephalic H-Tx and Sprague-Dawley rats as controls. Gene and protein expressions of HMGB-1 in the cerebellum were assayed by real-time polymerase chain reaction and Western blotting, respectively; furthermore, immunohistochemical analyses were performed by using HMGB-1 (indicator of apoptosis), single-stranded DNA; adhesion factor related to cell migration, HNK-1; and the Purkinje cell-specific antibody, calbindin. RESULTS: Cytoplasmic HMGB-1 expression observed in Purkinje cells in the 1-day-old hydrocephalic group was stronger than that in the nonhydrocephalic and Sprague-Dawley groups. Double fluorescent staining with single-stranded DNA confirmed that Purkinje cells were undergoing apoptosis. HNK-1 expression was lower in the Purkinje cell layer in the 7-day-old rats in the hydrocephalic group, and Purkinje cells were disrupted in comparison with the control groups. Morphological changes in the cerebellum were observed in the 7-day-old rats in the hydrocephalic group in comparison with the control groups. CONCLUSION: Our results suggest that cerebellar neuronal cell damage in the early postnatal period may be related to the higher expression of HMGB-1 in the Purkinje cells.

Intra-axial brainstem malignant melanoma mimicking cavernous angioma--case report.

A 41-year-old female presented with repeated pontine hemorrhage. Magnetic resonance imaging showed the pontine hemorrhage as a heterogeneously enhanced mass mimicking cavernous angioma. The mass lesion was removed via a midline suboccipital approach. Histological examination showed malignant melanoma cells. No cutaneous lesion was found and positron emission tomography found no abnormalities. Our presumptive diagnosis was primary intra-axial brainstem malignant melanoma. The possibility of malignant melanoma should be considered in patients with intra-axial brainstem lesion associated with repeated hemorrhages.