A nonbifurcating cervical carotid artery with a remnant of the proximal internal carotid artery that mimicked an aneurysm on magnetic resonance angiography.

PURPOSE: To describe a case of a nonbifurcating cervical carotid artery with remnant of the proximal internal carotid artery (ICA). METHODS: A 47-year-old man with unruptured cerebral aneurysm underwent magnetic resonance (MR) angiography of the neck region and intracranial region. The MR machine was a 3-T scanner. Catheter angiography was performed for the treatment of the aneurysm by coil embolization. RESULTS: The proximal main trunk of the left external carotid artery (ECA) was absent, and branches arose separately, indicative of a nonbifurcating cervical carotid artery. At the level of the ICA origin, aneurysmal protrusion was found on MR angiography. On catheter angiography, this protrusion was not an aneurysm but a remnant of the proximal ICA. CONCLUSION: The configuration of the nonbifurcating cervical carotid artery lacks the proximal ECA; however, segmental agenesis of the proximal ICA forms this rare cervical arterial variation. The present case had remnants of the proximal ICA. We speculate that the ICA channel, except for the origin, was occluded after the development of EC-ICA anastomosis, and a nonbifurcating cervical carotid artery with a remnant of the ICA may have formed. Including our patient, only four cases have been reported in the relevant English-language literature.

Ophthalmic artery arising from the presumed meningohypophyseal trunk of the cavernous internal carotid artery diagnosed by magnetic resonance angiography.

PURPOSE: The purpose of the study is to describe a case of ophthalmic artery (OA) arising from the presumed meningohypophyseal trunk (MHT) of the cavernous internal carotid artery (ICA). CASE REPORT: A 63-year-old woman suspected of having cerebral infarctions underwent cranial magnetic resonance (MR) imaging and MR angiography. MR angiography showed a right OA that was found to arise from the superolateral aspect of the proximal cavernous ICA. This OA entered the orbit via the superior orbital fissure (SOF). DISCUSSION: Rarely, an OA arises from the inferior aspect of the middle cavernous ICA at the level of the inferolateral trunk (ILT) and enters the orbit via the SOF. This OA variation was traditionally regarded as a persistent primitive dorsal OA but is now believed to be due to the persistence of the lateral branch of the primitive maxillary artery. The present case had an OA arising from the superolateral aspect of the more proximal cavernous ICA than the origin of the ILT, which was suggested to be the origin of the MHT. Persistence of the proximal segment of the trigeminal artery may play an important role in the formation of this extremely rare variation. CONCLUSION: To identify this extremely rare OA variation, careful observation of source images and the creation of volume-rendering MR angiography images are important.

Use of flexible endoscopic aspiration for an intraventricular small floating clot with hemorrhage: a technical note.

BACKGROUND: Although flexible endoscopy is effective for intraventricular lesions, it is less frequently used for hemorrhagic cases. In some hemorrhagic strokes, blood clots may plunge into the cerebral aqueduct and cause acute obstructive hydrocephalus. A flexible endoscope can aspirate clots and prevent acute hydrocephalus. METHODS: Here, we report four cases of hemorrhage: one of intracerebral hemorrhage and three of subarachnoid hemorrhages. RESULTS: In all cases, acute hydrocephalus was not apparent upon admission. Sudden comatose occurred; computed tomography revealed acute obstructive hydrocephalus with a strangulated clot in the cerebral aqueduct. We performed aspiration of the strangulated clot using a flexible endoscope. Consciousness improved in all cases, and acute hydrocephalus was prevented in all cases. CONCLUSION: The use of simple flexible endoscopic aspiration for clots might be a beneficial and less-invasive procedure for acute obstructive hydrocephalus caused by a small clot with hemorrhagic stroke.

Type 4 persistent primitive olfactory artery associated with contralateral accessory middle cerebral artery arising from the fenestrated segment of the distal anterior cerebral artery.

Persistent primitive olfactory artery (PPOA) is a rare variation of the proximal anterior cerebral artery (ACA) that generally follows an extreme anteroinferior course and takes a hairpin turn before continuing to the A2 segment of the ACA (type 1). There are four other types of extremely rare variations. The type 4 variation continues to the accessory middle cerebral artery (MCA) instead of the ACA. Only a few cases have been reported. We herein report a case of type 4 PPOA in which the contralateral side of the accessory MCA arose from the fenestrated segment of the distal ACA. No similar cases were found in the relevant English-language literature. For the identification of these variations on MR angiography, volume-rendering images were superior to maximum-intensity-projection images.

Ascending pharyngeal artery-posterior inferior cerebellar artery anastomosis via the jugular foramen: a case report and literature review.

The posterior inferior cerebellar artery (PICA) rarely arises from the cavernous segment of the internal carotid artery (ICA) and is called persistent trigeminal artery variant. The PICA also can arise from the cervical segment of the ICA, and it enters the posterior fossa via the hypoglossal canal, where it is called persistent hypoglossal artery variant. Using magnetic resonance angiography (MRA), we diagnosed a 79-year-old man with a PICA arising from the ascending pharyngeal artery and passing through the medial side of the jugular foremen pars vascularis. Only six cases of this variation have been reported previously in the English language literature. To identify this variation on MRA, the careful observation of source images is useful. Recognizing this variation is important in order to avoid ischemic cerebellar complications during neck surgery and endovascular therapy.

Medial type persistent trigeminal artery associated with a saccular aneurysm at its trunk.

There are four types of fetal anastomosis between the carotid and vertebrobasilar arteries at 5 weeks gestation; from caudal to cranial position, these involve the proatlantal intersegmental, hypoglossal, otic, and trigeminal arteries. Excluding otic artery, these arteries may persist rarely. Persistent trigeminal artery (PTA) is the most common carotid-vertebrobasilar anastomosis, and the medial type (intrasellar) PTA is quite rare, accounting for approximately 10% of all PTA cases. An aneurysm is occasionally found at the origin of the PTA. Rarely, an aneurysm arises at the trunk of the PTA. Using magnetic resonance angiography, we identified a case of medial type PTA with an unruptured saccular aneurysm at its trunk.

Right vertebral artery entering the third transverse foramen diagnosed by magnetic resonance angiography: a report of two cases.

The vertebral artery (VA) usually enters the sixth transverse foramen (TF). The VA sometimes enters the fifth, and rarely enters the fourth or seventh TF. Reports describing a VA entering the third TF, identified by anatomical dissection or computed tomography angiography, are extremely rare. We herein report two cases in which magnetic resonance (MR) angiography showed the right VA entering the third TF. Coronal reformatted MR angiography source images were useful for identifying the level at which the VA entered the TF. Because the anomalous VA takes an anterior course, it poses a risk during anterior neck surgery. Thus, a correct diagnosis is important when interpreting cervical MR angiography.

First external validation of sensitivity and specificity of the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for idiopathic inflammatory myopathies with a Japanese cohort.

OBJECTIVE: To externally validate the performance of the new European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria set for idiopathic inflammatory myopathies (IIM) with a Japanese cohort. METHODS: This study included 420 IIM and 402 non-IIM cases. Probability of having IIM in each patient was calculated using the collected data set. The cut-off probability was set at 55%, as recommended by EULAR/ACR. Patients classified as IIM by the criteria were further subclassified with classification trees. RESULTS: When the probability cut-off was set at 55%, the sensitivity/specificity of the new criteria to diagnose IIM were 89.3%/91.0% in the total cohort, 88.1%/95.1% without muscle biopsy data and 90.4%/65.5% with biopsy data. The cohort included 12 overlap syndrome patients with biopsy data, who were included as non-IIM cases in accordance with traditional Japanese methods. When they were included in the IIM cases, the specificity in patients with biopsy increased to 74.4%. The sensitivity/specificity of the new criteria to diagnose polymyositis/dermatomyositis (PM/DM) plus juvenile and amyopathic DM in the Japanese cohort was 87.4%/92.4%, which were greater than those of the Tanimoto's criteria revised to enable classification of amyopathic DM (ADM) (71.2%/87.8%) and were comparable with those of Bohan & Peter's criteria to diagnose those diseases except for ADM (88.4%/88.3%). CONCLUSIONS: Our study externally validated high specificity of the new criteria for the first time, although with several limitations, including low percentage of child patients. The new criteria have higher sensitivity and/or specificity in classification of PM/DM than the previously reported criteria, demonstrating its usefulness for interethnic patients.

Treatment consensus for management of polymyositis and dermatomyositis among rheumatologists, neurologists and dermatologists.

Although rheumatologists, neurologists and dermatologists see patients with polymyositis (PM) and dermatomyositis (DM), their management appears to vary depending on the physician's specialty. The aim of the present study was to establish the treatment consensus among specialists of the three fields to standardize the patient care. We formed a research team supported by a grant from the Ministry of Health, Labor and Welfare, Japan. Clinical questions (CQ) on the management of PM and DM were raised. A published work search on CQ was performed primarily using PubMed. Using the nominal group technique, qualified studies and results in the published work were evaluated and discussed to reach consensus recommendations. They were sent out to the Japan College of Rheumatology, Japanese Society of Neurology and Japanese Dermatological Association for their approval. We reached a consensus in 23 CQ and made recommendations and a decision tree for management was proposed. They were officially approved by the three scientific societies. In conclusion, a multidisciplinary treatment consensus for the management of PM and DM was established for the first time.

[Usefulness of Bilateral rSO2 Monitoring for Predicting Cerebral Hyperperfusion Syndrome after Carotid Artery Stenting].

OBJECTIVE: Cerebral hyperperfusion syndrome(CHS)and cerebral hyperperfusion phenomenon(CHP)induce intracranial hemorrhage and can become critical complications after carotid artery stenting(CAS). The purpose of the present study was to predict and avoid CHS after CAS using bilateral rSO2 intraoperative monitoring. METHODS: We retrospectively analyzed 100 consecutive patients who underwent CAS between January 2012 and May 2014 in our institution. We performed continuous bilateral rSO2 monitoring from anesthetic induction to the day following CAS. CHS was defined as the deterioration of neurological conditions post-CAS, no ischemic changes on post-CAS head CT or brain MRI, an increase in cerebral blood flow(CBF)and cerebral blood volume(CBV), and shortening of the mean transit time(MTT)or time to peak(TTP)on CT perfusion. To compare the CHS/CHP group and non-CHS/CHP group, we defined four parameters:rSO2 difference(rSO2 at the endpoint of the procedure-baseline rSO2), ΔrSO2 difference(affected side rSO2 difference-unaffected side rSO2 difference), rSO2 ratio(rSO2 at the endpoint of the procedure/baseline rSO2), and ΔrSO2 ratio(affected side rSO2 ratio/unaffected side rSO2 ratio). RESULTS: There were 2 CHS cases(2.2%)and 3 CHP cases(3.3%). In the CHS/CHP group, the ΔrSO2 difference and ΔrSO2 ratio were significantly higher than those in the non-CHS/CHP group(p value<0.05);however, no significant differences were found in the affected side rSO2 difference(p value=0.063)and affected side rSO2 ratio(p value=0.054)between the groups. CONCLUSION: We could promptly detect CHS and CHP in all cases by using continuous bilateral rSO2 monitoring and analysis of the ΔrSO2 difference and ΔrSO2 ratio.

Flexible Endoscopic Aspiration for Intraventricular Casting Hematoma.

BACKGROUND: Conventionally, patients suffering a massive intraventricular hemorrhage have undergone external ventricular drainage. However, long-term or repeated drainage increases the risk of complications due to infections or shunt dependency. Neuroendoscopic surgery may offer some advantages over more conventional procedures. METHODS: Thirteen patients suffering intraventricular hematoma associated with intracerebral hemorrhage, treated in our hospital between April 2011 and March 2014, were reviewed retrospectively. Casting hematomas in the ventricles were manually aspirated using a flexible endoscope. The timing of the operation, period of post-endoscopic ventricular drainage, additional internal shunt surgery, 3-month post-surgical outcome, and critical complications were evaluated. RESULTS: Two patients (treated during our earliest use of endoscope) who underwent surgery on the 7th and 16th day post-onset required subsequent cerebrospinal shunt surgery. In contrast, of the 11 patients who underwent endoscopic surgery on the day of onset, only 1 patient required an additional, third ventriculostomy due to a secondary obstruction of the aqueduct by adhesive fibrous membranes. After 3 months, all six patients with mRS scores of 2-3 satisfied all the following criteria: initial Glasgow Coma Scale scores higher than 8, flexible endoscopic surgeries performed on the day of onset, and period of ventricular drainage of less than 4 days. CONCLUSIONS: Early surgical intervention using a flexible endoscope and short period of post-surgical drainage can be highly effective for patients suffering from casting intraventricular hematomas associated with intracerebral hemorrhage. The advantages of this treatment may be a less invasive procedure, ICP control in the acute phase, breaking away from ventricular drainage in the early stage, and prevention of hydrocephalus or intracranial infectious complications in the long term.

Epidemiologic analysis of the clinical features of Japanese patients with polymyositis and dermatomyositis.

OBJECTIVES: This study aimed to investigate the clinical characteristics of polymyositis/dermatomyositis (PM/DM) in Japan by analyzing data from the nationwide registration system. METHODS: The data of the registration system in 2009 were analyzed to investigate patient numbers, sex, clinical symptoms, therapies, complications, and prognosis of PM/DM. RESULTS: The total number of PM/DM cases was approximately 17,000, and the female/male sex ratio was 2.7:1. Almost all patients improved as a result of therapy, but many suffered from sequelae such as muscle weakness. CONCLUSIONS: The results characterize significant aspects of Japanese PM/DM patients. However, a further prospective survey is required to clarify the true epidemiology and natural history of PM/DM.

Rupture of an aneurysm of the superior cerebellar artery feeding a dural arteriovenous fistula.

We experienced a very rare case of bleeding from an aneurysm of a branch of the superior cerebellar artery, which feeds a dural arteriovenous fistula (DAVF) of the posterior fossa. The aneurysm was not detected on initial angiography and 2 episodes of rebleeding resulted in deterioration of the patient's condition. Although rare, aneurysms of the pial feeding arteries should be considered as a cause of bleeding in cases of DAVF.

Ophthalmic artery arising from the anterior cerebral artery diagnosed by MR angiography.

Extremely rarely, the ophthalmic artery (OphA) arises from the A1 segment of the anterior cerebral artery (ACA). Discovery of this anomalous OphA during angiography or surgery has been reported in several patients. We report a case in which an OphA of ACA origin was diagnosed by magnetic resonance (MR) angiography and confirmed by selective cerebral angiography. To our knowledge, this is the first report of this variation with MR angiography. Careful observation of MR angiographic images is important for detecting rare arterial variations. Partial maximum-intensity-projection images are useful in identifying tiny anomalous arteries on MR angiography.

Memory impairment caused by cerebral hematoma in the left medial temporal lobe due to ruptured posterior cerebral artery aneurysm.

BACKGROUND: Cognitive disorders, such as memory disturbances, are often observed following a subarachnoid hemorrhage. We present a very rare case where rupture of a posterior cerebral artery aneurysm caused restricted damage to the hippocampus unilaterally, and caused memory disturbances. CASE PRESENTATION: A 56-year-old, right-handed man, with a formal education history of 16 years and company employees was admitted to our hospital because of a consciousness disturbance. He was diagnosed as having a subarachnoid hemorrhage due to a left posterior cerebral artery dissecting aneurysm, and coil embolization was performed. Subsequently, he had neither motor paresis nor sensory disturbances, but he showed disorientation, and both retrograde and anterograde amnesia. Although immediate recall and remote memory were almost intact, his recent memory was moderately impaired. Both verbal and non-verbal memories were impaired. Brain computed tomography (CT) and magnetic resonance imaging (MRI) revealed a cerebral hematoma in the left temporal lobe involving the hippocampus and parahippocampal gyrus, and single-photon emission computed tomography (SPECT) demonstrated low perfusion areas in the left medial temporal lobe. CONCLUSIONS: We suggest that the memory impairment was caused by local tissue destruction of Papez's circuit in the dominant hemisphere due to the cerebral hematoma.

Dysphagia following acute thalamic haemorrhage: clinical correlates and outcomes.

OBJECTIVE: A high incidence of dysphagia is seen during the acute phase of cerebral haemorrhage; however, there have been only rare reports of dysphagia caused by thalamic haemorrhage. We studied cases of thalamic haemorrhage during the acute period after hospitalization and examined the relationships between the frequency of dysphagia, stroke focus and haematoma volume. METHODS: There were 113 subjects in this study, selected from 247 patients with thalamic haemorrhage who visited our rehabilitation centre. Patients were excluded if they had a history of dementia, stroke, confusion, epileptic seizure, surgical treatment or tracheotomy. We conducted a bedside swallowing assessment (BSA) by using the repetitive saliva swallowing test and modified water swallowing test. The relationships between oral intake condition at the time of hospital discharge, discharge destination (e.g. home), type of haematoma and haematoma volume were examined. RESULTS: Abnormal BSA was found in 55% of the subjects (n = 62) with thalamic haemorrhage. Existence of a swallowing disorder was related to haematoma type and haematoma volume. A regular diet was possible in 41% of the subjects (n = 46). Age, haematoma volume, initial BSA evaluation and cognitive function had the greatest influence on a subject's ability to eat a general diet at the time of hospital discharge. Of those subjects in whom the initial BSA evaluation was normal, 70% were able to eat a regular diet at discharge. However, of those in whom the BSA evaluation was abnormal, >70% were discharged with enteral feeding. Furthermore, the rates of discharge to home were 95% in patients who could eat a regular diet and 30% in patients who needed enteral feeding. CONCLUSIONS: In the acute phase of thalamic haemorrhage, dysphagia was found in a high percentage of subjects. Dysphagia is associated with a poor prognosis for early hospital discharge.

Association between microbleeds observed on T2*-weighted magnetic resonance images and dysphagia in patients with acute supratentorial cerebral hemorrhage.

BACKGROUND: Approximately one-half of all patients with acute intracerebral hemorrhage (ICH) develop dysphagia that can lead to pneumonia or fatal outcomes; however, there are no reports about the relationship between swallowing function and cerebral microbleeds (CMBs) in these patients. Therefore, we investigated whether CMBs were associated with dysphagia in patients with ICH. METHODS: A total of 100 patients (mean age, 65.1 ± 14.1 years; range, 36-95 years) with acute supratentorial ICH were included in this study. CMBs were detected on T2*-weighted magnetic resonance imaging performed during admission using a 1.5-T scanner (mean duration from onset to magnetic resonance imaging, 12.6 ± 8.4 days). We assessed swallowing function by using bedside swallowing assessments on admission and by monitoring the mode of nutritional intake at discharge in relation to the number and location of CMBs. RESULTS: CMBs were detected in 60 of the 100 patients (60%) and were related to dysphagia. The number of CMBs and dysphagia were not related, but a significant relationship was observed in the presence of bilateral hematomas and CMBs. CONCLUSIONS: In addition to assessing just the presence or absence of CMBs, clinicians should identify the presence of bilateral or deep lesions in patients with ICH from the perspective of swallowing dysfunction.

Retrosplenial amnesia without topographic disorientation caused by a lesion in the nondominant hemisphere.

We report the case of a 68-year-old right-handed man who was admitted to our hospital because of sudden onset of headache. On admission, he presented with left homonymous hemianopsia, disorientation, and recent memory disturbance; however, he had normal remote memory and digit span. He was able to recall the room layout of his house and describe the route from the nearest station to his home on a map. However, at the hospital, he sometimes lost his way because of amnesia. Computed tomography (CT) and magnetic resonance imaging revealed a subcortical hematoma in the right occipital forceps and the parietal lobe, involving the cingulate isthmus. Single-photon emission CT imaging showed reduced perfusion not only in the retrosplenial region but also in the right thalamus. These findings suggested that the retrosplenial amnesia might have been caused by the interruption of hippocampal input into the anterior thalamus.

Subdural and intracerebral hemorrhage caused by spontaneous bleeding in the middle meningeal artery after coil embolization of a cerebral aneurysm.

Nontraumatic acute subdural hemorrhage (SDH) with intracerebral hemorrhage (ICH) is rare and is usually caused by severe bleeding from aneurysms or arteriovenous fistulas. We encountered a very rare case of spontaneous bleeding from the middle meningeal artery (MMA), which caused hemorrhage in the temporal lobe and subdural space 2 weeks after coil embolization of an ipsilateral, unruptured internal cerebral artery aneurysm in the cavernous portion. At onset, the distribution of hematoma on a computed tomography scan led us to believe that the treated intracavernous aneurysm could bleed into the intradural space. Emergency craniotomy revealed that the dura of the middle fossa was intact except for the point at the foramen spinosum where the exposed MMA was bleeding. Retrospectively, angiography just before and after embolization of the aneurysm did not show any aberrations in the MMA. Although the MMA usually courses on the outer surface of the dura and is unlikely to rupture without an external force, physicians should be aware that the MMA may bleed spontaneously and cause SDH and ICH.