[Herpes simplex encephalitis complicated with cerebral salt wasting syndrome: a case study].

A 78-year-old man was admitted to the hospital with a 4-day history of fever and confusion. Physical examination revealed oral dryness and decreased skin turgor. Blood tests showed hyponatremia (121.5 |mEq/l), and cerebrospinal fluid examination revealed positivity for herpes simplex virus 1 (HSV-1) via polymerase chain reaction. He was diagnosed with herpes simplex encephalitis and initiated acyclovir treatment. The hyponatremia was diagnosed as cerebral salt wasting syndrome (CSWS) and treated with hypertonic saline infusion and fludrocortisone. The cerebrospinal fluid HSV-1 DNA became negative, and the serum sodium levels normalized. Hyponatremia complicated with encephalitis is often caused by the syndrome of inappropriate secretion of antidiuretic hormone (SIADH), whereas CSWS is rare, mostly observed in tuberculous meningitis. Differentiating between the SIADH and CSWS is important as they require distinct therapeutic strategies.

Unusual Presentation of Double-seronegative Myasthenia Gravis with Positive Anti-LRP4 Antibody: Diagnostic Utility of a Videofluoroscopic Swallowing Study.

An 86-year-old woman was admitted to our hospital with cryptogenic progressive dyspnea and dysphagia following a tracheostomy procedure 4 months prior to presentation. She exhibited fluctuating diplopia, bilateral vocal fold paralysis, normal nerve test results, negative findings for serum anti-acetylcholine receptor and anti-muscle-specific kinase antibodies, and positive findings for anti-LDL-receptor related protein 4 (LRP4). A videofluoroscopic swallowing study (VFSS) with edrophonium revealed an improvement in bulbar paralysis. Consequently, the patient was diagnosed with double-seronegative myasthenia gravis (DSN-MG) and began immunomodulatory therapy. This case emphasizes the diagnostic challenges of bulbar-type DSN-MG and underscores the value of a VFSS with edrophonium for diagnosing this condition.

Delayed Superior Orbital Fissure Syndrome Arising More than One Month after Herpes Zoster Ophthalmicus and Meningitis.

A 79-year-old woman developed herpes zoster ophthalmicus (HZO) with a vesicular rash on the nasal root, which developed soon after intravenous acyclovir therapy. Although varicella zoster virus DNA was undetectable in the cerebrospinal fluid, she presented with ophthalmoplegia without optic nerve dysfunction 32 days after the onset of HZO. We diagnosed the patient with superior orbital fissure syndrome and administered intravenous immunoglobulin and systemic corticosteroids. Ophthalmoplegia did not immediately respond to these therapies but resolved 4 months later. We should be aware that ophthalmoplegia can occur, even after HZO and meningitis are completely treated.

Two Cases of Mechanical Thrombectomy in Patients with Fenestration of the M1 Segment of the Middle Cerebral Artery.

M1 fenestration of the middle cerebral artery is rare. We present two patterns of acute thromboembolic conditions in M1 fenestrations treated with mechanical thrombectomy. Case 1 was a male in his 60s presenting with right hemiparesis and aphasia. Angiography showed acute left M1 proximal occlusion; the first direct aspiration revealed two parallel routes, and the second aspiration achieved complete recanalization of the left M1 fenestration. Case 2 was a male in his 70s presenting right hemiparesis and aphasia. Angiography revealed a sudden stair-like narrowing of the left M1 in the intermediate part, and a retrograde blood flow cavity was observed on the upper side of the distal part. Mechanical thrombectomy was performed to diagnose the upper limb occlusion of the left M1 fenestration, and successful recanalization was achieved through direct aspiration. Anatomical variations, such as fenestration, should be considered to reduce complication risks.

Pembrolizumab-induced Myopathy with Anti-striated Muscle Antibodies Successfully Treated by Plasma Exchange.

A 70-year-old woman with advanced endometrial cancer developed right ptosis and muscle weakness in the right quadriceps after pembrolizumab administration. Serum creatine kinase (CK) levels were elevated, and anti-striated muscle antibodies were positive. On magnetic resonance imaging, the right vastus lateral muscle showed an abnormal signal. She was diagnosed with pembrolizumab-induced myopathy. We initiated plasma exchange (PE), and the ptosis immediately resolved. We then introduced oral corticosteroids, which improved her muscle weakness. We were able to rapidly diagnose her with ocular symptoms and serum CK level elevation. The early initiation of PE might prevent the exacerbation of pembrolizumab-induced myopathy.

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.

Bilateral occipital arteries arising from the thyrocervical trunks (ascending cervical artery-occipital artery anastomosis) diagnosed by magnetic resonance angiography.

We herein report a case of bilateral occipital arteries (OAs) arising from the thyrocervical trunks (TCTs). The patient was a 34-year-old woman with suspected basilar artery aneurysm underwent magnetic resonance (MR) angiography of the head and neck region using a 3-Tesla scanner. Cranial MR angiography revealed no aneurysm. Cervical MR angiography showed bilateral OAs arising from the TCTs. The extremely hyperplastic ascending cervical artery (ACA) arose from the transverse cervical artery, and continued to the OA, bilaterally. The OA usually arises from the proximal external carotid artery and runs posterosuperiorly; rarely, it arises from the internal carotid artery or the vertebral artery. The variation in our patient is regarded as bilateral ACA-OA anastomosis. Only one case of the unilateral type of this variation has been reported, having been diagnosed during dissection. Before cervical arterial intervention or head and neck surgery, identification of OA variation is important. During the interpretation of cervical MR angiography findings, careful observation of the origin and course of the OA is required.

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.

Successful treatment of ischemic stroke associated with brachiocephalic artery stenosis using alteplase.

Introduction: Brachiocephalic artery stenosis rarely causes right hemispheric infarction with associated left hemiparesis. To date, there have been no reported cases of stroke associated with brachiocephalic artery stenosis that were successfully treated with recombinant tissue-type plasminogen activator (rt-PA), alteplase. Case Report: An 80-year-old woman presented with left hemiparesis. Brain computed tomography showed no hemorrhage, and computed tomography angiography demonstrated brachiocephalic artery stenosis. Alteplase was administered based on a diagnosis of ischemic stroke. Brain magnetic resonance imaging showed multiple acute infarctions. Thereafter, the blood pressure of the right arm was found to be lower than that of the left arm. The patient's neurological deficits gradually improved; she was eventually able to walk again and was thus discharged home. Conclusion: While the combination of left hemiparesis and a decrease in blood pressure in the right arm are well known in patients with stroke associated with Stanford type A aortic dissections, it may also occur in patients with stroke due to brachiocephalic artery stenosis. Unlike stroke associated with Stanford type A aortic dissections, stroke due to brachiocephalic artery stenosis may be treated with alteplase.

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.

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.

Endovascular Treatment for Acute Ischaemic Stroke Caused by Vertebral Artery Dissection: A Report of Three Cases and Literature Review.

Vertebrobasilar artery dissection is an uncommon cause of acute ischaemic stroke (AIS). Optimal endovascular management has not been established. This study aimed to share our experience with endovascular reperfusion therapy for vertebrobasilar artery occlusion due to vertebral artery dissection (VAD). We retrospectively reviewed 134 consecutive patients with AIS who received urgent endovascular reperfusion therapy between November 2017 and November 2019. Three patients diagnosed with VAD were investigated. The evaluation included mechanisms of vertebrobasilar artery occlusion due to VAD, variations in endovascular procedures, and functional outcomes. Dissections at the V3, V4 and extension of V3 to V4 segments were seen in one patient each. The mechanism of AIS was different in each patient: occlusion of the distal non-dissected artery due to an embolus from the dissection site (distal occlusion), haemodynamic collapse of the entire vertebrobasilar artery system due to the arterial dissection itself (local occlusion), or coexistence of distal occlusion and local occlusion (tandem occlusion). The endovascular reperfusion therapy was performed corresponding to the abovementioned mechanisms: mechanical thrombectomy for distal occlusion, stenting for local occlusion, and a combination of thrombectomy and stenting for tandem occlusion. In all three patients, effective recanalization and functional independence (modified Rankin Scale scores of 0-2 at 90 days after the onset) were achieved. Endovascular treatment corresponding to the individual mechanism of AIS may improve patient outcomes.

Distribution of Deep Gray Matter Lesions on Magnetic Resonance Imaging in Lymphomatosis Cerebri.

We herein report the distribution of gray matter lesions on magnetic resonance imaging (MRI) in two patients with lymphomatosis cerebri (LC). In our patients, the fluid-attenuated inversion recovery sequence of brain MRI demonstrated a bilateral and diffuse high signal intensity, not only in the white matter but also in the thalamus, globus pallidus, putamen, and hippocampus. Among the deep gray matter, the caudate head and putamen (striatum) were relatively spared when compared with the globus pallidus, thalamus, and hippocampus. Interestingly, we found seven previous reports of similar MRI findings, with relative sparing of the striatum, in patients with LC. This finding may be characteristic of LC and help facilitate its diagnosis. Further investigations of a larger number of LC patients are necessary to confirm these findings.

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.