[A case of cerebral embolism treated with early surgical intervention for aneurysm of sinus of Valsalva as embolic source].

The patient was a 78-year-old man. He was transferred to the emergency room presenting with aphasia and right hemiplegia. Head CT and CT angiography demonstrated a narrow territory of early ischemic signs and occlusion of the horizontal segment of the left middle cerebral artery (MCA), respectively. Endovascular thrombectomy was performed and complete recanalization of MCA was achieved. After admission, transthoracic echocardiography revealed a cystic mass near the left coronary apex of the aortic valve, which was diagnosed as an aneurysm of the sinus of Valsalva (ASV) by contrast-enhanced chest computed tomography. Transesophageal echocardiography showed a severe smoke-like echo within ASV, despite being in sinus rhythm. Intravenous anticoagulant therapy was started, and patch closure was performed by cardiovascular surgeons on the 13th day despite of early period after stroke onset. During the operation, no thrombus was seen around the inlet of ASV. On the two days after the operation, paroxysmal atrial fibrillation was detected and anticoagulant therapy was continued. In this patient, ASV was regarded as the embolic source of cerebral embolism, and anticoagulant therapy and surgical intervention were selected in the early period after stroke onset to prevent embolism recurrence, resulting in a favorable clinical course.

Long-term effectiveness of anticoagulants in oldest-old stroke survivors with atrial fibrillation.

BACKGROUND: Long-term anticoagulant therapy in oldest-old persons poses the risk of bleeding complications. The aim of this study was to evaluate the long-term benefits of anticoagulant therapy for oldest-old stroke survivors with AF. METHODS: Patients with atrial fibrillation (AF) who were 90 years of age or older and were prescribed an anticoagulant on discharge were identified from a set of data from a prospective follow-up registry of 1,484 consecutive patients admitted for ischemic stroke or transient ischemic attack over a 4-year period beginning in 2014. The outcome measures were stroke and death following discharge. RESULTS: Of the 77 identified patients with AF who were 90 years of age or older, 71 were prescribed an anticoagulant (median age 93 years, 73% women). Thirty-nine patients were given a direct oral anticoagulant (DOAC) (median age 92 years, 69% women), and 32 were given warfarin (median age 93 years, 78% women). During the follow-up period (median 466 days), 9 patients (13%) had stroke recurrence (recurrence rate: 14%/year), and 25 patients (35%) died (mortality rate: 33%/year). The type of all recurrent strokes was ischemic, and no fatal bleeding occurred. There was no difference in the incidence of recurrent strokes according to anticoagulant type (DOAC 15%/year, warfarin 13%/year, P = 0.743), but a higher proportion of patients on warfarin died (21% vs. 47%, P = 0.002). CONCLUSIONS: Given that a higher proportion of oldest-old stroke survivors with AF on anticoagulant therapy have recurrent ischemic stroke rather than hemorrhagic stroke, long-term anticoagulant therapy may be justified for secondary stroke prevention.

[Eosinophilic granulomatosis with polyangiitis presenting with Guillain-Barré syndrome-like acute course. A case report].

A 57-years-old man with a history of bronchial asthma and pansinusitis developed acute progressive muscle weakness and sensory disturbance of the distal limbs after upper respiratory infection. On day 15 after onset of sensory disturbance and muscle weakness, the patient admitted to our hospital. A neurological examination revealed asymmetry weakness of both proximal and distal muscles, "glove and stocking type" hypoesthesia, and paresthesia without obvious pain. Blood tests and a nerve conduction study demonstrated eosinophilia and elevation of MPO-ANCA, axonal multiple mononeuropathy, respectively. The cerebrospinal fluid was normal. Eosinophilic granulomatosis with polyangiitis (EGPA) or Guillain-Barré syndrome (GBS) were suspected. So intravenous immunoglobulin therapy (IVIg) and high dose methylprednisolone pulse therapy (HDMP) followed by oral prednisolone were started. However, neurological symptoms did not improve. Sural nerve biopsy on day 31 revealed varying myelinating fiber loss at every nerve bundle and perivascular lymphocytic infiltration. The results did not fulfill the pathologic criteria for EGPA, but supported the changes of vasculitis. Cyclophosphamide (CPA) pulse therapy was administered for the additional therapy. Neurological symptoms did not improve and worsened again after decreasing oral prednisolone; therefore, combined therapy with IVIg, HDMP, and CPA was administered. Neurological symptoms then diminished gradually and the MPO-ANCA level and number of eosinophils normalized. This case suggests the importance of early nerve biopsy to obtain pathological findings supportive of EGPA diagnosis to allow introduction of aggressive immunosuppressive therapy such as CPA in a case with acute progressive motor-sensory neuropathy due to EGPA mimicking GBS.

[Autopsy case of cerebral embolism with lung cancer and atrial fibrillation in which the left atrium may have become an incubator for a tumor embolus].

An 82-year-old man with advanced lung cancer who had declined aggressive therapy was transferred to our hospital due to sudden-onset consciousness disturbance, global aphasia, and right hemiplegia. An electrocardiogram showed atrial fibrillation, and brain MRI and MRA revealed acute ischemic lesions of the left hemisphere and occlusion of the left internal carotid artery (ICA), respectively. We diagnosed acute ischemic stroke due to left ICA occlusion and performed endovascular thrombectomy, which resulted in complete recanalization of the left ICA after retrieval of the culprit embolus. Pathological examination of the retrieved thrombus revealed the presence of tumor tissue, as well as fibrin or red blood cells. Treatment was continued after admission, but the patient died of respiratory failure on day 40 of hospitalization. Autopsy revealed invasion of the tumor in the pulmonary vein, but not in the wall of the left atrium where thrombi were present. However, pathological examination of these thrombi in the left atrium revealed tumor tissue, along with fibrin or red blood cells. These findings suggest that the wall of the left atrium, in which lung cancer had not invaded, may be an incubator of a mixed embolus containing tumor tissue and thrombi in a case of cerebral embolism associated with both lung cancer and atrial fibrillation.

[Acute headache resulting from intracranial venous reflux due to occlusion of the brachiocephalic vein ipsilateral to a dialysis shunt].

A 72-year-old man on hemodialysis for 7 years with end-stage renal disease was admitted to our institution due to an acute headache. Physical examination revealed normal signs except for noise on the back of his neck. His head CT and brain MRI showed no abnormal findings, while his MRA demonstrated abnormal signals in the left transverse to sigmoid sinus (T-S) suggesting a left dural arteriovenous fistula. After admission, his headache persisted and left orbital numbness also occurred. His digital subtraction angiography performed on the 5th day after admission showed no vascular malformation of either the T-S or cavernous sinus (CS). However, it showed occlusion of the left brachiocephalic vein (BCV) and the origin of the left internal jugular vein (IJV) resulting in intracranial venous reflux. These findings indicated the possibility that his acute headache was caused by intracranial venous reflux and increase of intracranial pressure resulting from the occlusion of the BCV ipsilateral to a dialysis shunt. Percutaneous transluminal angioplasty (PTA) for occlusion of the left BCV was performed on the 9th day and successful dilation of the lesion with a residual stenotic ratio less than 30 percent was obtained. After the angioplasty, venous reflux to the intracranial vein was markedly reduced and his headache and orbital numbness disappeared. One day after the procedure, MRA demonstrated the disappearance of the abnormal signals of the left T-S. Twelve months after discharge, he felt discomfort in the left of his face and the re-occlusion of the left VCV was demonstrated by angiography, therefore he received re-PTA. We recommend that physicians consider occlusion of the BCV ipsilateral to a dialysis shunt and intracranial venous reflux as a cause of acute headache in patients on hemodialysis.

[Sudden-onset monoplegia of the upper limb due to traumatic subclavian artery pseudoaneurysm after an interval of three days from tumbling].

A 66-year-old woman was admitted to our institution with sudden-onset weakness of her left upper limb. Neurological examination revealed monoplegia and sensory loss of the limb. A brain MRI did not find evidence of an acute ischemic stroke. Her medical history revealed that she had fallen and bruised her shoulder 3 days earlier. Detailed physiological examination revealed that there was a mild subcutaneous ecchymosis with tenderness in the left shoulder. An additional contrast-enhanced chest CT scan showed a fracture of the clavicle diaphysis and a pooling contrast agent demonstrating a 60*40 mm mass near the left subclavian artery (SUB-A) which suggested a pseudoaneurysm. We determined that her symptoms were due to compression of the brachial plexus by immediate growth of a traumatic SUB-A pseudoaneurysm (TSAP) due to her earlier fall. For reduction of pressure to the brachial plexus by the TSAP and prevention of rupture, an endovascular treatment team performed endovascular internal trapping of the left SUB-A just distal to the orifice of the left vertebral artery and a cardiovascular surgeon performed percutaneous drainage of the pseudoaneurysm. After the procedure, the palsy and sensory loss of the left hand gradually improved. A TSAP could be one of the causes of sudden-onset palsy of the upper limb within a few days after a fall.

[A case of paraneoplastic limbic encephalitis associated with neck neuroendocrine carcinoma].

A 69-year-old man presented with a history of personality change for several years. He was admitted to our hospital due to partial seizure. A cerebrospinal fluid test and an electroencephalogram showed no specific abnormalities, but brain magnetic resonance imaging revealed abnormal findings in the right temporal pole, bilateral amygdala to hippocampus, and insular cortex. He was diagnosed with limbic encephalitis accompanied by partial seizure, and received infusion of an antiepileptic agent and acyclovir. Additional examinations for malignancy and autoimmune disease were performed, and neck CT and MRI revealed a neck tumor. Neck lymph node biopsy suggested lymph node metastasis of a neuroendocrine neoplasm derived from other organs. He did not want aggressive treatment involving surgical resection and chemotherapy, and thus, conservative treatment was chosen by an otorhinolaryngologist and immunotherapy was not used. After discharge, the neck tumor grew gradually. To manage the focal mass effect, chemotherapy and surgical resection followed by chemoradiotherapy were performed by the otorhinolaryngologist on days 244 and 325 of the disease course, respectively. Histology of resected tissues disclosed neck neuroendocrine carcinoma derived from a submandibular gland. His personality change improved temporarily after surgical resection, but then worsened again with regrowth of the tumor. He died on day 723. After death, a blood test revealed the presence of anti-amphiphysin antibody. This case suggests that neck neuroendocrine carcinoma can induce paraneoplastic limbic encephalitis, and in such cases, early surgical resection of the neck tumor with suspected lymph node metastasis is necessary both to control symptoms associated with encephalitis and to exclude carcinoma derived from the neck itself.

[A case of Wernicke encephalopathy with hypoacusia and MR high intensity of the inferior colliculi that normalized after thiamine administration].

A 61-year-old man was admitted to our institution with progressive hypoacusia, double vision, and lightheadedness. Neurological examination on day 6 of his illness showed severe hypoacusia, mild confusion, ocular motility disorder, truncal ataxia and absence of a deep tendon reflex. MRI fluid-attenuated inversion recovery imaging revealed symmetrical high intensities in the tectum of the midbrain, involving the bilateral inferior colliculi and the bilateral medial thalami, which suggested Wernicke encephalopathy (WE). Thiamine was administered immediately after completion of the MRI, and the patients' hearing and other abnormal neurologic signs improved rapidly within a few days, except for the absence of the deep tendon reflex. Whole blood examination at admission revealed very low levels of vitamin B1. The patient was discharged on day 19, and MRI on day 39 showed the disappearance of the abnormal high intensities involving the bilateral inferior colliculi. The present case indicates that hypoacusia and abnormal MRI signal due to WE might be normalized by administration of thiamine a few days after the onset of symptoms.

[A case of motor dominant neuropathy and focal segmental glomerulosclerosis associated with Sjögren's syndrome].

A 49-year-old woman was admitted to our hospital with gradually progressive weakness of the limbs for about 20 days. She presented with weakness of the limbs, predominantly in the proximal portion, and slight dysesthesia of the limbs, predominantly in the distal portion. Repeated nerve conduction examination revealed axonopathy dominantly in the motor neurons. Therefore, we suspected her as having Guillain-Barré syndrome, and initiated intravenous administration of high-dose immunoglobulin. However, her symptoms progressed gradually and finally she found it difficult to walk. Her urine analysis simultaneously demonstrated albuminuria, and a kidney biopsy indicated focal segmental glomerulosclerosis. At that point, laboratory examination showed high levels of anti SS-A antibody and salivary gland biopsy revealed infiltration of a significant number of lymphocytes around the gland, which led to the diagnosis of Sjögren's syndrome. We considered the etiology of the neural and renal dysfunction as due to the inflammatory mechanism associated with Sjögren's syndrome. Therefore, we administered a second course of immunoglobulin therapy and steroid therapy, which included both pulse and oral administration. Her neurologic symptoms and albuminuria improved rapidly after steroid therapy. The present case indicates that both motor dominant neuropathy and focal segmental glomerulosclerosis can occur in patients with Sjögren's syndrome.

[A case of acute progressive myelopathy due to intravascular large B cell lymphoma diagnosed with only random skin biopsy].

A 64-year old woman was admitted to our hospital with subacute onset paraparesis and sensory disturbance at a level below Th10. Spinal MRI showed a T2 weighted high-signal intensity lesion at a level from Th5 to Th12, and an abdominal CT showed a mass in the left kidney. Her paraparesis deteriorated rapidly, and administration of high dose methyl prednisolone followed by oral steroid therapy was started before obtaining of a definitive diagnosis. However her symptoms did not improve after the beginning of treatment. At the same time, a bone marrow puncture, and biopsies from kidney and spinal cord were performed. These biopsies demonstrated no clues, diagnostically. Therefore a random skin biopsy was performed at the five sites on the 17th day after the steroid dosage end. From this, pathological evidence of intravascular large B cell lymphoma (IVLBCL) was shown. For rapid diagnosis of acute myelopathy with mass lesion of another organ due to IVLBCL, a biopsy is taken not only from spinal cord or mass lesions, but is also taken of multiple sites in skin randomly. This must be performed without a delay before a sudden deterioration of neurologic symptoms can occur from ischemic events not responsive to steroid therapy.

[Neurologist and emergency neuroendovascular revascularization -training programs for endovascular procedures-].

Emergency neuroendovascular revascularization is a reperfusion therapy for acute stroke. The operator for this therapy has to obtain a license as a specialist in endovascular procedures. For neurologists wishing to acquire this license, there are two kinds of training programs: full-time training and concurrent training. Full-time training was chosen by the first author of this review, while concurrent training will be performed by staff in the author's department. The advantage of full-time training is the acquisition of a lot of experience of various diseases that are treated with endovascular procedures and managed in the periprocedural period. However, full-time training has the disadvantages of a requirement to discontinue medical care of neurological diseases except for stroke and employment at a remote institution. The advantages and disadvantages of concurrent training are the reverse of those of full-time training. Neither training system can succeed without cooperation from Departments of Neurology in neighboring universities and the institutional Department of Neurosurgery. It is particularly important for each neurologist to establish a goal of becoming an operator for recanalization therapy alone or for all fields of endovascular procedures because training will differ for attainment of each operator's goal.

Prediction of dysphagia severity: an investigation of the dysphagia patterns in patients with lateral medullary infarction.

OBJECTIVE: In order to identify the factors that influence the swallowing function in patients who develop Wallenberg syndrome (WS) following lateral medullary infarction (LMI), we examined various patient characteristics, including the passage pattern abnormality (PPA) of a bolus through the upper esophageal sphincter (UES). METHODS: Fifty-four pure LMI patients with dysphagia participated in this study. PPA, defined as the failure of bolus passage through the UES corresponding to the intact side of the medulla, was identified during videofluorographic swallowing evaluations of each patient. On brain magnetic resonance imaging, the subjects' lesions were classified vertically into three levels and horizontally into seven levels in relation to the involvement of the ambiguous and/or solitary nuclei. Logistic regression analyses were performed for age, sex, PPA and the vertical and horizontal sites of the lesions. RESULTS: In terms of severity, 15 subjects were categorized as having mild dysphagia, 26 subjects were categorized as having moderate dysphagia and 13 were categorized as having severe dysphagia. Subjects with cephalic lesions, greater vertical spread of the lesion and PPA were more likely to have severe dysphagia. PPA and a greater vertical spread of the lesion were related to the severity of the functional outcome (p<0.01). The horizontal extent of the lesion was not strongly related to the prognosis. CONCLUSION: The presence of PPA in LMI patients is suggestive of abnormalities in the swallowing pattern and, in turn, damage to the medullary central pattern generator. The presence of PPA and a greater vertical spread of the lesion can be useful predictors of severe dysphagia.

[Endovascular trapping using a tandem balloon technique for a spontaneous vertebrovertebral fistula associated with neurofibromatosis type 1].

We report a rare case of a young man who had spontaneous left vertebrovertebral fistula associated with neurofibromatosis Type 1. His complaints were severe pain in the left neck and numbness in the left upper extremity. Cervical MR images showed a large abnormal flow void to the left of the spinal canal. An angiogram demonstrated a fusiform aneurysm and a high flow arteriovenous fistula in the left vertebral artery that drained into the internal vertebral plexus and formed a large venous varix. The occipital artery, the thyrocervical artery and the contralateral vertebral artery were associated with the fistula. The arteriovenous fistula was treated by endovascular coil embolization, using a tandem balloon technique. For this fistula, exhibiting the combination of high flow and multiple associated arteries, the flow control technique during the coil embolization, using tandem balloons in both the subclavian artery and the distal portion of the fistula of the vertebral artery, was safe and feasible for preventing coil migration.

Characteristics of intracranial branch atheromatous disease and its association with progressive motor deficits.

BACKGROUND: Small deep brain infarcts are often caused by two different vascular pathologies: 1. atheromatous occlusion at the orifice of large caliber penetrating arteries termed branch atheromatous disease (BAD) and 2. lipohyalinotic degenerative changes termed lipohyalinotic degeneration (LD). We herein analyze and describe the characteristics of these 2 different pathologies. METHODS: We studied 394 patients with penetrating artery territory infarcts in the territories of the lenticulostriate arteries and anterior pontine arteries. Radiologically defined BAD of the lenticulostriate arteries was defined as infarcts with size more than 10mm in diameter on axial slice and visible for 3 or more axial slices, and that of the anterior pontine arteries was defined as unilateral infarcts extending to the basal surface of the pons. Within each of the 2 territory groups, differences between BAD and LD were compared. RESULTS: Ninety five patients in the lenticulostriate arteries group (36.1%) and 78 patients in anterior pontine arteries group (59.5%) were classified as BAD. Initial NIHSS, incidence of progressive motor deficits and poor functional outcome were significantly higher and incidence of concomitant silent lacunar infarcts tended to be lower in BAD than LD. In logistic regression analysis, BAD compared with LD was independently associated with PMD, in lenticulostriate arteries group (OR: 4.21, p=0.0001) and in anterior pontine arteries group (OR: 5.32, p=0.0018). CONCLUSIONS: Radiologically defined BAD and LD had different characteristics. BAD was significantly associated with progressive motor deficits and considered as a major vascular mechanism of progressive motor deficits in penetrating artery infarcts.

Predictive factors for progressive motor deficits in penetrating artery infarctions in two different arterial territories.

BACKGROUND: Progressive motor deficits (PMD) are common in cerebral penetrating artery disease (PAD) during the acute stage and leads to severe disability. Reliable predictors and stroke mechanism for PMD in PAD have been yet to be elucidated. Moreover, difference of predictors between topographically classified PAD has not ever been systematically studied. METHODS: Three hundred ninety two consecutive patients with acute PAD (<20 mm) who showed lacunar motor syndrome and admitted within 24 h after onset were selected for this study. Patients were divided into 2 groups whose infarcts were topographically located within the territories of lenticulostriate arteries (LSA), and anterior pontine arteries (APA). Within each of the 2 groups, factors associated with PMD were analyzed. RESULTS: Progressive motor deficits were found in 55 patients (21.0%) in LSA group and 38 patients (29.0%) in APA group. In multivariate analysis, female sex and severity of motor deficit on admission (NIHSS 5 or more) were common independent predictors for PMD in both groups. The specific predictors were single infarcts without concomitant silent lacunar infarcts and preceding TIAs in LSA group and diabetes mellitus in APA group. CONCLUSIONS: Predictive factors for PMD were different in the 2 different territory groups. Diabetes mellitus was particularly associated with PMD in APA group.

[A patient with small cerebellar infarcts at tonsil and nodulus who complained of vertigo, vomiting and chest oppression].

We reported a 66-year-old man who complained of headache, vertigo, vomiting and chest oppression sensation. He could not walk veering to right and spontaneous contrarateral horizontal nystagmus was noted. A MRI DWI showed scattered multiple small high signals within the territory of left medial branch of posterior inferior cerebellar artery. Cerebellar infarcts specifically affected the tonsil and nodulus, which has tight connections with vestibular labyrinth and vestibular nuclei. Digital subtraction angiography exhibited tapered occlusion on the V4 segment of the right vertebral artery. Stroke mechanism of artery to artery embolism from occlusive site was presumed. As small multiple infarcts were limited within the cerebellar tonsil and nodulus, repeated CT or MRI T2 weighted image did not showed obvious findings. It is worth noting for differential diagnosis that small cerebellar infarcts only detected by diffusion MRI can produce strong vertigo, nausea and chest oppression.