[A case of recurrent cerebral embolism associated with Lambl's excrescence].

A 75-year-old female had a history of prior ischemic stroke with aphasia and right hemiplegia. Magnetic resonance angiography showed left internal carotid artery occlusion. She was successfully treated with intravenous recombinant tissue plasminogen activator (IV t-PA) and underwent endovascular thrombectomy (EVT). She was diagnosed with cardioembolic stroke due to the presence of atrial fibrillation and mitral valve stenosis, and warfarin was administered. However, she experienced large vessel occlusion twice within 2 years. Upon further analysis, transesophageal echocardiography revealed a mobile hyperechoic structure on the aortic valve, which was assumed to be an embolic source. Thus, we decided to perform mitral and aortic valve replacement. The excised aortic valve structure was suggested to be an example of Lambl's excrescence, histopathologically. After surgery, the patient had no recurrence for 3 years. Several cases of ischemic stroke associated with Lambl's excrescence have been reported, but definitive guidelines for managing patients with Lambl's excrescence do not currently exist. Surgical intervention for Lambl's excrescence with recurrent ischemic events may be important for preventing further recurrence.

Vessel Wall Injury After Stent Retriever Thrombectomy for Internal Carotid Artery Occlusion with Duplicated Middle Cerebral Artery.

BACKGROUND: Experimental evidence that stent retriever thrombectomy (SRT) may cause arterial wall damage is increasing, although histopathologic findings focused on vessel wall injury after SRT have not fully been reported in humans. CASE DESCRIPTION: We describe an autopsy case of an 82-year-old woman who presented with acute left intracranial internal carotid artery occlusion and received SRT. When we attempted to catheterize the M1 segment of the normal middle cerebral artery (MCA), we were not aware of a duplicated MCA and advanced the microcatheter into the main MCA trunk with a smaller caliber than that of the normal M1 segment. A 6-mm Trevo thrombectomy device was deployed from the main MCA trunk but was insufficiently expanded in the MCA segment. Two passes with a Trevo resulted in failed recanalization. The patient died on postoperative day 4 because of extensive infarction. Autopsy revealed a duplicated MCA and subintimal arterial dissection in the proximal segment of the main MCA trunk. The supraclinoid internal carotid artery also revealed endothelial denudation and mural thrombus. CONCLUSIONS: We provide a histopathologic report of vessel wall injury after SRT that caused failed recanalization. Anatomic variations of the MCA should be considered when selecting suitable thrombectomy devices in order to avoid vessel wall injury.

Carotid artery stenting with proximal embolic protection via the transbrachial approach: sheathless navigation of a 9-F balloon-guiding catheter.

PURPOSE: Transbrachial carotid artery stenting (TB-CAS) is performed as an alternative procedure for patients with hostile vascular anatomy of the aortic arch and aortic or peripheral artery disease. Proximal protection during TB-CAS is not generally feasible because a small size of the brachial artery may preclude using a large-diameter sheath introducer. We, herein present a novel method that enables proximal protection during TB-CAS by sheathless navigation of a 9-F balloon-guiding catheter equivalent to a 7-F sheath. METHODS: We analyzed eight consecutive patients who underwent TB-CAS with proximal protection using the sheathless method from April 2016 to June 2017. Relevant demographic, radiographic, and procedural features were retrospectively reviewed. RESULTS: We performed TB-CAS using our method for five patients with a bovine or type 3 aortic arch, for one patient with combined peripheral artery disease, and for two patients with a type 1 or 2 aortic arch. We successfully navigated the balloon-guiding catheter via the brachial artery and performed CAS under proximal flow control in all patients. However, we experienced kinking and exchange of the balloon-guiding catheter in one patient and a periprocedural thromboembolic event occurred. A pseudoaneurysm at the access site developed in one patient. CONCLUSION: TB-CAS with proximal embolic protection using the sheathless method is feasible and may provide an alternative approach in carefully selected patients who have difficult anatomy in the transfemoral approach and plaques with a high risk of distal embolization.

Serum levels of renin-angiotensin system components in acute stroke patients.

AIM: The renin-angiotensin system (RAS) is involved in the pathogenesis of ischemic brain damage, and is suggested to have therapeutic potential in stroke by large clinical trials. However, the changes of serum RAS components in patients with acute stroke are totally unknown. We assessed the serum levels of RAS components in acute stroke patients, and investigated the relationship between RAS and stroke subtype. METHODS: Levels of angiotensin-converting enzyme (ACE), ACE2 and angiotensin II in serum from patients with acute stroke (n=117; male 75, female 42, age 69 ± 13 years) were measured by an established enzyme-linked immunosorbent assay method. Diagnosis of subtypes of ischemic stroke was based on the Trial of Org10172 in Acute Stroke Treatment classification. The Kruskal-Wallis test with post-hoc Mann-Whitney U-test with Bonferroni correction was carried out for statistical analysis. RESULTS: Classification of stroke was as follows: large-artery atherosclerosis (n=44), cardioembolism (n=33), small-vessel occlusion (n=31), stroke of other determined etiology (n=9). Levels of angiotensin II and ACE did not show significant differences among each group. However, serum ACE2 level was significantly higher in the cardioembolism group than in the small-vessel occlusion group (cardioembolism 13 ± 9.3 ng/mL, large-artery atherosclerosis 10.2 ± 6.8 ng/mL, small-vessel occlusion 7.2 ± 3.7 ng/mL, stroke of other determined etiology 10.2 ± 7.3 ng/mL). ACE2 level showed a positive correlation with serum brain natriuretic peptide level (P=0.031). In contrast, angiotensin II concentration showed a negative correlation with National Institute of Health Stroke Scale score on admission (P=0.023). CONCLUSIONS: These findings suggest that changes of serum RAS components could reflect stroke subtypes and predict stroke severity.

[A case of cerebral cardioembolism successfully treated by Merci retriever despite a large ischemic change on diffusion-weighted MR imaging].

A 63-year-old man with paroxysmal atrial fibrillation presented with aphasia (16:30) followed by right hemiplegia. The last known time that he was clinically well was 14:30. On admission (17:43), his baseline NIHSS score was 34. Head diffusion-weighted MR imaging (DWI) demonstrated large hyperintense signals throughout the left middle cerebral artery (MCA) territory. The left carotid angiogram (CAG) demonstrated occlusion of the left ICA 2 cm distal from the bifurcation. The right CAG showed a small branch laterally extending from the left anterior cerebral artery (ACA). Mechanical thrombectomy with a Merci retriever removed a large amount of thrombi after the first trial. The left ICA and MCA were recanalized to grade TICI 2b. The left hemiplegia was markedly improved, and he could walk independently. His NIHSS score was 11 at discharge. Revascularization therapy may improve a motor deficit in patients with possible penumbra of the precentral gyrus by collateral circulation from the ACA even if the ischemic lesion in the MCA territory is large on DWI.

Efficacy of High-frequency Magnetic Stimulation of the Sacral Root in Patients with Urinary Incontinence Following a Radical Prostatectomy.

OBJECTIVES: We report on our initial data from a prospective study to determine the efficacy of high-frequency magnetic stimulation on the sacral root (MSSR) for the intractable post-radical prostatectomy, stress urinary incontinence (SUI). METHODS: A total of 14 men with persistent SUI after a radical prostatectomy underwent treatment once every 2 weeks over a 40-week period for a total of 20 sessions. The outcome was assessed by these variables at baseline, at immediately after the first session, and at immediately after the final (20(th) ) session. RESULTS: Mean leak episodes (per day) consistently decreased after the first to the final session (from 6.1 ± 2.9 to 3.5 ± 2.6, and to 3.0 ± 2.3, P < 0.01), and it remained to be decreased following 2 months after the final session. The mean pad weight (per h) also decreased after the treatment (but no statistically significant change compared to the pretreatment level). The cystometric bladder capacity at the first desire to void and the capacity at the strong desire to void increased significantly following the high-frequency MSSR (first desire to void: from 146 ± 43 to 182 ± 52 mL; strong desire to void: from 224 ± 69 to 258 ± 60 mL, P < 0.01). No obvious complication was observed in any patients during or after the treatment. CONCLUSION: This study provides the preliminary evidence that high-frequency MSSR may potentially afford a useful option with minimal invasiveness for the patients with obstinate SUI after a radical prostatectomy.

[Recurrent idiopathic hypertrophic pachymeningitis after surgery of chronic otitis media with cholesteatoma: a case report].

A 70-year-old woman visited our hospital because of a chronic headache four years ago. MRI demonstrated almost symmetrically thickened dura mater in the frontal and parietal regions. She was diagnosed with idiopathic hypertrophic pachymeningitis and received corticosteroid therapy. Corticosteroid therapy improved her clinical symptoms and thickening of the dura mater. She remained free of neurological symptoms after prednisolone was tapered to 5 mg/day. However, three years ago, she developed ear pain, otorrhea and hearing loss on the left side. She was diagnosed as having otitis media with cholesteatoma last year, and underwent mastoidectomy and tympanoplasty on the left side three months ago. After surgery, she recovered from the ear symptoms, but noticed a headache on the left side. Three months after the surgery, MRI demonstrated the recurrence of hypertrophic pachymeningitis in the frontal and parietal regions, particularly on the left side. Corticosteroid therapy again improved the headache and thickening of the dura mater. The finding that the patient recovered after corticosteroid therapy alone suggests that non-infectious inflammation played a major role in the pathogenesis. Chronic inflammation associated with otitis media with cholesteatoma or surgical invasiveness might induce the recurrence of idiopathic hypertrophic pachymeningitis.

[Migraine improved by amlodipine medication in a case with hypertension].

A 64-year old woman visited our outpatient clinic with chronic headache on August 30(th), 2005. She had been suffering from migraine from her 30's. Although sumatriptan improved her symptoms, migraine attacks had occurred every other day recently. At presentation, her headache was moderate in intensity and lasted about four hours. Daily activity worsened her pulsating headache associated with nausea and vomiting. She diagnosed with migraine without aura, which met the diagnostic criterion for migraine by International Classification of Headache Disorders. Lomerizine was administered as a prophylactic agent, however it did not reduce the frequency of migraine attacks. Accordingly, lomerizine was withdrawn from November 2005. Her blood pressure gradually increased during the course, and amlodipine, 2.5 mg/day, was started for her hypertension May 2007. After starting amlodipine, she noticed a reduction in migraine attacks, only two times a month in August 2007. Assessment with the HIT-6 questionnaire showed great improvement in her QOL score for daily life. Although the underlying mechanisms have not been elucidated, amlodipine was effective in reducing migraine attacks in this patient. The findings in this case may suggest that amlodipine administration might be a useful strategy to control migraine attack.

[An autopsy case of amyotrophic lateral sclerosis with ampulla cardiomyopathy].

We herein report an autopsy case of a 63-year-old man with amyotrophic lateral sclerosis (ALS) who developed "ampulla cardiomyopathy." At the age of 56, he noticed a progressive weakness in his right upper extremity. One year later, a progressive weakness of the left upper extremity began. Dropped head and progressive weakness of the lower extremities emerged at the ages of 61 and 62, respectively. Intravenous immunoglobulin and plasma-exchange therapies did not improve his weakness. At the age of 63, one month before his death, he was hospitalized due to aspiration pneumonia. A tracheostomy was performed to secure his airway. Four days after the operation, an ST elevation of his electrocardiogram was incidentally found on the ECG monitor. An echocardiogram revealed diffuse hypokinesia of the wall of the left ventricle except in the basal portion, which is the typical finding of "ampulla cardiomyopathy." Wall motion of the left ventricle improved and his circulatory condition was stabilized after treatment, but his condition thereafter worsened again and he died 3 weeks later. An autopsy examination revealed diffuse fibrosis and degeneration of the cardiomyofibers. Neuropathological examination revealed neuronal cell loss, Bunina bodies and skein-like inclusions in the hippoglossal nuclei. In the thoracic spinal cord, degenarated anterior horn cells were seen and macrophage permeation in the corticospinal tract were shown by CD68 immunostaining. Therefore, the final neuropathological diagnosis was ALS. This report is the first autopsy case of ALS complicated with "ampulla cardiomyopathy."

[Case of recurrent transverse myelitis and diplopia after meningomyeloradiculitis caused by Epstein-Barr virus].

A 60-year-old woman was admitted to our hospital because of pyrexia, headache, gait disturbance, and sensory disturbance in the lower part of the body four days after she received an epidural block for postherpetic neuralgia. Neurological tests revealed neck stiffness, flaccid paraparesis in the legs with absent deep tendon reflexes, and hyperesthesia below Th7. According to a cerebrospinal fluid examination, the protein concentration was 245 mg/dl and the cell count was 176/mm3. PCR analysis of the cerebrospinal fluid was positive for Epstein-Barr virus (EBV). Serological tests for EBV yielded mild increases of serum IgG antibodies against EBV nuclear antigen (EBNA) and IgG antibodies against EBV viral capsid antigen (VCA). In a spinal MRI, the inferior lumbar meninges showed gadolinium enhancement. Nerve conduction studies revealed a decreased F wave ratio in the bilateral tibial nerves. The patient was diagnosed with meningomyeloradiculitis caused by EBV. The symptoms improved after steroid pulse therapy, but relapses of transverse myelitis and diplopia due to disturbance of the bilateral abducent nerves and left trochlear nerve occurred 7, 12, 16, and 26 months after treatment. The relapses were sometimes accompanied by small fluctuations in cerebrospinal fluid protein concentration, cell count, or serum anti-EBV antibody titer. These findings suggest that the myelopathy and diplopia were induced by a secondary immune reaction after inflammation caused by EBV infection.

[Case of relapsing polychondritis followed by cognitive impairment].

A 64-year-old man developed cognitive impairment. A neurological examination showed hypereactive tendon reflex. A cerebrospinal fluid (CSF) examination showed a mild increase in both the number of mononuclear cells (14 cells/mm3) and the protein level (45 mg/dl). An electroencephalogram showed diffuse slow wave activity. Brain MR images revealed multifocal lesions in the limbic area and white matter regions. He was suspected to have autoimmune encephalitis and his symptoms improved after treated with methylprednisolone (1 g/day for 3 days). Despite this treatment, his mental disorders later relapsed. His condition improved after undergoing repeated treatment. Thereafter, he developed bacterial meningoencephalitis which had been caused by Listeria monocytogenes during the period of the internal administration of steroids. Antibiotics successfully cured his Listeria meningoencephalitis. He thereafter developed conjunctivitis, polyarthritis, hearing difficulties, dizziness and auricular chondritis. Based on these findings, we diagnosed the patient to have relapsing polychondritis (RP). It was thus conceivable that his cognitive impairment has been caused by CNS vasculitis associated with RP. RP therefore should be carefully taken into consideration and be included in the differential diagnosis as a possible cause for CNS vasculitis.

[Titer of anti-muscle-specific receptor tyrosine kinase (MuSK) antibody correlated with symptomatic improvement in response to corticosteroid therapy in a patient with anti-MuSK antibody-positive myasthenia gravis: a case report].

A 54-year-old woman was admitted to our hospital because of diplopia, dysphagia, dropped head, and muscle weakness with easy fatigability. A neurological examination showed bilateral ptosis, ocular motility disorder, dysphagia, and weakness of the neck extensor muscles. Edrophonium and repetitive nerve stimulation tests of the thenar muscles showed positive results. The serum titer of anti-acetylcholine receptor antibody was negative. A thymoma was not detected in her chest CT. Finally, she was diagnosed with anti-MuSK antibody-positive myasthenia gravis based on the high serum titer of anti-MuSK antibody (239 nmol/l). Her symptoms improved after administration of prednisolone. However, the symptoms were aggravated when the prednisolone dosage was reduced, and the titer of anti-MuSK antibody rose at the same time. We evaluated the possible association between changes in the severity of her clinical symptoms and the titer of the antibody during prednisolone therapy. It was revealed that the titer of the antibody was correlated to the severity of clinical symptoms expressed by a QMG (Quantitative Myasthenia Gravis) score. These findings indicate that monitoring the titer of anti-MuSK antibody can be useful for assessing disease activity as well as decision making during treatment.

[A case of Werner syndrome with chromosomal abnormality].

A 52-year-old woman with diabetes mellitus (DM) complained of weakness of the arms and legs. She was referred to our hospital in November 2002 because of anemia, thyroid tumor and meningioma including DM. She was short in stature, juvenile bilateral cataract, intractable skin ulcers, clavus on the sole of her foot, a bird-like face and high-pitched voice. Typical physical features led to the final diagnosis of Werner's syndrome. Although the myelogram revealed no abnormal findings except erythroid hypoplasia, cytogenetic analysis of bone marrow cells showed deletion of chromosome 20 in 10% of the analyzed cells, which suggested the possibility of that myelodysplastic syndrome (MDS) or acute myeloblastic leukemia (AML) could occur. She had a thyroidectomy because both lobes of the thyroid gland were enlarged and caused hoarseness, In addition, it is common knowledge that the goiter could become malignant. We need to follow her carefully because she might be vulnerable to malignant disease, including leukemia and malignant meningioma.

Myeloradiculitis caused by Cryptococcus neoformans infection in a patient with ulcerative colitis: a neuropathological study.

Meningitis is the most common feature of cryptococcal infection of the nervous system. We herein describe the case of a 48-year-old man with fulminant cryptococcal myeloradiculitis, whose initial symptoms were impotence, dysuria and weakness of the lower extremities. He had been administered prednisolone and azathioprine for 7years for ulcerative colitis before onset of myeloradiculitis. He finally developed meningoencephalitis and died 2 months after onset despite treatment with amphotericin B and flucytosine. Post-mortem examination revealed numerous infiltrations of cryptococci in the spinal roots as well as in the meninges and subarachnoid space. Inflammatory cells and cryptococci had infiltrated the vessel walls in the spinal cord, and this was accompanied by necrotizing myelopathy. Myeloradiculitis is rare in cryptococcal infection, and this is the first case report to demonstrate direct cryptococcal infection in the spinal roots. Cryptococcal infection should be considered while managing myeloradiculopathy of unknown etiology, especially in immunocompromised patients.

[Venous infarction associated with carotid-cavernous fistula].

We report an 88-year-old woman who developed a hemorrhagic venous infarction in the left cerebral hemisphere and brainstem, in association with left carotid-cavernous fistula (CCF). Without aura the patient noticed diplopia due to left abducens palsy, and exophthal mos and congestion of the left eye. Brain CT revealed extrusion of the left eye, and dilatation of left superior orbital vein and cerebral cortical veins. She received diagnosis of CCF. Brain CT also revealed a small mass in the left ethmoidal sinus, which was not attached to the CCF. Biopsy of the mass was done under local anesthesia. On the following she had high fever. Her consciousness level deteriorated and she developed right hemiparesis FLAIR images of MRI showed, extensive high signal lesions in the left frontal and temporal cortices, basal ganglia, thalamus, midbrain and pons. These findings were consisted with venous infarction, possibly associated with peri-operative infection and hypovolemia. Intracranial hemorrhage occur in 3% of cases with CCF, but venous infarction was much rarer. The patients with CCF, who show dilatation of cortical veins in CT or MRI, have a higher risk of cerebral hemorrhage or infarction, and should be carefully observed.

Character changes from idiopathic cranial pachymeningoencephalitis.

A 66-year-old man with idiopathic cranial pachymeningoencephalitis was described. He suffered from left orbital pain, and character changes. He became short tempered, and was very attached to trifles. Two years prior to these symptoms, he had developed transient left abducent nerve palsy. Head MRI showed a thickening and enhancement of the dura mater on gadolinium-enhanced T1-weighted images, and high signal intensity lesions at bilateral frontal lobes predominantly in the white matter on T2-weighted images. Biopsies revealed microglial proliferation in the cerebral parenchyma, and mild lymphocytic perivascular infiltration. No evidence of intracranial infection was detected. We therefore treated him with methylprednisolone pulse therapy followed by oral prednisolone. His character became gradually normalized, and bilateral frontal lobe lesions seen on MRI disappeared. This is the first case to describe recurrent pachymeningoencephalitis with character changes, and symptoms were probably due to frontal lobe dysfunction.

[A patient with paraneoplastic limbic encephalitis induced by breast cancer presenting with hypersomnia].

A 50-year-old woman developed gait disturbance and hypersomnia over a period of a month. General physical examination revealed axillary lymph node swelling. On neurological examinations she was fully orientated but hypersomnic; short term memory disturbance, horizontal gaze evoked nystagmus and ataxic gait were observed. Electroencephalography disclosed a tendency for easily decreasing vigilance with delta activities but normal dominant rhythm. Cerebrospinal fluid examinations showed increased protein amounts (109 mg/dl) without either pleocytosis or atypical cells. An echogram of the breasts revealed a tiny mass in the left side. Pathological studies on a biopsied lymph node and the mass in her left breast showed a mammillary duct carcinoma. Brain MRI was normal, and no anti-neuronal antibody was detected in sera by two dimensional immunoblotting using human brain crude antigens. She was diagnosed as having paraneoplastic limbic encephalitis (PLE) associated with breast cancer. Over 42 hours polysomnography showed long total sleep time (TST) with a high ratio of sleep stage 1/TST and no REM sleep abnormalities; this resembled a thalamic-hypothalamic damaged sleep pattern. At first she was treated with plasma exchanges, but no improvement was observed. Hormonal and chemotherapies produced partial resolution of her neurologic symptoms and there were signs of reduction of the breast mass. Most reported PLE cases with hypersomnia have been associated with testicular cancer and anti-Ma antibodies. The present case is an extremely rare example manifesting hypersomnia without either testicular cancer or anti-Ma antibody. Since anti-tumor therapy successfully ameliorated her neurologic symptoms, cell-mediated immunity against a common tumor and neuronal antigens rather than hormonal immunity may have played a role in the development of her PLE.