Akio Iwasaki

The relationship between right-to-left shunt and brain white matter lesions in Japanese patients with migraine: a single center study

BackgroundThere may be a link between right-to-left shunt (RLs) and brain white matter lesions (WMLs) in patients with migraine. In this study, we assessed the relationship between WMLs and RLs in Japanese migraine patients.MethodsA total of 107 consecutive patients with migraine with (MA) and without aura (MWOA) were included in this study. Contrast transcranial Doppler ultrasound was used to detect RLs. WMLs were graded using brain magnetic resonance imaging based on well-established criteria.FindingsThe prevalence of RLs was significantly increased in the WMLs positive group (n = 24) compared with the WMLs negative group (n = 83) (75.0% vs. 47.0%, p = 0.015). In prevalence of WMLs between MA and MWOA patients, there were no statistical differences (p = 0.410). Logistic regression analysis adjusted by age and disease duration of migraine identified an RLs-positive status as the sole determinant for the presence of WMLs (OR = 6.15; 95% CI 1.82–20.8; p = 0.003)ConclusionOur study suggests a possible link between RLs and WMLs in Japanese patients with migraine.

Recurrent juvenile ischemic stroke caused by bow hunter’s stroke revealed by carotid duplex ultrasonography

Bow hunter’s stroke (BHS) is a rare cause of vertebrobasilar insufficiency due to rotational vertebral artery (VA) occlusion associated with head turning. We report a juvenile patient presenting with recurrent ischemic stroke caused by BHS, which was revealed by carotid duplex ultrasonography. Carotid duplex ultrasonography performed in the neutral position showed normal findings. However, disappearance of end-diastolic blood flow of contralateral VAs was observed with head rotation. Digital subtraction angiography confirmed occlusion at C1/2 levels in the VA contralateral to the head rotation, bilaterally. Importantly, our patient did not recognize the association of head rotation and previous episodes of stroke. We suggest that BHS should be considered in patients with cryptogenic stroke occurring in the vertebrobasilar artery territory.

Identification and functional characterization of novel mutations including frameshift mutation in exon 4 of CSF1R in patients with adult-onset leukoencephalopathy with axonal spheroids and pigmented glia

ObjectiveAdult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is caused by mutations in CSF1R. Pathogenic mutations in exons 12–22 including coding sequence of the tyrosine kinase domain (TKD) of CSF1R were previously identified. We aimed to identify CSF1R mutations in patients who were clinically suspected of having ALSP and to determine the pathogenicity of novel CSF1R variants.MethodsSixty-one patients who fulfilled the diagnostic criteria of ALSP were included in this study. Genetic analysis of CSF1R was performed for all the coding exons. The haploinsufficiency of CSF1R was examined for frameshift mutations by RT-PCR. Ligand-dependent autophosphorylation of CSF1R was examined in cells expressing CSF1R mutants.ResultsWe identified ten variants in CSF1R including two novel frameshift, five novel missense, and two known missense mutations as well as one known missense variant. Eight mutations were located in TKD. One frameshift mutation (p.Pro104LeufsTer8) and one missense variant (p.His362Arg) were located in the extracellular domain. RT-PCR analysis revealed that the frameshift mutation of p.Pro104LeufsTer8 caused nonsense-mediated mRNA decay. Functional assay revealed that none of the mutations within TKD showed autophosphorylation of CSF1R. The p.His362Arg variant located in the extracellular domain showed comparable autophosphorylation of CSF1R to the wild type, suggesting that this variant is not likely pathogenic.ConclusionsThe detection of the CSF1R mutation outside of the region-encoding TKD may extend the genetic spectrum of ALSP with CSF1R mutations. Mutational analysis of all the coding exons of CSF1R should be considered for patients clinically suspected of having ALSP.

Human herpesvirus 6 encephalitis followed by acute disseminated encephalomyelitis in an immunocompetent adult

A 26-year-old, otherwise healthy man presented with visual abnormality followed by loss of consciousness and convulsion. The patient then developed headache and fever 14 days later. Brain MRI showed hyperintensities in the left cingulate cortex. The cerrebrospinal fluid examinations showed mononuclear pleocytosis and positive PCR results for human herpesvirus 6 (HHV-6). A diagnosis of HHV-6 encephalitis and symptomatic epilepsy was made. The patients clinical symptoms improved promptly following acyclovir treatment. However, 3 months later the patient noticed dysesthesia in the trunk, the left upper limb and the right lower limb. Brain and spine MRI showed multiple brain white matter lesions, the middle cerebellar peduncle and cervical spinal lesions. The symptoms resolved following methylprednisolone pulse therapy only. We report an adult patient with HHV-6 encephalitis followed by acute disseminated encephalomyelitis whose initial presentation was epilepsy. HHV-6 encephalitis should be included in the differential diagnosis of encephalitis of unknown etiology in an immunocompetent adult.

Utility of chest X-ray in predicting left atrium dimension and brain natriuretic peptide

Objective: Detection of paroxysmal atrial fibrillation (PAF) in patients with acute stroke is often difficult. Dilatation of left atrium dimension (LAD) and increased levels of brain natriuretic peptide (BNP) are reported to indicate cerebral embolism due to PAF. We assessed the usefulness of chest X-ray in predicting LAD and BNP among patients with acute cerebral infarction. Methods: We included 141 consecutive patients with cerebral infarction [107 cerebral thromboses (cerebral thrombosis group)] and 34 cardiogenic cerebral embolisms due to PAF (PAF group). Transthoracic echocardiography (TTE) was used to evaluate LAD (TTE-LA). On chest X-ray, heart diameter at the third aortic arch (XPLA) and XP-LA divided by maximum intrathoracic diameter (XP-LA ratio) were measured. Results: TTE-LA, XP-LA, XP-LA ratio and BNP levels were significantly higher in PAF group compared with those in cerebral thrombosis group. XP-LA had a significant positive correlation with TTE-LA (r = 0.332, p < 0.0001) and BNP (r = 0.292, p < 0.001). XP-LA ratio also showed a significant correlation with TTE-LA (r = 0.210, p = 0.0126) and BNP (r = 0.515, p < 0.0001). Conclusion: We suggest chest X-ray is a simple and useful tool for predicting LAD and BNP.

Influence of pulsed wave doppler angle on diagnosis of internal carotid artery stenosis

WCN17-1276 SHIFT 3 STROKE Significance of hyperintense vessels in negative DWI area as a prognostic factor after thrombolysis M. Ichijo, S. Ishibashi, K. Miki, S. Itaya, T. Amino, T. Kamata, T. Yokota. Musashino Red Cross Hospital, Department of Neurology, Tokyo, Japan; Tokyo Medical and Dental University, Department of Neurology and Neurological Science, Tokyo, Japan; Tokyo Medical and Dental University, Department of Endovascular Surgery, Tokyo, Japan

Risk factors for deep venous thrombosis in patients with acute stroke

脳卒中の急性期では下肢麻痺や長期臥床,うっ血性心 不全の併発による静脈還流のうっ滞により,下肢深部静 脈血栓(deep venous thrombosis: DVT)を併発する例 が存在する .DVTは肺塞栓症の原因となり,予後不 良の転帰を示すことから,急性期からDVTの予防,早 期発見が重要となる. 脳卒中に対するDVT予防に明確なエビデンスはない ものの,弾性ストッキングの着用が勧められていた . しかし脳卒治療ガイドライン 2015では,脳出血に対し て弾性ストッキングの使用は推奨されておらず,脳梗塞 に対しても明らかな予防効果は示されていない .し たがって,現在では間欠的空気圧迫法による予防が主体 となるが,医療資源の問題からすべての脳卒中患者に対 して施行するのは困難と考えられる. そこでわれわれは脳卒中の急性期においてDVTを併 発する患者の危険因子を明らかにするため,急性期脳卒 28 Neurosonology 29(1):2016

Post-irradiation Skin Changes Associated with Lumbosacral Radiculopathy

We herein describe the cases of two patients with post-irradiation lumbosacral radiculopathy. The patients underwent postoperative radiation therapy to the abdomen due to testicular neoplasms 20 and 25 years prior to the onset of weakness, respectively. On physical examinations, asymmetric lower limb weakness and areflexia without apparent sensory loss were observed in both patients. Interestingly, artificial and squared atrophy of the skin and subcutaneous tissue, thought to correspond to the radiation fields, were observed in the lower back, and electromyography revealed selective motor axon loss localized to the lower extremities. The detection of skin changes in the area being irradiated is a valuable clue for diagnosing post-irradiation lumbosacral radiculopathy.

Ischemic Stroke as the Initial Manifestation of Neurosyphilis in a Young Adult Patient Positive for Human Immunodeficiency Virus

A 31-year-old man with pontine infarction was referred to our hospital for further evaluation and treatment. At admission, his neurological examination was unremarkable. No lymphadenopathy or skin lesions were found. The Treponema pallidum haemagglutination test, rapid plasma regain test and fluorescent treponemal antibody absorption test of immunoglobulin G were positive in both serum and cerebrospinal fluid (CSF). CSF analysis showed lymphocytic pleocytosis. The patient had male-to-male sexual contact and was found to be HIV positive. Physicians should be aware that acute ischaemic stroke may be the first manifestation of neurosyphilis in a young adult, especially with HIV infection.