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Background and ObjectivesThe functional outcome and mortality of patients with poststroke epilepsy (PSE) have not been assessed in a prospective study. Previous reports have suggested that patients with PSE may suffer from prolonged functional deterioration after a seizure. In this study, we prospectively investigated the functional outcome and mortality of patients with PSE and analyzed the effect of seizure recurrence on the outcomes.MethodsThis is part of the Prognosis of Post-Stroke Epilepsy study, a multicenter, prospective observational cohort study, where 392 patients with PSE (at least 1 unprovoked seizure more than 7 days after the onset of the last symptomatic stroke) were followed for at least 1 year at 8 hospitals in Japan. This study included only PSE patients with a first-ever seizure and assessed their functional decline and mortality at 1 year. Functional decline was defined as an increase in modified Rankin Scale (mRS) score at 1 year compared with baseline, excluding death. The associations between the seizure recurrence and the outcomes were analyzed statistically.ResultsA total of 211 patients (median age of 75 years; median mRS score of 3) were identified. At 1 year, 50 patients (23.7%) experienced seizure recurrence. Regarding outcomes, 25 patients (11.8%) demonstrated functional decline and 20 (9.5%) had died. Most patients died of pneumonia or cardiac disease (7 patients each), and no known causes of death were directly related to recurrent seizures. Seizure recurrence was significantly associated with functional decline (odds ratio [OR] 2.96, 95% CI 1.25–7.03, p = 0.01), even after adjusting for potential confounders (adjusted OR 3.26, 95% CI 1.27–8.36, p = 0.01), but not with mortality (OR 0.79, 95% CI 0.25–2.48, p = 0.68). Moreover, there was a significant trend where patients with more recurrent seizures were more likely to have functional decline (8.7%, 20.6%, and 28.6% in none, 1, and 2 or more recurrent seizures, respectively; p = 0.006).DiscussionOne-year functional outcome and mortality of patients with PSE were poor. Seizure recurrence was significantly associated with functional outcome, but not with mortality. Further studies are needed to ascertain whether early and adequate antiseizure treatment can prevent the functional deterioration of patients with PSE.

Poststroke epilepsy is a major ischaemic/haemorrhagic stroke complication. Seizure recurrence risk estimation and early therapeutic intervention are critical, given the association of poststroke epilepsy with worse functional outcomes, quality of life and greater mortality. Several studies have reported risk factors for seizure recurrence; however, in poststroke epilepsy, the role of EEG in predicting the risk of seizures remains unclear. This multicentre observational study aimed to clarify whether EEG findings constitute a risk factor for seizure recurrence in patients with poststroke epilepsy. Patients with poststroke epilepsy were recruited from the PROgnosis of POst-Stroke Epilepsy study, an observational multicentre cohort study. The enrolled patients with poststroke epilepsy were those admitted at selected hospitals between November 2014 and June 2017. All patients underwent EEG during the interictal period during admission to each hospital and were monitored for seizure recurrence over 1 year. Board-certified neurologists or epileptologists evaluated all EEG findings. We investigated the relationship between EEG findings and seizure recurrence. Among 187 patients with poststroke epilepsy (65 were women with a median age of 75 years) admitted to the lead hospital, 48 (25.7%) had interictal epileptiform discharges on EEG. During the follow-up period (median, 397 days; interquartile range, 337–450 days), interictal epileptiform discharges were positively correlated with seizure recurrence (hazard ratio, 3.82; 95% confidence interval, 2.09–6.97; P < 0.01). The correlation remained significant even after adjusting for age, sex, severity of stroke, type of stroke and generation of antiseizure medications. We detected periodic discharges in 39 patients (20.9%), and spiky/sharp periodic discharges were marginally associated with seizure recurrence (hazard ratio, 1.85; 95% confidence interval, 0.93–3.69; P = 0.08). Analysis of a validation cohort comprising 187 patients with poststroke epilepsy from seven other hospitals corroborated the association between interictal epileptiform discharges and seizure recurrence. We verified that interictal epileptiform discharges are a risk factor for seizure recurrence in patients with poststroke epilepsy. Routine EEG may facilitate the estimation of seizure recurrence risk and the development of therapeutic regimens for poststroke epilepsy.

Idiopathic systemic capillary leak syndrome (ISCLS) is a rare cryptogenic disorder characterized by recurrent hemoconcentration, hypoalbuminemia, edema, and hypotension due to extravascular fluid leakage. This is the first report that details uncommon extensive leukoencephalopathy caused by ISCLS upon a neuropathological investigation. A 68-year-old female had recurrent episodes of hemoconcentration, hypoalbuminemia, and generalized edema and was diagnosed with ISCLS. After 9 years, brain magnetic resonance imaging (MRI) incidentally revealed extensive leukoencephalopathy without neurological deficits. Thorough examinations ruled out other disorders, and the cerebral involvement due to ISCLS was finally diagnosed. Three years later, she developed an acute-onset coma and status epilepticus together with hypotension and hemoconcentration, which were compatible with ISCLS recurrence. Electroencephalogram and MRI were correlated with a seizure arising from the left hemisphere. Extensive leukoencephalopathy did not show notable changes for 3 years. Although treatment for ISCLS recurrence temporally improved hemoconcentration and consciousness, consciousness worsened again by marked edema of the left hemisphere, and she died of cerebral herniation. A brain autopsy revealed straggly perivascular plasma leakage around the small vessels of the deep white matter, which supported that the leukoencephalopathy was caused by ISCLS. Widespread myelin pallor and decreased axonal density with sparse astrogliosis and microgliosis were observed in the cerebral white matter and corresponded with a chronic change in the MRI. Current radiological and pathological observations revealed that frequent perivascular leakages could cause chronic leukoencephalopathy, were linked with the development of systemic capillary leakage in ISCLS, and provided insights into the mysterious pathophysiology.

We present the case of an 81-year-old woman who underwent aortic valve replacement and coronary artery bypass surgery by median sternotomy. Following the operation, she experienced distal muscle weakness in her left upper limb and numbness in the medial part of her left forearm and palm. Nerve conduction study revealed low amplitudes of her left ulnar compound muscle action potential (CMAP) and sensory nerve action potential (SNAP), radial CMAP, and medial antebrachial cutaneous SNAP. Needle electromyography showed denervation potentials in the extensor digitorum communis and abductor pollicis brevis. CT and MRI showed a left first rib fracture and a hematoma nearby. Short-T1 inversion recovery image (STIR) showed a high-intensity area in the left root of C8. Based on these findings, we diagnosed the patient with lower trunk brachial plexopathy due to hematoma.

Objective Various neurological manifestations have been increasingly reported in coronavirus disease 2019 (COVID-19). We determined the neurological features and long-term sequelae in hospitalized COVID-19 patients. Methods We retrospectively studied 95 consecutive hospitalized patients with COVID-19 between March 1 and May 13, 2020. Acute neurological presentations (within two weeks of the symptom onset of COVID-19) were compared between 60 non-severe and 35 severely infected patients who required high-flow oxygen. In the 12 ventilated patients (the most severe group), we evaluated neurological complications during admission, subacute neurological presentations, and neurological sequelae (51 and 137 days from the onset [median], respectively). Results Of the 95 patients (mean age 53 years old; 40% women), 63% had acute neurological presentations, with an increased prevalence in cases of severe infections (83% vs. 52%, p＜0.001). Impaired consciousness and limb weakness were more frequent in severe patients than in non-severe ones (0% vs. 49%; p＜0.001, and 0% vs. 54%; p＜0.001, respectively). In the most severe group (mean age 72 years old; 42% women), 83% of patients had neurological complications [cerebrovascular disease (17%), encephalopathy (82%), and neuropathy (55%)], and 92% had subacute neurological presentations [impaired consciousness (17%), higher brain dysfunction (82%), limb weakness (75%), and tremor (58%)]. Neurological sequelae were found in 83% of cases, including higher brain dysfunction (73%), limb weakness (50%), and tremor (58%). Conclusions Neurological manifestations are common in COVID-19, with the possibility of long-lasting sequelae.

Objective Various neurological manifestations have been increasingly reported in coronavirus disease 2019 (COVID-19). We determined the neurological features and long-term sequelae in hospitalized COVID-19 patients. Methods We retrospectively studied 95 consecutive hospitalized patients with COVID-19 between March 1 and May 13, 2020. Acute neurological presentations (within two weeks of the symptom onset of COVID-19) were compared between 60 non-severe and 35 severely infected patients who required high-flow oxygen. In the 12 ventilated patients (the most severe group), we evaluated neurological complications during admission, subacute neurological presentations, and neurological sequelae (51 and 137 days from the onset [median], respectively). Results Of the 95 patients (mean age 53 years old; 40% women), 63% had acute neurological presentations, with an increased prevalence in cases of severe infections (83% vs. 52%, p

Background and purpose The management of post‐stroke epilepsy (PSE) should ideally include prevention of both seizure and adverse effects; however, an optimal antiseizure medications (ASM) regimen has yet been established. The purpose of this study is to assess seizure recurrence, retention, and tolerability of older‐generation and newer‐generation ASM for PSE. Methods This prospective multicenter cohort study (PROgnosis of Post‐Stroke Epilepsy [PROPOSE] study) was conducted from November 2014 to September 2019 at eight hospitals. A total of 372 patients admitted and treated with ASM at discharge were recruited. Due to the non‐interventional nature of the study, ASM regimen was not adjusted and followed standard hospital practices. The primary outcome was seizure recurrence in patients receiving older‐generation and newer‐generation ASM. The secondary outcomes were the retention and tolerability of ASM regimens. Results Of the 372 PSE patients with ASM at discharge (median [IQR] age, 73 [64–81] years; 139 women [37.4%]), 36 were treated with older‐generation, 286 with newer‐generation, and 50 with mixed‐generation ASM. In older‐ and newer‐generation ASM groups (n = 322), 98 patients (30.4%) had recurrent seizures and 91 patients (28.3%) switched ASM regimen during the follow‐up (371 [347–420] days). Seizure recurrence was lower in newer‐generation, compared with the older‐generation, ASM (hazard ratio [HR], 0.42, 95%CI 0.27–0.70; p = .0013). ASM regimen withdrawal and change of dosages were lower in newer‐generation ASM (HR, 0.34, 95% CI 0.21–0.56, p, The current study is a real‐world prospective cohort study to evaluate seizure control, retention, and tolerability of older or newer‐generation anti‐seizure medication (ASM) treatment in post‐stroke epilepsy (PSE). Seizure recurrence was lower in newer‐generation, compared with the older‐generation, ASM (hazard ratio [HR], 0.42, 95%CI 0.27–0.70; p = .0013). ASM regimen withdrawal and change of dosages were lower in newer‐generation ASM (HR, 0.34, 95% CI 0.21–0.56, p < .0001). These findings suggest potential for newer‐generation ASM as the primary choice in the secondary prophylaxis of PSE.

Few studies have assessed the duration of the postictal state after a generalized convulsion (GC) in adults. This study aimed to investigate the postictal duration after a GC and the factors associated with it.Patients aged ≥16 years who presented to the emergency department of a community general hospital with an unprovoked GC from January 2015 through December 2016 were evaluated retrospectively. A GC was defined as a bilateral convulsion with apparent impaired consciousness including a generalized tonic-clonic seizure.We evaluated 209 consecutive GCs (median age, 42 years) with the median postictal duration of 0.75 h. The univariate analyses indicated that the median duration of the postictal state was significantly longer: in elderly patients (aged ≥65 years) than in younger patients (aged65 years) (2 h vs. 0.7 h, p = 0.0005); in patients with higher modified Rankin scale (mRS) scores (≥3) at baseline than in those with lower scores (≤2) (2.5 h vs. 0.7 h, p 0.0001); in patients with longer seizure duration (≥30 min) than in those with shorter duration (55 h vs. 0.7 h, p 0.0001); in patients who were given emergency antiepileptic drugs than in those who were not (16 h vs. 0.6 h, p 0.0001); and in patients who were intubated than in those who were not (63.5 h vs. 0.75 h, p = 0.0009). Multiple linear regression analyses indicated that older age, higher mRS scores at baseline, longer seizure duration, and administration of emergency antiepileptic drugs were independently associated with longer postictal duration.Age, baseline functional disability, and seizure duration were factors associated with the duration of the postictal state after a GC.

A 48-year-old woman developed painless bilateral acute visual loss with a progressive visual field defect over 2 weeks. At the initial visit, her best-corrected visual acuity was 6/9 (right) and 6/15 (left). There was a left relative afferent pupillary defect. There was no ptosis, and ocular motility was full. Her retina appeared normal without papilloedema. Her critical flicker fusion frequency was 17 Hz in the right eye and 13 Hz in the left eye (normal range >37 Hz).1 On Humphrey visual field analysis, she had a complete bitemporal hemianopia (figure 1A). MR scan of orbit showed enhanced optic chiasm enlargement with slight extension to the right posterior optic nerve, suggesting a neoplasm or inflammatory process such as sarcoidosis (figure 2A–C).2–4 Her cerebrospinal fluid (CSF) was clear with normal pressure, containing 6 cells/µL (≤5), protein 1.2 g/L (0.15–0.45) and glucose of …

Previous studies on status epilepticus (SE) in the elderly were conducted in tertiary centers. We aimed to delineate the features and outcomes of elderly SE patients by comparing them to those of younger patients in a comprehensive community hospital providing primary to tertiary care.We retrospectively investigated 197 consecutive adult SE patients admitted to our hospital. The clinical, electroencephalography, and diffusion-weighted imaging (DWI) findings of 112 elderly patients (aged ≥65 years) were compared to those of 85 younger patients (aged65 years).Compared to that in younger patients, SE in elderly patients more frequently presented de novo (68.8% vs. 52.9%, p = 0.02); was less likely to be tonic-clonic (55.4% vs. 83.5%, p 0.001) but more likely to be focal motor (36.6% vs. 12.9%, p 0.001); and was more frequently refractory (30.4% vs. 14.1%, p = 0.008), particularly refractory nonconvulsive SE (13.4% vs. 4.7%, p = 0.04). Lateralized periodic discharges on electroencephalography (20.9% vs. 4.8%, p = 0.001) and SE-associated hyperintensities on DWI (27.8% vs. 13.6%, p = 0.03) were more common in the elderly than in the younger SE patients. The mortality rates did not significantly differ between the groups (6.3% vs. 3.5%, p = 0.52). The percentage of poor functional outcomes did not significantly differ between the groups in cases with acute symptomatic etiology (52.0% vs. 45.7%, p = 0.63), but was higher in elderly patients with remote symptomatic and cryptogenic etiologies (33.3% vs. 12.0%, p = 0.006).SE in the elderly differed from that in younger adults in the semiology, refractoriness, electroencephalography and DWI findings, and functional outcome. Outcomes were better than previously reported.

Chronic inflammatory demyelinating polyradiculoneuropathy is a relapsing-remitting or chronic progressive demyelinating polyradiculoneuropathy. We report the case of a patient with chronic inflammatory demyelinating polyradiculoneuropathy who experienced relapses on four occasions after experiencing pyrexia and flu-like symptoms. Our patient showed characteristic features, such as relapse after pyrexia and flu-like symptoms, remission after pyretolysis without treatment, and the absence of remarkable improvement in a nerve conduction study in the remission phase. The serum level of tumor necrosis factor-α was elevated in the relapse phase and reduced in the remission phase; thus, the induction of cytokine release by viral infection might have caused the relapses.

Immune checkpoint inhibitors (ICIs) are promising drugs for various cancers. However, immune activation by ICIs can lead to immune-related adverse events (irAEs). Autoimmune encephalitis is a rare irAE, and its clinical features remain unknown. We herein report two patients with ICI-associated autoimmune encephalitis who, saliently, showed elevated adenosine deaminase (ADA) levels in the cerebrospinal fluid (CSF). This is the first report of increased ADA levels in the CSF of patients with ICI-induced autoimmune encephalitis. Although the mechanism of the ADA increase is poorly understood, elevated ADA in the CSF may be informative in the diagnosis of this rare disorder.

Neuronal intranuclear inclusion disease (NIID) has highly variable clinical manifestations. Sone et al. describe the clinical and pathological features of 57 adult-onset cases diagnosed by postmortem dissection/antemortem skin biopsy. They report ‘dementia dominant’ and ‘limb weakness’ subtypes, and recommend consideration of NIID in the differential diagnosis of leukoencephalopathy and neuropathy., Neuronal intranuclear inclusion disease (NIID) is a slowly progressive neurodegenerative disease characterized by eosinophilic hyaline intranuclear inclusions in the central and peripheral nervous system, and also in the visceral organs. NIID has been considered to be a heterogeneous disease because of the highly variable clinical manifestations, and ante-mortem diagnosis has been difficult. However, since we reported the usefulness of skin biopsy for the diagnosis of NIID, the number of NIID diagnoses has increased, in particular adult-onset NIID. In this study, we studied 57 cases of adult-onset NIID and described their clinical and pathological features. We analysed both NIID cases diagnosed by post-mortem dissection and by ante-mortem skin biopsy based on the presence of characteristic eosinophilic, hyaline and ubiquitin-positive intanuclear inclusion: 38 sporadic cases and 19 familial cases, from six families. In the sporadic NIID cases with onset age from 51 to 76, dementia was the most prominent initial symptom (94.7%) as designated ‘dementia dominant group’, followed by miosis, ataxia and unconsciousness. Muscle weakness and sensory disturbance were also observed. It was observed that, in familial NIID cases with onset age less than 40 years, muscle weakness was seen most frequently (100%), as designated ‘limb weakness group’, followed by sensory disturbance, miosis, bladder dysfunction, and dementia. In familial cases with more than 40 years of onset age, dementia was most prominent (100%). Elevated cerebrospinal fluid protein and abnormal nerve conduction were frequently observed in both sporadic and familial NIID cases. Head magnetic resonance imaging showed high intensity signal in corticomedullary junction in diffusion-weighted image in both sporadic and familial NIID cases, a strong clue to the diagnosis. All of the dementia dominant cases presented with this type of leukoencephalopathy on head magnetic resonance imaging. Both sporadic and familial NIID cases presented with a decline in Mini-Mental State Examination and Frontal Assessment Battery scores. Based on these clinicopathological features, we proposed a diagnosis flow chart of adult-onset NIID. Our study suggested that the prevalence rate of adult-onset NIID may be higher than previously thought, and that NIID may be underdiagnosed. We should take NIID into account for differential diagnosis of leukoencephalopathy and neuropathy.

Objective Guillain–Barre syndrome (GBS), Miller Fisher syndrome (MFS), and Bickerstaff brainstem encephalitis (BBE) are usually monophasic, but some patients experience recurrences after long asymptomatic intervals. We aimed to investigate clinical features of recurrent GBS, MFS, and BBE at a single hospital. Methods Records from 97 consecutive patients with GBS, MFS or BBE who were admitted to a tertiary hospital between 2001 and 2013 were reviewed. Clinical and laboratory features of patients with recurrent GBS, MFS, or BBE were investigated. Results Patients included 55 (32 males) with GBS, 34 (22 males) with MFS, and 8 (6 males) with BBE. Recurrent cases occurred in 2 (4%) of the 55 patients with GBS, 4 (12%) of the 34 patients with MFS, and 2 (25%) of the 8 patients with BBE. Patients with recurrent MFS had a tendency to be younger at the first episode than patients with non-recurrent MFS (median, 22 versus 37 years old). Symptoms and signs were less severe during relapses than during the initial episode in recurrent patients. Conclusions Recurrences occurred more frequently in patients with MFS or BBE compared with those with GBS. Patients with recurrent MFS might be younger than those with non-recurrent MFS.

IMPORTANCE: Reports on dermatomyositis (DM) sine dermatitis (DMSD) are scarce, and the concept of the disease has not been widely accepted. OBJECTIVE: To confirm the existence of DMSD, determine its prevalence, and characterize its serologic features. DESIGN, SETTING, AND PARTICIPANTS: This is a cohort study that reviewed clinical information, laboratory data, and muscle pathology slides from January 2009 to August 2019. We further assessed the follow-up data of 14 patients with DMSD. The median (interquartile range) follow-up period was 34 (16-64) months. Muscle biopsy samples, along with clinical information and laboratory data, were sent to a referral center for muscle diseases in Japan for diagnosis. Of patients whose myopathologic diagnosis was made at the National Center of Neurology and Psychiatry between January 2009 and August 2019, 199 patients were eligible for inclusion. These patients underwent full investigation for DM-specific autoantibodies (against transcriptional intermediary factor γ, Mi-2, melanoma differentiation–associated gene 5, nuclear matrix protein 2 [NXP-2], and small ubiquitin-like modifier activating enzyme ); however, 17 patients were excluded because their muscle fibers did not express myxovirus resistance protein A, a sensitive and specific marker of DM muscle pathology. MAIN OUTCOMES AND MEASURES: Diagnosis of DMSD was based on the absence of a skin rash at the time of muscle biopsy. RESULTS: Of the 182 patients, 93 were women (51%) and 46 were children (25%) (

A 67-year-old man presented with hoarseness, dysarthria and deviation of the tongue to the left side the day after the open-heart operation under general anesthesia. Brain MRI demonstrated no causal lesion, and laryngoscope showed left vocal cord abductor palsy, so we diagnosed him with Tapia's syndrome (i.e., concomitant paralysis of the left recurrent and hypoglossal nerve). His neurological symptoms recovered gradually and improved completely four months after the onset. Tapia's syndrome is a rare condition caused by the extra cranial lesion of the recurrent laryngeal branch of the vagus nerve and the hypoglossal nerve, and mostly described as a complication of tracheal intubation. In this case, transesophageal echo probe has been held in the left side of the pharynx, so compression to the posterior wall of pharynx by the probe resulted in this condition, and to the best of our knowledge, this is the first report of Tapia's syndrome due to transesophageal echocardiography during an open-heart operation. This rare syndrome should be considered as a differential diagnosis of dysarthria and tongue deviation after a procedure associated with compression to the pharynx.

Anti-NMDA receptor encephalitis is a paraneoplastic encephalitis characterised by psychiatric features, involuntary movement, and autonomic instability. Various EEG findings in patients with anti-NMDA receptor encephalitis have been reported, however, the correlation between the EEG findings and clinical course of anti-NMDA receptor encephalitis remains unclear. We describe a patient with anti-NMDA receptor encephalitis with a focus on EEG findings, which included: status epilepticus, generalised rhythmic delta activity, excess beta activity, extreme delta brush, and paroxysmal alpha activity upon arousal from sleep, which we term"arousal alpha pattern". Initially, status epilepticus was observed on the EEG when the patient was comatose with conjugate deviation. The EEG then indicated excess beta activity, followed by the emergence of continuous slow activity, including generalised rhythmic delta activity and extreme delta brush, in the most severe phase. Slow activity gradually faded in parallel with clinical amelioration. Excess beta activity persisted, even after the patient became almost independent in daily activities, and finally disappeared with full recovery. In summary, our patient with anti-NMDA receptor encephalitis demonstrated slow activity on the EEG, including extreme delta brush during the most severe phase, which gradually faded in parallel with clinical amelioration, with excess beta activity persisting into the recovery phase.

Introduction The most common form of spinal muscular atrophy (SMA) is a recessive disorder caused by SMN1 mutations in 5q13, whereas the genetic etiologies of non-5q SMA are very heterogenous and largely remain to be elucidated. We present a father and son with atrophy and weakness of the lower leg muscles since infancy. Genetic studies in this family revealed a novel BICD2 mutation causing autosomal dominant lower extremity-predominant SMA type 2. Patients The proband was the father, aged 30, and the son was aged 3. Both of them were born uneventfully to nonconsanguineous parents. While the father first walked at the age of 19 months, the son was unable to walk at age 3 years. In both, knee and ankle reflexes were absent and sensation was intact. Serum creatine kinase levels were normal. The son showed congenital arthrogryposis and underwent orthopedic corrections for talipes calcaneovalgus. Investigation of the father at the age of 5 years revealed normal results on nerve conduction studies and sural nerve biopsy. Electromyography showed chronic neurogenic change, and muscle biopsy showed features suggestive of denervation. The father was diagnosed clinically with a sporadic distal SMA. Follow-up studies showed very slow progression. Investigations and results Next-generation and Sanger sequencing revealed a deleterious mutation in BICD2: c.1667A>G, p.Tyr556Cys, in this family. Discussion BICD2 is a cytoplasmic conserved motor-adaptor protein involved in anterograde and retrograde transport along the microtubules. Next-generation sequencing will further clarify the genetic basis of non-5q SMA.

Background Orbital apex syndrome is a localized type of orbital cellulitis, where mass lesions occur at the apex of the cranial nerves. Although nasal septal abscess is uncommon, the organism most likely to cause nasal septal abscess is Staphylococcus aureus, and fungal septal abscesses are rare. Here we present an extremely rare and serious case of orbital apex syndrome secondary to fungal nasal septal abscess caused by Scedosporium apiospermum in a patient with uncontrolled diabetes. Case presentation A 59-year-old man with a 1-month history of headache underwent consultation in an otolaryngological clinic of a general hospital. He was diagnosed with nasal septal abscess and was treated with incisional drainage and 1 month of an antibiotic drip; however, his symptoms persisted. The patient later complained of diplopia due to bilateral abducens nerve palsy, and was then referred to the department of Otolaryngology – Head and Neck Surgery, Kobe City Medical Center General Hospital. The septal lesion was biopsied under general anesthesia, and S. apiospermum was detected using polymerase chain reaction. The patient was treated with an antifungal drug and surgical resection of the lesion was performed. Although the patient survived, he lost his eyesight. Conclusions This patient represents the second reported case of nasal septal abscess and orbital apex syndrome caused by S. apiospermum. If not treated properly, septal abscess can be life-threatening and cause severe complications, such as ablepsia.

Visual and semi-quantitative assessments of

We studied the fracture behaviour of extruded Mg–Zn–Y alloys at room temperature (RT) and at 523 K using microfracture testing. An Mg97Zn1Y2 alloy was used to obtain two-phase specimens consisting of α-Mg and long-period stacking ordered (LPSO) structure phases, and an Mg88Zn5Y7 alloy was used to obtain specimens consisting of an LPSO phase. The microfracture testing of the two-phase specimen revealed that the fracture behaviour changed from brittle to ductile as the testing temperature increased. By contrast, the LPSO-phase specimen remained brittle even at the elevated temperature and the intrinsic fracture toughness values obtained at both testing temperatures were nearly identical. Ex situ transmission electron microscopy of the two-phase specimen showed that mechanical twinning in the α-Mg phase did not occur at the elevated temperature, although it was activated at RT. This suggests that the plastic deformation mode in the α-Mg phase plays a crucial part in the enhanced crack growth resistance of the two-phase alloy at the elevated temperature.

Objective To delineate the clinical characteristics and functional outcome of status epilepticus (SE) in elderly people, and elucidate prognostic implications of SE-associated rhythmic and periodic patterns (RPPs) in electroencephalography and hyperintensities on diffusion-weighted imaging. Methods We retrospectively investigated 107 consecutive patients with SE aged ≥ 65 years in a comprehensive community hospital. RPPs were classified using the 2012 American Clinical Neurophysiology Society's Standardized Critical Care EEG Terminology. Poor outcome was defined as an increase in modified Rankin Scale (mRS) score at discharge compared with that at baseline, including death. Results Median age of patients was 80.0 years. Median mRS score at baseline was 3. Thirty-four patients (31.8%) had a previous diagnosis of epilepsy. Cerebrovascular disease and dementia were major etiologies. Poor outcome occurred in 41 (38.3%). In electroencephalography, periodic discharges (PDs) were present in 21.0% (22/105), rhythmic delta activity (RDA) in 10.5% (11/105), and conventional seizure patterns in 9.5% (10/105). Diffusion-weighted hyperintensities associated with SE were observed in 28.0% (26/93). With univariate analysis, poor outcome was significantly associated with no previous diagnosis of epilepsy, etiology, refractory SE, specific electroencephalographic patterns (PDs and conventional seizure patterns, but not RDA), and diffusion-weighted hyperintensities. With multivariate logistic regression analysis, diffusion-weighted hyperintensities (OR 6.13 [95% CI 1.72–21.9]) and refractory SE (OR 5.36 [95% CI 1.28–22.4]) were independently associated with poor outcome. Conclusions SE often occurred as the first seizure in already disabled elderly people, further worsening their functional disabilities. Diffusion-weighted hyperintensities and refractory SE, but not RPPs in electroencephalography, were independent functional prognostic factors.

：症例は生来健康な22歳男性．性交後に右後頭部痛と顔面を含む左半身感覚障害，左上同名性四分盲を生じ，頭部MRIで右後頭葉と右視床に脳梗塞巣，頭部MRAにて右後大脳動脈にpearl & string signを認めた．性交を契機とした後大脳動脈解離による脳梗塞と診断し，抗血小板薬による保存的加療を行った．経過は良好で，クモ膜下出血の合併や脳梗塞再発は認めず，MRA上右後大脳動脈の壁不整所見も経時的に改善した．脳動脈解離は若年性脳梗塞の原因として重要であるが，性交が誘因となることもあり，頭痛や神経症状発症時の状況を詳しく病歴聴取することが大切である．また，性行為に伴って生じる頭痛は一般に経過良好なprimary headache associated with sexual activityとして知られているが，本症例のような脳動脈解離を鑑別する必要がある．

Background Hb interference induces false test results and thus hinders the establishment of further applications that use the formazan method. We attempted to eliminate hemoglobin (Hb) interference on the formazan reaction. Methods Total serum bile acid (TBA) reagent was constructed to elucidate the mechanism of Hb interference. We determined the interaction between formazan reagents and Hb using spectral analysis and investigated the optimal condition of the reagent to establish a practical technique for its prevention. Results Hb caused overestimation during TBA measurement, and its influence was attributed to the errors of both the sample and reagent blank signals. These errors were triggered by the reaction between Hb and formazan reagents. The main finding was that the addition of both imidazole and sodium nitrite in the reagent considerably accelerated the oxidation of Hb, even at neutral pH. The overestimation of TBA concentration was reduced to Conclusions We elucidated the mechanism of Hb interference on the formazan reaction and succeeded in preventing its influence. This will help to solve the technical difficulties associated with utilizing formazan reactions as routine diagnostic tools.

Influenza-associated encephalopathy (IAE) usually occurs in children aged

Although nonconvulsive status epilepticus (NCSE) is a major neurological emergency, its frequency and clinical course are not well clarified. We investigated the clinical characteristics of status epilepticus focusing on the significance of NCSE. One thousand seven hundred twenty-three patients were admitted as neurological emergency cases in our hospital between October 2003 and September 2006. Of these cases, 94 (5.5%) were diagnosed as status epilepticus of which, 24 (25.5%) were diagnosed with NCSE on admission. Moreover, 8 patients who presented with convulsive status epilepticus on admission had episodes of NCSE during hospitalization. Thus, 32 patients (34.0%) suffered from NCSE during their clinical course. We analyzed the prognostic factors of status epilepticus using the Glasgow Outcome Scale. Poor outcome was significantly correlated with NCSE (p = 0.003) and acute cerebrovascular disease (p = 0.010), independent of age, sex, history of epilepsy, and other etiologies. Our study revealed that NCSE is not a rare condition and results in a poor outcome. Careful EEG evaluation of patients with consciousness disturbance might increase the diagnostic accuracy of NCSE, and aggressive treatment of patients with NCSE should be necessary to improve the prognosis of NCSE.

Circulating bilirubin is thought to function as a physiological antioxidant. One of the decomposition products of this process is the biopyrrins, which include two regioisomers: biotripyrrin‐a (1,14,15,17‐tetrahydro‐2,7,13‐trimethyl‐1,14‐deoxy‐3‐vinyl‐16H‐tripyrrin‐8,12‐dipropionic acid) and biotripyrrin‐b (1,14,15,17‐tetrahydro‐3,7,13‐trimethyl‐1,14‐deoxy‐3‐vinyl‐16H‐tripyrrin‐8,12‐dipropionic acid). We measured biopyrrins in random urine specimens and investigated whether the biopyrrin values obtained were valid when expressed as a ratio of the creatinine (Cr) concentrations. All of the random urine specimens collected over 48 hr were from presumably healthy adults. We measured the biopyrrins by means of an enzyme‐linked immunosorbent assay (ELISA) using an anti‐bilirubin monoclonal antibody. When the values were expressed in terms of the ratio to Cr, the within‐day coefficient of variation (%CV) of the excretion of biopyrrins was reduced to 27%±10% (P0.05), even if their values were corrected by Cr. The amounts of biopyrrins excreted in 24‐hr urine specimens were significantly correlated with the 24‐hr cortisol excretion (P0.05). J. Clin. Lab. Anal. 21:1–7, 2007. © 2007 Wiley‐Liss, Inc.

An 82-year-old woman with a history of asthma was admitted to our hospital because of dyspnea. On admission, laboratory testing showed a white blood cell count of 17,700/μl with hypereosinophilia of 9,204/μl (52% of all white blood cells). Various examinations, including a bone marrow biopsy for the cause of eosinophilia, were unremarkable. The patient was diagnosed with hypereosinophilic syndrome (HES). Treatment with intravenous methylprednisolone was initiated. The patient's eosinophil count normalized within 1 day. On the 6th day, she developed left-sided hemiparesis. Magnetic resonance imaging (MRI) of the brain showed acute multiple infarcts in arterial border zones of bilateral cerebral and cerebellar hemispheres, and in bilateral basal ganglia and the thalamus. Magnetic resonance angiography was normal. Coagulation factors were normal, except for an elevated D-dimer level (12.9 μg/ml). A transthoracic echocardiogram showed thickening of the left ventricular endocardium with immobile thrombus, compatible with Loffler endocarditis. Treatment with oral prednisolone was started at 30 mg/day and then tapered to a maintenance dose of 5 mg/day. Anticoagulation was concurrently started for prevention of stroke. Ten months later, an echocardiogram showed that the thrombus had decreased in size, and MRI revealed no new cerebral infarctions. The cause of cerebral infarction in patients with hypereosinophilia is thought to be thromboembolism or cerebrovascular endothelial toxicity of eosinophils. In this patient, the cerebral infarcts may have been the result of embolism from the left ventricular thrombus. Because HES with Loffler endocarditis is frequently associated with a poor prognosis, cardiovascular problems should be evaluated and treatment started as soon as possible.

We examined two enzymatic reagents for cholesterol measurement that contained cholesterol oxidase. One of the reagents gave higher values by up to 11 mg/dl in samples of 109 out of 3119 patients examined. We found that the positive errors were caused by a nonspecific reaction that has not previously been recognized in the determination of H(2)O(2) using Trinder reagents, and we further discovered its mechanism.We compared the total cholesterol concentration in 3119 patients between the 2 reagents. By examining the characteristics of the interferrent-susceptible reagent and affected patients' samples, we identified the cause and mechanism of the influence of the nonspecific reaction.The existence of potassium ferrocyanide in the reagent gave rise to positive errors when the concentration of unsaturated iron binding capacity (UIBC) in the patients' sample was70 microg/dl. Moreover, ceruloplasmin in the patients' sample was found to cause a nonspecific reaction and its influence was proportional to the serum copper concentration. The two types of reagents compared in the present study included buffers with different metal chelating capacities; the one with the lower capacity gave an erroneous measurement. The buffer in the reagent without influence had been acting as a chelating agent. Therefore, the influence of the interferrent-susceptible reagent could be prevented by addition of a chelating agent (e.g. EDTA).Trinder reagents containing potassium ferrocyanide have been widely used for clinical diagnostic tests such as creatinine, uric acid and so on. These reagents would have similar problems. Some test items might have a greater influence than cholesterol reagent. Physicians should be aware of the risks of diagnostic mistakes due to these errors of measurement.

Background: Existing studies have demonstrated the clinical significance of triglyceride content in VLDL (VLDL-TG) and intermediate-density lipoprotein (IDL-TG). We developed a homogeneous assay protocol to directly measure VLDL-TG. Methods: Possible reagents and conditions for measuring VLDL-TG were comprehensively tested, and the “best” combination was determined. Healthy persons were instructed to consume a fatty meal after 15-h overnight fasting. Serum VLDL-TG + IDL-TG concentrations were measured using the proposed method. Patients with serum LDL-cholesterol concentrations ≥3.62 mmol/L (140 mg/dL) were administered simvastatin at a daily dose of 5 mg, and serum VLDL-TG concentrations were then measured. Results: The combination of 2 nonionic surfactants played an important role in differentiating VLDL and IDL from other lipoproteins, probably via specific interactions with phospholipids and apolipoproteins. The regression line of the proposed method (y) and the ultracentrifugal assay (x) was: y = 0.98x + 0.31 mmol/L (r = 0.98; n = 73; P Conclusion: Our homogeneous method can measure TG content in VLDL and IDL.