Cerebrospinal fluid analysis is associated with enhancement on MRI in bacterial and tuberculous meningitis: A retrospective observational study.

OBJECTIVE: Along with cerebrospinal fluid (CSF) analysis, enhancement on contrast-enhanced MRI is useful to diagnose meningitis. However, the conditions for its appearance have not been clarified. This study aimed to investigate the association between CSF parameters and enhancement on contrast-enhanced head or spinal MRI in patients with bacterial meningitis (BM) or tuberculous meningitis (TM). METHODS: A total of 12 patients with BM and 23 patients with TM who underwent both CSF analysis and contrast-enhanced MRI were included. The correlation between CSF analysis and MRI findings has been examined using receiver operating characteristic (ROC) analysis. RESULTS: Contrast enhancement was found in 7 and 10 patients with BM and TM, respectively. Both CSF analysis and MRI were useful to distinct between BM and TM, and the enhancement implied the severity of them. In patients with BM, higher CSF protein and lower CSF glucose were associated with enhancement on MRI, while not only CSF protein and glucose but also leukocyte and lymphocyte counts were associated with it in TM. CSF adenosine deaminase (ADA) did not show discriminant ability of the MRI findings. CONCLUSIONS: CSF analysis is associated with enhancement on contrast-enhanced MRI both in BM and TM. Our findings indicate the importance of CSF analysis in assessing the need to perform contrast-enhanced MRI, which may be useful in diagnosis, distinction, and estimation of prognosis in those patients.

Status epilepticus severity score as a predictor for the length of stay at hospital for acute-phase treatment in convulsive status epilepticus.

To date, hospital length of stay (LOS) determinants for convulsive status epilepticus's (CSE) acute-phase treatment have not been sufficiently investigated, as opposed to those for status epilepticus's (SE) outcome predictors, such as status epilepticus severity score (STESS). Here, we aimed at assessing the significance of STESS in the LOS in patients with CSE. We retrospectively reviewed consecutive adult patients with CSE who were transported to the emergency department of our urban tertiary care hospital in Tokyo, Japan. The study period was from August 2010 to September 2015. The primary endpoint was the LOS of patients with CSE who were directly discharged after acute-phase treatment, and survival analysis for LOS until discharge was conducted. As a result, among 132 eligible patients with CSE admitted to our hospital, 96 (72.7%) were directly discharged with a median LOS of 10 days (IQR: 4-19 days). CSE patients with severe seizures, represented by higher STESS (≥3), had a significantly longer LOS after adjustments with multiple covariates (p = 0.016, in restricted mean survival time analysis). Additionally, prediction for the binomial longer/shorter LOS achieved better performance when STESS was incorporated into the prediction model. Our findings indicate that STESS can also be used as a rough predictor of longer LOS at index admission of patients with CSE.

Anti-myelin oligodendrocyte glycoprotein antibody-positive acute disseminated encephalomyelitis mimicking limbic encephalitis: A case report.

Anti-myelin oligodendrocyte glycoprotein antibodies (MOG-ab) have been detected in various disorders of the central nervous system including acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disorders (NMOSD), optic neuritis, myelitis, and cortical encephalitis. We report an atypical case of MOG-ab-associated encephalomyelitis with part of the clinical manifestations resembling limbic encephalitis. Multifocal, hyperintense, bilateral lesions predominantly affecting the white matter on brain magnetic resonance imaging and marked response to steroid therapy were compatible with a MOG-ab-associated disease. This case illustrates that MOG-ab-associated disease should be considered in encephalomyelitis involving the bilateral limbic system.

A favorable outcome of intensive immunotherapies for new-onset refractory status epilepticus (NORSE).

BACKGROUND: New-onset refractory status epilepticus (NORSE) is a newly defined critical disease entity characterized by prolonged periods of refractory epileptic seizure with no readily identifiable cause in otherwise healthy individuals. Its etiology is uncertain, but autoimmune encephalitis is a possible candidate for the underlying cause of this condition. Immunotherapies could be considered for this condition, but its efficacy is not established. CASE PRESENTATION: A 31-year-old man with no prior history presented with refractory status epilepticus. His seizure persisted even with multiple anti-epileptic drugs and required prolonged general anesthesia under mechanical ventilation. Magnetic resonance imaging and cerebrospinal fluid did not indicate the cause of seizure, and autoantibodies related to encephalitis were not detected. It was speculated that the patient had occult autoimmune encephalopathy because of its acute-onset clinical course preceded by fever, even without definite evidence of an autoimmune mechanism. The patient received intravenous methylprednisolone, plasma exchange, and intravenous immunoglobulin in succession and manifested a favorable outcome after these treatments. CONCLUSION: Our case supports the efficacy of immunotherapies for NORSE even though it does not manifest definite evidence for autoimmune background. Clinicians should consider these immunotherapies for NORSE as early as possible, because this condition is associated with high mortality and morbidity owing to prolonged seizure activity and long-term intensive care including general anesthesia and mechanical ventilation.

Steady-state relationship between average glucose, HbA1c and RBC lifespan.

HbA1c is used to estimate average glucose. Previous studies showed linear relationship between average glucose and HbA1c. We made a new theoretical relationship using recently proposed Γ-like function model of erythrocyte lifespan. We showed the relationship between average glucose and HbA1c; we approximated it into a simple hyperbolic function: HbA1c=MRBCkgAG/(1+(2/3)MRBCkgAG), whose inverse function is easily obtained. Apparent linear relationship is an approximation of the curved relationship. Hyperbolic function would provide a more accurate approximation than a linear equation. Physicians should keep in mind the curved relationship and be aware that extremely high HbA1c indicates acceleratingly high glucose level.

The Prehospital Predictors of Tracheal Intubation for in Patients who Experience Convulsive Seizures in the Emergency Department.

Objective To identify the prehospital factors predicting the performance of tracheal intubation (TI) at the emergency department (ED) in patients with convulsive seizure or epilepsy. Methods We performed a retrospective analysis of seizure patients who underwent TI at the ED soon after arrival. The clinical variables obtained in the prehospital setting were reviewed. Patients The study population included consecutive adult patients who were transported to an urban tertiary care ED due to convulsive seizure between August 2010 and September 2015. Results Among the 822 eligible patients, 59 patients (7.2%) underwent TI at the ED. Four independent prehospital predictors were identified using multivariate analysis: age ≥50 years (+1 point), meeting the definition of convulsive status epilepticus (+4 points), and an on-scene heart rate of ≥120 bpm (+1 point) led to a higher likelihood of TI, while a higher on-scene (alert or confused) level of consciousness (-3 points) led to a lower likelihood of TI. The derived prediction rule (the sum of all points) had good predictive performance with an area under the curve of 0.88 (95% confidence interval: 0.79-0.97), a sensitivity of 0.62, a specificity of 0.91, and a positive likelihood ratio of 10.6, when the cut-off value was set to 5 points. Conclusion We constructed a simple prehospital prediction rule to help predict the need for TI in seizure patients, even in the prehospital phase. This may possibly lead to the more effective management of seizure patients in the ED.

Improved 123I-Ioflupane Binding After Immunotherapy in Anti-NAE Antibody-Positive Hashimoto Encephalopathy That Clinically Mimicked Multiple System Atrophy.

We describe an 84-year-old man with anti-NH2-terminal of α-enolase antibody-positive Hashimoto encephalopathy that clinically mimicked multiple system atrophy who underwent investigation by dopamine transporter SPECT before and after immunotherapy. Before treatment, dopamine transporter SPECT showed reduced striatal I-ioflupane binding, with a mean specific binding ratio of 2.42, even though he had no apparent parkinsonism. After immunotherapy, mean specific binding ratio was improved to 3.22. Dopamine transporter SPECT was useful in this case to detect subclinical striatal dysfunction, and evaluation both before and after immunotherapy helped to distinguish between neurodegenerative disease and neuroimmunological disorder.

Old Stroke as an Independent Risk Etiology for Todd's Paralysis.

BACKGROUND: Todd's paralysis (TP) is a well-known postictal paresis in which patients present with transient weakness in their limb(s) after seizures. Although recognized as a stroke mimic in clinical practice, the pathophysiological mechanism and clinical features of TP remain unknown. Furthermore, its diagnosis can be erroneous in neurological emergency practice. We aimed to illustrate the clinical features and identify factors associated with TP. METHODS: This single-center, retrospective observational study included consecutive adult patients who presented with convulsive seizure and were referred to an urban tertiary care emergency department between August 2010 and April 2016. The diagnosis of TP was set as the primary outcome measure. Clinical and laboratory variables were evaluated. RESULTS: Of 1381 eligible convulsive seizures in 1187 patients, TP was observed in 89 seizures (6.4%) in 75 patients. Patients with TP were significantly older, more likely to have convulsive status epilepticus, and had a longer duration of convulsion than patients without TP. TP was found in 19.7% (39 of 198) of convulsive seizures with remote etiologies including those due to old stroke. These etiologies were identified as independent significant risk factors for TP compared with seizures with cryptogenic etiology. The positive likelihood ratio of TP seizures was 11.2 for remote seizure etiologies. CONCLUSIONS: Our results indicated that the diagnosis of TP highly suggests premorbid or comorbid structural lesions in the central nervous system, including old stroke. This consideration in seizure etiology may help in reducing the risk of misdiagnosis of acute stroke in emergency settings and further antiepileptic treatment.

Hyperammonaemia and associated factors in unprovoked convulsive seizures: A cross-sectional study.

PURPOSE: Hyperammonaemia is frequently observed in patients who have experienced convulsive seizures. Although excessive muscle contraction is presumed to be responsible for the elevated levels of ammonia, the underlying mechanism is poorly understood. The present study aimed to identify the independent factors associated with ammonia elevation using large-scale multivariate analysis. METHODS: We conducted a cross-sectional study involving 379 adult patients who had been transported to our emergency department and treated for unprovoked convulsive seizures between August 2010 and September 2015. Elevation of venous plasma ammonia levels was set as the primary endpoint, and patients' clinical and laboratory data were obtained. Those with severe liver dysfunction, known hepatic encephalopathy, or convulsions due to cardiovascular or psychogenic causes, and those taking valproate were excluded. RESULTS: Using a cut-off value of 50µg/dL, 183 patients (48.3%) were found to have elevated levels of plasma ammonia. Four factors were identified as independent variables associated with hyperammonaemia following seizures: elevated venous lactate, lowered venous pH, sex (male), and longer duration of convulsion. CONCLUSIONS: The results of the present study revealed independent factors associated with hyperammonaemia following unprovoked convulsive seizures in a larger scale and with more plausible statistical analysis. The authors further suggest that the excessive skeletal muscle contraction and/or respiratory failure during/after convulsive seizure may be the primary mechanism of hyperammonaemia.

Anti-TIF1-γ antibody and cancer-associated myositis: A clinicohistopathologic study.

OBJECTIVE: We aimed to analyze the clinical and histopathologic features of cancer-associated myositis (CAM) in relation to anti-transcriptional intermediary factor 1 γ antibody (anti-TIF1-γ-Ab), a marker of cancer association. METHODS: We retrospectively studied 349 patients with idiopathic inflammatory myopathies (IIMs), including 284 patients with pretreatment biopsy samples available. For the classification of IIMs, the European Neuromuscular Center criteria were applied. Patients with CAM with (anti-TIF1-γ-Ab[+] CAM) and without anti-TIF1-γ-Ab (anti-TIF1-γ-Ab[-] CAM) were compared with patients with IIM without cancers within and beyond 3 years of myositis diagnosis. RESULTS: Cancer was detected in 75 patients, of whom 36 (48%) were positive for anti-TIF1-γ-Ab. In anti-TIF1-γ-Ab(+) patients with CAM, cancers were detected within 1 year of myositis diagnosis in 35 (97%) and before 1 year of myositis diagnosis in 1. All the anti-TIF1-γ-Ab(+) patients with CAM satisfied the dermatomyositis (DM) criteria, including 2 possible DM sine dermatitis cases, and were characterized histologically by the presence of perifascicular atrophy, vacuolated fibers (VFs), and dense C5b-9 deposits on capillaries (dC5b-9). In contrast, 39 anti-TIF1-γ-Ab(-) patients with CAM were classified into various subgroups, and characterized by a higher frequency of necrotizing autoimmune myopathy (NAM). Notably, all 7 patients with CAM classified into the NAM subgroup were anti-TIF1-γ-Ab(-) and exhibited no dC5b-9 or VFs. CONCLUSIONS: CAM includes clinicohistopathologically heterogeneous disease entities. Among CAM entities, anti-TIF1-γ-Ab(+) CAM has characteristically shown a close temporal association with cancer detection and the histopathologic findings of dC5b-9 and VFs, and CAM with NAM is a subset of anti-TIF1-γ-Ab(-) CAM.

Reduced 123I Ioflupane Binding in Bilateral Diabetic Chorea: Findings With 18F FDG PET, 99mTc ECD SPECT, and 123I MIBG Scintigraphy.

We report a 64-year-old man with diabetic chorea whom we investigated with dopamine transporter SPECT, F FDG PET, Tc ethylcysteinate dimer (ECD) SPECT, and I metaiodobenzylguanidine (MIBG) scintigraphy. Dopamine transporter SPECT revealed reduced I ioflupane binding in the bilateral striatum. F FDG PET showed metabolic dysfunction in the bilateral striatum, as shown in earlier studies. Tc ECD SPECT revealed reduced brain perfusion in the bilateral caudate nucleus and putamen. I MIBG scintigraphy revealed no cardiac sympathetic nerve dysfunction. Our case suggests a possible nigrostriatal presynaptic dopaminergic involvement in diabetic chorea.

Exclusion of Isolated Cortical Swelling Can Increase Efficacy of Baseline Alberta Stroke Program Early CT Score in the Prediction of Prognosis in Acute Ischemic Stroke Patients Treated with Thrombolysis.

BACKGROUND: The accuracy of the Alberta Stroke Program Early CT Score (ASPECTS) as a prognostic indicator in the treatment of cerebral infarction with thrombolysis remains controversial. We hypothesized that ASPECTS can more accurately predict treatment outcomes by excluding isolated cortical swelling (ICS) from ASPECTS and retrospectively tested patients treated with thrombolysis. METHODS: This retrospective cohort study included 106 patients treated with intravenous thrombolysis for cerebral infarction in our hospital. We included only patients with middle cerebral artery infarction. For the modification of ASPECTS, we removed each ICS from the ASPECTS system. We compared the correlation coefficients between the ASPECTS and modified ASPECTS with regard to treatment outcome, and performed a multivariate logistic regression analysis to evaluate the association between modified ASPECTS and outcomes. The primary outcome was a modified Rankin Scale score equal to or less than 2 on discharge and the secondary outcomes included an improvement of National Institutes of Health Stroke Scale (NIHSS) score of 4 or greater within 24 hours. RESULTS: Seventy-two patients were included in this study. The correlation coefficient of modified ASPECTS was significantly higher than that of ASPECTS in the primary outcome (r = .249 versus r = .363, P < .001) and in the improvement of NIHSS score (r = .303 versus r = .443, P < .001). Multivariate analysis revealed that a modified ASPECTS greater than 7 was significantly associated with the primary outcome (odds ratio [OR] = 1.334, 95% confidence interval [CI] = 1.071-1.661, P = .012) and the improvement of the NIHSS score (OR = 1.555, 95% CI = 1.208-2.003, P = .001). CONCLUSIONS: The present study reveals that ASPECTS may be more strongly associated with outcome by excluding ICS.

Brainstem Infarction and Panuveitis due to Sarcoidosis Successfully Treated with Steroid Pulse Therapy.

A 36-year-old man visited our hospital because of blurred vision and redness of the conjunctiva. Slit-lamp examination showed panuveitis. Two days later, he suddenly experienced dizziness, speech disturbance, paralysis of his right extremities, and gait disturbances. Neurological examinations suggested that his symptoms were caused by a left lateral medullary lesion. He also had erythema mainly on his trunk. Magnetic resonance imaging (MRI) of his brain demonstrated a small infarct on the left side of the medulla oblongata. Clinical presentation and MRI findings were consistent with the diagnosis of a Wallenberg's syndrome. He also had bilateral hilar lymphadenopathy. A skin biopsy showed granulomatous nodular dermatitis compatible with sarcoidosis. He was treated with steroid pulse therapy and his neurological and ocular symptoms immediately improved. Only seven similar cases of intracranical sarcoidosis have been reported, but none had been treated with steroid pulse therapy. We recommend that steroid pulse therapy be considered to treat patients with sarcoidosis with signs of lesions in the central nervous system.

Pyrenes and pyrendiones from Uvaria lucida.

A chemical investigation of the chloroform extract of the roots of Uvaria ludida Benth. (Annonaceae), an important African traditional medicine, led to the isolation of six new compounds; three pyrenes, 2-hydroxy-1,8-dimethoxypyrene (1), 8-methoxy-1,2-methylenedioxypyrene (2), and 7-hydroxy-8-methoxy-1,2-methylenedioxypyrene (3), two pyrenediones, 2-hydroxy-1,8-pyrenedione (4) and 2-methoxy-1,8-pyrenedione (5), and a sesquiterpene, (-)-10-oxo-isodauc-3-en-15-oic acid (6), together with eight known compounds (7-14). The structural elucidation by spectroscopic studies of the compounds isolated is described. While pyrenes did not exhibit strong cytotoxicity against human promyelocytic leukemia HL-60 cells, pyrenediones showed strong cytotoxicity. The IC(50) of 4 was 70 ng mL(-1), which was close to that of etoposide (IC(50) = 60 ng mL(-1)).

[Case of Fisher syndrome with impairment of taste].

A 38-year-old man developed dysesthesia, diplopia, and an unsteady gait following an upper respiratory infection. IgG anti-GQ1b antibody was detected in his serum and he was diagnosed as Fisher syndrome. The patient also complained of loss of taste sensation, and it resolved along with improvement of other neurological manifestations. In Guillain-Barré syndrome, cranial nerve involvement is very common, though taste disturbance is a rare complaint. Impairment of taste has been reported in association with severe facial nerve involvement, but taste disturbance developed without facial nerve palsy in the present case and taste sensation was diminished in the area of all four nerves involved in taste sensation. These findings suggest that the impaired taste sensation in the present patient was not a complication of facial nerve palsy as in previous cases, but rather due to taste sensory specific involvement.

Diffusion-weighted MR imaging in acute spinal cord ischemia.

We report diffusion-weighted (DW) MR findings for acute spinal cord ischemia in a 56-year-old patient. MR imaging obtained approximately 3 h after symptom onset demonstrated an area of hyperintensity on DW images, but no conspicuous signal abnormality on T2-weighted images in the conus medullaris. DW imaging of the spinal cord can contribute to the early detection of spinal cord vascular compromise.