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Pei-Ya Chen,1,2 Guei-Chiuan Chen,1,2 Cheng-Lun Hsiao,1 Po-Jen Hsu,1 Fu-Yi Yang,1 Chih-Yang Liu,1 Adam Tsou,1 Wan-Ling Chang,1 Hsiu-Hsun Liu,1 Shinn-Kuang Lin1,2 1Stroke Center and Department of Neurology, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City, Taiwan; 2School of Medicine, Tzu Chi University, Hualien, TaiwanCorrespondence: Shinn-Kuang Lin, Tel +886-2-66289779 ext 3129, Fax +886-2-66289009, Email stuartlin0428@gmail.com; sk2022@tzuchi.com.twPurpose: We investigated the differences of clinical features, four immune-inflammatory markers, namely neutrophil counts, platelet-to-lymphocyte ratio, neutrophil-to-lymphocyte ratio (NLR), and systemic immune-inflammation index (SII), as well as outcomes between patients with in-hospital ischemic stroke (IHIS) and out-of-hospital ischemic stroke (OHIS).Patients and Methods: We retrospectively enrolled 72 patients with IHIS and 3330 patients with OHIS.Results: IHIS accounted for 2% of all patients with ischemic stroke and occurred more often in cardiology and orthopedic surgery wards. Infection, cardiac disease, and pulmonary disorder were the most common causes of hospitalization. Compared with those with OHIS, patients with IHIS had higher levels of immune-inflammatory markers, initial National Institute of Health Stroke Scale (NIHSS) scores, longer hospital stays, higher rates of heart disease, large-artery atherosclerosis or cardioembolism, received more intravenous thrombolysis (IVT) or endovascular thrombectomies (EVTs), more complications, unfavorable outcomes, and mortality. Every immune-inflammatory marker exhibited positive correlations with initial NIHSS scores and discharge modified Rankin Scale scores among patients with OHIS. NLR and SII were higher among patients with a fatal outcome in both groups. Among patients receiving IVT, most of treatment time intervals were shorter for those with IHIS than those with OHIS. Significant factors for mortality were NLR > 5.5, atrial fibrillation, and complications, with a C-statistic of 0.897 in those with IHIS; in those with OHIS, these factors were an initial NIHSS score of > 10, NLR > 6.0, atrial fibrillation, prior stroke, cancer history, and complications with a C-statistic of 0.902. The results were similar after replacing the NLR with SII.Conclusion: Patients with IHIS had more complicated clinical features, higher levels of immune-inflammatory markers, and higher rates of mortality than patients with OHIS. The most significant predictor for mortality among those with OHIS was NIHSS score > 10, and the predictors among patients with IHIS were NLR > 5.5 and SII > 2120.Keywords: immune-inflammatory markers, in-hospital ischemic stroke, intrahospital delay, neutrophil-to-lymphocyte ratio, out-of-hospital ischemic stroke, systemic immune-inflammation index

Shinn-Kuang Lin,1,2 Pei-Ya Chen,1,2 Guei-Chiuan Chen,1 Po-Jen Hsu,1 Cheng-Lun Hsiao,1 Fu-Yi Yang,1 Chih-Yang Liu,1 Adam Tsou1 1Stroke Center and Department of Neurology, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City, Taiwan; 2School of Medicine, Tzu Chi University, Hualien, TaiwanCorrespondence: Shinn-Kuang LinStroke Center and Department of Neurology, Taipei Tzu Chi Hospital, No. 289, Jian Guo Road, 231, Sindian District, New Taipei City, TaiwanTel +886-2-66289779 ext 3129Fax +886-2-66289009Email stuartlin0428@gmail.comPurpose: Immune–inflammatory processes are involved in all the stages of stroke. This study investigated the association of the neutrophil-to-lymphocyte ratio (NLR) with the hyperdense artery sign (HAS) observed on brain computed tomography (CT) and with clinical features in patients with acute ischemic stroke.Methods: We retrospectively enrolled 2903 inpatients with acute ischemic stroke from May 2010 to May 2019. Data collected included imaging studies, risk factors, laboratory parameters, and clinical features during hospitalization.Results: The HAS was identified in 6% of the 2903 patients and 66% of the 236 patients with acute middle cerebral artery occlusion. Patients with the HAS had a higher NLR. HAS prevalence was higher in men and patients with cardioembolism. The NLR exhibited positive linear correlations with age, glucose and creatinine levels, length of hospital stay, initial National Institutes of Health Stroke Scale (NIHSS) scores, and mRS scores at discharge. The NLR was significantly higher in patients with large-artery atherosclerosis and cardioembolism and was the highest in patients with other determined etiology. Multivariate analysis revealed that an initial NIHSS score of ≥ 10 and an NLR of > 3.5 were significant positive factors, whereas diabetes mellitus and age > 72 years were significant negative factors for the HAS, with a predictive performance of 0.893. An initial NIHSS score of ≥ 5, positive HAS, age > 75 years, diabetes mellitus, an NLR of > 3.5, female sex, a white blood cell count of > 8 × 103/mL, and elevated troponin I were significant predictors of unfavorable outcomes, with a predictive performance of 0.886.Conclusion: An NLR of > 3.5 enabled an efficient prediction of CT HAS. In addition to conventional risk factors and laboratory parameters, both an NLR of > 3.5 and CT HAS enabled improved prediction of unfavorable stroke outcomes.Keywords: acute ischemic stroke, hyperdense artery sign, neutrophil-to-lymphocyte ratio, NIHSS, unfavorable outcome

Background Atherosclerosis is a chronic inflammatory process that occurs in the arterial wall. This immuno-inflammatory process plays a role throughout all stages of stroke. Neutrophils, lymphocytes, and platelets are crucial blood cells for innate and adaptive immunity. This study investigated the associations of four types of immuno-inflammatory markers, namely the systemic immune-inflammation index (SII), neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and neutrophil count (NC), with clinical outcomes in patients with acute ischemic stroke. Methods In this retrospective study, we enrolled 2903 inpatients with acute ischemic stroke from May 2010 to May 2019. Data included risk factors, laboratory parameters, and clinical features during hospitalization. The National Institutes of Health Stroke Scale (NIHSS), and modified Rankin Scale (mRS) were used to assess stroke severity and outcomes. Results All four immuno-inflammatory markers exhibited positive linear correlations with age, glucose, creatinine, length of hospital stay, NIHSS score on admission, and mRS score at discharge. The levels of the four immuno-inflammatory markers were significantly higher in patients with large-artery atherosclerosis and cardioembolism and were highest in patients with other determined etiology. Patients with levels of immuno-inflammatory markers higher than their cutoff values for unfavorable outcomes also exhibited higher rates of cancer history (except for SII and NC), uremia (except for NC), elevated troponin I, and in-hospital complications. Multivariate analysis including SII revealed that admission NIHSS score ≥ 5, age > 75 years, SII > 724, diabetes mellitus, female sex, elevated troponin I, heart disease, and prior stroke were significant predictors for unfavorable outcomes. These significant predictors were retained after replacing SII > 724 with NLR > 143, PLR > 3.5, or NC > 6 × 10 3 /mL, except for prior stroke. For a basic model comprising seven significant predictors of unfavorable outcomes, the C-statistic was 0.860. The addition of SII, NLR, and PLR to the basic model resulted in a significant improvement in the prediction performance to 0.864, 0.863, and 0.863, respectively. Conclusions Immuno-inflammatory markers provide more useful information than conventional risk factors and other laboratory parameters for the prediction of stroke outcomes. SII > 724 is the most appropriate marker when combined with other predictors.

Background: Efficacy of thrombolytic therapy decreases with time elapsed from symptom onset. We sought to identify the impact of code stroke on the thrombolytic therapy. Methods: Code stroke is activated by the emergency physician when a patient is eligible for thrombolytic therapy. We retrospectively reviewed patients with acute ischemic stroke between January 2011 and December 2014. Results: In total, 1809 patients were enrolled. Code stroke was activated in 233 of 351 patients arriving at the emergency room (ER) within 3 h of symptom onset, and in 21 patients arriving >3 h. The sensitivity, specificity, and positive and negative predictive values of code stroke were 76%, 46%, 72%, and 51%, respectively. Thrombolytic therapy was provided to 58 patients, accounting for 3.4% of all cerebral infarcts. Code stroke was activated in 40 of these patients. The most common reasons for excluding thrombolytic therapy were: National Institute of Health Stroke Scale (NIHSS) 80 years. Mean liaison-to-neurological evaluation time was only 6 min. Code stroke activation significantly reduced all the intervals, except for the onset-to-ER and door-to-order times. During the 4-year study period, there were significant reductions of the door-to-neurology liaison time by 28 min and door-to-laboratory time by 22 min. The proportion of door-to-needle time within 60 min improved from 33% in 2011 to 67% in 2014. Improved NIHSS scores during hospitalization were most prominent in tPA-treated patients. Symptomatic ICH occurred in 3.6% patients arriving within 3 h. Death occurred in 50% of patients received tPA treatment on family's request, and only 13% of those patients had favorable outcome. Conclusion: Code stroke is effective in reducing in-hospital delays. The accuracy of code stroke activation has acceptable sensitivity but low specificity. Rapid patient assessment by neurologists increases the number of patients eligible for thrombolytic therapy. Keywords: Acute stroke, Brain infarction, Code stroke, In-hospital delays, Thrombolytic therapy

Background and aims Renal dysfunction is a potent risk factor for cardiovascular diseases, including stroke. This study aimed to evaluate the impact of admission estimated glomerular filtration rate (eGFR) levels on short-term (1-month) and long-term (1-year) mortality in patients with acute ischemic stroke. Methods From the Taiwan Stroke Registry data, we classified ischemic stroke patients, identified from April 2006 to December 2015, into 5 groups by eGFR at admission: ≥ 90, 60–89, 30–59, 15–29, and Results Among 52,732 ischemic stroke patients, 1480 died within one month. The 1-month mortality rate was over 5-fold greater in patients with eGFR Conclusions After acute ischemic stroke, patients with reduced eGFR are at elevated risks of short-term and long-term deaths in a graded relationship.

We investigated the impact of serum cholesterol levels on 30-day mortality after ischemic stroke in dialysis patients.From the Taiwan Stroke Registry data, we identified 46,770 ischemic stroke cases, including 1101 dialysis patients and 45,669 nondialysis patients from 2006 to 2013.Overall, the 30-day mortality was 1.46-fold greater in the dialysis group than in the nondialysis group (1.75 versus 1.20 per 1000 person-days). The mortality rates were 1.64, .62, 2.82, and 2.23 per 1000 person-days in dialysis patients with serum total cholesterol levels of 120 mg/dL, 120-159 mg/dL, 160-199 mg/dL, and ≥200 mg/dL, respectively. Compared to dialysis patients with serum total cholesterol levels of 120-159 mg/dL, the corresponding adjusted hazard ratios of mortality were 4.20 (95% confidence interval [CI] = 1.01-17.4), 8.06 (95% CI = 2.02-32.2), and 6.89 (95% CI = 1.59-29.8) for those with cholesterol levels of 120 mg/dL, 160-199 mg/dL, and ≥200 mg/dL, respectively.Dialysis patients with serum total cholesterol levels of ≥160 mg/dL or 120 mg/dL on admission are at an elevated hazard of 30-day mortality after ischemic stroke.

Background: Acute intermittent porphyria (AIP) is an inherited disorder of heme biosynthesis, the clinical manifestations of which are incompletely understood. In this report, we describe 12 cases of AIP, focusing on the neurological manifestations. Methods: Twelve patients were diagnosed with AIP on the basis of characteristic clinical findings, erythrocyte porphobilinogen deaminase (PBGD) activity, and molecular genetics. Central and peripheral nervous system manifestations were noted, and electrophysiological and radiological studies performed. Potential precipitating factors were recorded. Results: Eleven PBGD gene mutations were identified in 12 patients. Nine patients experienced neurological symptoms involving the central nervous system (consciousness disturbance, n = 8; convulsion/seizure, n = 4; behavior change, n = 1), while 7 patients experienced peripheral neuropathies (motor paresis, n = 7; impairment of bulbar or respiratory function, n = 4). The electrophysiological and electroencephalographic findings were consistent with the neurological symptoms of AIP. Urinary PBG and δ-aminolevulinic acid levels were elevated in all patients. PBGD enzyme activity levels were below normal in all patients. Eight patients had documented exposure to porphyrogenic agents. Conclusions: Our detailed description of a relatively large number of cases of AIP may help clinicians to recognize this often difficult-to-diagnose disorder.

Four briarane-type diterpenoids, including two new chlorinated metabolites, fragilides F (1) and G (2), along with two known compounds, junceellonoid D (3) and juncin Z (4), were isolated from the ...

Prenatal toluene exposure may lead to significant developmental neurotoxicity known as fetal solvent syndrome. Emerging evidence suggests that toluene embryopathy may arise from an elusive deviation of the neurogenesis process. One key event during neural development is synaptogenesis, which is essential for the progression of neuronal differentiation and the establishment of neuronal network. We therefore aim to test the hypothesis that toluene may interfere with synaptogenesis by applying toluene to cultured hippocampal neurons dissected from embryonic rat brains. In the presence of toluene, hippocampal neurons displayed a significant loss of the immunostaining of synapsin and densin-180 punctas. Notably, a dramatic reduction was also discerned for the colocalization of the two synaptic markers. Moreover, Western blotting analyses revealed that toluene exposure resulted in considerable down-regulation of the expression of synapse-specific proteins. None of the preceding observations can be attributed to toluene-induced cell death effects, since toluene treatments failed to affect the viability of hippocampal neurons. Overall, our data are consistent with the idea that toluene may alter the expression and localization of essential synaptic proteins, thereby leading to a disruption of synapse formation and maintenance.

Cleidocranial dysplasia (CCD; MIM 119600) is a rare autosomal dominant disorder characterized by facial, dental, and skeletal malformations. To date, rearrangement and mutations involving RUNX2, which encodes a transcription factor required for osteoblast differentiation on 6p21, has been the only known molecular etiology for CCD. However, only 70% patients were found to have point mutations, 13% large/contiguous deletion but the rest of 17% remains unknown. We ascertained a family consisted of eight affected individuals with CCD phenotypes. Direct sequencing analysis revealed no mutations in the RUNX2. Real time quantitative PCR were performed which revealed an exon 2 to exon 6 intragenic deletion in RUNX2. Our patients not only demonstrated a unique gene change as a novel mechanism for CCD, but also highlight the importance of considering “deletion” and “duplication” in suspected familial cases before extensive effort of gene hunting be carried.

A higher frequency of hypoplasic vertebral artery (VA) in patients with migraine with aura than in normal controls has been documented. However, the role of a hypoplasic VA in a migraine attack remains unclear. The aim of our work was to measure the net VA flow volume and related spectral parameters in patients with migraine with aura and a hypoplasic VA. From January 2005 to October 2005 we reviewed the records of 250 migraine outpatients (108 men and 142 women; mean age = 30.8 ± 14.0 years, range = 25–55). Ninety-two patients with migraine with aura were selected. Among these patients, 26 had a hypoplasic VA that was delineated by cervical magnetic resonance angiography. We performed a case-control study that included these 26 migrainous patients. Duplex color-coded ultrasonography was utilized to calculate the spectral parameters during attacks and headache-free periods. The net VA flow volume did not decrease during attacks. A reduction in the resistance index of the hypoplasic VA was noted during attacks in subjects who had migraine with aura. Our observation of VA vasomotor alteration during migraine attacks extends the understanding of the role of a hypoplasic VA. Vasomotor regulation of the VA could be neurogenic in origin. We hypothesize that VA hypoplasia contributes to migraine through complex neurovascular pathways rather than through its low flow volume.

Anterior cerebral artery A1 segment hypoplasia is an uncommon fetal variant of the circle of Willis. The frequency of this congenital variation is 1–13% as derived from angiograms and autopsy reports. Impaired collateral blood flow through the circle of Willis is a recognized risk factor for ischemic stroke. The A1 segment of the anterior cerebral artery is a principal supplier of anterior collateral blood flow. The aim of our study was to determine whether A1 segment hypoplasia may be responsible for acute ischemic stroke. We consecutively examined 280 acute ischemic stroke patients (aged 66.9 ± 14.2 years). Cerebral magnetic resonance angiography was performed within 72 h of ischemic stroke onset. The overall incidence of A1 variation in our experimental group was 15.0% (n = 42, agenesis/hypoplasia = 18/24), which was statistically higher than in the control group (n = 12). The majority (n = 30, 71.42%) had ipsilateral striatal lacunar infarctions. Based on our results, A1 agenesis/hypoplasia appears to be a risk factor contributing to ischemic stroke, especially to strokes in arteries penetrating the striatal area.

Infectious mononucleosis (IM) complicated with a neurological manifestation, including acute cerebellar ataxia, Guillain-Barre syndrome, meningitis, encephalitis, cranial nerve palsies, optic neuritis or transverse myelitis, has been rarely reported; however, IM complicated with acute cerebral infarction has never been reported in the literature.A 49-year-old man with diabetic mellitus suffered from IM with fever, pharyngitis, parotiditis with lymphadenopathies, thrombocytopenia and splenomegaly. After two weeks of conservative treatment, left upper limb paresis and left hemihypesthesia occurred. Neuroimaging demonstrated acute ischemic stroke involving the right frontal lobe. In view of the underlying infection, immediate intravenous rt-PA was not recommended; hence, oral aspirin 100 mg daily was prescribed and he received regular rehabilitation in the subsequent follow up.Although IM is known to be self-limited, it could contribute to acute cerebral infarction, which is a rare IM neurological complication.

BACKGROUND AND PURPOSE The requirement for neurology liaison is increasing in accordance with the growing health care demands associated with aging populations. The aim of this study was to characterize the nature of neurological inpatient liaisons (NILs) to help plan for the appropriate use of neurology resources. METHODS This was a retrospective cross-sectional study of NILs in a secondary referral hospital over a 12-month period. RESULTS There were 853 neurological consultations with a liaison rate of 3% per admission case. Chest medicine, gastroenterology, and infectious disease were the three most frequent specialties requesting liaison, and altered consciousness, seizure, and stroke were the three most frequent disorders for which a NIL was requested. Infection was the most common cause of altered consciousness. Epilepsy, infection, and previous stroke were common causes of seizure disorders. Acute stroke accounted for 44% of all stroke disorders. Electroencephalography was the most recommended study, and was also the most frequently performed. Ninety-five percent of emergency consultations were completed within 2 hours, and 85% of regular consultations were completed within 24 hours. The consult-to-visit times for emergency and regular consultations were 44±47 minutes (mean±standard deviation) and 730±768 minutes, respectively, and were shorter for regular consultations at intensive care units (p=0.0151) and for seizure and stroke disorders (p=0.0032). CONCLUSIONS Altered consciousness, seizure, and stroke were the most common reasons for NILs. Half of the patients had acute neurological diseases warranting immediate diagnosis and treatment by the consulting neurologists. Balancing increasing neurologist workloads and appropriate health-care resources remains a challenge.

Background and objectives: Botulinum toxin type A has been shown to relieve primary headaches, myofascial pain and various neuropathic pains. Carpal tunnel syndrome (CTS) is a common disorder resulting from median nerve compression at the wrist. As reports of botulinum toxin A in the treatment of CTS were limited, this study set out to evaluate the safety and tolerability of botulinum toxin A and its effects on the relief of nerve entrapment and pain in patients with CTS. Methods: We conducted an open-label, prospective pilot study using 60 units of botulinum toxin A injected intracarpally in patients with primary CTS. Changes in median nerve conduction velocities, distal latencies, compound muscle action potentials and visual analogue scale (VAS) pain scores were evaluated for 3 months following injection. All adverse experiences, reported spontaneously or observed directly by the investigator, were recorded. Results: Five women aged 52.2 ± 2.5 years with 1–2 years’ history of CTS were enrolled. Botulinum toxin A was well tolerated and safe. No exacerbated hand weakness was observed in any of the patients. At 3 months, pain was lessened in three patients, remained static in one patient, and was aggravated in one patient. The VAS pain score showed a trend to improvement during the 3 months of follow-up, although it did not reach statistical significance (p = 0.2). Conclusion: Our data suggest long-lasting antinociceptive effects of botulinum toxin A rather than electrophysiological restoration in patients with CTS. Intracarpal injection of botulinum toxin A was shown to be well tolerated and safe. A double-blind, placebo-controlled trial of botulinum toxin A in CTS is warranted since the current study may have been confounded by the placebo effect of intracarpal injection.

A new 3beta,5alpha,6beta-trihydroxysteroid, bebryceoid A (1), has been isolated from an octocoral Bebryce sp. In addition, an octocoral Carijoa sp. yielded two new 3beta,5alpha,6beta-trihydroxysteroids, carijoids A (2) and B (3). The structures of steroids 1-3 were elucidated by spectroscopic methods and by comparison of the spectral data with those of known steroid analogues.

We report a 6-month-old girl with episodic hyperventilation, myoclonus, disturbed consciousness, and lactic acidosis. Brain sonogram revealed progressive ventriculomegaly, and MRI showed symmetric low densities over the putamen bilaterally with diffuse cortical cystic lesions. Ragged-red fibers were noted in the muscle biopsy. Molecular analysis revealed a heteroplasmic T-->G mutation at nucleotide position (np) 8993 of mitochondrial DNA (mtDNA). The proportion of the mutant mtDNA in the muscle of the proband was estimated to be 86%. Her mother and maternal uncle also harbored the same mutant mtDNA (54 and 48% in their leukocytes, respectively). One of her sisters carried 64% mutant mtDNA in her leukocytes, but another sister did not. These results suggest that this mutant mtDNA is transmitted through the maternal lineage in this family in a randomly segregated manner. To our knowledge, this is the first report of a Chinese patient with Leigh syndrome associated with the T-->G substitution at np 8993 of mtDNA.

Objectives. Sacral root stimulation (SRS) is a technique to restore the idiopathic overactive bladder (IOB). However, its mechanism of action is yet to be elucidated. Hence, we studied whether SRS restored IOB through the mechanism of spinal neuromodulation. Materials and Methods. Six IOB patients and 10 healthy volunteers were included in the study. The spinal nociceptive reflex was used as the index of spinal excitability and was evoked by electrical stimulation at the foot, with recording at the ipsilateral tibialis anterior. Results. IOB patients had increased spinal excitability to somatic nociceptive stimuli of the lower limbs. This spinal excitability decreased and bladder function improved after SRS, an effect that outlasted actual stimulation by at least 30 min. Conclusions. Our results showed that spinal excitability was increased in response to somatic nociceptive afferents in IOB patients. SRS restored bladder function, at least, in part, through spinal neuromodulation.

Painful ophthalmoplegia due to extramedullary plasmacytoma is a rare initial manifestation of multiple myeloma. The present report describes a 48-year-old man who suffered an acute onset of retro-orbital pain, left abducens palsy and left facial hypoesthesia. In addition, he exhibited an elevated erythrocyte sedimentation rate and partial responsiveness to corticosteroid treatment, all of which resemble the features of Tolosa–Hunt syndrome. Imaging studies revealed a multilobulated tumour invading the left sphenoid bone and sphenoid sinus, later confirmed as a plasmacytoma at pathology. Multiple myeloma was also diagnosed by bone marrow examination. After completion of chemotherapy and radiotherapy, the patient has been free of symptoms for 10 months. Although cranial neuropathies with any combination of oculomotor, abducens, trochlear, ophthalmic and maxillary nerves may indicate a cavernous sinus lesion, neuropathies exclusive to the abducens and maxillary nerves may raise the possibility of extracavernous sinus origin. Cranial imaging is crucial in diagnosing painful ophthalmoplegia with additional minimal cranial nerve signs.

The novel hydroxysteroids (I)—(III) are isolated from the freeze-dried material of both marine species mentioned in the title.

Four briarane-type diterpenoids, including two new chlorinated metabolites, fragilides F (1) and G (2), along with two known compounds, junceellonoid D (3) and juncin Z (4), were isolated from the ...

Aims: Warfarin, a widely prescribed oral anticoagulant, is used for the prevention of thromboembolism. Several polymorphisms in VKORC1 have been shown to be associated with warfarin dose requirements. The frequencies of these VKORC1 polymorphisms display population differences; however, this has not been examined in many populations. In this study, we examined VKORC1 polymorphisms in five East-Asian populations (Han Chinese, Indonesian, Filipino, Thai and Vietnamese) and Indians. Materials & methods: A total of six SNPs in the VKORC1 gene (-1639G>A, 497T>G, 1173C>T, 1542T>G, 2255C>T and 3730G>A) were genotyped. Frequencies, linkage disequilibrium and haplotype structures of these six VKORC1 SNPs were analyzed. Results: Our data showed that 497T>G is only polymorphic in the Indian population. The 497G allele is very rare in the East-Asian populations (frequency < 1%). The remaining SNPs demonstrated high linkage disequilibrium and had similar frequencies and haplotype structures in all but the Indian population. The Indian population is mostly made up of the H7 haplotype (76%) while the rest of the recruited populations consisted of the H1 haplotype (>80%).

Posterior communicating artery (PCoA) hypoplasia is a fetal variant of the Circle of Willis. According to angiograms and autopsy reports, this congenital variation is found in 6-21% of the general population. PCoA hypoplasia only becomes a risk factor for ischemic stroke in the presence of ipsilateral internal carotid artery (ICA) occlusion. The aim of our study was to determine the role of PCoA hypoplasia in acute ischemic stroke in the absence of ICA occlusion. We examined 310 acute ischemic stroke patients (mean age+/-standard deviation; 68.9+/-15.6 years). Cerebral magnetic resonance angiography was performed within 72 hours of ischemic stroke onset. For comparison, a risk factor-matched control group was recruited. Conditional logistic regression was used to calculate odds ratios (OR) and 95% confidence intervals (CI) to estimate the independent effect of potential risk factors. The overall incidence of PCoA hypoplasia in our experimental group was 19.35% (n=60), which was significantly higher than in the control group (8.20%, n=22, p=0.036, OR, 3.21; 95% CI, 1.43-9.62). The most common ischemic event was ipsilateral thalamic lacunar infarctions with or without occipital lobe involvement. Based on our results, PCoA hypoplasia appears to be a contributor to the risk of ischemic stroke, even in the absence of ICA occlusion. This risk is especially pronounced for strokes involving arteries that penetrate the thalamus.

Electrical stimulation may produce excitation or inhibition of the motor neurons, as represented the blink reflex and masseter silent period in response to trigeminal nerve stimulation. Clinically, a light touch on the palm may evoke a mentalis muscle response (MMR), i.e. a palmomental reflex. In this study, we attempted to characterize the MMR to median nerve stimulation. Electrical stimulation was applied at the median nerve with recordings at the mentalis muscles. An inhibition study was done with continuous stimuli during muscle contraction (I1 and I2 of MMRaverage). Excitation was done with a single shot during muscle relaxation (MMRsingle) or by continuous stimuli during muscle contraction (E1 and E2 of MMRaverage). The characteristic differences between MMRaverage and MMRsingle were as follows: earlier onset latencies of MMRaverage (MMRaverage45 ms; MMRsingle60 ms), and a lower amplitude of MMRaverage (MMRaverage50 microV; MMRsingle150 microV). The receptive field of MMRsingle was widespread over the body surface and that of MMRaverage was limited to the trigeminal, median and index digital nerves. Series of stimuli usually significantly decreased the amplitude of MMRsingle, as a phenomenon of habituation. On the other hand, it was difficult to evoke the earlier response (i.e. MMRaverage) without continuous stimuli and an average technique. MMRaverage had the components of both excitation (E) and inhibition (I); for example, E1-I1-E2-I2 or I1-E2-I2. E2 was the most consistent component. In patients with dorsal column dysfunction, median nerve stimulation could successfully elicit MMRsingle, but not MMRaverage. Contrarily, in patients with pain sensory loss, it was more difficult to reproduce MMRsingle than MMRaverage. It seemed that MMRaverage and MMRsingle did not have equivalents across the different modalities of stimulation.

Mitochondrial diseases are heterogeneous disorders affecting multiple systems. Here, we presented the findings of multimodal evoked potential (EP) studies of three siblings with a specific A8344G mutation of mitochondrial DNA. One of them had DM and another two had a history of encephalopathy. Visual EPs were abnormal in one patient and motor, somatosensory and brainstem auditory EPs were observed in all three patients. Our EP studies showed that the A8344G mutation of mitochondrial DNA involved multiple levels of the central nervous system even though there were no correlated symptoms. Therefore EP is an adjunct of methods to detect the functional disturbance and to screen the distribution of the involvement of the nervous system in mitochondrial diseases.

Guillain-Barre syndrome (GBS) is an acute neuropathy and a clinical syndrome that includes a number of pathological and electrophysiological subtypes. Intravenous immunoglobulin (IVIG) and plasma exchange (PE) are both equally efficacious for the treatment of GBS; however, the cost of IVIG may be lower for both the patient and the healthcare system. To compare the pharmacoeconomics of PE and IVIG in GBS, a retrospective study was done from 1999 to 2004, which included a total of 24 patients with GBS who were admitted to Taipei Veterans General Hospital. This showed that except for the costs of the drugs used in IVIG, treatment of GBS with IVIG was more cost-effective (p=0.057) than that with PE in total length of hospitalization and the cost of procedures and hospitalization. The study also showed that the total costs were higher for patients on ventilators than those not requiring ventilators (p=0.008, t-test) and the length of hospitalization showed a very strong linear relationship to total costs (Pearson correlation coefficient=0.907). The regression analysis showed that each additional day of hospitalization increased the hospitalization costs by an average of 5599 New Taiwan Dollars (NT) (US$1.00=NT$33.50 in 2005).

A 68-year-old man developed progressive four-limb weakness and areflexia 17 days after an influenza vaccination. He was diagnosed with Guillain-Barre syndrome (GBS), and remained ventilator dependent and bed-bound for 3 months, despite plasmapheresis and immunoglobulin infusion. However, cognitive impairment, excessive daytime sleepiness, and motor disability were still present, even when he was no longer ventilator dependent. Brain computerized tomography scan and isotope cisternography was consistent with normal pressure hydrocephalus. His motor control and cognitive function recovered almost completely after insertion of a ventriculoperitoneal shunt. Although hydrocephalus is not frequently associated with GBS, our case report indicates that brain imaging is necessary in GBS patients whose cognitive functions deteriorates after disease onset.