bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neuroepithelial Cysts Presenting with Movement Disorders: Two Cases
Authors: Heran Navraj S. , Berk Caglar , Constantoyannis Constantine , Honey Christopher R. 
Published: December 2014

Background:The authors present two cases of movement disorders caused by neuroepithelial cysts and highlight their management. Neuroepithelial cysts are ependymal or epithelial lined fluid collections of unknown etiology within the central nervous system parenchyma with no obvious ventricular or subarachnoid connection. Most cysts are asymptomatic, however, some present with seizures, mass effect, or rarely with movement disorders.Case Reports:The first patient, a 27-year-old female, presented with progressive weakness, dystonic posturing, tremor, ballismus and choreoathetotic movements of her right upper extremity. Her symptoms improved after stereotactic drainage of a neuroepithelial cyst in her basal ganglia but recurred within a year. The second case, a 56-year-old female, presented with diplopia, nystagmus, gait imbalance and hemiparesis. Her symptoms improved after stereotactic drainage of a midbrain neuroepithelial cyst. The cyst reaccumulated over the next few years and she became symptomatic with left arm tremor and facial weakness. Aspiration was again performed with symptomatic improvement for nine months. Her tremor recurred and a cyst access device was placed stereotactically. She improved and has remained stable for over a year.Conclusion:Simple stereotactic drainage of neuroepithelial cysts has a high recurrence rate. The authors recommend considering placement of a drainage device to facilitate aspiration of the cyst fluid during follow-up, if needed.
Structural Abnormalities are Similar in Familial and Nonfamilial Mesial Temporal Lobe Epilepsy
Authors: Ferreira Fabio Thadeu , Kobayashi Eliane , Lopes-Cendes Iscia , Cendes Fernando 
Published: December 2014

Background/Objective:Diffuse temporal lobe abnormalities can be observed on MRI of patients with mesial temporal lobe epilepsy (MTLE). Our objective was to perform qualitative and quantitative analyses of temporal lobe structures in patients with familial MTLE (FMTLE) and nonfamilial MTLE.Methods:Two groups of patients were ascertained: 67 FMTLE patients (14 with refractory seizures) and 30 patients with nonfamilial refractory MTLE. We performed qualitative analyses of MRI (with multiplanar reconstruction) and volumes of hippocampi and anterior temporal lobes in all patients, and in a normal control group of 23 individuals. We used the Chi-square test and ANOVA for statistical analyses.Results:We identified anterior temporal lobe abnormalities by visual analysis in only 4% of FMTLE patients and atrophy of the anterior temporal lobe by volumetric analysis in 19%. In the group of nonfamilial MTLE patients we found anterior temporal lobe abnormalities by visual analysis in 17% of patients and anterior temporal lobe atrophy in 13%. Hippocampal atrophy was present in 90% of FMTLE and in 83% of nonfamilial MTLE. No signs of cortical dysplasia were observed.Conclusion:Anterior temporal lobe atrophy and other abnormalities outside the mesial portion of temporal lobes were infrequent in both familial and nonfamilial MTLE patients. Despite the genetic basis, hippocampal atrophy in FMTLE is not associated with other abnormalities outside the mesial temporal regions.
Trends in Hospital Admission for Stroke in Calgary
Authors: Field T.S. , Green T.L. , Roy K. , Pedersen J. , Hill M.D. 
Published: December 2014

Background:Stroke incidence has fallen since 1950. Recent trends suggest that stroke incidence may be stabilizing or increasing. We investigated time trends in stroke occurrence and in-hospital morbidity and mortality in the Calgary Health Region.Methods:All patients admitted to hospitals in the Calgary Health Region between 1994 and 2002 with a primary discharge diagnosis code (ICD-9 or ICD-10) of stroke were included. In-hospital strokes were also included. Stroke type, date of admission, age, gender, discharge disposition (died, discharged) and in-hospital complications (pneumonia, pulmonary embolism, deep venous thrombosis) were recorded. Poisson and simple linear regression was used to model time trends of occurrence by stroke type and age-group and to extrapolate future time trends.Results:From 1994 to 2002, 11642 stroke events were observed. Of these, 9879 patients (84.8%) were discharged from hospital, 1763 (15.1%) died in hospital, and 591 (5.1%) developed in-hospital complications from pneumonia, pulmonary embolism or deep venous thrombosis. Both in-hospital mortality and complication rates were highest for hemorrhages. Over the period of study, the rate of stroke admission has remained stable. However, total numbers of stroke admission to hospital have faced a significant increase (p=0.012) due to the combination of increases in intracerebral hemorrhage (p=0.021) and ischemic stroke admissions (p=0.011). Sub-arachnoid hemorrhage rates have declined. In-hospital stroke mortality has experienced an overall decline due to a decrease in deaths from ischemic stroke, intracerebral hemorrhage and sub-arachnoid hemorrhage.Conclusion:Although age-adjusted stroke occurrence rates were stable from 1994 to 2002, this is associated with both a sharp increase in the absolute number of stroke admissions and decline in proportional in-hospital mortality. Further research is needed into changes in stroke severity over time to understand the causes of declining in-hospital stroke mortality rates.
Cerebral Vein and Sinus Thrombosis in Isfahan-Iran: A Changing Profile
Authors: Saadatnia Mohammad , Mousavi Seyyed Ali , Haghighi Sassan , Aminorroaya Ashraf 
Published: December 2014

Objectives:This study was performed to investigate the clinical presentation and predisposing factors for cerebral vein and sinus thrombosis (CVST) in Isfahan, Iran.Methods:Data from the records of all patients with CVSTreferred to the largest tertiary-care hospital of Isfahan during a five-year period (1997 to 2001) were extracted and reviewed.Results:The number of cases with CVST diagnosed annually was 6, 9, 11, 14 and 15 patients, respectively. Thirteen men and 42 women were diagnosed to have CVST with the mean age of 35.1± 3.8 and 28.7±1.3 years, respectively. Headache was the most frequent complaint (95%) and 63% of patients had focal neurological symptoms, including seizure (58%). Among possible predisposing factors, oral contraceptive pill was the most prevalent one, which was used by 38.1% of affected women for a period of as short as 1-3 months. Anticardiolipin antibodies were detected in 14% of patients.Conclusion:It seems that the annual incidence of CVST is increasing in Isfahan, perhaps due to more extensive intake of oral contraceptive pills and usage of more accurate modern diagnostic tools. The use of oral contraceptive pills was the most frequent predisposing factor; infections and postpartum factors were infrequently observed. Despite other reports from the Middle East, Behçet's disease is not a principal risk factor for CVST in Isfahani patients.
Remission of Multiple Sclerosis Post-Liver Transplantation
Authors: Yoshida Eric M. , Devonshire Virginia A. , Prout Alister J.E. 
Published: December 2014

Background:The effect of liver transplantation on pre-existing multiple sclerosis (MS) has never been reported. We report the three year post-transplant neurological outcome of a patient with MS.Case report:A Caucasian woman with MS received an urgent liver transplant for fulminant liver failure at the age of 59. Her Extended Disability Scale Score (EDSS) pretransplant was 5.0 and clinically she had cerebellar and brainstem dysfunction. Post-transplant immunosuppression consisted of tacrolimus, mycophenolate mofetil and tapering corticosteroids that were discontinued after 1.5 years. Post-transplant her EDSS decreased to 2.0 and after three years she is clinically asymptomatic with only very mild dysarthria on neurologic examination. Long-term maintenance immunosuppression consists of low dose tacrolimus.Conclusion:Combination immunosuppression with tacrolimus may have a beneficial effect on MS although an effect of donor allograft itself can not be excluded.
Reversible Coma: A Rare Presentation of Spontaneous Intracranial Hypotension
Authors: Kashmere Jodi L. , Jacka Michael J. , Emery Derek , Gross Donald W. 
Published: December 2014

Background:Spontaneous intracranial hypotension (SIH) is a well-recognized neurologic disorder that typically presents with orthostatic headaches, low cerebral spinal fluid pressures and distinct abnormalities on magnetic resonance imaging.Methods:We present a case of a rare presentation of SIH.Results:A 49-year-old man presented with a two week history of orthostatic headaches that rapidly progressed to encephalopathy and coma, requiring intubation. Neuroimaging revealed abnormalities typical of SIH; diffusely enhancing pachymeninges, subdural fluid collections, and descent of the brain. Treatment with an epidural blood patch reversed his coma within minutes. Following a second blood patch, the patient became asymptomatic. No cerebral spinal leak could be identified on magnetic resonance imaging or on a nuclear medicine technetium cerebral spinal fluid flow study. At six month follow-up, he remained symptom free.Conclusion:The mechanism of coma in SIH is presumed to be compression of the diencephalon from downward displacement of the brain. Although it is very unusual for patients with SIH to present with coma, it is important to recognize since the coma may be reversible with epidural blood patches.
Intracranial Hypotension with Severe Neurological Symptoms Resolved by Epidural Blood Patch
Authors: Peng Philip W.H. 
Published: December 2014

Background:A patient with a constellation of severe neurological symptoms caused by spontaneous intracranial hypotension, which was successfully managed by epidural blood patch, was described.Case history:A 50-year-old woman presented to the neurological service with a two-month history of orthostatic headache. Associated clinical features included sensorineural deafness, ataxia and short-term memory loss. Magnetic resonance imaging showed downward displacement of brain structures, bilateral subdural collections and the postgadolinium images demonstrated diffuse dural enhancement suggestive of the diagnosis of spontaneous intracranial hypotension.Results:Following a lumbar epidural blood patch, all symptoms improved significantly and her headache resolved.Conclusion:The principal presentation of spontaneous intracranial hypotension is orthostatic headache. The patient described presented a combination of various neurological symptoms, ataxia, memory loss and deafness, which all responded well to epidural blood patch.
Pathology of Mitochondrial Encephalomyopathies
Authors: Sarnat Harvey B. , Marín-García José 
Published: December 2014

ABSTRACT:Muscle biopsy provides the best tissue to confirm a mitochondrial cytopathy. Histochemical features often correlate with specific syndromes and facilitate the selection of biochemical and genetic studies. Ragged-red fibres nearly always indicate a combination defect of respiratory complexes I and IV. Increased punctate lipid within myofibers is a regular feature of Kearns-Sayre and PEO, but not of MELAS and MERRF. Total deficiency of succinate dehydrogenase indicates a severe defect in Complex II; total absence of cytochrome-c-oxidase activity in all myofibres correlates with a severe deficiency of Complex IV or of coenzyme-Q10. The selective loss of cytochrome-c-oxidase activity in scattered myofibers, particularly if accompanied by strong succinate dehydrogenase staining in these same fibres, is good evidence of mitochondrial cytopathy and often of a significant mtDNA mutation, though not specific for Complex IV disorders. Glycogen may be excessive in ragged-red zones. Ultrastructure provides morphological evidence of mitochondrial cytopathy, in axons and endothelial cells as well as myocytes. Abnormal axonal mitochondria may contribute to neurogenic atrophy of muscle, a secondary chronic feature. Quantitative determinations of respiratory chain enzyme complexes, with citrate synthase as an internal control, confirm the histochemical impressions or may be the only evidence of mitochondrial disease. Biological and technical artifacts may yield falsely low enzymatic activities. Genetic studies screen common point mutations in mtDNA. The brain exhibits characteristic histopathological alterations in mitochondrial diseases. Skin biopsy is useful for mitochondrial ultrastructure in smooth erector pili muscles and axons; skin fibroblasts may be grown in culture. Mitochondrial alterations occur in many nonmitochondrial diseases and also may be induced by drugs and toxins.
Optimal Check Size and Reversal Rate to Elicit Pattern-reversal MEG Responses
Authors: Chen Wei-Ta , Ko Yu-Chieh , Liao Kwong-Kum , Hsieh Jen-Chuen , Yeh Tzu-Chen , Wu Zin-An , Ho Low-Tone , Lin Yung-Yang 
Published: December 2014

ABSTRACT:Objective:To determine the impact of check size and interstimulus interval (ISI) on neuromagnetic visual cortical responses.Methods:We recorded visual evoked fields to pattern-reversal stimulation with central occlusion in ten subjects. The ~100 ms magnetic activation (P100m) was analyzed by single dipole modeling.Results:With 1 s ISI, P100m strengths increased as check size increased from 15' up to 120' of visual arc, and larger checks elicited less P100m activation. With 120' checks, we found no P100m attenuation as ISI decreased from 4 s to 0.16 s. P100m sources around the calcarine sulcus did not vary with check size or ISI.Conclusions:The magnitude of cortical activation during visual contrast processing is check size-dependent and the 120' checks are optimum for future studies on neuromagnetic visual cortical functions using central-occluded stimulation. The corresponding neuronal activation demonstrated a short refractory period less than 0.16 s. We also found significantly overlapping cortical representation areas for different check sizes or ISIs.
Reliability of Carotid Doppler performed in a dedicated Stroke Prevention Clinic
Authors: Dean N. , Lari H. , Saqqur M. , Amir N. , Khan K. , Mouradian M. , Salam Abdul , Romanchuk H. , Shuaib A. 
Published: December 2014

ABSTRACT:Introduction:Doppler ultrasound (DUS) is used as a screening tool to assess internal carotid artery (ICA) disease. Recent reports suggest that the DUS may be inaccurate in over 28% of patients. We sought to evaluate the accuracy of DUS, when performed in a dedicated stroke prevention clinic (SPC).Methods:We retrospectively reviewed the charts of patients who had a DUS performed in our SPC, followed by conventional cerebral angiography. Three groups of patients were defined. Group 1 had DUS measured ICA stenosis of >50%; Group II had a DUS measured ICA stenosis of <50%; Group III had complete ICA occlusion on DUS.Results:Sixty-seven patients (69 arteries) were included in the study. There were 45 patients in Group I and based on the findings of cerebral angiography, carotid endarterectomy was considered inappropriate in only one patient. - a misclassification rate of 2.2% (95%CI: 0 – 6.5%). Group II consisted of 19 patients and on cerebral angiography, none of these patients had a stenosis of >50% - a misclassification rate of 0%. Group III consisted of five patients in whom DUS showed complete ICA occlusion. The angiogram confirmed the occlusion in all five patients – a misclassification rate of 0%. Overall, misclassification rate was 1.45% (95% CI: 0 - 4.3%).Conclusions:Doppler ultrasound when performed in a stroke prevention clinic (SPC), has a high accuracy in measuring ICA stenosis of >50%. Doppler ultrasound is reliable in detecting complete ICA occlusion and finally DUS is a reliable screening tool to rule out clinically significant ICA stenosis.
Spontaneous Spinal Epidural Hematoma During Pregnancy
Authors: Kelly Michael E.B. , Beavis R. Cole , Hattingh Suzanne 
Published: December 2014

ABSTRACT:Background:Spontaneous spinal epidural hematoma is a rare phenomenon that has no distinct etiology. Spontaneous spinal epidural hematoma (SSEH) during pregnancy is extremely rare. We present what we believe to be the fifth reported case of spontaneous spinal epidural hematoma associated with pregnancy in the English literature.Methods:A 31-year-old female presented with acute onset of paraplegia at 32 weeks of pregnancy. The patient had a T2 sensory level and complete paralysis of all lower extremity motor groups. Magnetic resonance imaging of the thoracic spine showed an acute epidural hematoma posterior to the thoracic spinal cord between the second and fourth thoracic vertebrae.Results:The patient was taken to the operating room were her child was delivered by caesarean section. She then underwent a posterior laminectomy and evacuation of a spinal epidural hematoma. Followup selective spinal angiography was negative for any vascular malformation. The patient gradually recovered lower extremity function and was independently ambulating at six month follow-up. Voluntary bowel and bladder function returned within four months but twice daily intermittent catheterization remained necessary for excessive post-void residual urine.Conclusions:Spontaneous spinal epidural hematoma in pregnancy is a rare phenomenon. It is postulated that elevated venous pressure associated with pregnancy may be a contributing factor. In the reported cases of SSEH in pregnancy most patients presented with acute symptoms, thoracic location and profound neurological deficits but, with prompt surgical treatment, generally had good long term recovery.
Duchenne De Boulogne: A Pioneer in Neurology and Medical Photography
Authors: Parent André 
Published: December 2014

ABSTRACT:Guillaume-Benjamin-Amand Duchenne was born 200 years ago in Boulogne-sur-Mer (Pas-de-Calais, France). He studied medicine in Paris and became a physician in 1831. He practiced general medicine in his native town for about 11 years and then returned to Paris to initiate pioneering studies on electrical stimulation of muscles. Duchenne used electricity not only as a therapeutic agent, as it was commonly the case earlier in the 19th century, but chiefly as a physiological investigation tool to study the anatomy of the living body. Without formal appointment he visited hospital wards across Paris searching for rare cases of neuromuscular disorders. He built a portable electrical device that he used to functionally map all bodily muscles and to study their coordinating action in health and disease. He gave accurate descriptions of many neuromuscular disorders, including pseudohypertrophic muscular dystrophy to which his name is still attached (Duchenne muscular dystrophy). He also invented a needle system (Duchenne's histological harpoon) for percutaneous sampling of muscular tissue without anesthesia, a forerunner of today's biopsy. Duchenne summarized his work in two major treatises entitled De l'électrisation localisée (1855) and Physiologie des mouvements (1867). Duchenne's iconographic work stands at the crossroads of three major discoveries of the 19th century: electricity, physiology and photography. This is best exemplified by his investigation of the mechanisms of human physiognomy in which he used localized faradic stimulation to reproduce various forms of human facial expression. The album that complements his book on this issue is considered a true incunabulum of photography. Duchenne de Boulogne, a shy but hard-working, acute and ingenious observer, became one of most original clinicians of the 19th century. He died in Paris in 1875.
Diffusion Tensor Imaging Abnormalities in Focal Cortical Dysplasia
Authors: Gross Donald W , Bastos Alexandre , Beaulieu Christian 
Published: December 2014

ABSTRACT:Purpose:Focal cortical dysplasia (FCD) is one of the most common underlying pathologic substrates in patients with medically intractable epilepsy. While magnetic resonance imaging (MRI) evidence of FCD is an important predictor of good surgical outcome, conventional MRI is not sensitive enough to detect all lesions. Previous reports of diffusion tensor imaging (DTI) abnormalities in FCD suggest the potential of DTI in the detection of FCD. The purpose of this study was to study subcortical white matter underlying small lesions of FCD using DTI.Methods:Five patients with medically intractable epilepsy and FCD were investigated. Diffusion tensor imaging images were acquired (20 contiguous 3mm thick axial slices) with maps of fractional anisotropy (FA), trace apparent diffusion coefficient (trace/3 ADC), and principal eigenvalues (ADC parallel and ADC perpendicular to white matter tracts) being calculated for each slice. Region of interest analysis was used to compare subcortical white matter ipsilateral and contralateral to the lesion.Results:Three subjects with FCD associated with underlying white matter hyperintensities on T2 weighted MRI were observed to have increased trace/3 ADC, reduced fractional anisotropy and increased perpendicular water diffusivity which was greater than the relative increase in the parallel diffusivity. No DTI abnormalities were identified in two patients with FCD without white matter hyperintensities on conventional T2-weighted MRI.Conclusions:While DTI abnormalities in FCD with obvious white matter involvement are consistent with micro-structural degradation of the underlying subcortical white matter, DTI changes were not identified in FCD lesions with normal appearing white matter.
The Impact of a Stroke Prevention Clinic in Diagnosing Modifiable Risk Factors for Stroke
Authors: Mouradian Mikael S. , Hussain Muhammad S. , Lari Harris , Salam Abdul , Senthilselvan Ambikaipakan , Dean Naeem , Shuaib Ashfaq 
Published: December 2014

ABSTRACT:Objective:To evaluate the referral patterns of patients to a stroke prevention clinic (SPC) and to test the adequacy of pre-referral diagnosis and management of modifiable risk factors for stroke.Methods:We collected prospective data on consecutive patients referred to the SPC at University of Alberta Hospital in Edmonton, Alberta, Canada. Outcome measures included: alternate diagnoses to stroke or transient ischemic attack (TIA), uncontrolled or undiagnosed hypertension, hyperlipidemia and diabetes, therapies, and investigations leading to carotid endarterectomy.Results:Two thousand and eleven patients were referred to SPC. Nearly 25% of the referrals originated from the emergency room and the rest from general physicians. Of the referrals, 68.7% were confirmed as TIA or stroke at the SPC. Among 1381 patients with TIA or stroke, 736 had history of hypertension. Uncontrolled hypertension was found in 265 patients (36.0% of those with hypertension: 95% CI: 32.5–39.5) while undiagnosed hypertension was found in 103 (15.9% of those without hypertension: 95%CI: 13.14-18.79). History of hyperlipidemia was present in 451 patients (32.6%) and 356 (78.9%: 95% CI: 75.2-82.69) of these patients were not at target for secondary prevention. Among 930 patients without history of hyperlipidemia, 739 (79.5%: 95% CI: 76.8-82.1) were diagnosed with hyperlipidemia through the SPC. Fasting blood glucose levels above 7.1 mmol/L in patients with and without history of diabetes were 221 (79.2%: 95% CI: 74.5-83.9) and 66 (6%: 95%CI: 4.6-7.4) respectively.Conclusions:Management of risk factors for stroke needs improvement. SPCs should consider actively managing the classical modifiable risk factors of stroke.