bluuebunny/crossref_metadata_2025_split huggingface dataset:
Prognostic Indicators in an Aggressive Pituitary Crooke's Cell Adenoma
Authors: Kovacs K. , Diep C.C. , Horvath E. , Cusimano M. , Smyth H. , Coire C. , Lombardero M. , Scheithauer B.W. , Lloyd R.V. 
Published: December 2014

ABSTRACT:Objective:To investigate prognostic indicators in an aggressive Crooke's cell adenoma of the pituitary.Methods:The surgically removed tumor was studied by histology, immunohistochemistry and transmission electron microscopy.Results:An aggressive invasive sellar tumor removed by repeated surgeries from a 43-year-old woman with pituitary related Cushing's disease was classified as a Crooke's cell adenoma of the pituitary. The application of several cell proliferation markers confirmed the aggressive nature of the tumor.Conclusions:The investigation of the present case provides additional evidence that pituitary Crooke's cell adenomas may possess aggressive behavior.
Progress in Clinical Neurosciences: Frontotemporal Dementia-Pick's Disease
Authors: Kertesz Andrew 
Published: December 2014

ABSTRACT:Frontotemporal dementia (clinical Pick's disease) is a relatively common, but underdiagnosed degenerative disease in the presenium. Estimated prevalence ranges from 6-12% of dementias. The behavioural, aphasic and extrapyramidal presentations are labeled FTD-behavioural variant, Primary Progressive Aphasia (PPA) and Corticobasal Degeneration/Progressive Supranuclear Palsy (CBD/PSP). The diagnostic features and course of each are described and their overlap in the evolution of the illness is emphasized. The neuropathology ranges from the most common tau negative ubiquitin positive amyotrophic lateral sclerosis (ALS) type inclusions to the tau positive classical Pick bodies and more or less distinct changes of PSP and CBD. The genetics of the relatively frequent tau mutations and the yet unsolved problem of tau negative families are discussed. The tau negative cases tend to be associated with the behavioural presentation and semantic dementia and the tau positive ones with PPA and the CBD/PSP syndrome. However the overlap is too great to split the disease. A glossary to navigate the proliferating terminology is included.
Triphasic Waves Versus Nonconvulsive Status Epilepticus: EEG Distinction
Authors: Boulanger Jean-Martin , Deacon Charles , Lécuyer Diane , Gosselin Sylvie , Reiher Jean 
Published: December 2014

ABSTRACT:Background:Triphasic waves (TWs) and generalized nonconvulsive status epilepticus (GNCSE) share morphological features that may create diagnostic ambiguity.Objective::To describe electroencephalographic differences between TWs and GNCSE.Methods:We retrospectively compared the electroencephalograms (EEGs) of two groups of patients presenting with decreased level of consciousness; those with TWs associated with metabolic encephalopathy and those with GNCSE. We studied the following: demographics, etiology and EEG morphological features. All EEGs were classified blindly (TWs or GNCSE) by two expert EEGers. Agreement between experts and concordance with clinical diagnosis were measured.Results:We analysed 87 EEGs (71 patients) with TWs and 27 EEGs (13 patients) with GNCSE. Agreement between experts and concordance with clinical diagnosis were excellent. When compared to TWs, epileptiform discharges associated with GNCSE had a higher frequency (mean=2.4Hz vs 1.8Hz) (p<0.001), a shorter duration of phase one (p=0.001), extra-spikes components (69% vs 0%) (p<0.001) and less generalized background slowing (15.1% vs 91.1%) (p<0.001). Amplitude predominance of phase two was common with TWs (40.8% vs 0%) (p=0.01). Lag of phase two was absent in all cases of GNCSE but present in 40.8% of patients with TWs. Noxious or auditory stimulation frequently increased the TWs (51%) while it had no effect on the epileptiform pattern (p=0.008).Conclusion:Certain EEG morphological criteria and the response to stimulation are very helpful in distinguishing TWs from GNCSE.
Subdural Effusions in the Posterior Fossa Associated with Spontaneous Intracranial Hypotension
Authors: Watanabe Arata , Horikoshi Toru , Uchida Mikito , Koizumi Hidehito , Yamazaki Hiromichi , Kinouchi Hiroyuki 
Published: December 2014

ABSTRACT:Background:Misdiagnosis of spontaneous intracranial hypotension remains a problem, despite increasing recognition.Methods:Three patients with spontaneous intracranial hypotension presented with typical findings on lumbar puncture, magnetic resonance (MR) imaging, and radioisotope cisternography. All patients showed subdural effusions in the posterior fossa on axial T2-weighted MR imaging. Axial MR images of 112 patients with other conditions were also screened for this finding.Results:One of three patients had typical orthostatic headache, and the other two had continuous headache. The finding of subdural effusions in the posterior fossa on axial T2-weighted MR imaging disappeared after treatment. Similar findings were found in 14 of 112 patients with other conditions. Most of the patients were over 60 years old or had dementia or previous radiation therapy.Conclusion:Subdural effusions in the posterior fossa can be identified by T2-weighted axial MR imaging, and are useful for the diagnosis of spontaneous intracranial hypotension and for verifying the effectiveness of treatment.
Acute Anterior Circulation Stroke: Recanalization Using Clot Angioplasty
Authors: Lum Cheemun , Stys Peter K. , Hogan Matthew J. , Nguyen Thanh B. , Srinivasan Ashok , Goyal Mayank 
Published: December 2014

ABSTRACT:Background and Purpose:Different strategies have been employed to recanalize acutely occluded middle cerebral and internal carotid arteries (ICA) in the setting of acute stroke including intravenous and intra-arterial tPA. However, pharmaceutical thrombolysis alone, may not be effective in patients with a large amount of clot volume (complete M1, terminal internal carotid artery). We report our initial experience with endovascular clot disruption using a soft silicone balloon in addition to intravenous or intraarterial thrombolysis with tPA.Methods:This is a retrospective review of nine patients with symptoms of acute stroke from clot in the middle cerebral or internal carotid territories who were treated with intracranial balloon angioplasty. All patients presented with symptoms of acute anterior circulation stroke less than six hours from onset. Patients in whom computed tomography (CT) angiography confirmed the presence of large vessel clot (terminal ICA, M1 or proximal M2) were included in the study. A CT perfusion was performed providing maps of cerebral blood volume, flow and mean transit time. If the patient presented less than three hours from onset then intravenous tissue plasminogen activator (tPA) was also administered. Intra-arterial tPA was delivered into the clot. If the volume of clot was judged to be significant by the treating neurointerventionist, then a limited trial of tPA was administered intraarterially followed by balloon angioplasty of persistant clot. The time from imaging to vessel recanalization was recorded. Clinical outcomes were assessed using the modified Rankin scale and Barthel Index.Results:Diagnostic CT perfusion studies were performed in 7 (78%), all of which showed a significant amount of salvageable tissue as judged by the treating neurointerventionist and neurologist. Recanalization (TIMI 2 or 3) was possible in 8 (89%). There were no cases of symptomatic intracranial hemorrhage and 2 (22%) asymptomatic hemorrhages. The average time from performance of the initial emergency CT to vessel recanalization was 2.1 hours with mean time from symptom onset to vessel recanalization of 4.1 hours. Five (56%) patients had good outcomes, 1 (11%) had mild and 3 (33%) had moderate to severe disability.Conclusion:Clot angioplasty can potentially shorten recanalization times in well-selected patients and can be an effective complimentary procedure in patients with tPA resistant clot. Angioplasty can be performed with a very low complication rate using the technique described and may be associated with good outcomes.
Demographics and Clinical Features of Patients Referred to Headache Specialists
Authors: Jelinski Susan E. , Becker Werner J. , Christie Suzanne N. , Giammarco Rose , Mackie Gordon F. , Gawel Marek J. , Eloff Arnolda G. , Magnusson Jane E. 
Published: December 2014

ABSTRACT:Objective:To examine demographic characteristics and clinical features of headache patients referred to neurologists specializing in headache in Canada.Methods:Demographic and clinical data were collected at the time of consultation for 865 new headache patients referred to five headache-specialty clinics in Canada. The Headache Impact Test (HIT-6) and Migraine Disability Questionnaire (MIDAS) were used to measure headache impact and disability. Data were analyzed as part of the Canadian Headache Outpatient Registry and Database (CHORD) Project.Results:The average age of the patients was 40 years and the majority were female (78%). Most were employed either full time (49%) or part time (13%). The majority of patients were diagnosed with either migraine or tension-type headache (78%). Over a third of patients experienced headache every day, and half had experienced a headache in the previous month which was of severe intensity. Most (80%) scored in the “very severe” category of the HIT-6 and over half (55%) were severely disabled as measured by the MIDAS.Conclusion:Patients referred to headache specialists in Canada are severely disabled by their headache disorders. These patients are in the most productive phase of their lives in terms of age and employment. It is important to provide the best available treatment to headache patients in order to minimize the disability and impact of their headache disorders.
Rasmussen's Encephalitis in a 58-Year-Old Female: Still a Variant?
Authors: Hunter Gary R. W. , Donat Jeffrey , Pryse-Phillips William , Harder Sheri , Robinson Christopher A. 
Published: December 2014

ABSTRACT:Background:We report the case of a 58-year-old female with clinical, radiological, and histopathological evidence of Rasmussen's encephalitis, representing the oldest confirmed case to date.Case Summary:The patient presented with complex partial seizures characterized by numbness of the left face and staring spells. These progressed to a state of epilepsia partialis continua with jerking of the left face, as well as severe cognitive impairment and loss of all communication. The patient responded well to Intravenous Immunoglobulin (IVIG) therapy despite early complications and with ongoing treatment is living independently with minimal cognitive impairment.Conclusions:This represents the oldest confirmed case of Rasmussen's encephalitis and suggests that this diagnosis should be considered in patients of any age with an appropriate clinical picture. We recommend IVIG as a first line therapy for adult cases of Rasmussen's encephalitis.
Canadian Association of Neurosciences Review: Learning at a Snail's Pace
Authors: Parvez Kashif , Rosenegger David , Orr Michael , Martens Kara , Lukowiak Ken 
Published: December 2014

ABSTRACT:While learning and memory are related, they are distinct processes each with different forms of expression and underlying molecular mechanisms. An invertebrate model system,Lymnaea stagnalis, is used to study memory formation of a non-declarative memory. We have done so because: 1) We have discovered the neural circuit that mediates an interesting and tractable behaviour; 2) This behaviour can be operantly conditioned and intermediate-term and long-term memory can be demonstrated; and 3) It is possible to demonstrate that a single neuron in the model system is a necessary site of memory formation. This article reviews howLymnaeahas been used in the study of behavioural and molecular mechanisms underlying consolidation, reconsolidation, extinction and forgetting.
Intractable SUNCT Cured After Resection of a Pituitary Microadenoma
Authors: Leroux Elizabeth , Schwedt Todd J. , Black David F. , Dodick David W. 
Published: December 2014

Background:SUNCT is a rare primary headache disorder that is associated with activation of the posterior hypothalamus and often poorly responsive to medication. Recently, a relationship between between pituitary microadenoma and SUNCT has been suggested, and reports of both amelioration and exacerbation by dopamine-agonists have been published. These findings suggest a functional role for the hypothalamic-pituitary axis in SUNCT.Methods:We report the long-term 4 year follow-up of a 35 year-old patient with a 14-year history of medically and surgically intractable SUNCT who experienced immediate and complete resolution of symptoms after resection of a 6 mm pituitary microadenoma.Results:This patient was first seen at the age of 28 years with a 10-year history of attacks of right retro-orbital pain satisfying the IHS criteria for SUNCT. Many medical and surgical treatments were attempted without success. An MRI demonstrated a 6 mm microadenoma without compression of surrounding structures. A trial of bromocriptine caused marked exacerbation of his pain. The patient underwent a trans-sphenoidal resection of the pituitary lesion. SUNCT attacks worsened for the first 24h post-operatively, then disappeared. He has been completely headache-free, without medication, for the past 43 months with the last follow-up being January 2006.Conclusion:This case emphasizes the relationship between pituitary microadenomas and SUNCT, supports the role of the hypothalamic-pituitary axis in the genesis of SUNCT, and illustrates the importance of careful imaging of the pituitary region in patients with SUNCT.
Biological Markers in Alzheimer's Disease
Authors: Bailey Peter 
Published: December 2014

abstractBiomarkers are required to improve our diagnostic sensitivity and specificity and to monitor the biological activity of the Alzheimer’s disease (AD) in terms of the burden of neural involvement and the tempo of disease progression. Biomarkers will initially supplement our more traditional neuropsychological and imaging markers but may eventually evolve into useful surrogate endpoints in AD research. These markers may also provide important mechanistic clues to the pharmacological action of anti-dementia compounds. At this point, the combination of elevated cerebrospinal fluid phosphorylated TAU (CSF p-TAU) proteins and low CSF A²1-42are the only biomarkers with the sensitivity and specificity to serve as useful diagnostic biomarkers capable of distinguishing AD from other dementias in the early stages. Advances in non CSF tests is urgently required. Markers assessing the progression of disease do not necessarily require the same high disease specificity as diagnostic markers, but need to be sensitive to changes in disease state. At present, candidate markers fall under four main biological rationales: 1. Specific markers of AD neuropathology; 2. Non-specific markers of neural degeneration; 3. Markers of oxidative stress; 4. Markers of neural inflammation. It is foreseeable that a panel of such markers might prove advantageous. It will be important to develop “non-invasive “ markers utilizing readily obtainable tissue samples such as serum or urine to monitor disease progression (or hopefully regression). Repeated sampling would allow for comparison with traditional neuropsychological and imaging measures. The assays themselves will need to be reproducible, reliable and relatively inexpensive. Unfortunately, these biomarkers are in the formative stages of testing and results at present are inconclusive. To facilitate biomarker development in the future it would be highly advantageous to begin to collect and store biological specimens as an adjunct to current research in AD.
Clinical Equipoise and Treatment Decisions in Cervical Spondylotic Myelopathy
Authors: Benatar Michael 
Published: December 2014

Objective:The primary objective of this study is to evaluate clinician attitudes towards the treatment of cervical spondylotic myelopathy (CSM) in order to determine whether clinical equipoise exists for a segment of this patient population. The secondary objective is to examine the factors that influence treatment decisions.Methods:Cross-sectional internet-based survey of neurologists, neurosurgeons and orthopedic surgeons.Results:Between 40-60% of respondents recommended surgery for (1) patients with minimal or no symptoms, but incidentally discovered increased T2 signal within the cervical cord on MRI, (2) patients with mild symptoms and indentation of the cervical cord but without increased T2 signal and (3) those with at least moderately severe clinical findings accompanied by MRI showing effacement of the thecal sac but without indentation of the cord or increased T2 signal. The severity of the radiological abnormalities most strongly influence treatment decisions.Conclusion:We conclude that clinical equipoise does exist for certain groups of patients with CSM, suggesting that a randomized controlled trial could be performed in this population.
The G2019S LRRK2 Mutation is Rare in Korean Patients with Parkinson's Disease
Authors: Cho Jin-Whan , Kim Sung-Yeon , Park Sung-Sup , Kim Han-Jun , Ahn Tae-Beom , Kim Jong-Min , Jeon Beom-Seok 
Published: December 2014

Background:A number of causative mutations such as a-synuclein, parkin, UCHL1, Pink-1, DJ-1 have been identified in Parkinson's disease (PD). They are usually found in the familial cases. One mutation of great interest is the G2019S mutation in the LRRK2 gene, which has been reported in both familial and sporadic PD. Its prevalence has been reported to vary markedly among different races. We examined the prevalence of the G2019S mutation in the Korean PD population for genetic study planning.Methods:We conducted a genetic analysis of the G2019S mutation by standard PCR and restriction digestion method. 453 PD patients were studied, 34% of whom had an age at onset of <50 years and 3.8% had a positive family history.Results:None of the 453 study subjects carried the G2019S mutation.Conclusion:Our result confirms previous reports that the G2019S mutation is rare among PD patients in the Asian population. This result supports the notion that the prevalence of this LRRK2 mutation is population specific, and that there may be a founder effect within western populations.
Axial Signs and Magnetic Resonance Imaging Correlates in Parkinson's Disease
Authors: Acharya Hernish J. , Bouchard Thomas P. , Emery Derek J. , Camicioli Richard M. 
Published: December 2014

Background:Age-related brain changes may contribute to axial features in Parkinson's disease (PD).Objectives:To determine if ventricular volume and white matter high signal changes (WMC) are related to motor signs in PD and controls independent of age.Methods:Patients were rated with the Unified Parkinson's Disease Rating Scale (subscore A: tremor, rigidity, bradykinesia, and facial expression; subscore B: speech and axial impairment). Steps and time taken to walk 9.144 meters were measured. Total ventricular volume (TVV) and intracranial volume (ICV) were measured on T1-weighted MRI using manual tracing software. WMC were rated on axial T2-weighted, dual-echo or FLAIR MR images using a visual scale.Results:TVV (cm3) (PD: 36.48 ± 15.93; controls: 32.16 ± 14.20, p = 0.21) and WMC did not differ between groups (PD: 3.7 ± 4.2; controls: 3.2 ± 3.1, p = 0.55). Age correlated positively with ICV-corrected TVV and WMC in PD (cTVV: r = 0.48, p = 0.003; WMC: r=0.42, p=0.01) and controls (cTVV: r = 0.31, p = 0.04; WMC: r=0.44, p=0.003). Subscore B (r = 0.42, p = 0.01) but not subscore A (r = 0.25, p = 0.14) correlated with cTVV in PD. Steps and walking time correlated with cTVV and WMC in PD; cadence correlated with cTVV and steps with WMC in controls. Age-adjustment eliminated correlations.Conclusion:Subscore B, but not subscore A correlated positively with ventricular volume in PD, though this association was accounted for by age. Age-related brain change super-imposed on PD may contribute to axial features.
Tumor Effects on Cerebral White Matter as Characterized by Diffusion Tensor Tractography
Authors: Wei Corie W. , Guo Gang , Mikulis David J. 
Published: December 2014

Background:Diffusion tensor MRI fiber tractography (DTT) is the first non-invasive in vivo technique for delineating specific white matter (WM) tracts. In cerebral neoplasm, DTT can be used to illustrate the relationship of the tumor with respect to adjacent WM trajectories.Methods:Fiber tractography was used in this study to assess tumor-induced changes in WM trajectories in three cases of cerebral neoplasm: glioblastoma multiforme, meningioma, and anaplastic astrocytoma.Results:Three patterns of WM alteration were identified: 1) disruption, 2) displacement, and 3) infiltration. Tumor disruption of WM tracts was observed in glioblastoma multiforme, which terminated fibers crossing the corpus callosum. In meningioma, DTT illustrated bulk displacement of the corticospinal tract in the affected hemisphere as well as preservation of the deviated axons. In anaplastic astrocytoma, fiber tracking demonstrated disruption of WM tracts at the tumor origin as well as intact axons through areas of tumor infiltration.Conclusions:Fiber tracking results correlated with the clinical and histopathological features of the tumor. Larger case series will be required to determine if fiber tracking can add accuracy to existing imaging methods for grading tumors.
Recurrent Stroke/TIA in Cryptogenic Stroke Patients with Patent Foramen Ovale
Authors: Casaubon Leanne , McLaughlin Peter , Webb Gary , Yeo Erik , Merker Darren , Jaigobin Cheryl 
Published: December 2014

Background:Patent foramen ovale (PFO) is present in 40% of patients with cryptogenic stroke and may be associated with paradoxical emboli to the brain. Therapeutic options include antiplatelet agents, anticoagulation, percutaneous device and surgical closure. We assessed the hypothesis that there are differences in rates of recurrent TIA or stroke between patients in the four treatment groups.Methods:Patients presenting from January 1997 with cryptogenic stroke or TIA and PFO were followed prospectively until June 2003. Treatment choice was made on an individual case basis. The primary outcome was recurrent stroke. The secondary outcome was a composite of stroke, TIA, and vascular death.Results:Baseline. Our cohort consisted of 121 patients; 64 (53%) were men. Median age was 43 years. Sixty-nine percent presented with stroke and 31% with TIA. One or more vascular risk factor was present in 40%. Atrial septal aneurysm (ASA) was present in 24%. Treatment consisted of antiplatelet agents (34%), anticoagulation (17%), device (39%) and surgical closure (11%). Follow-up. Recurrent events occurred in 16 patients (9 antiplatelet, 3 anticoagulation, 4 device closure); 7 were strokes, 9 were TIA. Comparing individual treatments there was a trend toward more strokes in the antiplatelet arm (p=0.072); a significant difference was seen for the composite endpoint (p=0.012). Comparing closure versus combined medical therapy groups, a significant difference was seen for primary (p=0.014) and secondary (p=0.008) outcomes, favoring closure. Age and pre-study event predicted outcome.Conclusion:Patent foramen ovale closure was associated with fewer recurrent events. Complications of surgical and device closure were self-limited.