bluuebunny/crossref_metadata_2025 huggingface dataset:
An Unusual Presentation of Tarsal Tunnel Syndrome Caused by an Inflatable Ice Hockey Skate
Authors: Watson B.V. , Algahtani H. , Broome R.J. , Brown J.D.
Published: December 2014

Background:

Tarsal tunnel syndrome is a rare form of entrapment neuropathy. In athletes, it is usually the result of repetitive activity, local injury or a space-occupying lesion. Rarely, athletic footwear has been described as the primary cause of this syndrome.

Methods:

A 37-year-old male recreational hockey player was examined clinically and electrophysiologically because of spreading numbness in the toes of his left foot while playing hockey and wearing inflatable ice hockey skates designed to promote a better fit.

Results:

Clinical and electrophysiological studies revealed evidence of left medial and lateral plantar nerve involvement. Reduced amplitudes of mixed and motor plantar nerve responses with fibrillation potentials and positive sharp waves and no evidence of conduction block suggest that the primary pathology was axonal loss. Follow-up examination showed significant clinical and electrophysiological improvement after the patient stopped wearing his inflatable ice hockey skates.

Conclusion:

We report an unusual case of tarsal tunnel syndrome caused by an inflatable ice hockey skate. The patient improved clinically and electrophysiologically when he stopped wearing the boot.
Intracranial Malignant Glioma Presenting as Subarachnoid Hemorrhage
Authors: Hentschel Stephen , Toyota Brian
Published: December 2014

Objective:

Cerebral aneurysms are the predominant cause of spontaneous subarachnoid hemorrhage (SAH). However, if an aneurysmal cause has been excluded, there remains but a short list of other potential etiologies. Cerebral neoplasms are clearly on this list but are most commonly meningiomas or metastatic lesions. This article details a case of a neoplasm that presented exclusively with SAH.

Clinical Presentation:

A 40-year-old male presented with a SAH with normal cerebral angiography. The initial magnetic resonance image revealed a lesion in the right insula thought to be resolving hemorrhage. Subsequent images, however, revealed the mass to be enlarging.

Intervention:

Craniotomy and resection of the lesion established a diagnosis of a malignant oligodendroglioma.

Conclusion:

An affirmation is made that patients experiencing ’angiographically-negative’ SAH should undergo MRI, occasionally on a serial basis, to exclude other etiologies for hemorrhage, including neoplasia.
CANADIAN ASSOCIATION OF NEUROSCIENCE REVIEW: Development and Plasticity of the Auditory Cortex
Authors: Yan Jun
Published: December 2014

The functions of the cerebral cortex are predominantly established during the critical period of development. One obvious developmental feature is its division into different functional areas that systematically represent different environmental information. This is the result of interactions between intrinsic (genetic) factors and extrinsic (environmental) factors. Following this critical period, the cerebral cortex attains its adult form but it will continue to adapt to environmental changes. Thus, the cerebral cortex is constantly adapting to the environment (plasticity) from its embryonic stages to the last minute of life. This review details important factors that contribute to the development and plasticity of the auditory cortex. The instructive role of thalamocortical innervation, the regulatory role of cholinergic projection of the basal forebrain and the potential role of the corticofugal modulation are presented.
Cannabis Use as Described by People with Multiple Sclerosis
Authors: Page S.A. , Verhoef M.J. , Stebbins R.A. , Metz L.M. , Levy J.C.
Published: December 2014

Background:

Multiple sclerosis (MS) is one of the most common neurological diseases affecting young adults. The prevalence of MS in Alberta has been described as among the highest reported in the world, estimated at 217 per 100,000. Numerous anecdotal reports, and a few small empirical investigations have suggested that cannabis use may relieve the symptom experience of those with MS. The present study was undertaken to describe cannabis use by this patient group. Information on peoples’beliefs, practices and experiences related to use were investigated.

Methods:

Aquestionnaire was mailed to a sample of 780 adults with MS in southern Alberta, Canada.

Results:

Completed questionnaires were returned by 420/673 eligible subjects (response rate 62%). Mean sample age was 48 years and 75% were women. Respondents ranged from mildly to severely impaired. The majority of respondents (96%) was aware cannabis was potentially therapeutically useful for MS and most (72%) supported legalization for medicinal purposes. Forty-three percent had tried cannabis at some point in their lives, 16% for medicinal purposes. Symptoms reported to be ameliorated included anxiety/depression, spasticity and chronic pain. Reasons given for not trying cannabis were the fact that it is an illegal substance, concern about side effects and lack of knowledge on how to obtain it.

Conclusions:

Subjective improvements in symptom experience were reported by the majority of people with MS who currently use cannabis. Further evaluation of this substance is warranted.
Multiple Brain Abscesses Caused by Fusobacterium nucleatum Treated Conservatively
Authors: Heckmann Josef G. , Lang Christoph J.G. , Hartl Heinz , Tomandl Bernd
Published: December 2014

Background:

Multiple brain abscesses are serious neurological problems with high mortality and disabling morbidity. The frequency is rising as a result of AIDS and the increasing number of immunocompromised patients.

Case study:

A 59-year-old woman developed signs and symptoms of diffuse brain dysfunction including fever and neck stiffness. A brain CT scan demonstrated nine contrast-enhancing ring-shaped lesions. Analysis of the cerebrospinal fluid using PCR-technique revealed DNA of Fusobacterium nucleatum. Conservative treatment with antibiotics was successful. The patient recovered with only mild cognitive deficits.

Results:

The experience of our patient and the review of the literature indicate that multiple brain abscesses due to Fusobacterium nucleatum are rare. The most probable source is oral infection.

Conclusion:

Multiple brain abscesses may be caused by Fusobacterium nucleatum. Cerebrospinal fluid analysis using PCR technique is helpful with diagnosis. Conservative management can be successful.
Paraneoplastic Sensory Neuronopathy and Spontaneous Regression of Small Cell Lung Cancer
Authors: Gill Sharlene , Murray Nevin , Dalmau Josep , Thiessen Brian
Published: December 2014

Background:

Spontaneous tumour regression in small cell lung cancer has previously been suggested in patients with paraneoplastic neurologic syndromes. Rare documentation of this event has occurred in the literature.

Case Report:

The authors report a patient with anti-Hu associated paraneoplastic sensory neuronopathy who had a spontaneous regression of her small cell lung cancer.

Conclusions:

This case supports the hypothesis that anti-Hu neurologic syndromes are the consequence of a misdirected immune response to small cell tumours.
The Burden of Seizures in Manitoba Children: A Population-Based Study
Authors: Kozyrskyj Anita L. , Prasad Asuri N.
Published: December 2014

Background:

Population-based studies are necessary to better understand the risk factors for developing seizure disorders and the impact of these conditions on children. We undertook an assessment of the prevalence of seizure disorders in a population of children on the basis of health care utilization records.

Methods:

Using Manitoba’s population-based prescription and health care data for 1998/99, the prevalence of children with seizure disorders, on the basis of at least one physician visit or hospitalization for epilepsy or a prescription for an antiepileptic drug, was determined by age, urban/rural region and socioeconomic status. The latter was measured as neighbourhoods stratified by income quintiles according to Census data.

Results:

Age-specific prevalence rates for seizure disorders in Manitoba children, determined from health care administrative records, were similar to published data on the prevalence of epilepsy, with one exception. Prevalence rates in adolescents were higher than those reported in the literature. No statistically significant differences in prevalence rates were observed between urban and rural populations. However, a higher prevalence was found among children of all ages living in lower socioeconomic neighbourhoods in urban areas, which presented as a gradient of increased prevalence with decreased levels of income.

Conclusion:

Population-based health care administrative data can be used to describe the geographical distribution of seizure disorders. Our data suggest that the burden of seizure disorders is not evenly distributed among children.
The Endovascular Management of Superior Cerebellar Artery Aneurysms
Authors: Haw Charles , Willinsky Robert , Agid Ronit , TerBrugge Karel
Published: December 2014

Background:

Superior cerebellar artery aneurysms are rare. We present a clinical series of twelve of these aneurysms that were treated exclusively with endovascular coils.

Method:

A retrospective analysis of a prospectively collected database of cerebral aneurysms treated with coil embolization was performed. Clinical notes and radiological images were reviewed.

Results:

Twelve superior cerebellar artery aneurysms were treated in eleven patients between 1992 and 2001. Seven patients presented with subarachnoid hemorrhage, two with neurologic deficit, and two had asymptomatic aneurysms. Coiling resulted in complete aneurysm obliteration in six patients and incomplete obliteration in the other six. No subsequent hemorrhage occurred with follow-up between 6 and 119 months (mean follow-up 50 months). Procedural morbidity was one superior cerebellar artery infarct with good recovery. Management morbidity was one middle cerebral artery embolus during a follow-up angiogram that required thrombolysis with a good clinical result. Nine out of 11 patients on follow-up were performing at Glasgow Outcome Scale (GOS) 5. One patient with GOS 3 presented with a poor grade subarachnoid hemorrhage and the other patient with GOS 4 presented with a parenchymal hemorrhage due to an arteriovenous malformation.

Conclusion:

Endovascular treatment of superior cerebellar artery aneurysms is an effective treatment strategy with low morbidity.
Do General and Multiple Sclerosis-Specific Quality of Life Instruments Differ?
Authors: Moore Fraser , Wolfson Christina , Alexandrov Lubo , Lapierre Yves
Published: December 2014

Background:

Quality of life instruments provide information that traditional outcome measures used in studies of multiple sclerosis do not. It is unclear if longer, disease-specific instruments provide more useful information than shorter, more general instruments, or whether patients prefer one type to another.

Methods:

We conducted a cross-sectional study of quality of life in a multiple sclerosis clinic population using a mailed questionnaire that combined three different quality of life instruments; the SF-36, the Multiple Sclerosis Quality of Life Instrument-54, and the EuroQol EQ-5D. We assessed the feasability of using each instrument and patient preference for each, calculated correlation coefficients for the summary scores of each instrument and other measures of disease severity, and calculated odds ratios from proportional odds models comparing each instrument with the Expanded Disability Status Scale.

Results:

We did not find substantial differences between the three instruments. All were well-received by patients, and over 75% felt that the combination of the three instruments best assessed their quality of life. For each instrument there was substantial variability between patients with similar quality of life scores in terms of their disability (as assessed by the Expanded Disability Status Scale and their own perception of their disease severity and quality of life (on simple 1-10 scales).

Conclusion:

Quality of life instruments are easy to use and well-received by patients, regardless of their length. There do not appear to be clinically important differences between general and disease-specific instruments. Each instrument appears to measure something other than a patient’s disability or perception of their own disease severity or quality of life.
Wernicke Encephalopathy in a Patient with T-Cell Leukemia and Severe Malnutrition
Authors: Lacasse Lynn , Lum Cheemun
Published: December 2014

The patient was a 20-year-old male with an eight-month history of T-cell acute lymphoblastic leukemia (ALL). He presented to the emergency room with confusion and decreased level of consciousness that had worsened over the prior two weeks. He complained of generalized fatigue, weakness, intermittent nausea and vomiting and depression. His diet had consisted of only soda pop over the six weeks preceding admission.

The patient was disoriented and had a depressed level of consciousness. He appeared pale, cachectic, and had poor hygiene. Examination of the central nervous system showed lateral nystagmus and slowed motor activity in the both upper and lower extremities. Reflexes were bilaterally 2+ except at the ankles where they were 1+. Plantar responses were bilaterally downgoing.
Can rt-PA be Administered to the Wrong Patient? Two Patients with Somatoform Disorder
Authors: Mouradian Mikael S. , Rodgers Jennifer , Kashmere Jodi , Jickling Glen , McCombe Jennifer , Emery Derek J , Demchuk Andrew M. , Shuaib Ashfaq
Published: December 2014

Background:

Intravenous rt-PA (IV rt-PA) for acute stroke has raised many concerns, including its inadvertent use in patients presenting with acute stroke-like symptoms as the expression of their somatoform disorder. Diagnosis of the somatoform disorder is often delayed, and thrombolytics in these patients for their stroke-like presentation subjects them to risk for hemorrhage.

Methods:

The presentation, neurological findings, and the therapeutic decision making was audited in 85 patients who received IV rt-PA for a diagnosis of acute stroke. All the surviving patients were re-examined neurologically at least three months after IV rt-PA. Baseline and follow-up brain CT scans were re-reviewed by a neuroradiologist who was blinded to clinical presentation and outcome. Patients whose clinical presentation, brain CT and neurological outcome did not fit into known or expected anatomical and clinical patterns of stroke underwent psychological assessment using the Minnesota Multiphasic Personality Inventory-2.

Results:

In two patients three stroke-like presentations of somatoform disorder inadvertently were treated with IV rt-PA. This was primarily caused by abbreviated neurological examination and narrow differential diagnosis.

Interpretation:

Patients with somatoform disorder may present with symptoms mimicking acute stroke. Under the time constraints of IV rt-PA use, a diagnosis of somatoform disorder can be missed, subjecting such patients to the potential complications of thrombolytics.
Lacunar Stroke Associated with Methylphenidate Abuse
Authors: Sadeghian Hamid
Published: December 2014

Background:

Methylphenidate is a central nervous system stimulant used for the treatment of attention deficit hyperactivity disorder and narcolepsy and like other psychostimulants has a potential for abuse.

Case study:

A young man with a cerebral lacunar infarction following chronic oral abuse of methylphenidate is presented.

Conclusion:

The experience of our patient and a review of the literature suggest that cerebral infarction is a potential side effect of chronic consumption of methylphenidate.
Autosomal Dominant Spinocerebellar Ataxias: An Asian Perspective
Authors: Tan E.K.
Published: December 2014

Autosomal dominant cerebellar ataxias, frequently referred to as spinocerebellar ataxias (SCAs) have been under intense scientific research limelight since expansions of coded CAG trinucleotide repeats were demonstrated to cause several dominantly inherited SCAs. The number of new SCA loci has expanded dramatically in recent years. At least ten genes have been identified for SCAs 1, 2, 3, 6, 7, 8, 10, 12, 17, dentatorubral-pallidoluysian atrophy (DRPLA), and six loci responsible for SCAs 4, 5, 11,13, 14, and 16 have been mapped. Genetic testing is essential for diagnosis due to the overlapping and varied phenotypic features of the different SCAs. While there is no effective treatment available, genetic counseling is important for addressing the many ethical, social, legal, and psychological issues facing SCA patients. Researchers have recently provided valuable information on the pathogenesis of the disease and hopefully a cure will be available in the near future.
Incomplete Assessment of Experimental Cytoprotectants in Rodent Ischemia Studies
Authors: DeBow Suzanne B. , Clark Darren L. , MacLellan Crystal L. , Colbourne Frederick
Published: December 2014

Background:

Inadequate preclinical testing (e.g., rodent studies) has been partly blamed for the failure of many cytoprotectants to effectively treat stroke in humans. For example, some drugs went to clinical trial without rigorous functional and histological assessment over long survival times. In this study, we characterized recent experimental practices in rodent cytoprotection experiments to determine whether the limitations of early studies have been rectified.

Methods:

We identified 138 rodent cytoprotection studies published in several leading journals (Journal of Neuroscience, Stroke, Journal of Cerebral Blood Flow and Metabolism and Experimental Neurology) for 2000 - 2002 and compared these to those published in 1990. From each study we determined the ischemia model, age and sex of the animal, the histological and functional endpoints used, and the methodology used to assess intra- and postischemic temperature.

Results:

Ninety-eight percent of recent studies used young adult rodents and most used males. Most studies (60%) did not assess functional outcome and survival times were often ≤ 48 hr (66%) for focal ischemia and ≤ 7 days (80%) for global ischemia. Over 60% of the experiments relied solely upon rectal temperature during ischemia and only 32.6% of ischemia studies measured temperature after surgery. The 1990 data were similar.

Conclusion:

Many investigators ignore the need to assess long-term functional and histological outcome and do not accurately represent clinical conditions of ischemia (e.g., use of aged animals). In addition, intra- and postischemic temperature measurement and control is frequently neglected or inadequately performed. Further clinical failures are likely.
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.
Etiology of Parkinson's Disease
Authors: Huang Zhigao , Fuente-Fernández Raúl de la , Stoessl A. Jon
Published: December 2014

There is growing recognition that Parkinson's disease (PD) is likely to arise from the combined effects of genetic predisposition as well as largely unidentified environmental factors. The relative contribution of each varies from one individual to another. Even in situations where more than one family member is affected, the predominant influence may be environmental. Although responsible for only a small minority of cases of PD, recently identified genetic mutations have provided tremendous insights into the basis for neurodegeneration and have led to growing recognition of the importance of abnormal protein handling in Parkinson's as well as other neurodegenerative disorders. Abnormal protein handling may increase susceptibility to oxidative stress; conversely, numerous other factors, including oxidative stress and impaired mitochondrial function can lead to impaired protein degradation. A limited number of environmental factors are known to be toxic to the substantia nigra; in contrast, some factors such as caffeine intake and cigarette smoking may protect against the development of PD, although the mechanisms are not established. We review the various genetic and environmental factors thought to be involved in PD, as well as the mechanisms that contribute to selective nigral cell death.
New Perspectives on Dystonia
Authors: Langlois Mélanie , Richer Francois , Chouinard Sylvain
Published: December 2014

Dystonia is a syndrome of sustained muscular contractions with numerous underlying etiologies. This review examines the varied phenomenology of dystonias, its evolving classification including recent genetic data as well as its clinical investigation and treatment. Although age of onset, anatomical distribution and family history are key elements of the investigation of dystonia, classification increasingly relies on etiologic and genetic criteria. Physiological abnormalities in striatocortical circuits are common in dystonia but the pathophysiology is still unclear. In recent years, a great deal has been learned on the more common primary dystonias such as primary torsion dystonia and on dystonia-plus syndromes such as dopamine responsive dystonia. Treatment of dystonia has also evolved and there are now a number of therapeutic agents with clear beneficial effects including anticholinergics, benzodiazepines, and botulinum toxin and there is growing interest in neurofunctional surgery including deep brain stimulation.
Tremor - Easily Seen but Difficult to Describe and Treat
Authors: Grimes D.A.
Published: December 2014

Tremor is a common movement disorder yet many physicians struggle with its terminology as well as with its treatment. Attempts have been made to develop standard terminology and criteria for tremors but this process continues to evolve. In this review, a summary of the currently-proposed phenomenology and syndromic classification of all types of tremor is presented. The diagnosis and management of essential tremor is presented in more detail, as it is the most commonly encountered tremor.
Neuropsychology for Movement Disorders Neurosurgery
Authors: Saint-Cyr Jean A.
Published: December 2014

The neuropsychologist plays a crucial role in three phases of the neurosurgical treatment of movement disorder patients, namely screening, outcome evaluation and research. In screening patients, the differential diagnosis of dementia, impact of depression or other psychiatric conditions, and the influence of disease and medication-induced symptoms on cognitive performance must be determined. Postoperatively, systematic evaluations elucidate the cognitive costs or benefits of the procedure. The neuropsychologist is then able to provide feedback and counselling to the professional staff, patient and family to inform management strategies. Neuropsychologists also study alteration of cognitive processing due to lesions or stimulation, which, in tandem with functional imaging, shed light on plasticity in cortical and subcortical processing.
Revised 2004 International Classification of Headache Disorders: New Headache Types
Authors: Gladstone Jonathan P. , Dodick David W.
Published: December 2014

In 1988, the International Headache Society created a classification system that has become the standard for headache diagnosis and research. The International Classification of Headache Disorders galvanized the headache community and stimulated nosologic, epidemiologic, pathophysiologic, and genetic research. It also facilitated multinational clinical drug trials that have led to the basis of current treatment guidelines. While there have been criticisms, the classification received widespread support by headache societies around the globe. Fifteen years later, the International Headache Society released the revised and expanded International Classification of Headache Disorders second edition. The unprecedented and rapid advances in the field of headache led to the inclusion of many new primary and secondary headache disorders in the revised classification. Using illustrative cases, this review highlights 10 important new headache types that have been added to the second edition. It is important for neurologists to familiarize themselves with the diagnostic criteria for the frequently encountered primary headache disorders and to be able to access the classification (www.i-h-s.org) for the less commonly encountered or diagnostically challenging presentations of headache and facial pain.
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.
Charcot et Babinski: au-delà de la simple relation professeur-élève
Authors: Massie Rami
Published: December 2014

Charcot and Babinski: beyond a simple teacher-student relationship. Jean-Martin Charcot (18251893) is now considered to be the father of clinical neurology in France. He trained a generation of eminent neurologists, among them Joseph Babinski, with whom he had a special relationship. Babinski was undoubtedly Charcot's favorite pupil and they enjoyed an excellent collaboration at la Salpétrière. Even though both men felt tremendous respect for each other, it is sad that this relationship may, in one instance, have been detrimental to Babinski. This is probably the reason why Bouchard denied him full professorship, a decision with eventual consequences for both men. In spite of this, the neurologist of Polish origin held his master in tremendous admiration, even as he pursued Charcot's research on hysteria after his death. Even though Babinski eventually contradicted his master on many fundamental issues, it did not affect his devotion to him. The relationship between the two men can be considered as more than a simple relationship between a teacher and his pupil and may be compared to a father-son relationship, which is a reminder of the original model of Hippocratic teaching.
Progress in Clinical Neurosciences: Migraine-Stroke: A Causal Relationship, but Which Direction?
Authors: Kern Ralph Z.
Published: December 2014

A significant association between migraine and ischemic stroke has been demonstrated in population and case-control studies. The risk of ischemic stroke appears to be higher in migraine with aura (MWA) than migraine without aura (MwoA). Migraine-stroke comprises a number of distinct entities, including migrainous infarction, in which ischemic stroke occurs during an attack of MWA and migraine-related stroke, in which the causal link is less clear. Migrainous infarction accounts for only one-third of migraine-stroke, strokes may occur during attacks of MwoA, and a number of cerebrovascular disorders may present as MWA or MwoA. Migraine may occur as a consequence of conditions that are known to cause stroke; therefore it remains to be determined whether migraine predisposes to stroke in the absence of any known disease associations, if it is an epiphenomenon of an underlying stroke diathesis, or if it requires the presence of another stroke risk factor to produce cerebral ischemia. Furthermore, it is unclear if ischemia results in migraine more often than migraine results in ischemia. Careful clinical studies that evaluate this bidirectional relationship are needed to determine why migraine patients are subject to a higher risk of ischemic stroke.
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

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

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.
Herpes Simplex Encephalitis
Authors: Taylor Sean W. , Smith Roger M. , Pari Giovanna , Wobeser Wendy , Rossiter John P. , Jackson Alan C.
Published: December 2014

A 28-year-old woman presented with a one day history of high fever and partial seizures with secondary generalization. This was preceded by a three week history of headache, ataxia, and fatigue. An initial computed tomogram head scan showed a low density mass lesion in the right frontal operculum without enhancement. On the next day, a repeat scan showed a new frontopolar, expansile, low density cortical lesion (Figure 1A) suggestive of encephalitis. Cerebrospinal fluid showed a pleocytosis of 311 mononuclear white blood cell count per μL and an elevated protein of 1.57 g/L. She received intravenous acyclovir and antibiotics. She remained febrile and became mute. A magnetic resonance (MR) scan under general anesthesia on her fourth hospital day showed frontal and perisylvian lesions with restricted diffusion (Figure 1B - D and Figure 2). A right frontal brain biopsy showed meningoencephalitis and immunohistochemical staining was positive for herpes simplex virus (HSV) antigen (Figure 3). Subsequently, HSV-1 DNA was demonstrated in both cerebrospinal fluid and brain tissue with polymerase chain amplification. She improved after a course of intravenous therapy with acyclovir with residual frontal lobe signs, including marked executive dysfunction, and her speech became normal.
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

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

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

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.