bluuebunny/crossref_metadata_2025 huggingface dataset:
The Failure of Punishment as Social Control
Authors: L. Abel Richard
Published: February 2016

I must begin with several disclaimers, which express not the obligatory protestations of false modesty but the real limitations of this article. First, I am not a criminologist, penologist or philosopher of law but a lawyer and sociologist of law. Second, I inevitably reflect the unique social environment within the United States, which may be very different from that of the other countries represented at this conference. Third, my remarks are impressionistic and synthetic, not the conclusions of a careful empirical study.

My argument is simple. In the contemporary United States, stateimposed sanctions are seen as a central mechanism of social control. (Of course, they also are seen as incapacitation, retribution, and rehabilitation; but most people would view these as secondary justifications.) Indeed, I believe most Americans would view state punishment as the most important mechanism of social control. By state-imposed sanctions I mean both criminal penalties (execution, imprisonment, fine, probation, and community service) and civil damages (compensatory and punitive).
“What Must Be the Answer of the United States to Such a Proposition?” Anarchist Exclusion and National Security in the United States, 1887–1903
Authors: NOONAN ALEXANDER
Published: February 2016

This article examines the debates around anarchist restriction that shaped the eventual passage of the Immigration Act of 1903 and argues that domestically oriented conceptions of national security are both challenged and constituted by transnational and international processes and currents. While discussions of transnational immigration control became important features of both scholarly discourse and popular debate in the wake of the terrorist attacks of 2001, these discussions were not new. Similar debates about immigration policy, security, and civil liberties shaped discussions between the mid-1880s and early 1900s, when an unprecedented wave of attacks against heads of state fed rumors of wide-ranging conspiracies, and reports of anarchist outrages in cities far and wide spread fear. Anarchist exclusion was far more than an example of a rising nativist tide raising all boats and excluding a widening spectrum of undesirable aliens. Such measures set the foundation for restriction based on political beliefs and associations that, over subsequent decades, would become critical to suppressing political dissent. Consequently, understanding how the fear of anarchist violence helped shape the contours of the domestic and diplomatic debates over anarchist restriction is critical as these old questions of transnational immigration control reemerge.
Quality of Life Measurements in Epilepsy
Authors: Chadwick David
Published: February 2016

In the treatment of the patient with epilepsy not only seizure control but quality of life issues are important aspects of management. Aspects that have an impact on the quality of life include seizure frequency and severity, social functioning, psychological well-being, disease specific measures, and general health status.
Regional HmPAO SPECT and CT Measurements in the Diagnosis of Alzheimer's Disease
Authors: Mattman A. , Feldman H. , Forste B. , Li D. , Szasz I. , Beattie B.L. , Schulzer M.
Published: February 2016

Background:

This study investigated the hypothesis that the combination of regional CT brain atrophy measurements and semiquantitative SPECT regional blood flow ratios could produce a diagnostic test for Alzheimer's disease (AD) with an accuracy comparable to that achieved with the present clinical gold standard of the NINCDS-ADRDA criteria.

Methods:

Single proton emission computed tomography (SPECT) and CT head scans were performed on 122 subjects referred an UBC Alzheimer clinic and diagnosed as either ‘not demented’ (ND-37) or ‘possible/probable AD’ (AD-85) by the NINCDS-ADRDA criteria. Stepwise discriminant analysis (SDA) was performed on the bilateral SPECT regions of interest and compared to bilateral CT qualitative/quantitative assessment in the frontal, parietal and temporal lobes to determine which were most accurate at ND/AD distinction. Receiver operating curves (ROC) were then constructed for these variables individually and for their combined discriminant function.

Results:

The left temporal qualitative cortical atrophy score (CT) and left temporal perfusion ratio (SPECT) were selected in the SDA. The combined discriminant function was more specific at AD/ND distinction than either of CT or SPECT alone. The accuracy of AD/ND distinction with the combined discriminant function was below that achieved by clinical diagnosis according to the NINCDS-ADRDA criteria and was not significantly different from that achieved with SPECT or CT alone as defined by ROC curve analysis.

Conclusion:

The measurements of left temporal cortical atrophy and regional cerebral blood flow were most indicative of AD; however they lacked the sensitivity and specificity to recommend their use as a diagnostic test for AD.
Marked Hyperprolactinemia Caused by Carotid Aneurysm
Authors: Kahn Susan R. , Leblanc Richard , Sadiko Abbas F. , Fantus I. George
Published: February 2016

Background:

Pituitary dysfunction caused by intracranial aneurysms is rare. We report a patient with the unique feature of hyperprolactinemia to a degree previously seen only with prolactinsecreting tumours.

Method:

Case report.

Result:

A 42-year-old woman had a galactorrhea, left-sided headache, reduced vision in the left eye and a left temporal hemianopsia. Serum prolactin was elevated (365 μg/L). Cranial computed tomography (CT) revealed a suprasellar mass, which carotid angiography showed to be a left internal carotid artery aneurysm. At craniotomy, this aneurysm and a smaller one of the ophthalmic artery were repaired, and the patient's vision returned to normal. The prolactin level fell to normal. Follow-up CT showed no evidence of pituitary adenoma or hypothalamic lesion.

Conclusions:

Carotid aneurysm can cause reversible pituitary dysfunction. A prolactin level >300 μg/L is not a reliable cut-off for distinguishing prolactin-secreting adenomas from other causes of elevated prolactin. A co-existing prolactinoma was felt to be ruled out by both a normal CT scan and normal prolactin levels following aneurysm repair. Patients with marked hyperprolactinemia should be considered for angiography or MRI to rule out carotid aneurysm, since the consequences of pituitary exploration in this setting are potentially grave.
Syringomyelia Developing as an Acute Complication of Tuberculous Meningitis
Authors: Daif Abdu Kader , Rajeh Al , Ogunniy Adesola , Boukai Amer Al , Tahan Abdulrahman Al
Published: February 2016

Background:

Tuberculosis of the nervous system has protean manifestations. Syringomyelia, though an uncommon complication of it, is usually of late onset.

Methods:

We report two patients with tuberculosis meningitis who developed syringomyelia acutely. The diagnosis was supported by neuroimaging and findings at laminectomy.

Results:

The two patients developed syringomyelia between 11 days and 6 weeks of the onset of tuberculous meningitis. They both had cord swelling and softening

Conclusions:

Acute-onset syringomyelia should be suspected in any patient being treated for tuberculosis meningitis who subsequently develops limb weakness and/or sphincteric dysfunction. Inflammatory edema and cord ischemia appeared to be the underlying mechanisms in these early onset cases rather than arachnoiditis which is important in late-onset cases.
Lhermitte-Duclos Disease: Literature Review and Novel Treatment Strategy
Authors: Tuli Sagun , Provias John P. , Bernstein Mark
Published: February 2016

Background:

Lhermitte-Duclos disease (LDD) is a rare pathologic entity involving the cerebellum. The fundamental nature of the entity and its pathogenesis remain unknown, and considerable debate has centered on whether it represents a neoplastic, malformative or hamartomatous lesion. The cell or cells of origin remain incompletely defined. Previous reports of cases in the English literature have dealt predominantly with the clinical and pathological aspects yet few address issues of treatment.

Methods:

A case of Lhermitte-Duclos disease (LDD) in a 54-year-old female leading to local compressive symptoms and obstructive hydrocephalus is presented. A craniectomy, in addition to a CI laminectomy followed by a decompressive duroplasty (using autologous fascia lata graft) was performed.

Results:

The patient clinically improved and follow-up MRI 11 months post-operatively revealed improvement in hydrocephalus.

Conclusions:

The histological and immunohistochemical features of the lesion are described, emphasizing the role of an abnormal dysplastic granule cell layer. The evidence in favor of each of the major theories of pathogenesis, malformative and neoplastic is discussed. Based on these facts a form of surgical intervention involving decompressive duroplasty is proposed.
Magnetic Resonance Spectroscopy Guided Brain Tumor Resection: Differentiation Between Recurrent Glioma and Radiation Change in Two Diagnostically Difficult Cases
Authors: Preul Mark C. , Leblanc Richard , Caramanos Zografos , Kasrai Reza , Narayanan Sridar , Arnold Douglas L.
Published: February 2016

Background:

It is often difficult to differentiate a recurrent glioma from the effects of post-operative radiotherapy by means of conventional neurodiagnostic imaging. Proton magnetic resonance spectroscopic imaging (1H-MRSI), that allowsin vivomeasurements of the concentration of brain metabolites such as choline-containing phospholipids (Cho), may providein vivobiochemical information helpful in distinguishing areas of tumor recurrence from areas of radiation effect.

Patients and Methods:

Two patients who had undergone resection and post-operative radiotherapy for a cerebral glioma became newly symptomatic. Computed tomographic (CT) and magnetic resonance imaging (MRI) performed after the intravenous infusion of contrast material, and in one case, [18F] fluorodeoxyglucose positron emission tomography (PET), could not differentiate between the possibilities of recurrent glioma and radiation effect. The patients underwent1H-MRSI prior to reoperation and the1H-MRSI results were compared to histological findings originating from the same locations.

Results:

A high Cho signal measured by1H-MRSI was seen in areas of histologicallyproven dense tumor recurrence, while low Cho signal was present where radiation changes predominated.

Conclusions:

The differentiation between the recurrence of a cerebral glioma and the effects of post-operative irradiation was achieved using1H-MRSI in these two patients whose conventional neurodiagnostic imaging was equivocal for such a distinction. Where these two conditions are present, metabolite images from1H-MRSI, such as that based on Cho, can be co-registered with other imaging modalities such as MRI and may also be integrated with functional MRI or functional PET within a multimodal imaging-guided surgical navigation system to assure maximal resection of recurrent tumor while minimizing the risk of added neurological damage.
Severe Tension Pneumocephalus Complicating Frontal Sinus Osteoma
Authors: Marras L.C. , Kalaparambath T.P. , Black S.E. , Rowed D.W.
Published: February 2016

Background:

Tension pneumocephalus, the accumulation of intracranial gas under pressure, is a rare but potentially life-threatening condition which can complicate craniofacial surgery, trauma, or cranial tumor. It presents as an acute or subacute expanding mass lesion.

Case Report:

We present a case of a 40-year-old male who developed tension pneumocephalus as a consequence of a previously detected but untreated frontal sinus osteoma. Despite prompt decompression and repair of the fistulous connection between the sinus and the intracranial compartment, the patient suffered permanent frontal lobe damage with significant neurocognitive sequelae and seizures.

Conclusions:

This case illustrates that tension pneumocephalus can be a dangerous entity with potential for early mortality and long-term morbidity. We recommend, therefore, early treatment and close follow up of destructive lesions involving the posterior frontal sinus wall.
Outcome Evaluation of Gabapentin as Add-on Therapy for Partial Seizures
Authors:   on behalf of the "NEON" Study Investigators Group
Published: February 2016

Objective:

The safety, tolerability, efficacy, and impact on quality of life of gabapentin (Neurontin®) as adjunctive therapy to carbamazepine (CBZ) and/or phenytoin (PHT) was assessed in epileptic patients with partial seizures.

Methods:

NEON (Neurontin Evaluation of Outcomes in Neurological Practice) was an open-label, prospective, multicentre study conducted in patients on a stable dose of CBZ and/or PHT and experiencing an average of up to 4 complex partial seizures with or without secondary generalization per month, with no seizure-free months. The treatment lasted 20 weeks. Gabapentin was started at 400 mg/day and was individually titrated to effective tolerable dose up to 2400 mg/day. Quality of life was evaluated using the QOLIE-10 questionnaire.

Results:

A total of 141 patients were enrolled at 36 sites; 114 patients were evaluable for efficacy analyses. The mean maintenance dose of gabapentin was 1600 mg/day (range = 300-3200). A decrease of 50% or more in frequency of complex partial + secondarily generalized seizures was observed in 81 (71 %) patients (p = 0.0001). Fifty two (46%) patients were seizure-free during the last 8 weeks of treatment. A significant improvement (p < 0.05) was observed in 5 of the 10 questions of the QOLIE-10, as well as in the composite QOL score (p = 0.0002). The most frequent adverse events included somnolence (16%), dizziness (9%), and asthenia (6%). Twenty-five (18%) patients prematurely discontinued the study, 16 (11%) of them due to adverse events.

Conclusion:

This study indicates that treatment with gabapentin as adjunctive therapy to standard antiepileptic drugs in this group of patients not only provides significant improvement in seizure control, but also has a positive impact on quality of life. The clinical benefits in efficacy, safety and tolerability demonstrated at 20 weeks are sustained, and no tolerance develops with gabapentin in longer term use.
Respiratory Muscle Training in Patients With Moderate to Severe Myasthenia Gravis
Authors: Weiner Paltiel , Gross Ditza , Meiner Zeev , Ganem Rushrash , Weiner Margalit , Zamir Down , Rabner Marinella
Published: February 2016

Background:

Myasthenia gravis (MG) is a specific autoimmune disease characterized by weakness and fatigue. MG may affect also the respiratory muscles causing symptoms that may vary from dyspnea on severe exertion to dyspnea at rest. This study was undertaken in order to determine the effects of respiratory muscle training on respiratory muscle performance, spirometry data and the grade of dyspnea in patients with moderate to severe generalized MG.

Methods:

Eighteen patients with MG were studied and divided into 2 groups: Group A included 10 patients (3 males and 7 females aged 29-68) with moderate MG, and Group B that included 8 patients (5 males and 3 females aged 21-74) with severe MG. Patients in Group A received both inspiratory and expiratory muscle training for 1/2 h/day, 6 times a week, for 3 months, while patients in Group B followed the same protocol but had inspiratory muscle training only.

Results:

Mean PImax increased significantly from 56.6 ± 3.9 to 87.0 ± 5.8 cm H20 (p < 0.001) in Group A, and from 28.9 ± 5.9 to 45.5 ± 6.7 cm H20 (p < 0.005) in Group B. The mean PEmax also increased significantly in patients in Group A, but remained unchanged in the patients in Group B. The respiratory muscle endurance also increased significantly, from 47.9 ± 4.0 to 72.0 ± 4.2%, p < 0.001, in patients of Group A, and from 26.0 ± 2.9 to 43.4 ± 3.8, p < 0.001, in patients in Group B. The improved respiratory muscle performance was associated with a significant increase in the FEV, values, and in the FVC values, in patients of both groups. Mean dyspnea index score also increased significantly from 2.6 ± 0.8 to 3.6 ± 0.4 (p < 0.005) in Group A, and from 0.7 ± 0.2 to 2.0 ± 0.2 (p < 0.001) in Group B.

Conclusions:

Specific inspiratory threshold loading training alone, or combined with specific expiratory training, markedly improved respiratory muscle strength and endurance in patients with MG. This improvement in respiratory muscle performance was associated with improved lung function and decreased dyspnea. Respiratory muscle training may prove useful as a complementary therapy with the aim of reducing dyspnea symptoms, delay the breathing crisis and the need for mechanical ventilation in patients with MG.
Cervical Artery Dissection is Associated with Widened Aortic Root Diameter
Authors: Skljarevski Vladimir , Turek Michele , Hakim Antoine M.
Published: February 2016

Objective:

Dissection of the internal carotid and vertebral arteries is a well recognized cause of stroke, especially in the middle-aged. The exact etiology of this condition is controversial. According to one theory there is an underlying vasculopathy originating from disturbed development of the neural crest. The neural crest gives rise to several tissues, including the tunica media of large cervical arteries and the outflow tract of the heart. We attempted to test the theory that developmental abnormality at the level of the neural crest may play a role in dissection of the large cervical arteries.

Methods:

We designed a retrospective case control study. By means of transthoracic echocardiography we measured the aortic root diameter in a group of patients with radiographically determined dissection of at least one large artery in the neck. The results were compared to a control group.

Results:

In comparison to age matched controls, male patients were found to have a significantly larger aortic root. Although a similar trend was apparent in females, the difference between the patient and control group of females was not statistically significant.

Conclusions:

Patients with cervical artery dissections may have other abnormalities in organs arising from the neural crest. A larger prospective clinical study and further research are needed to establish a firm link between dissection of the cervical arteries and abnormalities in other organs.
Topiramate: Pharmacokinetics and Pharmacodynamics
Authors: Schneiderman J.H.
Published: February 2016

Topiramate is a structurally novel anti-epileptic drug with at least 3 postulated mechanisms of action including: 1) potentiation of GABA responses, 2) impairment of AMPA/kainate glutamate receptors and 3) suppression of high frequency action potential firing. It has a favourable pharmacokinetic profile with rapid absorption, good bio-availability, linear pharmacokinetics, relatively long half-life and limited pharmacokinetic drug interactions. However, topiramate can reduce the estrogen component of oral contraceptive medications. Women may require birth control preparations containing 50 (Xg of estrogen. Topiramate clearance is reduced in severe renal failure and increased by enzyme-inducing antiepileptic drugs. The dose of topiramate may have to be reduced in renal failure or when withdrawing enzyme inducers.
Calmodulin-Dependent Cyclic Nucleotide Phosphodiesterase in Human Cerebral Cortex and Glioblastoma Multiforme
Authors: Lal Sumeer , Raju Rajala V.S. , Macaulay Robert J.B. , Sharma Rajendra K.
Published: February 2016

Background: Calmodulin-dependent cyclic nucleotide phosphodiesterase (CaMPDE) has been extensively studied and characterized in normal mammalian tissues; however very little is known about this enzyme in human brain tumors. It has been established that high levels of this enzyme exist in non-central nervous system tumors, PDE inhibitors or cAMP analogues have been used to treat them. This study has examined the levels of CaMPDE in glioblastoma multiforme from six patients and has compared these to the levels of CaMPDE in four patients with normal cerebral tissue. In addition, an enzyme immune assay method (EIA) was developed in this study for the detection of CaMPDE in human cerebral tissue. This method is proposed to be used as an adjunct to the spectrophotometric method presently utilized. This would be beneficial in cases where small tissue samples, for example in stereotactic biopsy, are available.Methods: The CaMPDE activity and corresponding levels of expression in cerebral tissue from temporal lobectomies and both surgical extraction or stereotactic biopsy in patients with primary tumors were determined by spectophotometric and EIA, respectively. The EIA was developed from the production of a polyclonal antibody against bovine brain 60 kDa CaMPDE isozyme. Cross reactivity of the antibody with human was confirmed using transblot and immunohistochemistry.Results: Utilising the EIA, there was found to be significant reduction in both catalytic activity (p < 0.001) and in quantitative protein expression (p < 0.001) in glioblastoma multiforme from patients when compared to normal cerebral cortex. Immunoblotting experiments and immunohistochemistry demonstrated that CaMPDE in glioblastoma multiforme failed to react with a polyclonal antibody raised against bovine brain 60 kDa CaMPDE isozyme, whereas the enzyme from normal tissue reacted with antibody.Conclusions: Contrary to other studies on non-CNS tumors, the catalytic activity and the protein expression of CaMPDE is reduced in glioblastoma multiforme. The EIA method is a more sensitive in detecting CaMPDE than in the spectrophotometric method, especially when a small amount of tissue is available. Immunohistochemistry and the EIA may be useful in the future to use as markers for other types of brain tumors and not for glioblastoma multiforme as demonstrated.
Impaired Incentive Learning in Treated Parkinson’s Disease
Authors: Charbonneau D. , Riopelle R.J. , Beninger R.J.
Published: February 2016

Objective: To quantify the performance of patients with Parkinson’s disease (PD) in incentive learning, or learning to respond to stimuli that signal the imminent presentation of a reinforcer, and in paired-associate learning, or learning of word associations. Methods: The performance of 32 patients with idiopathic PD was compared to that of 25 healthy control subjects, and 32 subjects suffering from arthritis, matched for age and education. The PD and arthritic groups were comparable on a self-report measure of physical disability. All subjects were physically capable of satisfying the contingencies of the incentive learning task. The avoidance task that quantified incentive learning used money loss as an aversive stimulus. The word paired-associate learning task was presented on a computer and feedback was not given on performance. Results: The normal and arthritic groups performed equally well on the avoidance task, whereas the PD group was impaired despite dopaminergic replacement therapy. The groups did not differ significantly in paired-associate learning. Conclusions: These findings are among the first to suggest that the nigrostriatal dopamine dysfunction associated with PD may play a role in incentive learning but not in paired-associate learning and are consistent with a role for dopamine in certain forms of learning and memory. The findings may highlight differences between tonic and modulated function in the nigrostriatal system.
Relationship Between Sleep, Neck Muscle Activity, and Pain in Cervical Dystonia
Authors: Lobbezoo Frank , Thu Thon Marc , Rémillard Guy , Montplaisir Jacques Y. , Lavigne Gilles J.
Published: February 2016

Objective: The interactions between sleep, neck muscle activity, and cervical spinal pain were examined in a controlled study with nine patients suffering from idiopathic cervical dystonia (ICD; also referred to as spasmodic torticollis), and nine gender- and age-matched controls. Methods: From each participant, two all-night polysomnograms with additional electromyographic recordings from the sternocleidomastoid and upper trapezius muscles were obtained. The first night was for habituation to the laboratory environment; the second night for experimental data collection. Visual analogue scales were used to collect intensity and unpleasantness ratings of cervical spinal pain before and after the second sleep recording. Results: None of the standard sleep variables showed statistically significant differences between average values of both groups of participants. However, a significantly larger variance in sleep latency was obtained for the ICD patients. In general, abnormal cervical muscle activity decreased immediately when lying down without the intention to go to sleep. Subsequently, abnormal muscle contractions were gradually abolished in all ICD patients during the transition from relaxed wakefulness to light NREM sleep. Following this transition phase, no more abnormal EMG activity was found in any of our patients. Finally, cervical spinal pain intensity and unpleasantness were reduced by about 50% overnight. Conclusions: Both supine position and sleep can be associated with an improvement of symptoms of ICD, and this disorder does not induce any sleep perturbations.
Triphasic Waves During Post-Ictal Stupor
Authors: Ogunyemi Abayomi
Published: February 2016

Background: The term, “triphasic wave” originally described an EEG pattern believed to be a marker for a specific stage of hepatic coma. For 4 decades, the diagnostic and prognostic specificity of the pattern remains controversial. Its pathophysiology also continues to be elusive. Methods: EEG recordings were obtained in three patients known or suspected to have primary generalized epilepsy. In 2 patients, the EEGs were part of long-term monitoring using simultaneous video-EEG telemetry. For the third patient, the EEG was secured only during the post-ictal unconsciousness. These 3 patients were specifically selected because of the presence of triphasic waves in their EEGs. Results: Triphasic waves were observed in the EEG of the 3 patients only during post-ictal unconsciousness. The pattern was transient, being preceded by generalized suppression and delta slow waves and followed by theta activities. Alpha rhythms supervened when the patients became fully alert. Conclusion: A post-ictal state should be considered in unconscious patients with triphasic EEG waves.
Stereotactic Management of Bacterial Brain Abscesses
Authors: Shahzadi Sohrab , Lozano Andres M. , Bernstein Mark , Guha Abhijit , Tasker Ronald R.
Published: February 2016

Background:

CT and MR guided stereotactic techniques have provided promising results in the management of brain abscesses. We reviewed our results of stereotactic management of brain abscesses in 20 consecutive patients with 28 abscesses from 1986 to 1993.

Methods:

13 abscesses were in the cerebral hemispheres, 12 in the cerebellum, 2 in the pons and 1 in the thalamus. The bacterial organism was isolated in 12 of the 20 cases. All patients, except one who had a tuberculous abscess, were on antibiotics for less than 7 weeks.

Results:

Although there were 3 patients in coma before surgery, the mortality rate was zero and 17 patients had an excellent recovery with 3 patients having a persistent mild neurologic disability. Stereotactic aspiration of the largest lesion in the patients with multiple brain abscesses combined with intravenous antibiotic therapy was sufficient for the resolution of all lesions. Two of our patients treated with antibiotics alone showed abscess progression with neurologic worsening.

Conclusion:

Stereotactic aspiration is safe, accurate, and when combined with the appropriate antibiotics, should be considered the procedure of choice in the management of brain abscesses.
Narcolepsy Secondary to Fourth Ventricular Subependymoma
Authors: Ma T.K.F. , Ang L.C. , Mamelak M. , Kish S.J. , Young B. , Lewis A.J.
Published: February 2016

Background:

Secondary (symptomatic) narcolepsy is rare. We report a subependymoma of the fourth ventricle associated with narcolepsy. The patient was a 50-year-old woman with a long history of narcolepsy who died of colonic carcinoma with no cerebral metastasis. She was positive for HLA-DR2. At autopsy there was a tumour dorsal to the fourth ventricle which involved the midbrain tectum and rostral pons. Histologic examination of the tumour confirmed it to be a subependymoma.

Methods:

Review of the previous cases of secondary narcolepsy was made with particular reference to the anatomical location of the lesions.

Results:

Most of the lesions were found around the third ventricle and rostral brainstem.

Conclusions:

Knowing the anatomical localization of the pathological changes in secondary narcolepsy could be important in improving our understanding of its pathogenesis.
Vertebral Artery Injury Associated with a Jefferson Fracture
Authors: Walsh Gregory S. , Cusimano Michael D.
Published: February 2016

Background

The evaluation and treatment of Jefferson fractures, a burst fracture of the ring of CI, has been well documented in the medical literature. Vertebral artery injury associated with a Jefferson fracture is very rare.

Methods

The case study technique was used to summarize the case. Review of the literature was performed to discuss the case. Retrospective chart review of the 174 patients with cervical fractures admitted to St. Michael’s Hospital from 1989–1994 was also performed.

Results

The case of a patient with a Jefferson fracture, with bilateral lateral displacement of the lateral masses causing bilateral vertebral artery occlusions resulting in a lateral medullary and cerebellar infarction is reported. A review of the literature is provided.

Conclusion

A high index of suspicion for this injury is paramount, especially in patients with multiple trauma, where the diagnosis of Jefferson fractures can be delayed.
Long Term Intrathecal Baclofen Therapy in Patients with Intractable Spasticity
Authors: Becker W.J. , Harris C.J. , Long M.L. , Ablett D.P. , Klein G.M. , DeForge D.A.
Published: February 2016

Background

Severe spasticity unresponsive to oral drugs may respond satisfactorily to baclofen delivered intrathecally.

Methods

Intrathecal baclofen (IB) therapy delivered by means of implanted infusion pumps was used for nine patients with severe spasticity. Six patients had multiple sclerosis, two cervical spinal cord injury, and one head injury. All were non-ambulatory.

Results

Patients showed improvement in many areas, including ability to transfer, seating, pain control, personal care, and liability to skin breakdown. Before IB therapy, only three of the nine patients were able to live at home in the community and six were institutionalized. At the end of our follow-up period, only one patient remained institutionalized, three lived in group homes and five lived at home in the community. In the year preceding pump implantation, the nine patients spent a total of 755 days in acute care hospitals. In the year following onset of IB therapy, they spent only 259 days in hospital.

Conclusions

IB therapy can improve patient quality of life and can be cost-effective in carefully selected patients with severe spasticity and disability. The drug delivery catheter is that part of the therapeutic system most vulnerable to failure. Because of the varied expertise required to manage these patients effectively, and the potential for a variety of complications, it is essential that an IB program is supported by a well-organized multi-disciplinary medical team.
Brain Herniation: A Revision of Classical Concepts
Authors: Fisher C. Miller
Published: February 2016

This paper is an update on evolving ideas about brain herniations. Following observations on cerebellar pressure coning that raised doubts about its reputed lethal connotations, herniation at the tentorium was re-examined for its role in critically damaging the brain stem. Combining clinical, pathologic, computed tomography and magnetic resonance imaging data, it is concluded that temporal lobe herniation is not the means by which the midbrain sustains irreversible damage in acute cases, but rather lateral displacement of the brain at the tentorium is the prime mover and herniation a harmless accompaniment. Transtentorial herniation has been investigated with computed tomography using the three calcification relationship and descent through the tentorial opening could not be documented. Bilateral brain stem compression in acute bilateral cases must be distinguished from herniation. Upward cerebellar herniation is only the sign of an overfull posterior fossa. Subfalcial herniation is tolerated unless lateral displacement is excessive.
Pyridoxine Dependent Epilepsy with Iatrogenic Sensory Neuronopathy
Authors: McLachlan Richard S. , Brown William F.
Published: February 2016

An 18-year-old man was treated from birth with chronic high dose pyridoxine (vitamin B6) up to 2000 mg per day for pyridoxine-dependent seizures. Within two years of onset of treatment, he developed a sensory neuropathy which did not progress over the following 16 years. Electrophysiological studies were consistent with a pure sensory neuronopathy expressed as centripetal degeneration of processes of the dorsal root ganglion cells.
Bilateral Central Ptosis in Acquired Immunodeficiency Syndrome
Authors: Barton Jason J.S. , Kardon Randy H. , Slagel Daniel , Thompson H. Stanley
Published: February 2016

A man with acquired immune deficiency syndrome suddenly developed bilateral complete ptosis and minor vertical gaze limitation. Magnetic resonance imaging revealed a lesion in the midbrain surrounding the sylvian aqueduct in addition to bilateral masses in the caudate nuclei. Pathologic examination showed that the caudate lesions were central nervous system lymphoma of B cell origin, but the midbrain lesion contained only signs of AIDS encephalopathy. The periaqueductal lesion involved the caudal central subnucleus and probably also the subnuclei of the superior and inferior recti of the oculomotor nuclear complex bilaterally.
Pathological Definition and Evaluation of Diabetic Neuropathy and Clinical Correlations
Authors: Sima Anders A. F.
Published: February 2016

Diabetic neuropathy is a major complication of diabetes mellitus affecting to an equal extent type 1 and type 2 patients. Various mechanisms including metabolic and vascular abnormalities have been proposed to explain the progressive pathological changes that occur in peripheral nerve. Regardless of the precise mechanisms, structural abnormalities lead to functional changes which eventually result in the clinical manifestations of diabetic neuropathy. Possible interventions that may influence the progressive disease process include intensive insulin therapy and aldose reductase inhibitors. This article reviews the proposed pathogenetic mechanisms underlying diabetic neuropathy, their effects on nerve histomorphometric changes and ultrastructure typical of diabetic neuropathy, and potential therapeutic interventions and their impact on disease progression.
Unilateral Creutzfeldt-Jakob Disease Presenting as Rapidly Progressive Aphasia
Authors: Kirk Andrew , Ang L.C.
Published: February 2016

A 64-year-old man presented with a three day history of progressive Broca’s aphasia, followed within 3 weeks by exclusively right-sided myoclonus, rigidity, and dystonia. Within 4 weeks he was globally aphasie. He died within 7 weeks of onset. In the final week, rigidity and myoclonus became bilateral. CT and MRI were normal. SPECT showed diminished perfusion of the left hemisphere. EEG showed periodic discharges on the left. At autopsy, there were marked cortical spongiform change, neuronal loss, and gliosis throughout the left hemisphere and in the right occipital cortex. Elsewhere in the right hemisphere, spongiform change was non-existent to minimal. There was moderate spongiform change in the molecular layer of the cerebellar cortex, much more marked on the left. Clinical and pathological unilateral cerebral predominance extended to the ipsilateral cerebellum. Creutzfeldt-Jakob disease is an important consideration in patients with rapidly progressive unilateral cerebral signs associated with a movement disorder.
Somatosensory Evoked Potentials and Intracranial Pressure in Severe Head Injury
Authors: Konasiewicz Stefan J. , Moulton Richard J. , Shedden Peter M.
Published: February 2016

The purpose of this study was to explore the relationship between neurologic function, using a quantitative measurement of continuous somatosensory evoked potentials (SSEPs), and intracranial pressure (ICP) following traumatic brain injury. During a 6 year period, severely head-injured patients with a Glascow Coma Scale ≤ 8 who were not moribund were monitored with SSEPs and ICP measurements. SSEPs from each hemisphere and ICP were recorded hourly for each patient. Neurologic outcomes were scored using the Glasgow Outcome Scale at three months post injury. Although initial SSEP amplitude did not correlate well with outcome, final SSEP summed peak to peak amplitude from both hemispheres (p = .0001), the best hemisphere (p = .0004), and the worst hemisphere (p = .0001) correlated well with the Glasgow Outcome Scale groups. Of a total of 72 patients, 40 had deteriorating SSEPs and 32 had stable or improving SSEPs. Peak ICP values were not statistically different in these groups (p = .6). Among patients with deteriorating SSEPs, 52.5% lost the greatest proportion of hemispheric electrical activity prior to ICP elevation. In the remaining patients, the percent reduction of SSEP activity after peak ICP levels was not statistically different from the percent reduction in SSEP activity prior to the peak ICP levels (p = .9). This data suggests that in a select group of patients with severe head injury, ICP does not cause SSEP deterioration, but rather is the consequence of deterioration of brain function.
Acidic Fibroblast Growth Factor Infusion Reduces Ischemic CA1 Hippocampal Damage in the Gerbil
Authors: MacMillan V. , Walton-Roche K. , Davis J.
Published: February 2016

Occlusion of the carotid arteries for 5 minutes in the Mongolian gerbil results in selective necrosis of CAI pyramidal neurons. In the present experiments we studied whether intraventricular infusion of acidic fibroblast growth factor (aFGF) could attentuate this damage. Intraventricular infusions of bovine serum albumin (BSA-10 ng/h) or aFGF (1, 10 or 100 ng/h) were started 2 days prior to 5 minutes of bilateral carotid occlusion and continued for 5 days post-ischemia. The brains were perfused and fixed at 5 days post-ischemia and histological assessment of CAI damage was made. Animals receiving intraventricular infusions of 10 or 100 ng/h aFGF showed a significant reduction of CA1 neuronal damage in comparison to no treatment ischemic controls (no treatment − 8 + 1 ; aFGF 10 ng/h − 147 ± 28; aFGF 100 ng/h − 168 ± 35 cells/mm CAI; P < 0.05 for both aFGF groups). The results indicate that aFGF infusion can attenuate the severity of ischemic neuronal necrosis in the gerbil hippocampus.
Horner's Syndrome Secondary to Angiogram Negative, Subadventitial Carotid Artery Dissection
Authors: Assaf M. , Sweeney P.J. , Kosmorsky G. , Masaryk T.
Published: February 2016

A 59-year-old man presented with the acute onset of paresthesias and pain in the left neck, face, and forehead. On subsequent investigation he was found to have a subadventitial type of carotid artery dissection, producing an ipsilateral Horner's syndrome with normal carotid angiography. MRI imaging of the neck structures, using fat saturation technique, showed the subadventitial dissection, sparing the vessel lumen. MRI offers a non-invasive method of diagnosis and follow-up for carotid artery dissection.
The Focal Encephalopathies Associated with Mycoplasma Pneumoniae
Authors: Raj D. Sheth , Keith J. Goulden , William E. Pryse-phillips
Published: February 2016

Five patients with evidence of focal encephalopathy are reported. In each case, evidence ofmycoplasma pneumoniaeinfection was detected. No patient improved with conventional antibiotic therapy, but in three subjects, rapid and complete recovery did occur contemporaneously with the administration of high dose steroid therapy. It is suggested that focal as well as diffuse cerebral or cerebellar lesions may occur as manifestations of auto-immune disease complicating mycoplasmal infections in young people and that this illness may be designated as acute mycoplasma-associated encephalopathy.
Cell Interactions Underlying Purkinje Cell Replacement by Neural Grafting in the pcd Mutant Cerebellum
Authors: Constaino Sotelo
Published: February 2016

The results obtained with neuronal grafting in an animal model of heredo-degenerative ataxia (the pcd mutant mouse) have been extremely useful to unmask new aspects of neural plasticity. The grafted embryonic Purkinje cells invade the deficient molecular layer of the host by migrating radially through adult Bergmann fibers. There, they start building their dendritic trees and, by promoting the axonal sprouting of specific adult neuronal population in a timed sequence, they receive appropriate synaptic contacts, starting ten days after grafting. Twenty-one days after grafting, the grafted Purkinje cells have acquired their adult dendritic pattern and synaptic investment. Both the detailed timetable and the nature of the cellular interactions between embryonic and adult neural cells are remarkably similar to those occurring during normal development. These results raise the possibility that embryonic Purkinje cells can induce in adult neural cells a new type of plasticity, that of recreating a permissive microenvironment for the synaptic integration of the grafted neurons, leading to the anatomical restoration of the cortical circuit of the mutant cerebellum.
Mild Hypothermia Preserves Contractile Function and Inhibits Prostaglandin E2 Release from Metabolically Stressed Skeletal Muscle
Authors: Cwik Valerie A. , Majumdar Ramanath , Brooke Michael H.
Published: February 2016

An in vitro model of muscle damage was used to investigate the protective effect of mild hypothermia in muscle injury. Rat epitrochlearis muscles were dissected in their entirety and suspended in Krebs-Ringer solution and DNP, a mitochondrial uncoupler, was added. PGE2, and lactate release and the contractile response to stimulation were measured and compared to untreated controls. Experiments were done at 37, 35, 33 and 27°C. At 37°C, DNP stimulated muscle releases large amounts of PGE2 and lactate and is unable to contract. As the temperature is reduced, there is progressive preservation of contractile force, although high lactate levels at the lowest temperatures indicate that the metabolic stress is still present. In contrast, DNP stimulated PGE2 release is completely inhibited at or below 35°C and may be related to a similar protective phenomenon seen in experimental ischemic neuronal death.
Surgical Referral for Carotid Artery Stenosis — The Influence of NASCET
Authors: Coyne T.J. , Wallace M.C.
Published: February 2016

A retrospective review was undertaken of 139 consecutive patients with presumed carotid artery stenosis referred to a vascular neurosurgeon. The review period included three years prior and one year subsequent to the publication of the North American Symptomatic Carotid Endarterectomy Trial (NASCET) preliminary results showing surgery to be superior to best medical therapy for patients with symptomatic, high grade (> 70% linear diameter) carotid stenosis. The aims of this analysis were to determine any changes in the referral pattern following the NASCET publication (post-NASCET), and to examine the use and reliability for surgical decision making of pre-referral carotid artery imaging. Patient referral rate increased markedly post-NASCET, particularly from neurologists. There was a trend for more post-NASCET referrals to be for high grade stenosis and fewer referrals to be for intermediate grade (30-69% linear diameter) stenosis, although continued referral of patients with intermediate grade stenosis is desirable as randomization into NASCET continues for this group of patients.
Ninety-six patients (69%) were referred with carotid duplex ultrasonography having been performed. There was poor correlation of these results with angiography, which remains necessary for planning management. A majority of patients (65%) referred to this surgical practice did not come to surgery.
Pseudogout of the Transverse Atlantal Ligament: An Unusual Cause of Cervical Myelopathy
Authors: Griesdale Donald E.G. , Boyd Mike , Sahjpaul Ramesh L.
Published: February 2016

Background:

Calcium pyrophosphate dihydrate deposition in the cervical spine is infrequently symptomatic. This is especially true at the craniocervical junction and upper cervical spine.

Case Report:

A 70-year-old previously healthy woman presented with a progressive cervical myelopathy of four months duration.

Results:

Examination revealed sensorimotor findings consistent with an upper cervical myelopathy. Radiological studies (plain radiographs, computed tomography, and magnetic resonance imaging) revealed C1-2 instability, and a well-defined extradural 3cm x 1cm retro-odontoid mass causing spinal cord compression. Transoral resection of the mass was performed followed by posterior C1-2 stabilization. Histological examination of the mass confirmed calcium pyrophosphate dihydrate deposition. Follow-up examination showed marked clinical and radiological improvement.

Conclusion:

Although uncommon, calcium pyrophosphate dihydrate deposition disease should be considered in the differential diagnosis of extradural mass lesions in the region of the odontoid.
Multimodal Longitudinal Imaging of Focal Status Epilepticus
Authors: Doherty Colin P. , Cole Andrew J. , Grant P. Ellen , Fischman Alan , Dooling Elizabeth , Hoch Daniel B. , White Tessa Hedley , Cosgrove G. Rees
Published: February 2016

Background:

Little is understood about the evolution of structural and functional brain changes during the course of uncontrolled focal status epilepticus in humans.

Methods:

We serially evaluated and treated a nine-year-old girl with refractory focal status epilepticus. Long-term EEG monitoring, MRI, MRA, SPECT, intraoperative visualization of affected cortex, and neuropathological examination of a biopsy specimen were conducted over a three year time span. Imaging changes were correlated with simultaneous treatment and EEG findings.

Results:

The EEG monitoring showed almost continuous spike discharges emanating initially from the right frontocentral area. These EEG abnormalities were intermittently suppressed by treatment with anesthetics. Over time, additional brain areas developed epileptiform EEG abnormalities. Serial MRI studies demonstrated an evolution of changes from normal, through increased regional T2 signal to generalized atrophy. An MRAdemonstrated dilatation of the middle cerebral artery stem on the right compared to the left with a broad distribution of flow-related enhancement. An 18FDG-PET scan showed a dramatically abnormal metabolic profile in the same right frontocentral areas, which modulated in response to treatment during the course of the illness. A right frontotemporal craniotomy revealed a markedly hyperemic cortical focus including vascular shunting. A sample of resected cortex showed severe gliosis and neuronal death.

Conclusions:

The co-registration of structural and functional imaging and its correlation with operative and pathological findings in this case illustrates the relentless progression of regional and generalized abnormalities in intractable focal status epilepticus that were only transiently modified by exhaustive therapeutic interventions. Increased flow through large vessels appeared to be shunted and did not translate into increased microvascular perfusion.