bluuebunny/crossref_metadata_2025 huggingface dataset:
Transient Hemichorea/Hemiballismus Associated with New Onset Hyperglycemia
Authors: Ifergane G. , Masalha R. , Herishanu Y.O.
Published: December 2014

Objective:

To describe three patients suffering from transient hemichorea/hemiballismus associated with hyperglycemia, review previous reports and propose a possible pathophysiological explanation for this phenomenon.

Results:

Our original cases and previously reported ones reveal a uniform syndrome: mostly female patients (F/M ratio of 11/2), 50-80 years old, usually with no previous history of diabetes mellitus (9/13), develop choreic or ballistic movements on one side of the body over a period of hours. Serum glucose levels are elevated. In most of the patients, a lowering of the blood sugar level reverses the movement disorder within 24-48 hours.

Conclusions:

We believe that the combination of a recent or old striatal lesion (causing increased inhibition of the subthalamic nucleus) and hyperglycemia (causing decreased GABAergic inhibition of the thalamus) may be responsible for the appearance of this unilateral hyperkinetic movement disorder. Undiagnosed diabetes mellitus should always be suspected in patients who develop hemiballistic or hemichoreic movements. When hyperglycemia is detected and corrected, the movement disorder usually resolves within two days and may not require symptomatic therapy with dopamine receptor antagonists.
Progress in Clinical Neurosciences: The Neuropathogenesis of HIV Infection: Host-Virus Interaction and the Impact of Therapy
Authors: Power C. , Gill M.J. , Johnson R.T.
Published: December 2014

Despite the availability of highly active antiretroviral therapy (HAART), primary HIV-related neurological diseases remain major problems in HIV clinics. The present review examines the pathogenesis of HIV-related dementia and the less severe minor cognitive and motor deficit, together with distal sensory and drug-induced toxic polyneuropathies. Abnormal host immune responses within the nervous system and the role of viral expression and diversity are emphasized in relation to neurovirulence. Induction of innate immune responses within the central and peripheral nervous systems, largely mediated by cells of macrophage lineage, appear to be common to the development of primary HIV-related neurological disease. Activation of these cell types results in the release of a cascade of inflammatory molecules including cytokines, chemokines, matrix metalloproteinases, and arachidonic acid metabolites that influence neuronal survival. Individual viral proteins encoded by envelope and tat genes and discrete sequences within these genes influence the extent to which these pro-inflammatory molecules are induced. At the same time, systemic immune suppression may influence the occurrence and severity of HIV-related neurological diseases. Implementation of HAART and neuroprotective treatments improves neurological function although the evolution of drug-resistant viral strains limits the sustained benefits of HAART.
Lactate Stress Testing in 155 Patients with Mitochondriopathy
Authors: Finsterer Josef , Milvay Erika
Published: December 2014

Objective:

Few data are available about the diagnostic yield of the lactate stress test (LST) in a large group of patients with mitochondriopathy (MCP).

Methods:

Serum lactate was determined once before, three times during, and once after a 15-minute, constant 30W workload on a bicycle in 62 controls, aged 17 to 84 years, 155 patients with MCP, aged 17 to 87 years, and 31 patients with neurological disorders other than MCP.

Results:

Lactate's upper reference limits at rest, 5, 10, 15 minutes after starting, and 15 minutes after finishing the exercise were 2.0, 2.1, 2.1, 2.1 and 1.8 mmol/l respectively. The test was regarded abnormal if more than two of the five lactate values exceeded the cut-off levels. Among the 103 patients with abnormal LST, 64 (62 %) had normal resting lactate. The sensitivity of the test was 67% and the specificity 94%.

Conclusion:

The LST proved to have a high sensitivity and specificity in the detection of patients with MCP, being thus a simple but powerful tool to assess the impaired oxidative metabolism in MCP patients.
The Clinical Profile of Nonmotor Fluctuations in Parkinson's Disease Patients
Authors: Gunal Dilek Ince , Nurichalichi Kerim , Tuncer Nese , Bekiroglu Nural , Aktan Sevinç
Published: December 2014

Objective:

Recently described nonmotor fluctuations may cause disability in Parkinson's disease patients. These fluctuations are generally grouped as sensory, autonomic and psychiatric. The clinical spectrum and frequency of these fluctuating symptoms are not well-described.

Methods:

We studied the relationship of nonmotor fluctuations with motor symptoms and determined the influence of age at disease onset, duration of disease, dosage and duration of levodopa treatment in the appearance of nonmotor fluctuations.

Results:

Statistical analysis showed a relationship of disease-related parameters with sensory and autonomic fluctuations but psychiatric fluctuations were only found to be associated with the duration of levodopa usage. The nonmotor fluctuations included in the study were observed during “on” periods as well as “off” periods.

Conclusion:

Nonmotor fluctuations had variable presentations. Moreover, their co-appearance with different types of motor fluctuations may be linked to the effect of other neurotransmitter systems acting synchronously with dopamine. Risk factors for sensory and autonomic fluctuations in patients with Parkinson's disease were early age of disease onset, longer duration and higher dose of levodopa use. Psychiatric fluctuations were only associated with higher doses of levodopa.
Tapping and Peg Insertion after Levodopa Intake in Treated and de novo Parkinsonian Patients
Authors: Müller Thomas , Benz Sabiene , Przuntek Horst
Published: December 2014

Background:

Investigators use instrumental tasks for objective assessment of parkinsonian motor disability and its drug response. To date, such studies on treated parkinsonian patients have not addressed acute and long-term effects of dopaminergic drugs.

Objectives:

To determine the impact of long-term dopaminergic therapy within a standardized levodopa challenge test design in combination with two repeatedly performed instrumental tasks, peg insertion and tapping, in previously treated and untreated parkinsonian patients.

Results:

Tapping significantly deteriorated in previously untreated, but not in treated parkinsonian patients after levodopa intake. In contrast, motor symptoms and peg insertion significantly improved in both groups of parkinsonian patients. Results of both tests differed between parkinsonian patients and matched controls.

Conclusion:

Worsening of cognitively less demanding tapping may result from upregulated presynaptic inhibitory feedback regulation, sedative effects of levodopa or dopamine overflow in untreated parkinsonian patients, who are sensitive to these effects in contrast to treated parkinsonian patients. Tapping is a task with autonomic repetitive performance and programming of standardised movements with a low need for cognitive effort. This autonomic functioning of attentional control and selective processing is intact in Parkinson's disease. Peg insertion depends on more complex movements and thus hypothetically on dopamine-associated cognitive processes. Therefore, impairment of peg insertion responded to dopaminergic stimulation in both groups of parkinsonian patients. Future studies on the efficacy of antiparkinsonian drugs, using instrumental tasks for objective assessment, should consider long-term impact of antiparkinsonian drug therapy and associated cognitive efforts.
The Communication of Neurological Bad News to Parents
Authors: Jan Mohammed M.S. , Girvin John P.
Published: December 2014

Communicating disappointing or unexpected neurological news to parents is often both difficult and emotionally unwelcome. At the same time, it is important that transfer of such information is done well and, indeed, if done well, can be a very rewarding experience. Limited references are available for physicians regarding the proper communication of neurological bad news to parents. This paper attempts to provide general guidelines regarding this process. The review is based on the available medical literature, detailed discussions with many senior physicians from different medical systems and the authors'personal experience. The manner in which neurological bad news is conveyed to parents can significantly influence their emotions, their beliefs and their attitudes towards the child, the medical staff, and the future. This review of the literature, combined with clinical experience, attests to the fact that most families describe emotional shock, upset, and subsequent depression after the breaking of news of a bad neurological disorder. However, the majority find the attitude of the news giver, combined with the clarity of the message and the news giver's knowledge to answer questions as the most important aspects of giving bad news.
Idiopathic Free-Floating Thrombus of the Common Carotid Artery
Authors: Silver Brian , Gulka Irene , Nicolle Michael , Sahjpaul Ramesh , Hachinski Vladimir
Published: December 2014

Background:

The observation of an intraluminal common carotid artery thrombus overlying a wall defect at ultrasonography or angiography is unusual. To our knowledge, there are no previous reports of a free-floating thrombus in the common carotid artery.

Case Report:

A 45-year-old woman who was previously healthy and on no medications presented with acute hemiparesis and aphasia. Following testing that included carotid duplex and trancranial Doppler ultrasonography, diffusion-weighted magnetic resonance imaging, and digital subtraction angiography, the patient underwent emergency open embolectomy. No underlying wall defect was seen at the time of imaging or surgery. No obvious hypercoagulable state could be identified. Her NIH Stroke Scale score improved from 26 at admission to 2 at three months and 1 at one year.

Conclusions:

Multimodal imaging may have improved diagnosis and management in this patient with a unique finding. The source of the thrombus remains obscure.
Evaluating Migraine Disability: The Headache Impact Test Instrument in Context
Authors: Pryse-Phillips William
Published: December 2014

Migraine impairs physical, social and emotional functioning but the diagnosis is not always made despite the availability of helpful tools. Poor patient-physician communication is one reason cited for this. It is arguable that if the impact that headaches are having on a person's life can be communicated adequately to the physician, the likelihood of appropriate management will increase. The tools currently employed for assessing headache impact are reviewed briefly and the Headache Impact Test (HIT) and HIT-6 described as validated and reliable measures of the effect that headaches are having on patients. The availability of the standard test on the internet, with feedback provided, indicates that this is a potentially useful tool enabling headache sufferers to realize the extent of the burden of migraine and empowering them to seek appropriate management strategies.
Improving Patient Compliance to Prophylactic Migraine Therapy
Authors: Aubé Michel
Published: December 2014

As in many other chronic conditions, adherence to prophylactic treatment in migraine is probably poor. In chronic diseases, compliance at one year does not exceed 50%. That could explain the low therapeutic gain seen with migraine preventive medications. It also renders difficult the evaluation of clinical trials on migraine prophylaxis since in most of these trials compliance is not properly assessed. From the patients' perspective, there are several factors that could explain poor adherence to recommended treatments. Essentially, these factors are the expression of the patients'subjective perception of their disease and potential remedies in a context of a positive patient-physician relationship. When migraine prophylactic treatment is considered, patients should be informed of the natural history of their disease and a diagnosis of an accelerated form of migraine should be confirmed. Prophylactic treatment at best would reduce by 50% the frequency of migraine attacks. In most studies, however, the therapeutic gain is in the order of 30-40%. Treatment should be instituted for a minimum time of two to three months and if effective maintained for 6-12 months. The outcome of prophylaxis can rarely be determined in a prospective way. The choice of prophylactic regimens remains empirical, often based on the physician's experience and perception of the mechanism of migraine. A better adherence to prophylactic treatment of migraine could possibly improve outcomes but current methods of improving adherence for chronic health problems are mostly complex and not very effective.
Progress in Clinical Neurosciences: Status Epilepticus: A Critical Review of Management Options
Authors: Wijdicks Eelco F.M. , Lawn Nicholas D.
Published: December 2014

Although generalized tonic-clonic status epilepticus (SE) is frequently seen, an evidence-based approach to management is limited by a lack of randomized clinical studies. Clinical practice, therefore, relies on a combination of expert recommendations, local hospital guidelines and dogma based on individual preference and past successes. This review explores selected and controversial aspects of SE in adults and provides a critical appraisal of currently recommended management strategies.
Single Dose versus Fractionated Stereotactic Radiotherapy for Meningiomas
Authors: Lo Simon S. , Cho Kwan H. , Hall Walter A. , Kossow Ronald J. , Hernandez Wilson L. , McCollow Kim K. , Gerbi Bruce J. , Higgins Patrick D. , Lee Chung K. , Dusenbery Kathryn E.
Published: December 2014

Objective:

To evaluate the safety and efficacy of stereotactic radiosurgery (SRS) compared to fractionated stereotactic radiation therapy (FSRT) for meningiomas treated over a seven year period.

Methods and materials:

Of the 53 patients (15 male and 38 female) with 63 meningiomas, 35 were treated with SRS and the 18 patients with tumors adjacent to critical structures or with large tumors were treated with FSRT. The median doses for the SRS and the FSRT groups were 1400 cGy (500- 4500 cGy) and 5400 cGy (4000-6000 cGy) respectively. Median target volumes for SRS and FSRT were 6.8 ml and 8.8 ml respectively. The median follow-up for the SRS and FSRT groups were 38 months (4.1-97 months) and 30.5 months (6.0-63 months) respectively.

Results:

The five-year tumor control probability (TC) for benign versus atypical meningiomas were 92.7% vs. 31% (P=.006). The three-year TC were 92.7% vs. 93.3% for SRS vs. FSRT groups respectively (P=.62). For benign meningiomas, the three-year TC were 92.9% vs. 92.3% for the SRS group (29 patients) vs. FSRT group (14 patients) respectively (P=.77). Two patients in the SRS group and one in the FSRT group developed late complications.

Conclusion:

Preliminary data suggest that SRS is a safe and effective treatment for patients with benign meningiomas. Fractionated stereotactic radiation therapy with conventional fractionation appeared to be an effective and safe treatment alternative for patients not appropriate for SRS. A longer follow-up is required to determine the long-term efficacy and the toxicity of these treatment modalities.
Outpatient Lumbar Microdiscectomy: A Prospective Study in 122 Patients
Authors: Singhal Ashutosh , Bernstein Mark
Published: December 2014

Background:

Outpatient surgery saves the risk of nosocomial complications and health care dollars. Patients undergoing lumbar microsurgical discectomy are excellent candidates for outpatient surgery. The object of this study was to examine the feasibility of performing lumbar microdiscectomy on an outpatient protocol and to examine the potential savings associated with such a protocol.

Methods:

From February 1997 to September, 2001, 122 consecutive patients of the senior author were entered into a protocol of outpatient lumbar microdiscectomy. Only elective cases were considered for this study. Patients were excluded if they had significant co-morbidities, lived a significant distance out of town, or if their surgery was scheduled too late in the day. Success was defined as discharge home from the day-surgery unit approximately four hours after surgery.

Results:

During the study period, 150 elective lumbar microdiscectomies were performed. Twenty-four patients were excluded based on the above criteria and four patients requested not to participate in the study. Of the remaining 122, 116 successfully completed the protocol (95.1%). Six patients were admitted from the day surgery unit; two patients with dural tears and four patients with anaesthetic side-effects. No patient was readmitted to hospital after discharge and no complications of early discharge were observed. There was a total reduction in hospitalization of 1.2 nights per elective procedure considering the 150 patients, when compared with the hospitalization times prior to outpatient lumbar microdiscectomy.

Conclusion:

Lumbar microdiscectomy can be performed safely as an outpatient procedure, resulting in a substantial reduction in hospitalization times.
Rett Syndrome: Investigation of Nine Patients, including PET Scan
Authors: Dunn Henry G. , Stoessl A. Jon , Ho Helena H. , MacLeod Patrick M. , Poskitt Kenneth J. , Doudet Doris J. , Schulzer Michael , Blackstock Derek , Dobko Teresa , Koop Ben , Amorim Giovana V. de
Published: December 2014

Background:

We describe nine females with Rett Syndrome (RS), aged 14 to 26 years. All had had developmental delay before the end of their first year and had subsequently regressed to profound dementia with apraxia, ataxia, irregular respirations and often also seizures.

Methods:

The Revised Gesell developmental assessment and Alpern-Boll Developmental Profile were used in modified form. Volumetric measurements of basal ganglia using MRI were compared with the findings in nine age-matched volunteer females. Positron emission scans with [18F]-6-fluorodopa and [11C]-raclopride were performed under light anesthesia with intravenous Propofol, and the findings were compared with those in healthy control girls. Bidirectional sequencing of the coding regions of the MECP2 gene was investigated in blood samples for mutational analyses.

Results:

The RS females functioned at a mental age level ranging from about 4 to 15 months. The scores correlated with height, weight and head circumference. Magnetic resonance scans of basal ganglia showed a significant reduction in the size of the caudate heads and thalami in the Rett cases. Positron emission scans demonstrated that the mean uptake of fluorodopa in RS was reduced by 13.1% in caudate and by 12.5% in putamen as compared to the controls, while dopamine D2 receptor binding was increased significantly by 9.7% in caudate and 9.6% in putamen. Mutations in the coding regions of the MECP2 gene were present in all nine patients. No significant correlation between type and location of mutation and volumetric changes or isotope uptake was demonstrable.

Conclusion:

Our findings suggest a mild presynaptic deficit of nigrostriatal activity in Rett syndrome.
AZT Myopathy and HIV-1 Polymyositis: One Disease or Two?
Authors: Walsh Kimberley , Kaye Kathy , Demaerschalk Bart , Stewart Sarah , Crukley Jeff , Hammond Robert
Published: December 2014

Purpose:

This paper discusses the association between inflammatory and mitochondrial pathologies in patients with HIV-1/AIDS treated with zidovudine (AZT).

Methods:

We present the clinical and pathological details of a 52-year-old HIV-1 positive male who presented with progressive muscle weakness. We also review the current literature and address the debated pathogenesis of the inflammatory pathology.

Results:

Muscle biopsy revealed evidence of both HIV-1 polymyositis and AZT myopathy. Six months after initiation of corticosteroid therapy and discontinuation of AZT, the patient’s symptoms had greatly improved. The biopsy was repeated to show that both pathologies had resolved.

Conclusion:

The perceived overlap in the pathological spectra of HIV-1 polymyositis and AZT myopathy has produced some debate on causation and treatment. Unfortunately, there have been very few reports where a repeat biopsy following a drug washout period confirmed resolution of the pathology. Furthermore, affected patients have not been treated in a uniform fashion. Whether this represents one disease or two remains uncertain. The clinical relevance of this issue lies in the potential for harm from the unnecessary use of corticosteroids. This question may be best addressed by a randomized clinical trial.
Epileptogenesis, Ictogenesis and the Design of Future Antiepileptic Drugs
Authors: Weaver Donald F.
Published: December 2014

There is still no medical cure for epilepsy. Clinical epileptology is in need of a “paradigm shift” when it comes to the continuing development of therapeutics. An important first step in this conceptual evolution is differentiating between the notions of ictogenesis and epileptogenesis. All traditional therapeutics are anti-ictogenic, not antiepileptogenic. The future of antiepileptic drug development lies in the discovery of antiepileptogenics. Just as aspirin is not the drug of choice for meningitis, an anticonvulsant is not the drug of choice for epilepsy. Drug design for epilepsy needs to discover a penicillin, not more aspirins.
Combined Endovascular/Neurosurgical Therapy of Blister-Like Distal Internal Carotid Aneurysms
Authors: Pelz D.M. , Ferguson G.G. , Lownie S.P. , Kachur E.
Published: December 2014

Objective:

To describe a novel therapeutic strategy for the treatment of “blister-like” aneurysms of the distal internal carotid artery (ICA). Direct surgical treatments of these fragile lesions have been associated with generally poor outcomes.

Methods:

Two consecutive patients presenting with acute subarachnoid hemorrhage from “blister-like” aneurysms were treated with preliminary balloon occlusion of the ICA, followed by surgical trapping of the ICA beyond the aneurysm.

Results:

The treatment resulted in complete thrombosis of both aneurysms with no clinical complications.

Conclusion:

This combined endovascular-neurosurgical approach offers a controlled, safer alternative to primary surgical therapy of “blister-like” aneurysms.
Natural History of Absence Epilepsy in Children
Authors: Wirrell Elaine C.
Published: December 2014

Absence seizures may be seen in a variety of epileptic syndromes in childhood. Identification of the specific syndrome is important to determine medical prognosis. With childhood absence epilepsy, approximately two thirds of children can be expected to enter long-term remission, while in juvenile absence epilepsy, seizure control is often achieved, however, lifelong treatment is usually required. Other absence syndromes have a poorer prognosis, with lower rates of seizure control and remission. Psychosocial outcome is often poor, even in patients with more benign forms of absence epilepsy. Remission of epilepsy does not preclude psychosocial morbidity.
The Consortium to Investigate Vascular Impairment of Cognition: Methods and First Findings
Authors: Rockwood Kenneth , Davis Heather , MacKnight Chris , Vandorpe Robert , Gauthier Serge , Guzman Antonio , Montgomery Patrick , Black Sandra , Hogan David B. , Kertesz Andrew , Bouchard Remi , Feldman Howard
Published: December 2014

Background:

The Consortium to Investigate Vascular Impairment of Cognition (CIVIC) is a Canadian, multi-centre, clinic-based prospective cohort study of patients with Vascular Cognitive Impairment (VCI). We report its organization and the impact of diagnostic criteria on the study of VCI.

Methods:

Nine memory disability clinics enrolled patients and recorded their usual investigations and care. A case report form included all vascular dementia (VaD) individual criteria for each of four sets (National Institute of Neurological Disorders and Stroke (NINDS-AIREN), Alzheimer’s Disease Diagnostic Treatment Centers (ADDTC), the ICD-10 Classification of Mental and Behavioural Disorders (ICD-10), and the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV)) of consensus-based diagnostic criteria and for the Hachinski Ischemia Score (HIS). Investigators, having completed the case report form, were asked to make a clinical judgement about the cognitive diagnosis based on the best available information, including neuroimaging.

Results:

Of 1,347 patients (mean age 72 years; 56% women), 846 (63%) were diagnosed with dementia and 324 (24%) were diagnosed with VCI. The proportion of patients diagnosed with VaD by the diagnostic criteria was: 23.9% (n=322) by DSM-IV, 10.2% (n=137) by HIS, 4.3% (n=58) by ICD-10, 3.8% (n=51) by ADTCC, and 3.6% (n=48) by NINDS-AIREN. Judged against a clinical diagnosis of VaD, the sensitivity/specificity of each was: DSM-IV (0.77/0.80); HIS (0.41/0.92); ICD-10 (0.29/0.98); ADTCC (0.24/0.98); NINDS-AIREN (0.42/0.995). Compared with a clinical diagnosis of VCI, sensitivities were lower for the diagnostic criteria, reflecting the exclusion of patients who did not have dementia.

Conclusions:

Consensus-based criteria for VaD omit patients who do not meet dementia criteria that are modeled on Alzheimer’s disease. Even for patients who do, the proportion identified with VaD varies widely. Criteria based on empirical analyses need to be developed and validated.
Artificial Disc Insertion Following Anterior Cervical Discectomy
Authors: Pickett Gwynedd E. , Duggal Neil
Published: December 2014

Objective and importance:

Fusion following anterior cervical discectomy has been implicated in the acceleration of degenerative changes in the adjacent spinal segments. Discectomy followed by implantation of an artificial cervical disc maintains the functionality of the spinal unit, while still providing excellent symptomatic relief. We describe our preliminary experience with implantation of the Bryan Cervical Disc System in two cases of single-level cervical disc herniation.

Clinical presentation:

Two male patients presented with a left C6 radiculopathy, without evidence of myelopathy. Magnetic resonance imaging revealed a disc herniation at C5-6 in both cases. Pre-operative flexion and extension radiographs demonstrated preserved motion at the involved levels.

Intervention/technique:

Following a standard anterior cervical decompression, precision drilling of the vertebral endplates was carried out using a drill attached to a bed-mounted, gravitationally-referenced retraction frame. An artificial cervical disc, composed of a polyurethane nucleus with titanium endplates, was fitted between the contoured endplates without fixation to the vertebral bodies. No complications were experienced during the insertion of the prosthesis, or in the postoperative course. Both patients experienced immediate postoperative resolution of their radicular pain and were discharged from hospital the following day. At nine months following surgery, both patients continue to have complete relief of radicular symptoms. Postoperative radiographs at six months following surgery confirm accurate placement of the prosthesis and preserved mobility of the functional spinal unit.

Conclusion:

Insertion of the Bryan artificial cervical disc prosthesis following anterior cervical discectomy is a relatively straightforward procedure, which appears to be safe and provides good clinical results, without requiring additional surgical time. Long-term follow-up is required to assess its safety, efficacy, and ability to prevent adjacent segment degeneration.
Progress in Clinical Neurosciences: Parkinson's Disease with Dementia and Dementia with Lewy Bodies
Authors: Camicioli Richard , Fisher Nancy
Published: December 2014

Dementia occurs in up to 30% of people with Parkinson's disease and is a major cause of disability. Pathologically, Parkinson's dementia, where dementia follows the onset of parkinsonism by at least one year, overlaps with dementia with Lewy bodies. We review the functional impact, definitions, neuropsychology, epidemiology and pathophysiology of Parkinson's dementia, dementia with Lewy bodies and their overlap. Associated psychiatric and imaging findings are also considered. Lastly, current and emerging approaches to assessment and treatment in patients with these Lewy body associated dementias are presented.
Carotid Endarterectomy: A Review
Authors: Findlay J. Max , Marchak B. Elaine , Pelz David M. , Feasby Thomas E.
Published: December 2014

Background:

Since the validation of carotid endarterectomy (CEA) as an effective means of stroke prevention, there has been renewed interest in its best indications and methods, as well as in how it compares to carotid angioplasty and stenting (CAS). This review examines these topics, as well as the investigation of carotid stenosis and the role of auditing and reporting CEAresults.

Investigation:

Brain imaging with CTor MRI should be obtained in patients considered for CEA, in order to document infarction and rule out mass lesions. Carotid investigation begins with ultrasound and, if results agree with subsequent, good-quality MRAor CTangiography, treatment can be planned and catheter angiography avoided. An equally acceptable approach is to proceed directly from ultrasound to catheter angiography, which is still the gold-standard in carotid artery assessment.

Indications:

Appropriate patientsfor CEA are those symptomatic with transient ischemic attacks or nondisabling stroke due to 70-99% carotid stenosis; the maximum allowable stroke and death rate being 6%. Uncertain candidates for CEA are those with 50 - 69% symptomatic stenosis, and those with asymptomatic stenosis ≥ 60% but, if selected carefully on the basis of additional risk factors (related to both the carotid plaque and certain patient characteristics), some will benefit from surgery. Asymptomatic patients will only benefit if surgery can be provided with exceptionally low major complication rates (3% or less).Inappropriate patientsare those with less than 50% symptomatic or 60% asymptomatic stenosis, and those with unstable medical or neurological conditions.

Techniques:

Carotid endarterectomy can be performed with either regional or general anaesthesia and, for the latter, there are a number of monitoring techniques available to assess cerebral perfusion during carotid cross-clamping. While monitoring cannot be considered mandatory and no single monitoring technique has emerged as being clearly superior, EEG is most commonly used. “Eversion” endarterectomy is a variation in surgical technique, and there is some evidence that more widely practiced patch closure may reduce the acute risk of operative stroke and the longer-term risk of recurrent stenosis.

Carotid angioplasty and stenting:

Experience with this endovascular and less invasive procedure grows, and its technology continues to evolve. Some experienced therapists have reported excellent results in case series and a number of randomized trials are now underway comparing CAS to CEA. However, at this time it is premature to incorporate CAS into routine practice replacing CEA.

Auditing:

It has been shown that auditing of CEA indications and results with regular feed-back to the operating surgeons can significantly improve the performance of this operation. Carotid endarterectomy auditing is recommended on both local and regional levels.
Multiple Sclerosis in its European Matrix: Some Aspects of History, Mechanisms and Treatment
Authors: McDonald Ian
Published: December 2014

Susceptibility to multiple sclerosis is influenced by several genes which are relatively common in populations of European origin. Their precise identification is currently being intensively investigated. The pathophysiology of the main clinical features is better understood.Relapseresults from abnormalities of conduction to which both demyelination and inflammation contribute. At the membrane level,remissiondepends on the formation of new sodium channels which restore conduction even in persistently demyelinated axons. Remyelination presumably contributes and synaptic reorganisation may also do so. Axonal degeneration contributes to irrecoverable deficit andprogressionof disability. These observations suggest new therapeutic strategies. The management of multiple sclerosis needs to be improved. Progress requires an ethically based partnership between patients, whose needs are paramount, the research and caring communities and the pharmaceutical industry.
Effectiveness of a Multidisciplinary Treatment Program for Chronic Daily Headache
Authors: Magnusson Jane E. , Riess Connie M. , Becker Werner J.
Published: December 2014

Background:

Chronic daily headache (CDH: headache on fifteen days a month or more) is one of the most common forms of chronic pain. The relative efficacy of different treatment methods for these patients needs to be determined.

Objective:

To compare treatment outcomes for patients with CDH treated in a traditional office-based pharmacological treatment program with a second group treated in a multidisciplinary management program.

Methods:

Patient outcomes were measured using changes in the Headache Disability Inventory (HDI) and the Short-Form-36 (SF-36) over the treatment period. Outcomes from seventy patients treated in an office setting were compared to thirty-seven patients treated in a multidisciplinary headache treatment program. Both groups received similar pharmacological treatment. All patients treated in the office setting and the majority of patients in the multidisciplinary program had transformed migraine.

Results:

Even though a reduction in headache days per month occurred, mean headache related disability (measured by HDI) and mean Health Related Quality of Life (HRQoL measured by SF-36) did not improve for the patient group treated in the office setting but did improve significantly for the patient group treated in the multidisciplinary headache program.

Conclusion:

For patients with CDH, headache-related disability and HRQoL is more likely to improve with management in a multidisciplinary headache treatment program as compared to the traditional specialist consultation - family physician office-based setting.
Jugular Bulb Oximetry for Prediction of Vasospasm Following Subarachnoid Hemorrhage
Authors: Heran Navraj S. , Hentschel Stephen J. , Toyota Brian D.
Published: December 2014

Background:

Cerebral vasospasm adversely impacts the outcome of those suffering aneurysmal subarachnoid hemorrhage (SAH). Prediction of vasospasm could improve outcomes. We hypothesized that preclinical vasospasm would be heralded by an increase in cerebral oxygen extractions (AVDO2) which could be detected by jugular bulb oximetry. A pilot study was conducted to address this hypothesis.

Methods:

Fourteen consenting patients with aneurysmal SAH, undergoing early surgery, were entered into the study. Four patients were withdrawn from the study secondary to failure of catheters or religious belief. At the time of craniotomy, a jugular bulb catheter was placed. Post-operatively, arterial and jugular bulb blood samples were taken every 12 hours to calculate AVDO2. As this was an observational study, no change in management occurred based on measurements.

Results:

Four of 10 patients had clinical vasospasm. These patients had a significant rise in AVDO2approximately one day prior to the onset of neurologic deficits (P<0.001). Symptoms resolved along with a significant improvement in AVDO2on instituting hypertensive, hemo-dilutional, and hypervolemic therapy in these patients. The six patients who did not exhibit clinical vasospasm did not demonstrate significant rise in AVDO2.

Conclusion:

Jugular bulb oximetry is simple and cost effective. Increases in AVDO2using this technique were predictive of clinically evident vasospasm in the subsequent hours to days. This investigation supports a larger study to assess the utility of jugular bulb oximetry in predicting vasospasm in aneurysmal SAH.
Contralateral Motor Automatisms in Neocortical Temporal Lobe Epilepsy
Authors: Mirsattari Seyed M. , Lee Donald H. , Blume Warren T.
Published: December 2014

Background:

The lateralizing value of the motor automatisms is generally doubted in most patients with temporal lobe epilepsy. However, subgroup analysis of the seizures of temporal lobe origin suggests a role for motor automatisms in discriminating seizures of neocortical versus mesial temporal lobe origin.

Methods:

Video-EEG of a patient with well-defined neocortical temporal lobe epilepsy was reviewed to assess the localizing value of motor automatisms.

Results:

We report a patient with left upper extremity motor automatisms and clonic movements of the proximal left lower extremity with altered awareness as the sole manifestations of right temporal neocortical seizures.

Conclusion:

Early onset unilateral motor automatisms without dystonic posturing can localize the seizure origin to the contralateral temporal lobe neocortex.
“From Sheep to Babe” - Menkes Disease
Authors: Ho Alice , Mah Jean , Casey Robin , Gaul Penney
Published: December 2014

A four-month-old boy presented with a new onset focal seizure lasting 18 minutes. During the seizure, his head and eyes were deviated to the left, and he assumed a fencing posture to the left with pursing of his lips. He had been unwell for one week, with episodes of poor feeding, during which he would become unresponsive, limp and stare for a few seconds. Developmentally he was delayed. He was not yet rolling, and he had only just begun to lift his head in prone position. He could grasp but was not reaching or bringing his hands together at the midline. He was cooing but not laughing. His past medical history was significant for term delivery with fetal distress and meconium staining. He was flat and blue at birth, with birth weight of 3.5 kg, and Apgar scores of 1 at one minute, 4 at 5 minutes, and 8 at 10 minutes. He required resuscitation with positive pressure ventilation for two minutes, and then had no further postnatal complications. Family history was remarkable for a paternal cousin with cortical malformation, epilepsy, and developmental delay. His mother and maternal grandmother had migraine headaches and fibromyalgia.
Simulation of Brain Death from Fulminant De-efferentation
Authors: Friedman Yael , Lee Liesly , Wherrett John R. , Ashby Peter , Carpenter Stirling
Published: December 2014

Background:

Guillain-BarrÈ syndrome (GBS) classically presents with a subacutely evolving areflexic paralysis, with typical laboratory findings of elevated cerebrospinal fluid protein and abnormal nerve conduction studies. There is now an increasing recognition of GBS variants that differ in clinical presentation, prognosis, electrophysiology and presumed pathogenesis. Fulminant cases of GBS have been reported in which a rapid deterioration evolves to a clinical state resembling ìbrain deathî.

Methods:

A retrospective analysis of two such cases of fulminant neuropathy are described, that includes the clinical course, electrophysiology and neuropathology where available.

Results:

We describe two patients that presented with a rapid course of neurological deterioration, lapsing into what resembled a ìclinically brain-deadî state that was subsequently ascribed to a fulminant polyneuropathy. Investigations (electrophysiological, pathological) and the clinical course suggested an axonal neuropathy.

Conclusion:

A fulminant neuropathy can result in a clinical state resembling ìbrain deathî through diffuse de-efferentation. Although generally attributed to aggressive demyelination with secondary axonal degeneration, a primary axonopathy can also lead to a similar clinical presentation.
Treatment of Parkinson's Disease
Authors: Martin W.R. Wayne , Wieler Marguerite
Published: December 2014

Parkinson's disease is a progressive neurodegenerative disorder that demands a holistic approach to treatment. Both pharmacologic and nonpharmacologic interventions play an important role in the comprehensive management of this disorder. While levodopa remains the single most effective medication for symptomatic treatment, dopamine agonists are playing an increasingly important role. Motor complications of dopaminergic therapy are a significant issue, particularly in patients with more advanced disease who have been on levodopa for several years. All therapeutic interventions must be tailored to the individual and modified as the disease progresses, with the goal of minimizing significant functional disability as much as possible.
Progress in Clinical Neurosciences: Pharmacotherapies for the Secondar Prevention of Stroke
Authors: Hackam Daniel G. , Kapral Moira K.
Published: December 2014

Stroke is a leading cause of mortality and long-term disability worldwide. Survivors of a previous stroke or transient ischemic attack are vulnerable to further cerebrovascular events, as well as myocardial infarction, peripheral vascular disease, congestive heart failure and vascular death. Traditional approaches to the secondary prevention of stroke have included aspirin after ischemic stroke, warfarin for stroke associated with cardioembolic sources, and carotid endarterectomy for eligible candidates with significant carotid artery stenosis. In recent years, much evidence has emerged to support a broader array of pharmacotherapies, including newer antiplatelet agents, lipid lowering drugs, and several classes of blood pressure lowering therapies. Also under study are B vitamins for patients with cerebrovascular disease and hyper-homocysteinemia, and oral direct thrombin inhibitors for high-risk patients with atrial fibrillation. We review the literature to determine the clinical significance of these therapies, and provide recommendations regarding their use in the prevention of recurrent stroke.
Mixed Migraine and Tension-type: A Common Cause of Recurrent Headache in Children
Authors: Seshia Shashi S.
Published: December 2014

Objective:

Determine relative frequency of recurrent headache (HA) types in children and adolescents referred to a pediatric neurologist. Methods and subjects: Study design: Prospective, sequential, and observational. Setting: Private practice Pediatric Neurology Clinic in a Canadian city (Winnipeg). Patients and data collection: Information on those referred with HA between September 1998 and December 2001 was entered on data sheets. Patients were followed up for one month to four years.

Results:

Three hundred and twenty (69%) of 463 referred with HAhad recurrent HA. There were 172 males (54%) and 148 (46%) females. Their ages ranged from two years to 19 years (median: 11 years). They had had their HA disorder for one month to 14 years (median: two years) prior to assessment. Migraine was the main HA type in 124 (38%), tension-type headache (TTH) in 57 (18%) and mixed migraine and TTH in 101 (32%). Thus, 101 (45%) of 225 with migraine as one HAtype also had TTH.

Conclusion:

Tension-type headache and migraine frequently co-exist and may represent a distinct headache type, at least in children; the association will likely influence response of affected children and adolescents to specific migraine treatments in clinical trials or practice.
Thalamic Deep Brain Stimulation Essential Tremor: Recommendations Long-Term Outcome Analysis
Authors: Putzke J.D. , Wharen, Jr. R.E. , Obwegeser A.A. , Wszolek Z.K. , Lucas J.A. , Turk M.F. , Uitti R.J.
Published: December 2014

Objectives:

Determine the efficacy of thalamic deep brain stimulation (DBS) for tremor control among individuals with essential tremor (ET).

Methods:

A clinical series of 52 consecutive individuals undergoing placement of a DBS system for treatment of ET completed an unblinded battery of subjective and objective measures at postoperative intervals of one, three, and 12 months, and annually thereafter up to three years. The assessment battery included measures of tremor and activities of daily living.

Results:

Both subjective and objective measures showed that stimulation was associated with significant improvement at nearly every postoperative interval as compared to pre-operative and stimulation 'off' ratings of activities of daily living functioning, midline tremor, contralateral upper extremity tremor, and contralateral lower extremity tremor. Ipsilateral tremor showed some improvement with stimulation, but only within the first three months. Trend analysis showed stable tremor control. Stimulation settings remained largely unchanged after the first three months. Dysarthria was more common among those with bilateral stimulation. A range of missing data estimation methods were performed, and subsequent analyses corroborated the main findings of the study.

Conclusion:

Thalamic DBS is generally a well-tolerated and effective treatment for ET. Methodological and analytical recommendations are provided for the evaluation of long-term outcome.
Electrophysiological Evaluation of Peripheral and Autonomic Involvement in Leprosy
Authors: Soysal Aysun , Atay Turan , Ozu Tacettin , Arpaci Baki
Published: December 2014

Objective:

Motor and sensory nerve conductions, F responses, sympathetic skin responses and R-R interval variations (RRIV) were studied to determine the type of peripheral neuropathy among patients with leprosy.

Methods:

Twenty-nine consecutive patients with leprosy (25 male, 4 female) hospitalized in the “Istanbul Leprosy Hospital'' between January - December, 1999 were included in this study. Ten patients had borderline lepromatous leprosy, and 19 had lepromatous leprosy. None of the patients studied had the tuberculoid form. The mean age was 55±12 years. The control group consisted of 30 (26 male, 4 female) healthy volunteers (mean age: 58.1±7.8 years). All subjects included in the study underwent neurological examination and electrophysiological evaluation. Standard procedures were performed for evaluating sensory and motor conduction studies. Motor studies were carried out on both left and right median, ulnar, tibial and common peroneal nerves while median, ulnar, sural and superficial peroneal nerves were examined for sensory studies. Sympathetic skin response recordings on both hands and RRIV recordings on precordial region were done in order to evaluate the autonomic involvement.

Results:

The lower extremity was found to be more severely affected than the upper, and sensory impairment predominated over motor. Of 58 upper limbs examined, no sympathetic skin responses was recorded in 46 (79.3%). Compared with the controls, the RRIVs of the leprosy patients were found to be reduced during both resting and deep forced hyperventilation.

Conclusion:

Our results indicate that leprosy causes a predominantly axonal polyneuropathy that is more severe in the lower extremities. Sensory nerve damage is accompanied by autonomic involvement.