bluuebunny/crossref_metadata_2025 huggingface dataset:
The Treatment of Trigeminal Neuralgia in Patients with Multiple Sclerosis using Percutaneous Radiofrequency Rhizotomy
Authors: Berk Caglar , Constantoyannis Constantine , Honey Christopher R.
Published: December 2014

Background:

Trigeminal neuralgia (TN) has a higher incidence among patients with multiple sclerosis (MS) than in the general population. This cohort of MS patients with TN presents a series of management challenges including poor tolerance of antineuralgic medications and occasional bilateral presentation. We analyzed our surgical series of MS patients presenting with TN who were treated with percutaneous radiofrequency rhizotomy to estimate the success, failure and recurrence rate of this procedure for those patients.

Methods:

Surgical reports were retrospectively reviewed between the years 1996-2000. Patients with MS and TN who received a percutaneous rhizotomy during that time were included in the study and followed until the end of 2002. Data regarding age, sex, duration of MS and pain, response to medical treatment, pain distribution and surgical outcome were evaluated.

Results:

There were thirteen patients with MS and medically refractory TN treated with percutaneous radiofrequency rhizotomy. The average age at diagnosis for MS was 41 with TN beginning an average of eight years later. Following rhizotomy, complete pain relief without the need for any medication was achieved in 81% of the patients. The addition of medications resulted in pain control in the remaining patients. During a mean follow-up period of 52 months, there was a 50% recurrence rate. There were no complications related to the procedure and the associated facial numbness was well-tolerated.

Conclusions:

Percutaneous radiofrequency rhizotomy is a safe and effective method for the treatment of TN in patients with MS. The unique susceptibility of this cohort to the side effects of antineuralgic medications may require early consideration of rhizotomy.
Temporal Lobe Epilepsy as a Unique Manifestation of Multiple Sclerosis
Authors: Gambardella Antonio , Valentino Paola , Labate Angelo , Sibilia Grazia , Ruscica Francesca , Colosimo Eleonora , Nisticò Rita , Messina Demetrio , Zappia Mario , Quattrone Aldo
Published: December 2014

Objective:

To report on five patients with temporal lobe epilepsy (TLE) as the unique manifestation of multiple sclerosis (MS).

Methods:

Among 350 consecutive MS patients, we identified 16/350 (4.6%) who also had epileptic seizures. Here, we review their electrophysiological and clinical features.

Results:

Five of these 16 patients (four female, one male; mean age 34.2 years; range 31 to 38) with MS and epileptic seizures had an extremely homogeneous clinical picture characterized by TLE as the unique manifestation of MS, even at long follow-up (mean: five years; range 4 to 10). In all patients, seizures started in the second or third decade. Brain MRI revealed at least one juxta-cortical lesion within the temporal region. Antiepileptic medication was always effective.

Conclusions:

The present study provides the first evidence of a peculiar form of MS characterized by TLE as the unique manifestation of the disease with no disability or MS relapses at long-term follow-up.
Auguste Forel on Ants and Neurology
Authors: Parent André
Published: December 2014

Auguste Forel was born in 1848 in the French part of Switzerland. He developed a lifelong passion for myrmecology in his childhood, but chose medicine and neuropsychiatry to earn his living. He first undertook a comparative study of the thalamus under Theodor Meynert in Vienna and then, from 1872 to 1879, he worked as Assistant Physician to Bernhard von Gudden in Munich. This led in 1877 to his seminal work on the organization of the tegmental region in which he provides the first description of the zona incerta and the so-called H (Haubenfeld) fields that still bear his name. In 1879, Forel was appointed Professor of Psychiatry in Munich and Director of the Burghölzli cantonal asylum. He became interested in the therapeutic value of hypnotism, while continuing his work on brain anatomy and ants. His neuroanatomical studies with Gudden’s method led him to formulate the neuron theory in 1887, four years before Wilhelm von Waldeyer, who received most of the credit for it. Forel then definitively turned his back on neuroscience. After his retirement from the Burghölzli asylum in 1898, and despite a stroke in 1911 that left him hemiplegic, Forel started to write extensively on various social issues, such as alcohol abstinence and sexual problems. Before his death in 1931 at the age of 83, Forel published a remarkable book on the social world of the ants in which he made insightful observations on the neural control of sensory and instinctive behavior common to both humans and insects.
Lipoatrophy in Patients with Multiple Sclerosis on Glatiramer Acetate
Authors: Edgar Catherine M. , Brunet Donald G. , Fenton Paul , McBride E. Vee , Green Peter
Published: December 2014

Background:

Patients with relapsing remitting multiple sclerosis on the disease modifying therapy of glatiramer acetate may be experiencing an adverse reaction of lipoatrophy at the sites of their subcutaneous injections. The purpose of this study was to complete a full examination of the injection site areas for users of glatiramer acetate, and to examine the relationship between lipoatrophy and patient characteristics.

Methods:

Glatiramer acetate users were identified by means of chart review. Over six months, during regular clinic appointments, assessment included a full examination of injection site areas including visual inspection and manual palpation. Additional patient and clinical characteristics were obtained by means of chart review and patient questioning.

Results:

Seventy-six patients had been or were current users of glatiramer acetate. Of these, 34 (45%) had evidence of lipoatrophy in at least one injection site area. All were female, and five had severe, nine had moderate and 20 had mild lipoatrophy. In some cases, lipoatrophy occurred within months of therapy initiation. Case reviews are included for five of the 34 patients, along with photographs of the lipoatrophy, a magnetic resonance image and comments on skin biopsies.

Conclusion:

Prevalence of lipoatrophy was much higher than expected. Possible reasons for this adverse reaction are explored and suggested treatment recommendations are reviewed. Lipoatrophy can be very disfiguring and is thought to be permanent, and the psychological impact can be significant. It is, therefore, important that patients be aware of the possibility of lipoatrophy, be able to identify it and discontinue injecting in areas where it is identified.
Bony Metastases of Anaplastic Oligodendroglioma Respond to Temozolomide
Authors: Morrison Tara , Bilbao Juan M. , Yang Guisheng , Perry James R.
Published: December 2014

Background:

Fewer than 30 cases of oligodendroglioma or anaplastic oligodendroglioma metastatic to bone are reported in the literature. Prolonged survival even with therapy is uncommon.

Methods:

We report a case of anaplastic oligodendroglioma metastatic to bone with a dramatic and durable response to temozolomide therapy. A retrospective case review, molecular analysis, and literature search were performed.

Results:

The patient presented with a right frontal mass in 1990. Progression led to resection of the lesion in 1995. Histology revealed an anaplastic oligodendroglioma and the tumour was found to have allelic loss of heterozygosity (LOH) of chromosome 1p (1p-). He received standard radiation therapy. In 2000 he developed hip and pelvic pain. A bone scan showed multiple skeletal lesions. Magnetic resonance imaging of the brain showed stability of intracranial disease. Resection of one lesion found metastatic anaplastic oligodendroglioma with identical morphology to the patient’s original tumour, including glial fibrillary acidic protein expression. The patient was started on standard temozolomide chemotherapy and celecoxib with prompt pain relief, and rapid normalization of serum alkaline phosphatase. He received a total of 12 cycles of combined therapy with no toxicity and no evidence of progression until increasing pain marked disease recurrence. The patient underwent palliative chemo- and radiation therapy but eventually succumbed.

Discussion:

Loss of heterozygosity 1p- is associated with prolonged survival in anaplastic oligodendroglioma and may increase the cumulative risk for development of systemic metastases. We speculate that metastases from oligodendroglioma harbouring loss of heterozygosity at chromosome 1p- retain the chemosensitivity of the initial lesion.
Motor Vehicle Accidents: the Most Common Cause of Traumatic Vertebrobasilar Ischemia
Authors: Beaudry Michel , Spence J. David
Published: December 2014

Background:

Recent media exposure of strokes from chiropractic manipulation have focused attention on traumatic vertebrobasilar ischemia. However, chiropractic manipulation, while the easiest cause to recognize, is probably not the most common cause of this condition.

Methods:

We reviewed all consecutive cases of traumatic vertebrobasilar ischemia referred to a single neurovascular practice over 20 years, from the office files and hospital records.

Results:

There were 80 patients whose vertebrobasilar ischemia was attributed to neck trauma. Five were diagnosed as due to chiropractic manipulation, but the commonest attributed cause was motor vehicle accidents (MVAs), which accounted for 70 cases; one was a sports injury, and five were industrial accidents. In some cases neck pain from an MVA led to chiropractic manipulation, so the cause may have been compounded. In most vehicular cases the diagnosis had been missed, even denied, by the neurologists and neurosurgeons initially involved. The longest delay between the injury and the onset of delayed symptoms was five years.

Conclusions:

Traumatic vertebrobasilar ischemia is most often due to MVAs; the diagnosis is often missed, in part because of the delay between injury and onset of symptoms and, in part, we hypothesize, because of reluctance of doctors to be involved in medicolegal cases.
Imaging of Murine Brain Tumors Using a 1.5 Tesla Clinical MRI System
Authors: Furth Wouter R. van , Laughlin Suzanne , Taylor Michael D. , Salhia Bodour , Mainprize Todd , Henkelman Mark , Cusimano Michael D. , Ackerley Cameron , Rutka James T.
Published: December 2014

Background:

In this study, we investigated the feasibility of using a 1.5 Tesla (T) clinical magnetic resonance imaging (MRI) system for in vivo assessment of three histopathologically different brain tumor models in mice.

Methods:

We selected mouse models in which tumor growth was observed in different intracranial compartments: Patched+/- heterozygous knock-out mice for tumor growth in the cerebellum (n = 5); U87 MG human astrocytoma cells xenografted to the frontal lobe of athymic mice (n =15); and F5 (n = 15) or IOMM-Lee (n = 15) human malignant meningioma cells xenotransplanted to the athymic mouse skull base or convexity. Mice were imaged using a small receiver surface coil and a clinical 1.5 T MRI system. T1- and fast spin echo T2-weighted image sequences were obtained in all animals. Gadolinium was injected via tail vein to better delineate the intracranial tumors. Twenty mice were followed by serial MRI to study tumor growth over time. In these mice, images were typically performed after tumor implantation, and at two week intervals. Mice were euthanized following their last imaging procedure, and their tumors were examined by histopathology. The histopathological preparations were then compared to the last MR images to correlate the imaging features with the pathology.

Results:

Magnetic resonance imaging delineated the tumors in the cerebellum, frontal lobes and skull base in all mouse models. The detection of intracranial tumors was enhanced with prior administration of gadolinium, and the limit of resolution of brain tumors in the mice was 1-2 mm3. Sequential images performed at different time intervals showed progressive tumor growth in all animals. The MR images of tumor size and location correlated accurately with the results of the histopathological analysis.

Conclusion:

Magnetic resonance imaging of murine brain tumors in different intracranial compartments is feasible with a 1.5 T clinical MR system and a specially designed surface coil. Tumors as small as 1-2 mm3 can be detected with good image resolution. Mice harbouring nascent brain tumors can be followed sequentially by serial MR imaging. This may allow for a noninvasive means by which tumor growth can be measured, and novel therapies tested without resorting to sacrifice of the mice.
Dexamethasone for Morbidity After Subdural Electrode Insertion – A Randomized Controlled Trial
Authors: Sahjpaul Ramesh L. , Mahon Jeff , Wiebe Samuel
Published: December 2014

Background:

Invasive monitoring with subdural electrodes (SDE) for investigation of medically intractable epilepsy may be associated with undesirable immediate postoperative morbidity such as headache, nausea, vomiting, fever, and meningism. We undertook to evaluate the potential beneficial role of perioperative dexamethasone in reducing these symptoms.

Methods:

In a double-blind placebo controlled clinical trial 30 patients undergoing SDE insertion were randomized to receive either placebo or a course of dexamethasone beginning one hour prior to surgery and tapering to discontinue over 72 hours postoperatively. Pain, pain relief, nausea, nausea relief, temperature, and meningism were assessed regularly in the postoperative period, and analgesic, antipyretic, and antiemetic drug requirements were tabulated.

Results:

One patient was withdrawn from the dexamethasone group due to lack of data. With regards to postoperative pain, the direction of benefit favoured dexamethasone but a significant treatment by time interaction prevented further analysis of treatment effect. The dexamethasone group did have significantly lower temperatures and higher nausea relief scores. There was no statistically significant difference between the groups with regards to pain relief, nausea, and meningism scores. The beneficial effects of dexamethasone were delayed in onset, of limited duration, and not uniform over the observation period.

Conclusion:

Dexamethasone appears to have a role in reducing immediate morbidity following SDE insertion but its effect is not uniform in the postoperative period; it appears to be delayed in onset, and of limited duration. Further study is necessary to determine the ideal dosing schedule.
Subacute Sclerosing Panencephalitis Presenting with Unilateral Periodic Myoclonic Jerks
Authors: Shivji Zaitoon M. , Al-Zahrani Ibrahim S. , Al-Said Yousef A. , Jan Mohammed M.S.
Published: December 2014

Background:

Subacute sclerosing panencephalitis (SSPE) is a rare complication of measles virus infection. The disease is characterized by behavioural abnormalities, intellectual deterioration, motor weakness, and generalized myoclonic jerks progressing to coma and death in one to two years in 80% of the cases. The myoclonic jerks are associated with characteristic generalized slow periodic complexes on electroencephalography (EEG). The symptoms and signs of SSPE are frequently quite variable. The clinical course is equally variable and difficult to predict. The characteristic periodic myoclonus can rarely occur unilaterally particularly in the early stages of the disease. As well, the periodic EEG complexes have been reported unilaterally in up to 3% of cases.

Case Report:

A 12-year-old boy, who was seen at a later stage with atypical manifestation of myoclonic body jerks confined entirely unilaterally, combined with contralateral periodic EEG complexes. One could assume clinically that the more diseased hemisphere was responsible for generating the jerks. However, brain magnetic resonance imaging revealed asymmetric hemispheric changes suggesting that the less neurologically damaged hemisphere is responsible for generating the unilateral myoclonic jerks. This has led to the interpretation that the more severely damaged hemisphere has lost the neuronal connectivity required to generate these periodic myoclonic jerks.

Conclusion:

Subacute sclerosing panencephalitis may have asymmetric hemispheric involvement, not only early, but also in the advanced stages of the disease, which can result in unilateral periodic myoclonic jerks.
Clinical Manifestations of Parkinson's Disease and Parkinsonism
Authors: Hobson Doug Everett
Published: December 2014

The most common disorder in a patient presenting to a movement disorder clinic will be parkinsonism. The challenge is to provide the patient with the most accurate diagnosis and prognosis possible. The assumption at the time of initial presentation of the clinical diagnosis of Parkinson's disease is often wrong (20-25%). Waiting to see the pattern of progression, and response to medication provides invaluable additional information. This manuscript summarizes the clinical manifestations of Parkinson's disease and the main akinetic-rigid syndromes (progressive supranuclear palsy, multiple system atrophy, cortical-basal ganglionic degeneration, and dementia with Lewy bodies) that make up the differential diagnosis.
Myoclonus
Authors: Rivest Jean
Published: December 2014

Myoclonus refers to brief muscle jerks caused by neuronal discharges. Etiologies are numerous, ranging from physiological jerks to myoclonus secondary to severe neurodegenerative conditions. The source of myoclonus may be in the cerebral cortex, the brain stem or the spinal cord and multiple generators may be involved in a single patient. The clinical approach to myoclonus relies on both etiological and physiological classifications. Pharmacological therapy is largely based on the presumed site of origin of myoclonus. Polytherapy may be required, particularly in severe cases of cortical myoclonus.
Gilles de la Tourette's Syndrome
Authors: Faridi Kia , Suchowersky Oksana
Published: December 2014

Tourette's syndrome (TS) is a childhood onset neurological disorder characterized by motor and vocal tics. It may be associated with a number of co-morbidities including attention deficit hyperactivity disorder, obsessive compulsive symptomatology, and behaviour disorders. Prevalence of TS is higher than previously thought, and may be present in up to 2% of the population. Tourette's syndrome has a significant genetic component. Inheritance may involve several mechanisms including autosomal dominant, bilinear, or polygenic mechanisms. Pathophysiology is still unknown, although is thought to involve striatocortical circuits. Treatment begins with modification of the work and home environment. For more severe cases, medications such as tetrabenazine and neuroleptics may be helpful. Treatment of co-morbidities needs to be considered, as these may result in more disability than the tics themselves.
Antipsychotic Drug-Induced Movement Disorders
Authors: Blanchet Pierre J.
Published: December 2014

Very early in the process of diagnosing abnormal involuntary movement (AIM) disorders, one can be rewarded by keeping a high index of suspicion for possible drug-induced causes, not only through a complete list of current medications, but also identification of the drugs the patient used to take and other possible offending medications that might be available from family members and other sources. Among drug-induced movement disorders, antipsychotic drugs and other dopamine receptor blocking agents occupy a central place. Their various acute and tardive motor complications provide the template of this short review. Movement disorders caused by antidepressants, lithium, antiemetics, antiparkinsonian agents, anticonvulsants, calcium channel blockers, sympathomimetics and others are only briefly covered in table form.
Chronic Daily Headache in Children and Adolescents
Authors: Seshia Shashi S.
Published: December 2014

Objective:

Characterize chronic daily headache in those less than 20 years of age. Material and

Methods:

Study design: Prospective, observational, and sequential.

Setting:

Private practice Pediatric Neurology Clinic in a Canadian city (Winnipeg).

Patients and data collection:

Data on those referred with headache between September 1998 and December 2001 were entered on data sheets. Patients were followed up for one month to four years.

Results:

One hundred and forty-three (31%) of 463 referred with headache had chronic daily headache with duration of one month to five years (median: eight months). The age range was 5.5 years to 20 years (median: 13 years). There were significantly more females (N=93) than males (N=50). The main groups were: (1) transformed migraine: 6 (4%), (2) transformed tension-type headache: 80 (56%) and (3) transformed comorbid migraine and tension-type headache: 39 (27%). Tension-type features dominated in 94%. Analgesia overuse occurred in two. Stressors were recognized in 60 (42%); anxiety disorder was diagnosed in 8 (6%), and depression in 13 (9%). Computed tomography scans were done in 31 (22%), and MRI/MR angiography in 8 (6%) and were normal or showed nonspecific incidental findings. Twenty-two (15%) were lost to follow-up; 115 of the remaining 121 (95%) were headache free or greatly improved, 63 (55%) without specific treatment.

Conclusion:

Chronic daily headache is a common headache disorder in children and adolescents, especially in teenage girls. A prospective neuropsychiatric approach is necessary for evidenced-based management, since the condition has mental health, social and economic ramifications.
Comparison of Monitoring Techniques for Intraoperative Cerebral Ischemia
Authors: Rowed David W. , Houlden David A. , Burkholder Lee M. , Taylor Amanda B.
Published: December 2014

Objective:

To prospectively compare somatosensory evoked potentials, electroencephalography (EEG) and transcranial Doppler ultrasound (TCD) for detection of cerebral ischemia during carotid endarterectomy (CEA).

Methods:

Somatosensory evoked potentials and EEG recordings were attempted in 156 consecutive CEAs and TCD was also attempted in 91 of them. Recordings from all three modalities were obtained for at least 10 minutes before CEA, during CEAand for at least 15 minutes after CEA. Somatosensory evoked potentials peak-to-peak amplitude decrease of >50%, EEG amplitude decrease of >75%, and ipsilateral middle cerebral artery mean blood flow velocity (mean VMCAi) decrease >75% persisting for the entire period of internal carotid artery occlusion were individually considered to be diagnostic of cerebral ischemia. Clinical neurological examination was performed immediately prior to surgery and following recovery from general anaesthesia.

Results:

Somatosensory evoked potentials, EEG, and TCD were successfully obtained throughout the entire period of internal carotid artery occlusion in 99%, 95%, and 63% of patients respectively. Two patients (1.3%) suffered intraoperative cerebral infarction detected by clinical neurological examination and subsequent magnetic resonance imaging. Somatosensory evoked potentials accurately predicted intraoperative cerebral infarction in both instances without false negatives or false positives, EEG yielded one false negative result and no false positive results and VMCAi one true positive, four false positive and no false negative results. Transcranial Doppler ultrasound detection of emboli did not correlate with postoperative neurological deficits. Nevertheless the sensitivity and specificity of each test was not significantly different than the others because of the small number of disagreements between tests.

Conclusion:

A >50% decrease in the cortically generated P25 amplitude of the median somatosensory evoked potentials, which persisted during the entire period of internal carotid artery occlusion, appears to be the most reliable method of monitoring for intraoperative ischemia in our hands because it accurately detected both intraoperative strokes with no false positive or false negative results.
Magnetic Resonance Diffusion W Imaging in Cerebral Fat Embolism
Authors: Marshall G.B. , Heale V.R. , Herx L. , Abdeen A. , Mrkonjic L. , Powell J. , Sevick R.J. , Morrish W.
Published: December 2014

The use of diffusion weighted imaging with apparent diffusion coefficient mapping in the diagnosis of cerebral fat embolism is shown here to demonstrate infarcts secondary to fat emboli more intensely than T2 weighted sequences 24 hours after the onset of symptoms. Embolic foci are hypointense on apparent diffusion coefficient mapping consistent with cytotoxic edema associated with cell death and restricted water diffusion. This technique increases the sensitivity for detecting cerebral fat embolism and offers a potentially important tool in its diagnosis.
Spinal Injuries in Canadian Ice Hockey: Documentation of Injuries Sustained from 1943-1999
Authors: Tator C.H. , Provvidenza C.F. , Lapczak L. , Carson J. , Raymond D.
Published: December 2014

Objectives:

Study objectives were: (a) to examine the causes and incidence of major spinal cord injuries sustained by ice hockey players; and (b) to add recently reported Canadian cases to the Canadian Ice Hockey Spinal Injury Registry to determine the effectiveness of prevention efforts.

Methods:

The study was a review of questionnaires returned retrospectively by physicians and other sources reporting ice hockey related spinal injuries in Canada. Physicians reported on the mechanism of injury, vertebral level of injury, presence of neurologic deficit, type of event, and type of fracture.

Results:

Between 1943 and 1999, 271 major spinal injuries were reported in Canadian ice hockey players, of which 49.0% occurred to players 16-20 years of age. Ontario has had a disproportionately large number of injuries compared to some provinces, especially Quebec. Of the spinal cord injuries, 65.8% resulted from colliding with the boards, and 36.6% were due to players being pushed or checked from behind. The recent survey shows that there has been a decline in the number of major spinal cord injuries in Canadian ice hockey, especially those causing paralysis due to checking or pushing from behind.

Conclusion:

Impact of the head with the boards after being checked or pushed from behind was the most common mechanism of spinal cord injury. Injury prevention programs are becoming effective in reducing the overall number of injuries, especially those due to checking from behind. Greater awareness of the occurrence and mechanisms of injury through educational programs and rules changes by organized hockey have reduced the annual incidence of catastrophic spinal injuries in Canadian ice hockey.
Rotational Acceleration Measurements - Evaluating Helmet Protection
Authors: Kis M. , Saunders F. , Hove M.W. ten , Leslie J.R.
Published: December 2014

Purpose:

Current helmet testing standards do not address the rotational components of an impact to the head. We describe a new testing paradigm used to measure the rotational acceleration of a headform and a protective helmet following an impact to the head in the horizontal plane. This impact simulation allows for the testing of currently available head protection devices in conditions thought to be important for the generation of cerebral concussion. The degree to which a particular helmet dampens rotational acceleration, and thus protects against concussion, can be assessed.

Methods:

Our testing device consists of a pneumatic piston that provides a measured impact to a standard headform. Four different helmets were tested using the described paradigm.

Results:

Acceleration curves for each helmet and the corresponding headform are presented.

Conclusion:

Clear differences in rotational acceleration were demonstrated. Possible avenues of further investigation are discussed.
Benign Febrile Myoclonus in Childhood
Authors: Dooley J.M. , Hayden J.D.
Published: December 2014

Background:

Myoclonus is often associated with progressive myoclonic epilepsy or neurodegenerative conditions. Febrile myoclonus is a benign phenomenon, which has only been reported previously in one child.

Methods:

The clinical features of three children with fever-induced myoclonus are described.

Results:

Fever-induced myoclonus is characterized by frequent myoclonus, which resolves with resolution of the fever in otherwise healthy children.

Conclusion:

Recognition of fever-induced myoclonus as a benign phenomenon may prevent unnecessary investigations and interventions.

Conclusion:

Le fait de reconnaître le myoclonus induit par l'hyperthermie comme un phÉnomène bÉnin peut Éviter des investigations et des interventions inutiles.
The Academic Half-Day in Canadian Neurology Residency Programs
Authors: Chalk Colin
Published: December 2014

Background:

The academic half-day (AHD) appears to have become widespread in Canadian neurology residency programs, but there is little published information about the structure, content, or impact of the AHD.

Methods:

A written questionnaire was sent to the directors of all active Canadian adult and child neurology residency programs.

Results:

All 21 program directors responded. An AHD was operating in 15/15 adult and 5/6 child neurology programs. The AHD typically lasts three hours, and occurs weekly, 10 months per year. Most of the weekly sessions are lectures or seminars, usually led by clinicians, with about 90% resident attendance. Course-like features (required textbook, examinations) are present in many AHDs. There is a wide range of topics, from disease pathophysiology to practice management, with considerable variation between programs.

Conclusion:

Almost all Canadian neurology programs now have an AHD. Academic half-days are broadly similar in content and format across the country, and residents now spend a substantial portion of their training attending the AHD. The impact of the AHD on how residency programs are organized, and on the learning, clinical work, and professional development of residents merits further study.
Metastatic Hemangiopericytoma Presenting as an Epidural Spinal Cord Lesion
Authors: Brass Steven David , Guiot Marie-Christine , Albrecht Steffen , Glikstein Rafael , Mohr Gérard
Published: December 2014

Objective:

Hemangiopericytoma is an aggressive vascular tumour that rarely affects the central nervous system and is even more rarely spinal in presentation. The clinical features of a patient with a recurrent extraspinal hemangiopericytoma presenting with an epidural spinal cord compression by local invasion are described, including a review of the literature on metastatic hemangiopericytoma to the spine.

Methods:

A case of a 53-year-old male, with a recurrent extraspinal hemangiopericytoma which metastasized to the thoracic spine five years after detection of the primary tumour is presented. A chart review was conducted where all pertinent history, physical, laboratory, and radiological data were collected. A Pub-Med search using the keyword “hemangiopericytoma” identified all reported cases documenting clinical features, treatment, recurrence and outcome with respect to metastatic hemangiopericytoma to the spine.

Results:

Nine patients have been reported to have metastatic hemangiopericytoma to the spine. The median patient age was 47 years and there was a slight male preference. An unusual feature of the hemangiopericytoma is the prolonged period, up to 16 years, between the diagnosis of the primary hemangiopericytoma and the metastases to the spine. All patients were treated with a combination of radiation and surgery.

Conclusion:

Hemangiopericytomas show a slow clinical evolution with a strong propensity to relapse long after previous treatment and thus, once identified, prolonged follow-up for recurrence is indicated. A close follow-up of these patients is required because of frequent recurrences and delayed metastases even if the primary lesion was well-controlled. Although overall uncommon, hemangiopericytoma should be kept in mind in the differential diagnosis of vascular epidural spinal cord tumours.
REM Sleep Behaviour Disorder in a Child with Tourette's Syndrome
Authors: Trajanovic Nikola N. , Voloh Inna , Shapiro Colin M. , Sandor Paul
Published: December 2014

Purpose:

To describe an association of Tourette's syndrome with rapid eye movement sleep behaviour disorder (RBD) in a prepubescent boy.

Methods:

A four year longitudinal single-case study.

Results:

The co-existence of Tourette's syndrome and RBD was confirmed after polysomnographic studies using the standard criteria. The authors propose possible overlap in the pathophysiological mechanisms underlying the two disorders.
Giovanni Aldini: From Animal Electricity to Human Brain Stimulation
Authors: Parent André
Published: December 2014

Two hundred years ago, Giovanni Aldini published a highly influential book that reported experiments in which the principles of Luigi Galvani (animal electricity) and Alessandro Volta (bimetallic electricity) were used together for the first time. Aldini was born in Bologna in 1762 and graduated in physics at the University of his native town in 1782. As nephew and assistant of Galvani, he actively participated in a series of crucial experiments with frog's muscles that led to the idea that electricity was the long-sought vital force coursing from brain to muscles. Aldini became professor of experimental physics at the University of Bologna in 1798. He traveled extensively throughout Europe, spending much time defending the concept of his discreet uncle against the incessant attacks of Volta, who did not believe in animal electricity. Aldini used Volta's bimetallic pile to apply electric current to dismembered bodies of animals and humans; these spectacular galvanic reanimation experiments made a strong and enduring impression on his contemporaries. Aldini also treated patients with personality disorders and reported complete rehabilitation following transcranial administration of electric current. Aldini's work laid the ground for the development of various forms of electrotherapy that were heavily used later in the 19th century. Even today, deep brain stimulation, a procedure currently employed to relieve patients with motor or behavioral disorders, owes much to Aldini and galvanism. In recognition of his merits, Aldini was made a knight of the Iron Crown and a councillor of state at Milan, where he died in 1834.
Overview of Traumatic Brain Injury Patients at a Tertiary Trauma Centre
Authors: Guise Elaine de , Feyz Mitra , LeBlanc Joanne , Richard Sylvain-Luc , Lamoureux Julie
Published: December 2014

Objective:

The goal of this study was to provide a general descriptive and cognitive portrait of a population with traumatic brain injury (TBI) at the time of their acute care stay.

Material and methods:

Three hundred and forty-eight TBI patients were assessed. The following data were collected for each patient: age, level of education, duration of post-traumatic amnesia, Galveston Orientation Amnesia Test score, Glasgow Coma Scale score, results of cerebral imaging, Neurobehavioral Rating Scale score, the Functional Independence Measure cognitive score and the Glasgow Outcome Scale score.

Results:

The clinical profile of the population revealed a mean age of 40.2 (±18.7) and a mean of 11.5 (±3.6) years of education. Most patients presented with frontal (57.6%) and temporal (40%) lesions. Sixty-two percent had post-traumatic amnesia of less than 24 hours. Seventy percent presented with mild TBI, 14% with moderate and 15% with severe TBI. The cognitive deficits most frequently observed on the Neurobehavioral Rating Scale were in the areas of attention, memory and mental flexibility as well as slowness and mental fatigability. Most patients had good cognitive outcome on the Functional Independence Measure and scores of 2 and 3 were frequent on the GOS. Forty-five percent of the patients returned home after discharge, 51.7% were referred to in or out patient rehabilitation and 3.2% were transferred to long-term care facilities.

Conclusion:

Because of the specialized mandate of acute care institutions, the information provided here concerning characteristics of our TBI population is essential for more efficient decision-making and planning/programming with regards to care and service delivery.
Reducing Hardware-Related Complications of Deep Brain Stimulation
Authors: Constantoyannis Constantine , Berk Caglar , Honey Christopher R. , Mendez Ivar , Brownstone Robert M.
Published: December 2014

Background:

Deep brain stimulation (DBS) is used increasingly worldwide for the treatment of Parkinson's disease, dystonia, tremor and pain. As with any implanted system, however, DBS introduces a new series of problems related to its hardware. Infection, malfunction and lead migration or fracture may increase patient morbidity and should be considered when evaluating the risk/benefit ratio of this therapy. This work highlights several factors felt to increase DBS hardware complications.

Methods:

The authors undertook a prospective analysis of their patients receiving this therapy in two Canadian centres, over a four-year period.

Results:

One hundred and forty-four patients received 204 permanent electrode implants. The average follow-up duration was 24 months. Complications related to the DBS hardware were seen in 11 patients (7.6%). There were two lead fractures (1.4%) and nine infections (6.2%) including two erosions (1.4%). There was a significantly greater risk of infection in patients who underwent staged procedures with externalization. In patients with straight scalp incisions, the rate of infection was higher than that seen with curved incisions.

Conclusion:

Hardware complications were not common. A period of externalization of the electrodes for a stimulation trial was associated with an increased infection rate. It is also possible that a straight scalp incision instead of curvilinear incision may lead to an increase in the rate of infection. With a clear understanding of the accepted DBS device indications and their potential complications, patients may make a truly informed decision about DBS technology.
Pseudoephedrine-induced Hemorrhage Associated with a Cerebral Vascular Malformation
Authors: Baker Steven K. , Silva Jamie E. , Lam Ken K.S. , Baker Steven K.
Published: December 2014

Background:

Sympathomimetic-related intracerebral hemorrhage is well-documented. Most cases are associated with phenylpropanolamine use.

Case Report:

We report a case of intracerebral hemorrhage occurring in a middle-aged man who suffered from chronic sinusitis and had been ingesting pseudoephedrine daily for one year. This patient was previously well with no known cardiovascular risk factors. Clinical examination revealed no evidence of vasculitis nor coagulopathy and initial neuroimaging (i.e., computed tomography, angiography, magnetic resonance imaging) demonstrated no features consistent with aneurysm, arteriovenous malformation (AVM), cavernoma, nor cerebral metastases. A follow-up cerebral angiogram demonstrated a small AVM arising off a branch of the pericallosal artery and a small arteriovenous fistula arising off the costal marginal branch. The AVM was embolized without incident, however, the AVF was not accessible.

Conclusions:

Sympathomimetics have long been associated with intracerebral hemorrhage. Since 1979, over 30 published case reports have documented the relationship between phenylpropanolamine and stroke. Only one report links phenylpropanolamine consumption to an intracerebral hemorrhage in a patient with an AVM. There is a paucity of literature etiologically inculpating other ephedra alkaloids in the causation of intracerebral hemorrhage. This is a case of pseudoephedrine-induced intracerebral hemorrhage in a patient with an underlying vascular malformation.
Adrenomyeloneuropathy: Report of a New Mutation in a French Canadian Female
Authors: Dionne Annie , Brunet Denis , McCampbell Alexander , Dupré Nicolas
Published: December 2014

Background:

X-linked adrenoleukodystrophy is a peroxisomial disorder caused by mutations in the ABCD1 gene. Adrenomyeloneuropathy is the second most frequent phenotype (25-46%) of this disease and classically presents in adulthood with spastic paraparesis. Female heterozygotes can be symptomatic, but they are frequently misdiagnosed as having multiple sclerosis.

Case report:

We report a novel missense mutation in the ABCD1 gene in a 47-year-old French-Canadian female with spastic paraparesis and no confirmed family history of X-linked adrenoleukodystrophy. The mutation is located on exon 1 and causes the amino acid substitution of a valine for an alanine in a region of the protein highly conserved between mouse and man.

Conclusion:

Adrenomyeloneuropathy must be considered in the differential diagnosis of spastic paraparesis in men or women. This is an initial report of an ABCD1 gene mutation in the French-Canadian population, which should lead to the recognition of other cases in the future.
Canadian Association of Neuroscience Review: Respiratory Control and Behavior in Humans: Lessons from Imaging and Experiments of Nature
Authors: Moss Immanuela Ravé
Published: December 2014

The purpose of this review is to demonstrate that respiration is a complex behavior comprising both brainstem autonomic control and supramedullary influences, including volition. Whereas some fundamental mechanisms had to be established using animal models, this review focuses on clinical cases and physiological studies in humans to illustrate normal and abnormal respiratory behavior. To summarize, central respiratory drive is generated in the rostroventrolateral medulla, and transmitted to both the upper airway and to the main and accessory respiratory muscles. Afferent feedback is provided from lung and muscle mechnoreceptors, peripheral carotid and aortic chemoreceptors, and multiple central chemoreceptors. Supramedullary regions, including cortex and subcortex, modulate or initiate breathing with volition, emotion and at the onset of exercise. Autonomic breathing control can be perturbed by brainstem pathology including space occupying lesions, compression, congenital central hypoventilation syndrome and sudden infant death syndrome. Sleep-wake states are important in regulating breathing. Thus, respiratory control abnormalities are most often evident during sleep, or during transition from sleep to wakefulness. Previously undiagnosed structural brainstem pathology may be revealed by abnormal breathing during sleep. Ondine's curse and 'the locked-in syndrome' serve to distinguish brainstem from supramedullary regulatory mechanisms in humans: The former comprises loss of autonomic respiratory control and requires volitional breathing for survival, and the latter entails loss of corticospinal or corticobulbar tracts required for volitional breathing, but preserves autonomic respiratory control.
Visual-Sensitive Epilepsies: Classification and Review
Authors: Seshia Shashi S. , Carmant Lionel
Published: December 2014

Photosensitivity, photosensitive seizures, and photosensitive epilepsy are discussed. The International League against Epilepsy has suggested the term “visual-sensitive” replace “photosensitive”. Visual-sensitive seizures may be more common than is realized. A classification for visual-sensitive epilepsies is presented. Chromosomal and DNA testing may help to refine the classification further. A standardized approach for neurophysiologic testing, such as that proposed by European experts, is recommended. These steps should promote evidenced-based management for this group of disorders.
Inventory of Pediatric Neurology “Manpower” in Canada
Authors: Keene Daniel L. , Humphreys Peter
Published: December 2014

Objective:

To review the demographics and workload characteristics of pediatric neurology in Canada.

Method:

A standardized survey questionnaire was mailed out to practicing pediatric neurologists in Canada in 2001. Variables examined were age, gender, hours on call, regular hours worked per week, type of practice and projected changes in practice over next five to ten years. Results were compared to the 1994 Pediatric Neurology Manpower Survey which had used the same survey instrument.

Results:

Fifty-six (70%) pediatric neurologists practicing in Canada returned the survey. As was the case in 1994, no significant differences in workload were found based on age or gender. The average age of the practicing pediatric neurologist in 2001 was 51 years compared to 45 years in 1994. The proportion of physicians over 55 years in 2001 was 35% compared to 25% in 1994.

Conclusions:

Pediatric neurology in Canada is an aging specialty needing a significant recruitment of new members.
Clinical and Biochemical Heterogeneity in an Italian Family with CPT II Deficiency due to Ser 113 Leu Mutation
Authors: Rafay Mubeen F. , Murphy E. Gordon , McGarry J. Denis , Kaufmann Petra , DiMauro Salvatore , Tein Ingrid
Published: December 2014

Background:

Carnitine palmitoyltransferase II (CPT II) deficiency is an autosomal recessive disorder which presents with recurrent myoglobinuria. Heterozygotes are usually asymptomatic.

Methods:

We correlate the clinical, biochemical and molecular features of a family in which the proband is homozygous for CPT II deficiency, due to the common Ser 113 Leu mutation.

Results:

The 20-year-old female proband presented at age three years with episodic myalgia and myoglobinuria, elevated creatine kinase (CK) of 3600 IU/L and had a 33% residual CPT II activity in cultured skin fibroblasts. Her 25-year-old dizygotic twin brothers presented with muscle stiffness following prolonged exercise but no overt pigmenturia and had interictal CKs up to 662 IU/L. Her parents and a 13-year-old brother are asymptomatic. An elder sister, not investigated, had recurrent pigmenturia and died at eight years with myoglobinuria. Molecular analysis revealed that the proband is homozygous for the Ser 113 Leu mutation. Her parents are heterozygotes with CPT II activities of 55% to 70%. Her younger brother is normal with 83% activity. The symptomatic twin brothers are heterozygous but demonstrated unexpectedly low CPT II activities of 40%, which may explain their phenotype.

Conclusion:

We postulate that there may be genetic, environmental and sex hormonal factors accounting for this manifesting heterozygosity and biochemical heterogeneity in CPT II deficiency.