bluuebunny/crossref_metadata_2025 huggingface dataset:
The Pentapeptide QYNAD Does Not Inhibit Neuronal Network Activity
Authors: Otto F. , Kieseier B.C. , Görtz P. , Hartung H.-P. , Siebler M.
Published: December 2014

Background:

Controversial data was published about the sodium channel-blocking effect of the endogenous pentapeptide QYNAD, which is elevated in patients with multiple sclerosis and Guillain-Barré-syndrome. In some experiments with single cells and nerve preparations QYNAD inhibited sodium currents to the same extent as the known sodium channel blocker lidocaine whereas in other laboratory testing QYNAD failed to show any effect at all.

Methods:

Micro-electrode arrays with cultured neuronal networks are highly suitable to determine neuroactive activity of applied substances. The impact on electrophysiological parameter changes was compared between QYNAD and the established sodium channel blockers lidocaine and tetrodotoxin (TTX).

Results:

QYNAD did not alter network activity whereas the sodium channel blockers lidocaine (IC50 14.9 µM) and tetrodotoxin (IC50 1.1 nM) reversibly decreased network activity in similar concentrations as in patch-clamp experiments. This decrease of spontaneous electrophysiological activity was achieved by prolonging the interburst-interval.

Conclusion:

Although QYNAD might have mild effects on single-cell sodium currents, there is no significant effect on neuronal network function. These results raise concerns about QYNAD exhibiting a relevant impact on functional disability of the central nervous system in patients.
Processing and Interpretation Times of CT Angiogram and CT Perfusion in Stroke
Authors: Srinivasan Ashok , Goyal Mayank , Lum Cheemun , Nguyen Thanh , Miller William
Published: December 2014

Objective:

To determine the mean time for acquiring computed tomogram perfusion (CTP) and CT angiogram (CTA) images in acute stroke. To determine and compare processing and interpretation times amongst three groups of radiologists with varying degree of expertise: two radiology residents (Group I), two neuroradiology fellows (Group II) and four consultant neuroradiologists (Group III).

Methods:

The mean time of acquisition of CTA and CTP studies was calculated among ten patients presenting with acute stroke. All readers had to process the CTA and CTP images, interpret them (for presence or absence of thrombus and penumbra) and save them on the GE Advantage Windows workstation. The mean time for processing and interpreting these studies was calculated.

Results:

The mean time for acquisition of CTA and CTP studies in the ten patients was 14.6 ± 5.9 minutes. The time taken for CTA processing and interpretation in Groups I, II and III was 2.3 ± 1.3 min, 1.6 ± 0.4 min and 1.5 ± 0.7 min respectively. The time required for CTP processing and interpretation by the same groups was 5.2 ± 1.7 min, 4.5 ± 1.5 min and 4.1 ± 1.1 min respectively. There was a statistically significant difference of means between Groups I and III in the CTA and CTP processing and interpretation times (p=0.02, p=0.01 respectively) but no statistical difference between Groups I and II (p=0.15, p=0.22 respectively) or Groups II and III (p=0.31, p=0.30 respectively).

Conclusion:

The CTA and CTP studies can be performed, processed and interpreted quickly in acute stroke.
Vertebroplasty in Osteoporotic Spine Fractures: A Quality of Life Assessment
Authors: Kumar Krishna , Verma A.K. , Wilson Jefferson , LaFontaine Alika
Published: December 2014

Objective:

Our goal was to perform a quantitative evaluation of the improvement in functional capacity, quality of life, mental function, reduction in drug intake and impact on hospital admissions after vertebroplasty in the treatment of osteoporotic compression fractures. The efficacy of vertebroplasty in relief of pain has been addressed in previous publications but the quantitative evaluation of improvement in quality of life has not been addressed before.

Methods:

This is a prospective study of 42 patients with 83 symptomatic vertebral fractures treated by vertebroplasty with a mean follow-up of 9.1 months. The outcome was measured by pre and postoperatively utilizing the Visual Analogue Scale, the Oswestry Disability Index, the Rolland Morris Scale for Back Pain and EuroQol-5D questionnaire (EQ-5D). The postoperative evaluations were performed at one week, one month, three month, and six month intervals thereafter.

Results:

In 34 out of 39 active patients, marked pain relief was noted (87%). The Visual Analogue Scale score improved from a mean preoperative score of 8.2 to a mean postoperative score of 2.9 (p=0.0000003) at one week follow up and 3.9 at the last follow-up. The Rolland Morris Scale for Back Pain showed a drop from a mean preoperative rating of 13 to a mean postoperative rating of 10, showing a 25% improvement (p= 0.0207). The Oswestry Disability Index preoperatively was 64.4 which improved to 43.8 postoperatively, showing a 32% improvement (p= 0.0207). The EQ-5D showed a mean preoperative index value of 0.097 and mean postoperative index value of 0.592 (p = 0.0000003). All p-values were determined by the Willcoxin sign-ranked test.

Conclusion:

Vertebroplasty is a safe and efficacious procedure with a resulting improvement in pain and quality of life.
A Reappraisal of Rhythmic Coma Patterns in Children
Authors: RamachandranNair Rajesh , Sharma Rohit , Weiss Shelly K. , Otsubo Hiroshi , Cortez Miguel A.
Published: December 2014

Objective:

This study was designed to determine the prevalence of rhythmic coma patterns in comatose children and to ascertain the prognostic significance of reactive rhythmic coma patterns.

Methods:

We retrospectively analyzed and classified electroencephalogram (EEGs) in comatose children between two months and 18 years of age during the period 1996 - 2003 according to modified Young's classification. Outcome at one-year was scored according to the Paediatric Cerebral and Overall Performance Category Scale. Outcomes were compared using Fisher's exact test and Mann-Whitney test.

Results:

Analysis of 63 electroencephalogram (EEG) records in 38 patients showed rhythmic patterns in 19 records (30.2%; 9 alpha, 4 spindle, 4 theta and 2 beta coma patterns, total number of children = 14). Aetiology and outcome of alpha coma patterns and other rhythmic coma patterns were similar. In five children, one type of rhythmic pattern changed to another. Records with reactive rhythmic coma 66.7% (6/9), were associated with favourable outcome. Sixty percent of the records (6/10 records in seven children) with non-reactive pattern were associated with unfavourable outcome. This clinically significant difference did not reach statistical significance (lower Paediatric Cerebral and Overall Performance Category Scale score p= 0.14; favourable outcome p=0.19).

Conclusion:

Rhythmic coma patterns in comatose children are not uncommon. Aetiology, reactivity and outcome of individual patterns are similar and thus make the rhythmic coma patterns distinct EEG signatures in comatose children. There was a clinically significant better outcome with reactive rhythmic coma patterns.
Intracranial Invasion of a Basal Cell Carcinoma of the Scalp
Authors: Mathieu David , Fortin David
Published: December 2014

Objective and importance:

Basal cell carcinomas are common lesions that can have a locally invasive behavior. The authors present a case of a scalp lesion with skull and dura-mater invasion that was unrecognized prior to surgical undertaking.

Clinical presentation:

A 66-year-old female presented with an extensive ulcerating lesion on the frontal scalp, deemed to be a basal cell carcinoma. The plastic surgery team brought her to the operating room for resection but subsequently backed off after the discovery of bony invasion. Imaging revealed extensive bony and epidural extension.

Intervention:

Resection of the invaded frontal scalp, bone, dura-mater and proximal part of the superior sagittal sinus was performed. This was followed by a reconstructive procedure consisting of a duraplasty, a cranioplasty, and a skin rotation flap and partial-thickness skin grafting.

Conclusion:

Radiological evaluation of scalp basal cell carcinomas should be done prior to surgery to rule out any bony or intracranial invasion.
Unemployment in an Underserviced Specialty?: The Need for Co-ordinated Workforce Planning in Canadian Neurosurgery
Authors: Woodrow S.I. , O'Kelly C. , Hamstra S.J. , Wallace M.C.
Published: December 2014

Background:

A recent report suggested that newly trained Canadian neurosurgeons are experiencing difficulty finding employment in Canada. Such occurrences, in combination with recent certification restrictions imposed in the US, have resulted in increasing concern that we will shortly be seeing a surplus of graduating neurosurgeons in Canada. The purpose of this study was to develop a better understanding of training and employment patterns in the Canadian neurosurgical workforce.

Methods:

Using a database provided by the Royal College of Physicians and Surgeons of Canada, the current practice location of recent (1990-2002) neurosurgical certificants and a list of all neurosurgeons practicing in Canada were generated. From these data the number of surgeons per 100,000 patient population, and the number of residents required to maintain this workforce were determined.

Results:

Practice location could be identified for 183/189 individuals who passed their qualifying examination in neurosurgery during this time. Only 45% of them are currently practicing in Canada. The current service ratio for this specialty is 0.65 per 100,000 population overall. Although 14.6 residents/ year are being trained, only 6.5/ year are required to maintain the existing neurosurgical workforce.

Conclusion:

Our data supports the concern about an imminent employment crisis for young neurosurgeons in Canada with more than twice the required number of residents being trained. However, this shortfall of staff positions is at a time when the specialty may be underservicing the country's population. These results highlight the necessity for more cohesive workforce planning in Canada, and in particular, ensuring the appropriate balance between training and need.
Nα -Methyl Histamine Safety and Efficacy in Migraine Prophylaxis: Phase III Study
Authors: Millán-Guerrero Rebeca O. , Isais-Millán Rebeca , Benjamín Trujillo-Hernández , Tene Carlos E.
Published: December 2014

Background:

The histamine catabolite, Nα-methylhistamine, possesses a selective affinity for H3 receptors. For this reason, we considered evaluating the efficacy of this histaminergic H3 agonist in migraine prophylactic treatment.

Objective:

To study the therapeutic potential of the subcutaneous administration of Nα-methylhistamine in migraine prophylaxis, in a Phase III clinical pharmacological study.

Methods:

Using a controlled double-blind, placebo controlled clinical trial for 12 weeks, 60 patients with migraine, who fit the criteria established by the International Headache Society, were selected. The efficacy of subcutaneous administration of Nα-methylhistamine 1 to 3 ng twice a week against placebo was studied, evaluating the outcome of headache intensity, frequency, duration, and analgesic intake.

Results:

Comparison between the groups treated with placebo (n=30) and Nα-methylhistamine (n=30), on data collected for the 4th, 8th and 12th weeks of treatment, revealed that Nα-methylhistamine exerted a significant (p<0.0001) reduction (compared to placebo) in intensity, frequency, and duration of migraine attacks, as well as on the use of analgesic intake. No significant (p>0.05) adverse experiences or side effects developed in either group.

Conclusions:

The present study provides evidence of the efficacy of Nα-methylhistamine, given subcutaneously at doses of 1 to 3 ng twice a week, offering a new therapeutic alternative and laying the clinical and pharmacological groundwork for the use of histaminergic H3-agonists in migraine prophylaxis, which may specifically inhibit the neurogenic edema response involved in migraine pathophysiology.
Reduction in IV t-PA Door to Needle Times Using an Acute Stroke Triage Pathway
Authors: Mehdiratta M. , Woolfenden A.R. , Chapman K.M. , Johnston Dean C. , Schulzer M. , Beckman J. , Teal P.A.
Published: December 2014

Objective:

To determine the effectiveness of an Acute Stroke Triage Pathway in reducing door to needle times in acute stroke treatment with IV t-PA.

Background:

A previous study at our tertiary referral centre, examining IV t-PA door to needle times, was completed in 2000. The median door to needle time was beyond the recommended National Institute for Neurological Disorders and Stroke (NINDS) standard of 60 minutes. In November 2001, an Acute Stroke Triage Pathway was introduced in the emergency room (ER) to address this issue. The goal of this pathway was to rapidly identify patients eligible for treatment for IV t-PA, so that CT scans and lab studies could be arranged immediately upon ER arrival. Our hypothesis was that the Triage Pathway would shorten door to CT and door to needle times.

Design/Methods:

Using retrospective data, pre (n=87) and post (n=47) triage pathway times were compared. The door to CT time was reduced by 11 minutes (p=0.015) and door to needle time was reduced by 18 minutes (p=0.0036) in a subgroup of patients that presented directly to our hospital.

Conclusions:

These results indicate that the Acute Stroke Triage Pathway is effective in reducing Door to CT and Door to Needle Times in patients presenting directly to our ER. However, a majority of treatment times were still beyond NINDS recommendations. Stroke Centers require periodic review of their efficiency to ensure that target times are being obtained and may benefit from the use of an Acute Stroke Triage Pathway.
Brain & Chiasmal Herniations into Sella after Medical Treatment of Prolactinoma
Authors: Bangash M.H. , Clarke D.B. , Holness R.O.
Published: December 2014

Background:

Dopamine agonists are widely used in the treatment of pituitary prolactinomas. We report a case of inferior mesial frontal lobe (gyrus rectus) and chiasmal herniations into an enlarged sella following successful medical treatment of a pituitary macroadenoma.

Method:

A 71-year-old healthy man presented to medical attention with visual complaints. On examination, he was found to have bitemporal hemianopsia. Endocrine evaluation revealed an elevated prolactin level. He was treated medically with a dopamine agonist (bromocriptine).

Results:

Evaluation after one year of medical treatment revealed stabilization of the patient's vision, with a significant bitemporal field loss. Serum prolactin levels normalized (5.16 ng/ml). The MRI of the sella showed almost complete disappearance of the tumor, resulting in right mesial frontal lobe herniation inferiorly into an enlarged sella with associated chiasmal deformation.

Conclusions:

We report a case where successful medical treatment of a large pituitary prolactinoma has resulted in inferior frontal lobe and chiasmal herniatons into an enlarged sella.
Recovery in a Patient with Locked-In Syndrome
Authors: Silver Brian , Grover Kavita M. , Arcila Ximena , Mitsias Panayiotis D. , Bowyer Susan M. , Chopp Michael
Published: December 2014

Background and Purpose:

Sildenafil citrate has been shown to enhance neurogenesis, angiogenesis, synaptogenesis, and neurological outcome by augmentation of cyclic guanosine monophosphate (cGMP) levels in animal models of ischemic stroke. Whether sildenafil citrate may be helpful for recovery in human stroke is unknown at this time.

Methods:

A 41-year-old woman with locked-in syndrome due to pontine infarction began receiving 150 mg of oral sildenafil citrate daily on a compassionate use basis in August 2003 and continues treatment at this time. Magnetoencephalography (MEG) was performed at 12 and 17 months after stroke.

Results:

No serious adverse events have occurred. Significant milestone recoveries including standing, use of both arms, talking, and full return of swallowing have occurred, particularly after nine months of treatment. The MEG showed a significantly increased amplitude in the somatosensory cortex.

Conclusion:

Daily use of high dose sildenafil citrate appears to be safe in this patient with stroke resulting in locked-in syndrome. Further studies will be required to establish safety and efficacy.
Responsabilités Neuroéthiques
Authors: Racine Eric , Illes Judy
Published: December 2014

Neuroscience represents a dynamic area of biomedical research where neuroethical responsibilities for researchers are emerging. This paper is the companion piece to the English-language one also published in this issue of the Canadian Journal of Neurological Sciences. It serves as a review of recent advances in neuroethics through the lens of three cases: (1) incidental finding of anomalies in neuroimaging research; (2) creation of neurotechnologies that can lead to cognitive enhancement, and (3) responsible communication of research results. We propose and discuss a multidimensional framework of neuroethical responsibilities to help tackle these issues. The framework reiterates the fundamental role of scientific integrity, puts in the foreground social responsibilities pertaining to the eventual use of neuroscience knowledge, and highlights self-reflection in research and training of researchers.
Neuroethical Responsibilities
Authors: Racine Eric , Illes Judy
Published: December 2014

Neuroscience represents a dynamic area of biomedical research where neuroethical responsibilities for researchers are emerging. This paper is the companion piece to the French-language one also published in this issue of the Canadian Journal of Neurological Sciences. It serves as a review of recent advances in neuroethics through the lens of three cases: (1) incidental finding of anomalies in neuroimaging research; (2) creation of neurotechnologies that can lead to cognitive enhancement, and (3) responsible communication of research results. We propose and discuss a multidimensional framework of neuroethical responsibilities to help tackle these issues. The framework reiterates the fundamental role of scientific integrity, puts in the foreground social responsibilities pertaining to the eventual use of neuroscience knowledge, and highlights self-reflection in research and training of researchers.
Intractable Childhood Epilepsy and Maternal Fatigue
Authors: Jan Mohammed M.S.
Published: December 2014

Background:

Mothers of children with intractable epilepsy are generally stressed and experience more emotional problems. Fatigue may affect their productivity, social interactions, and their ability to adequately take care of their children. The objectives were to examine the relationship between intractable childhood epilepsy and maternal fatigue, and explore possible contributing factors.

Methods:

Sixty-four consecutive mothers of children with intractable epilepsy were identified prospectively. Exclusion criteria included degenerative/metabolic disorders or life threatening illness, such as brain tumors. Fatigue was measured using a standardized 11-item questionnaire, which has been revalidated in an Arabic speaking population.

Results:

Mothers' ages were 24-45 years (mean 34) and ages of their epileptic children were 1-15 years (mean 6.7). Most children (64%) had epilepsy for >2 years, were on >1 antiepileptic drug (AED) (72%), and had daily seizures (47%). Thirty-four (54%) of the children had motor deficits and 83% had mental retardation (severe in 41%). Twenty-eight (44%) mothers were fatigued. Factors associated with increased maternal fatigue included child's age <2 years (p=0.01), cryptogenic epilepsy (p=0.03), and severe motor deficits (p=0.04). Factors associated with lowered fatigue included performing regular exercise (p=0.006), lack of mental retardation (p=0.01), seizure control (p=0.05), using one AED (p=0.002), infrequent ER visits (p=0.005), and lack of recent hospitalization (p=0.005).

Conclusion:

Mothers of children with intractable epilepsy are increasingly fatigued. Several correlating factors were identified, mostly related to seizure control, mental and physical handicap. Strategies to manage the problem include proper education, seizure control, participation in regular exercise, social support, and psychological counseling.
A Novel GDAP1 Mutation 439delA is Associated with Autosomal Recessive CMT Disease
Authors: Georgiou Domna-Maria , Nicolaou Paschalis , Chitayat David , Koutsou Pantelitsa , Babul-Hirji Riyana , Vajsar Jiri , Murphy Jillian , Christodoulou Kyproula
Published: December 2014

Background:

Charcot-Marie-Tooth (CMT) disease is the most common form of inherited motor and sensory neuropathy. Based on neurophysiological and neuropathological criteria CMT has been sub-classified into two main types: demyelinating and axonal. Furthermore, it is genetically heterogeneous with autosomal dominant, autosomal recessive (AR) and X-linked modes of inheritance. Thus far, seven genes have been identified in association with the demyelinating AR-CMT disease. We hereby report our clinical and molecular genetic findings in a consanguineous family with AR-CMT.

Methods:

Two young sisters with AR-CMT and other non-affected family members were clinically and electrophysiologically evaluated and then molecular genetic investigation was carried out in order to identify the pathogenic mutation.

Results:

Following an initial indication for linkage of the family to the CMT4A locus on chromosome 8, we sequenced the Ganglioside-induced differentiation-associated protein 1 (GDAP1) gene and identified a single nucleotide deletion in exon 3 that is associated with AR-CMT in the family.

Conclusion:

We identified a novel GDAP1 439delA mutation that is associated with AR-CMT in a consanguineous family of Iranian descent with two affected young girls and a history in other members of the family.
Unilateral Hypoglossal Nerve Palsy Following the Use of the Laryngeal Mask Airway
Authors: Lo Timothy S.
Published: December 2014

Hypoglossal nerve injury is a rare complication of anesthetic airway management. We report a case of unilateral hypoglossal nerve injury following the use of the laryngeal mask airway (LMA). A 48-year-old man with no past medical history sustained a proximal humerus fracture. His pre-operative examination was normal except for a humerus fracture. Anesthesia was induced and a size-3 LMA was inserted successfully on first attempt without difficulty. The cuff was inflated with the recommended 20 ml of air until there was no leak and maintained at a pressure of 10-15 cm H20. The surgery was uneventful and the duration of anesthesia was two hours. Peri-operatively the patient was hemodynamically stable without episodes of hypotension. The recovery staff noted approximately three hours after the surgery that his tongue was deviated to the left. The patient complained of difficulty swallowing and slurred speech. He had normal sensation of his tongue, taste was unchanged, and the gag reflex was intact. No other cranial nerves were involved and no other neurological deficiency was found. The following day, there was significantly less dysarthria and slight improvement in tongue movement. Conservative management included speech therapy and regular assessment. He continued to improve and complete recovery took place within two weeks.
Occipital Condyle Fracture with Associated Hypoglossal Nerve Injury
Authors: Chugh Shaan , Kamian Kambiz , Depreitere Bart , Schwartz Michael L.
Published: December 2014

Occipital condyle fracture (OCF) is a rare injury that was first described by Bell in 1817. In fact, there have been only 96 more reported cases of occipital condyle fractures from 1817 to 1994 of which only 58 survived. Occipital condyle fractures can sometimes go unnoticed or under-diagnosed as they are not always evident on plain radiographs of the cervical spine. Also, in rare cases OCFs can cause damage to the hypoglossal nerve which passes through the hypoglossal canal which is near the occipital condyle. The presence of specific symptoms and clinical signs should lead to the correct diagnosis. This paper describes a patient who was diagnosed with OCFs, but not hypoglossal nerve damage until 20 days following admission to hospital. We point out many factors that contributed to this delayed diagnosis, which ultimately caused severe discomfort to the patient.
Fibrous Dysplasia of the Skull Base Presenting Acutely in an Adult
Authors: Amoozegar F. , Guberman A.
Published: December 2014

Fibrous dysplasia (FD) of the bone is an idiopathic disorder in which bone-forming mesenchyme develops abnormally. Here, we describe an unusual case in an adult, with recurrent acute headache and third nerve palsy due to FD of the skull base and low-grade fibrosarcoma.

A previously healthy 32-year-old female office clerk, presented with an eight day history of acute-onset, severe, right occipital and retro-orbital continuous headache, associated with nausea and vomiting. She had required dimenhydrinate, acetaminophen with 15 mg codeine, and meperidine with only mild relief of the headache. She also described a 5-day history of right eye ptosis which had progressed to eye closure by the time of presentation. She had otherwise been well, with no systemic or constitutional symptoms and no previous history of
headaches.
Sepsis from Neurofibromatosis
Authors: Tangri Navdeep , Sirhan Shireen , Crelinsten Gordon
Published: December 2014

Neurofibromatosis Type I or von Recklinghausen’s neurofibromatosis is an autosomal dominant disorder with a high index of spontaneous mutations and extremely varied and unpredictable clinical manifestations. We present a case of sepsis secondary to an infected hematoma, enclosed within a massive neurofibroma.

A 42-year-old man presented to the emergency department with a one week history of fever and chills. He reported an increase in pain and size of a growth near his chest. The patient was noted to be febrile on arrival. On physical examination, a very large neurofibroma was seen extending from the right upper chest. Wound and blood cultures were obtained. Computed Tomography (CT) of the thorax revealed a hematoma contained within the large mass.
Pituitary Carcinoma with Subependymal Spread
Authors: Kumar Krishna , Wilson Jefferson R. , Li Qiuyan , Phillipson Ryan
Published: December 2014

Pituitary carcinomas have been reported to metastasize systemically and, less commonly, along the craniospinal axis. Metastatic lesions have been reported in the cerebral cortex, cerebellum, spinal cord, leptomeninges, cervical lymph nodes, liver, ovaries, and bone. The authors are unaware of any other examples of subependymal metastases of a pituitary carcinoma. We report such a case.

Pituitary carcinoma is rare, accounting for roughly 0.2%1,3-5 of all pituitary tumors, with approximately 140 cases reported in the literature. These tumors are associated with a very high mortality, with 66% of patients dying within the first year after diagnosis. Pituitary carcinomas are differentiated from invasive pituitary adenomas by the presence of non-contiguous craniospinal tumor deposits and/or distant systemic metastases. The majority (88%) of these carcinomas prove to be hormone secreting, with prolactin secreting tumors being the most common. Invasive carcinomas evolve from hormone secreting pituitary adenomas after a latency period. Pituitary carcinomas have a predilection for systemic spread. The rate of systemic metastasis approaches 71% for prolactin producing tumors and 57% for ACTH producing tumors. Thirteen percent of tumors demonstrate both systemic and craniospinal patterns of metastatic spread.
Progress in Clinical Neurosciences: Stroke Recovery and Rehabilitation
Authors: Teasell Robert , Bayona Nestor , Salter Katherine , Hellings Chelsea , Bitensky Jamie
Published: December 2014

Background:

Recent literature has provided new insights into the role of rehabilitation in neurological recovery post-stroke. The present review combines results of animal and clinical research to provide a summary of published information regarding the mechanisms of neural recovery and impact of rehabilitation.

Methods:

Plasticity of the uninjured and post-stroke brain is examined to provide a background for the examination of brain reorganization and recovery following stroke.

Summary and Conclusions:

Recent research has confirmed many of the basic underpinnings of rehabilitation and provided new insight into the role of rehabilitation in neurological recovery. Recovery post stroke is dependent upon cortical reorganization, and therefore, upon the presence of intact cortex, especially in areas adjacent to the infarct. Exposure to stimulating and complex environments and involvement in tasks or activities that are meaningful to the individual with stroke serves to increase cortical reorganization and enhance functional recovery. Additional factors associated with neurological recovery include size of stroke lesion, and the timing and intensity of therapy.
Saccadic Adaptation in Chiari Type II Malformation
Authors: Salman Michael S. , Sharpe James A. , Eizenman Moshe , Lillakas Linda , To Teresa , Westall Carol , Steinbach Martin J. , Dennis Maureen
Published: December 2014

Background:

Saccadic adaptation corrects errors in saccadic amplitude. Experimentally-induced saccadic adaptation provides a method for studying motor learning. The cerebellum is a major participant in saccadic adaptation. Chiari type II malformation (CII) is a developmental deformity of the cerebellum and brainstem that is associated with spina bifida. We investigated the effects of CII on saccadic adaptation.

Method:

We measured eye movements using an infrared eye tracker in 21 subjects with CII (CII group) and 39 typically developing children (control group), aged 8-19 years. Saccadic adaptation was induced experimentally using targets that stepped horizontally 12º to the right and then stepped backward 3º during saccades.

Results:

Saccadic adaptation was achieved at the end of the adaptation phase in participants in each group. Saccadic amplitude gain decreased by 6.9% in the CII group and 9.3% in the control group. The groups did not differ significantly (p = 0.27). Amplitude gain reduction was significantly less in the CII participants who had multiple shunt revisions. Regression analyses revealed no effects of spinal lesion level, presence of nystagmus, or cerebellar vermis dysmorphology on saccadic adaptation.

Conclusion:

The neural circuits involved in saccadic adaptation appear to be functionally intact in CII.
Susac Syndrome with Frontal Intermittent Rhythmic Delta Activity (FIRDA)
Authors: Woolridge Danielle , Stefanelli Mark , Hoppe Barbara
Published: December 2014

Background:

Susac syndrome is a rare condition involving the brain, retina, and cochlea. Electroencephalogram (EEG) findings from published case reports show mainly generalized slowing.

Case report:

A 30-year-old man presented with acute onset of superior vision loss, unsteady gait, and hearing loss. This was accompanied by short-term memory loss and behavioral and mood changes. MRI showed multiple white matter hyperintensities. The EEG showed frontal intermittent rhythmic delta activity. A diagnosis of Susac syndrome was made and treatment with methylprednisolone, ASA, and Nimodipine was instituted. At one-year follow-up, bilateral hearing loss, mild visual impairment, and mood changes were still apparent.

Conclusion:

Frontal intermittent rhythmic delta activity can be seen in Susac syndrome.
West Nile Virus Encephalomyelitis with Polio-like Paralysis & Nigral Degeneration
Authors: Schafernak Kristian T. , Bigio Eileen H.
Published: December 2014

Background:

Patients infected with West Nile virus (WNV) may develop acute neurologic disease, which can be severe or even fatal, including WNV meningitis, encephalitis, and an irreversible acute flaccid paralysis or poliomyelitis-like syndrome. Movement disorders have also been described.

Report:

We report combined neuronal loss, gliosis, and neurofibrillary tangle formation in the substantia nigra of a 41-year-old man with a history of WNV encephalomyelitis and poliomyelitis-like paralysis.

Conclusion:

Clinically our patient did not display parkinsonism, however, it is interesting to speculate whether, in the absence of the residual subacute poliomyelitis-like syndrome, the neuropathologic findings could have eventually evolved clinically into WNV-associated postencephalitic parkinsonism.
Chronic Cluster-Like Headache Secondary to an Epidermoid Clival Lesion
Authors: Massie Rami , Sirhan Denis , Andermann Frederick
Published: December 2014

Cluster headache is recognized by the International Headache Society as a primary headache disorder, consisting of seasonal and daily attacks of severe unilateral periorbital pain accompanied by autonomic symptoms. As with other primary headache disorders, intracranial lesions can rarely mimic cluster headaches, in particular if it presents in an atypical fashion. We report a patient with chronic cluster-like headache secondary to a clival based epidermoid cyst. This is the first such association reported in the literature.

A 39-year-old man with no significant past medical history and no current medications was referred with a three-year history of strictly right-sided headaches. Two to three times daily, he suffered attacks of excruciating pressure-like pain in the periorbital and frontal area on the right. These lasted between 10 and 90 minutes and occasionally woke him from sleep. They
were always accompanied by numbness of the upper part of his face, increased lacrimation, rhinorrhea and ptosis on that side. Occasionally, he had nausea and vomiting as well. Since the
onset of symptoms, attacks occurred almost daily and never remitted for more than four weeks. He tried methysergide, gabapentin, carbamazepine, lithium carbonate and corticosteroids without success. Later, his attacks have occasionally involved the left side.
Unilateral Atrophy of Fungiform Papillae Associated with Lingual Nerve Injury
Authors: Phan Cecile L. , Kashmere Jodi L. , Kalra Sanjay
Published: December 2014

The lingual nerve is at risk of injury during common dental procedures because of its proximity to the inferior alveolar nerve and the roots of the third molar tooth. Lingual nerve injury has been documented following extraction of wisdom teeth, dental anesthetic injections, and other endodontic procedures. We present a case of loss of somatosensory function and taste associated with the intriguing finding of fungiform papillae atrophy as a result of lingual nerve injury.
Outcomes for Assessment of Symptomatic and Stabilization/Disease Modifying Drugs
Authors: Gauthier Serge
Published: December 2014

The safety and efficacy of current symptomatic drugs for AD was established using parallel groups taking different doses of active drugs vs placebo over three to twelve months, whereas drugs with potential stabilizing/disease modifying effects are being tested by adding new compounds or placebo to standard symptomatic drugs over 12 to 18 months. Delaying progression to disease milestones may offer additional validity to these studies. It is unclear if biological and neuroimaging markers will add to the clinical evidence.
Ethical Considerations for the Conduct of Antidementia Trials in Canada
Authors: Fisk John D.
Published: December 2014

Clinical trials in persons with dementia bring into focus the ethical dilemmas frequently confronting the clinician-scientist. Despite the existence of various ethical guidelines, most with common underlying principles, few are specific to dementia. A particular difficulty is finding a balance between respect for the autonomy of the individual and the protection of vulnerable persons, while at the same time defining an acceptable risk/benefit ratio for the study. The availability of symptomatic treatments for Alzheimer's disease also now make it difficult to argue that withholding treatment from those in the placebo arm of a clinical trial fulfills one's duty to provide best care. Those conducting clinical trials must be knowledgeable about existing legislation and ethical guidelines in order to justify to themselves and others, the design of clinical trials and their risks. They must be prepared to educate patients and family members about dementia and research, determine each potential subject's competence to consent, and ensure that decisions about participation are in accordance with the best interests of the subject. Ethical conduct of clinical trials of new antidementia therapies will require that everyone involved understands the values and beliefs that guide their decision-making and the potentially conflicting roles facing the clinician-scientist.
Disease Staging and Milestones
Authors: Rabheru Kiran
Published: December 2014

This new section to the guidelines was added due to the recognition that clinical milestones are useful indices of the progression of dementia in patients with Alzheimer's disease and could help in the development of stage-specific targeted therapy. This review specifically looks at clinical milestones that could be used in clinical trials, such as global function, function, behaviour, caregiver burden, and quality of life milestones. It also addresses the possible use of biological and surrogate markers for use as milestones - which may eventually replace clinical milestones. It concludes that current definitions of dementia must be broadened beyond cognition alone to include some of the domains listed.
Therapeutic Issues in Vascular Dementia: Studies, Designs and Approaches
Authors: Black Sandra E.
Published: December 2014

Vascular dementia (VaD) is a heterogeneous disorder resulting from various cerebrovascular diseases (CVD) causing cognitive impairment that reflects severity and location of damage. Epidemiological studies suggest VaD is the second commonest cause of dementia, but autopsy series report that pure VaD is infrequent, while combined CVD and Alzheimer's Disease(AD) is likely the commonest pathological-dementia correlate. Both diseases share vascular risk factors and benefit from their treatment. The most widely used diagnostic criteria for VaD are highly specific but not sensitive. Vascular Cognitive Impairment (VCI) is a dynamic, evolving concept that embraces VaD, Vascular Cognitive Impairment No Dementia (VCIND) and mixed AD and CVD. Clinical trials to date have focused on probable and possible VaD with beneficial effects evident for different drug classes, including cholinergic agents and NMDA agonists. Limitations have included use of cognitive tools suitable for AD that are insensitive to executive dysfunction. Disease heterogeneity has not been adequately controlled and subtypes require further study. Diagnostic VaD criteria now 13 years old need updating. More homogeneous subgroups need to be defined and therapeutically targeted to improve cognitive-behavioural outcomes including optimal control of vascular risk factors. More sensitive testing of executive function outlined in recent VCI Harmonization criteria and longer trial duration are needed to discern meaningful effects. Imaging criteria must be well-defined, with centralized review and standardized protocols. Serial scanning with quantification of tissue atrophy and lesion burden is becoming feasible, and cognitive interventions, including rehabilitation pharmacotherapy, with drugs strategically coupled to cognitive -behavioural treatments, hold promise and need further development.
Canadian Association of Neurosciences Review: The Role of Dopamine Receptor Function in Neurodegenerative Diseases
Authors: Lebel Manon , Robinson Pierre , Cyr Michel
Published: December 2014

Dopamine (DA) receptors, which are heavily expressed in the caudate/putamen of the brain, represent the molecular target of several drugs used in the treatment of various neurological disorders, such as Parkinson's disease. Although most of the drugs are very effective in alleviating the symptoms associated with these conditions, their long-term utilization could lead to the development of severe side-effects. In addition to uncovering novel mediators of physiological DA receptor functions, recent research advances are suggesting a role of these receptors in toxic effects on neurons. For instance, accumulating evidence indicates that DA receptors, particularly D1 receptors, are central in the neuronal toxicity induced by elevated synaptic levels of DA. In this review, we will discuss recent findings on DA receptors as regulators of long term neuronal dysfunction and neurodegenerative processes.
Uveitis Associated with Primary Angiitis of the Central Nervous System
Authors: Woolfenden A.R. , Wade N.K. , Tang P. , Chalmers A. , Reid G. , Teal P.A.
Published: December 2014

Background:

In Primary Angiitis of the Central Nervous System (PACNS), disease is typically limited to the brain and spinal cord although other organs may be affected. Uveitis is occasionally seen in systemic vasculitides but is not a recognized manifestation of PACNS. We describe two patients who developed PACNS following the onset of uveitis.

Case Descriptions:

Case 1-A 47-year-old male suffered multiple TIAs and left pontine stroke shortly after two episodes of diffuse uveitis. A cerbral angiogram demonstrated multiple caliber changes within several intracranial vessels. Cyclophosphamide was added after his stroke occurred during pulse methylprednisolone therapy. Case 2- A 35-year-old male suffered a spinal cord TIA followed by hemispheric and brainstem infarctions two months after an episode of uveitis and Bell's palsy treated with oral prednisone. A cerebral angiogram demonstrated multiple caliber changes within several intracranial vessels. He was successfully treated with oral prednisone and cyclophosphamide.

Conclusions:

Uveitis should be considered a recognized feature of PACNS. Combination immunosuppressive therapy with prednisone and cyclophosphamide may be necessary for successful treatment.
A Retrospective Study of Multiple Sclerosis in Siriraj Hospital, Bankok, Thailand
Authors: Siritho Sasitorn , Prayoonwiwat Naraporn
Published: December 2014

Objective:

To determine the demographic and clinical data of Thai multiple sclerosis (MS) patients.

Methods:

A retrospective study of 72 patients attending the MS clinic at Siriraj Hospital, Mahidol University, Thailand between January 1997 and June 2004.

Results:

Fifty-eight patients (81%) were classified as clinically definite MS, 5 (7%) as Devic's syndrome, and 9 (13%) as possible MS. There were 62 females (86%) and 10 males (14%). Age at onset was 33 ± 12 years with a mean relapse rate of 1.2 ± 1.0 attacks per annum. None had a family history of MS. Visual impairment (53%) was the most common manifestation. Only 16% had classic (western) form of MS. Positive oligoclonal bands were found in 21%, visual evoked potentials with a typical delayed latency in 28%. MRI brain lesions compatible with McDonald's criteria were seen in only 24%, and spinal MRI brain longer than 2 vertebral bodies in 62%. The mean Kurtzke's Expanded Disability Status Scale (EDSS) was 3.0.

Conclusion:

Thai MS patients had much more female occurrence, no family history, common optico-spinal form, long spinal MRI lesions and low positive CSF oligoclonal bands.
Factors Associated with Health-Related Quality of Life in Multiple Sclerosis
Authors: Hopman Wilma M. , Coo Helen , Edgar Cathy M. , McBride Evelyn V. , Day Andrew G. , Brunet Donald G.
Published: December 2014

Background:

Much research has gone into the assessment of function and health-related quality of life (HRQOL) in those with multiple sclerosis (MS). The Medical Outcomes Study 36-item short form (SF-36) has been widely used in this population but current recommendations are that it be supplemented with condition-specific measures such as the MS Quality of Life Inventory (MSQLI) and the MS Functional Composite (MSFC). The goal of the baseline component of this study was the measurement of generic and condition-specific HRQOL, and the identification of factors associated with these outcomes.

Methods:

HRQOL was assessed at the baseline phase of a longitudinal study. Participants completed the assessment during their regularly scheduled clinic visit.

Results:

300 of 387 eligible patients agreed to participate, for a response rate of 77.5%. Age ranged from 22 to 77 years, while duration of MS ranged from 1 to 47 years. Mean SF-36 scores were well below age- and sex-adjusted normative data. Only 240 completed the MSFC component. Higher EDSS, use of support services, pain medications, clinical depression and antidepressant use were associated with poorer HRQOL, while higher income and education were associated with better HRQOL.

Conclusions:

There is a substantial burden of illness associated with MS when compared to normative HRQOL data. This was more pronounced in physically- than in mentally-oriented domains. Assessment of HRQOL provides a valuable complement to the EDSS by providing information about the patient perception of function and HRQOL beyond that which can be obtained by physical assessment alone.
Headache During Gestation: Evaluation of 1101 Women
Authors: Melhado Eliana M. , Maciel Jayme A. , Guerreiro Carlos A.M.
Published: December 2014

Objective:

The purpose of this study was to evaluate the presence of headache in women with a previous history or new-onset headache during the current gestation, classify the findings, and describe the clinical characteristics and outcome of the headache.

Methods:

From January/1998 to June/2002 we prospectively evaluated 1101 pregnant women (12-45 years old), with a history of headache, at two prenatal clinics and an inpatient obstetric public hospital. Women were interviewed using a semi-structured questionnaire during the first, second, and third gestation trimesters and immediately after delivery. All interviews were conducted by one of the authors, using the International Headache Society Classification (IHSC-2004).

Results:

In 1029 women there was a history of headache prior to the current pregnancy, 36 (3.4%) women first experienced headache during this pregnancy and 40 patients experienced new types of headache. In these 76 patients with new onset headache during pregnancy, 40 had secondary headache (52.6%), 31 had primary headache (40.8%), and 5 had headache not classified elsewhere (6.6%). According to IHSC- 2004 criteria, we found migraine in 848/1029 women (82.4%), with pregestational headache.

Conclusions:

Most of the pregnant women presented with headache, mainly in migraine, prior to pregnancy, and most of the headaches improved or disappeared during the second and third gestation trimester. In a relatively small number of pregnant women, a new type of headache started during the gestation.
Ataxia and the Role of Antigliadin Antibodies
Authors: Wong D. , Dwinnel M. , Schulzer M. , Nimmo M. , Leavitt B. R. , Spacey S. D.
Published: December 2014

Background:

Although it is acknowledged that patients with celiac disease can develop neurological complications such as ataxia, the association of antigliadin antibodies in the etiology of sporadic ataxia and the usefulness of this testing in diagnosis of ataxia is controversial.

Methods:

We investigated this association by testing for the presence of IgG and IgA antigliadin antibodies in 56 ataxic patients and 59 controls. The ataxia patients were subsequently classified into three groups: sporadic, hereditary and MSA.

Results:

Of the total ataxic patients, 6/56 (11%) were positive for either IgG or IGA antigliadin antibodies compared to the controls of which 5/59 (8%) were positive (p = 0.68). In a subgroup analysis, 4/29 (14%) of the samples in the sporadic ataxic subgroup were positive for antigliadin antibodies (IgG or IgA) compared to control (p = 0.44). Similar negative results were found in the remaining subgroup analyses.

Conclusions:

These results do not support an association between antigliadin antibodies and sporadic ataxias.