bluuebunny/crossref_metadata_2025 huggingface dataset:
La dystrophie myotonique: I caractéristiques socio-économiques et résidentielles des malades
Authors: Perron Michel , Veillette Suzanne , Mathieu Jean
Published: July 2016

Myotonic Dystrophy: I Socio-Economic and Home-Environment Characteristics of the Patients. Myotonic dystrophy is a dominantly inherited genetic disorder which, in medical literature, has been linked to peculiar socio-economical conditions. In order to document this assertion, a sociological study was conducted in the Saguenay-Lac-Saint-Jean region (Quebec), using a representative sample (N = 218) drawn from the myotonic dystrophy population. A study of the patients' places of residence was also carried out. The results clearly indicate that myotonic dystrophy patients exhibit a socio-economic profile associated with disadvantaged milieux: high unemployment, low income and limited schooling. Many of these patients must rely on social welfare. Two families out of five (42%) live beneath the poverty line. The wage-earning history of these persons is closely linked to their self-evaluation of their working capabilities. After the age of forty, a high proportion of these people are unable to hold down a paying job and claim they are unable to do so. Within a well-screened urban area, a study of the patients' places of residence indicates census tracts where myotonic dystrophy is concentrated (ecological niches). These social areas are close to the downtown area and display characteristics of disadvantaged milieux. Accordingly, the My D gene seems to be confined to a specific socio-economic and socio-geographical environment, through which the spread of the gene is channelled among the population.
Traumatic Brain Injury, Aging and Reaction Time
Authors: Stuss D.T. , Stethem L.L. , Picton T.W. , Leech E.E. , Pelchat G.
Published: July 2016

The effects of traumatic brain injury (TBI) and aging were compared on tests of simple and complex reaction time (RT). Simple RT was not significantly affected by aging or TBI. TBI patients, however, tended to be slower on Simple RT tasks, and had a larger standard deviation. Individuals over age 60 and patients of any age with TBI demonstrated slower RT with choice RT tests. In addition, both groups (those over 60 and TBI patients) were less able than other groups to inhibit the processing of redundant information. For the TBI patients, this occurred primarily on reassessment. These results suggest that the deficit in both aging and TBI is not only a generalized neuronal slowing but a more specific impairment in attentional control processes, exhibited as a deficit in focused attention.
Objective Investigation of Visual Function Using a Nondestructive Zoom-FFT Technique for Evoked Potential Analysis
Authors: Regan M.P. , Regan D.
Published: July 2016

We describe an ultra-high resolution technique for recording evoked potentials (EPs) that are thousands of times smaller than the total EEG power. We report that two superimposed visual stimulus patterns, one modulated at F1 Hz and the other at F2 Hz , can generate 20 or more EP frequency components, each of which is contained within a bandwidth of no more than 0.004 Hz. We have developed a theoretical method for calculating the amplitudes and phases of these various cross-modulation components for different arrangements of model neurons. The relative amplitude of the various cross-modulation components seems to be a 'signature' of the particular neural model and this offers a way of testing theoretical neural models against EP data. As an illustration we compare the nonlinear EP components evoked by dichoptic stimulation with unpatterned flickering light against several models of binocular interaction. These nonlinear binocular interaction terms may offer a means of investigating binocularity in amblyopic children or infants who have low acuity in one or both eyes.
Flunarizine as a Supplementary Medication in Refractory Childhood Epilepsy: A Double-Blind Crossover Study
Authors: Keene D. , Whiting S. , Humphreys P. , Jacob P.
Published: July 2016

We report a double blinded cross-over study involving Flunarizine versus placebo in the treatment of refractory childhood epilepsy. The patients studied were between the ages of 2 and 18; and were having more than 4 seizures per month not responsive to regular anticonvulsant medications. Of the 34 patients treated, 8 had a 50% decrease in their seizures during the placebo phase, 5 had a 50% decrease during the Flunarizine phase, and 1 patient had a 50% increase in seizures while taking Flunarizine. The remaining 25 patients showed no change in seizure activity in either phase. Patients having partial seizures with secondary generalization tended to do better on Flunarizine than those with other seizure types. Monitoring serum Flunarizine levels showed no significant difference between patients having improved seizure control and those who were unimproved. No significant side effects were noted with this medication, nor were any significant drug interactions noted.
HTLV-1 Associated Myelopathy in Canada
Authors: Power Christopher , Weinshenker Brian G. , Dekaban Gregory A. , Ebers G.C. , Francis Gordon S. , Rice George P.A.
Published: July 2016

We report the clinical and laboratory findings of six Canadian patients with progressive myelopathy associated with HTLV-1 infection. The diagnosis was suspected on clinical grounds and supported by serological studies and positive gene amplification. Only five had emigrated from an area endemic for HTLV-1 infection. Tropical spastic paraparesis should be considered in all patients with myelopathy, even those without standard serological markers of HTLV-1 infection. The pathogenesis of this condition and the serological and molecular biological means by which this diagnosis can be made are reviewed.
Pure Motor Stroke Due to Vertebral Artery Dissection
Authors: Phillips Stephen J. , Maloney William J. , Gray Jean
Published: July 2016

A 39-year-old man presented with a pure motor stroke 9 days after cervical chiropractic manipulation. Computerised tomographic scanning showed a pontine infarct. Cerebral angiography showed changes consistent with the diagnosis of bilateral vertebral artery dissection. It is postulated that the infarct resulted from artery-to-artery embolism.
A Study of Monoamine Metabolism in Human Epilepsy
Authors: Louw D. , Sutherland G.R. , Glavin G.B. , Girvin J.
Published: July 2016

Fourteen patients with intractable epilepsy underwent surgical resection of their epileptogenic focus. Hippocampal and temporal cortical samples were obtained, and subjected to high performance liquid chromatography with electrochemical detection. Levels of 5-hydroxyindoleacetic acid (5-HIAA) in actively spiking temporal cortex were 0.454 ± 0.012 ng/mg (mean ± SEM), contrasting with less actively spiking cortex values of 0.248 ± 0.042 ng/mg and normal literature values, obtained from post-mortem material, of 0.140 ± 0.050 ng/mg. Similarly, homovanillic acid (NVA) levels were significantly increased in epileptic tissue compared to normal literature values. Actively spiking cortex values were 0.172 ± 0.001 pg/mg, less active cortex values were 0.058 ±0.012 pg/mg, and literature values were 0.011 ± 0.002 pg/mg. Although a direct statistical comparison between the data reported here and literature values may not be valid, the findings show a continuum from normal through increasingly active electrode sites, likely reflecting a true biologic phenomena. Similarly, statistically significant increases in 5-HIAA and HVA levels were found in hippocampal tissue, which also showed significantly lower dihydroxyphenylacetic acid levels. We conclude that raised levels of serotonin and dopamine metabolites in actively spiking cortex likely reflect an increase in their turnover, and are an epileptic epiphenomenon. Exaggeration of turnover may represent the “metabolic noise” of epilepsy, rather than a concerted strategy of local or distal neurons to contain an epileptogenic focus.
Transcranial Doppler Studies in Aneurysm Patients
Authors: Hutchison Kenneth , Weir Bryce
Published: July 2016

Fifty patients with ruptured intracranial aneurysms and 8 patients with elective clipping of unruptured aneurysms had daily transcranial Doppler (TCD) measurements performed. The highest mean middle cerebral artery velocity (MCA-Vel) was considered to be the best single parameter forjudging a patient's susceptibility to clinically significant vasospasm (VSP). Surgery for the clipping of unruptured aneurysms by itself does not lead to an increase in MCA-Vel. There is a progressive increase in MCA-Vel after subarachnoid hemorrhage (SAH) from aneurysms which peaks between 7 and 10 days. The MCA-Vel is higher on the side of the ruptured aneurysm and the degree of rise is greater if blood is seen on the initial CT scan. It is highly unlikely that a patient whose MCA-Vel remains under 100 cm/sec has a degree of angiographic VSP which causes clinical symptomatology. Patients whose MCA-Vel is > 200 cm/sec are at great risk of developing clinical symptomatology of VSP and are very likely to have significant angiographic VSP. There is a transitional zone in between these two levels.
Myelopathy: An Unusual Presentation of Toxoplasmosis
Authors: Nag S. , Jackson A.C.
Published: July 2016

Central nervous system toxoplasmosis is a well known disease of immunocompromised patients. Neuropathologic examinations have only rarely demonstrated spinal cord involvement. This report describes a fatal case of toxoplasmosis that presented with a subacute myelopathy. Toxoplasmosis should be considered in immunocompromised patients, including patients with the acquired immune deficiency syndrome, that develop intramedullary lesions of the spinal cord.
Pain in Gilles de la Tourette Syndrome and Related Tic Disorders
Authors: Riley David E. , Lang Anthony E.
Published: July 2016

Although pain is not generally recognized as a symptom of tic disorders, we have seen a number of patients in whom this was a prominent feature, at times even the symptom of greatest concern. The commonest pain complaints are those arising from the actual performance of a tic. Most often this follows directly from the discomfort produced by sudden or repeated extreme exertion. Here the origin of the pain is usually musculoskeletal, though rare examples of neuropathic pain may occur. Pain also may arise from striking or being struck by a moving body part involved in large amplitude tics. Other related painful acts include deliberate self-injury and pain inflicted upon others. A second major category is represented by a smaller number of patients who complain of pain during voluntary efforts to suppress their tics. Finally, there are patients who obtain relief from tics while experiencing pain, to such an extent that they will deliberately provoke pain to obtain its benefit. We feel that pain should be recognized as a common complaint, and occasionally a source of significant disability, in patients with tics.
Nuclear Inclusions in Oculopharyngeal Muscular Dystrophy in Quebec
Authors: Bouchard Jean-Pierre , Gagné François , Tomé Fernando M. S. , Brunet Denis
Published: July 2016

Seven French-Canadian cases of clearcut oculopharyngeal muscular dystrophy (OPMD) had their muscle studied for the presence of intranuclear inclusions, and they were all positive. Inclusions of both “mature” and “immature” types were seen in our material. The presence of such intranuclear structures should be added to the criteria of the clinical picture and the family history for diagnosis of a case and inclusion of a family in further genetic studies. Reverse genetic studies of large families and biochemical studies of these intranuclear structures may help to understand the pathogenesis of this common disease in Quebec.
Expression of Neuronal mRNAs in Alzheimer Type Degeneration of the Nervous System
Authors: Clark Arthur W. , Parhad Irma M.
Published: July 2016

There is extensive evidence for decrements of gene expression in AD, at several levels in the process. There is also evidence for increments of expression of some genes. Message for the amyloid precursor protein (APP), for example, is elevated in surviving neurons of certain subcortical populations in AD. We evaluated expression of message for APP as well as for certain neuronal and glial cytoskeletal proteins in the cortex of six cases of AD. Neuronal mRNAs, including that for APP, were significantly decreased when compared with control cortex, whereas the glial mRNA was increased. We have projected studies to determine the evolution of these mRNA decrements in Alzheimer-type degeneration. The rationale for these studies and preliminary findings are discussed.
Progress Report on the Canadian Multicentre Trial of Tetrahydroaminoacridine with Lecithin in Alzheimer's Disease
Authors: Gauthier S. , Bouchard R. , Bacher Y. , Bailey P. , Bergman H. , Carrier L. , Charbonneau R. , Clarfield M. , Collier B. , Dastoor D. , Gauthier L. , Germain M. , Kissel M. , Krieger M. , Kushnir S. , Lamontagne A. , Morin J. , Nair N.P.V. , Neirinck L. , Ratner J. , St-Martin M. , Suissa S. , Tesfaye Y.
Published: July 2016

Since the discovery of a significant depletion of acetylcholine in discrete areas of the brain of patients affected by Alzheimer's disease, attempts at symptomatic therapy have concentrated on acetylcholine supplementation, an approach that is based upon the efficacy of dopaminergic supplementation therapy for Parkinson's disease. Choline, then lecithin, used orally, failed to improve symptoms but the hypothesis that long-term choline supplementation might stabilize the course of Alzheimer's disease remains to be tested. Nerve growth factor may also offer that possibility. Bethanechol administered intracerebroventricularly did not help when a fixed dose was used but individual titration of more selective muscarinic agonists may prove more effective. In this article we report that tetrahydroaminoacridine (THA), given together with highly concentrated lecithin, appears to bring improvement in cognition and in functional autonomy using the Mini Mental State and the Rapid Disability Rating Scale-2 respectively, without change in behavior as reflected by the Behave-AD. Double-blind cross-over studies are in progress to establish its efficacy. Improvement in study design and means of assessment of cognition, functional autonomy and behavior have been made possible by these drug trials.
Prolactin-Secreting Adenomas - Surgical Results
Authors: Maira Giulio , Anile Carmelo , Marinis Laura De , Barbarino Antonino
Published: July 2016

The long-term follow-up of the transsphenoidal microsurgical treatment in 119 consecutively operated women with a PRL-secreting adenoma is presented. An apparent total removal of the tumor was achieved in 98 cases with an enclosed tumor (58 grade I and 40 grade II). In the remaining cases the removal was considered partial. The achievement of persistent normal PRL basal levels was verified in 61 patients (44 grade I and 17 grade II) who had an apparent total removal of the adenoma. In the 37 remaining patients who were thought at surgery to have had total removal we have distinguished two groups: 30 patients showed a “relapse” or “persistence” of PRL levels below 200 ng/ml without clinical and radiological signs of tumor regrowth, and 7 patients with a PRL level higher than 200 ng/ml who had evidence of PRL-secreting tumor recurrence.
Results of Primary Treatment with Bromocriptine of Prolactinomas with Extrasellar Extension
Authors: Verlaat Johanna W. van't , Croughs Ronald J.M. , Hendriks Martin J. , Bosma Nicolaas J.
Published: July 2016

Nineteen patients, seven women and twelve men, with macroprolactinomas characterized by extrasellar extension and basal prolactin levels above 6 U/I were treated with 10-20 mg bromocriptine daily in four divided doses for a mean period of 3.4 years (range 1.5-5.5 years). Plasma prolactin levels fell dramatically in all patients and values in the low normal range were obtained in sixteen patients. Tumor size was reduced by more than 75% in seventeen patients and by 50-75% in two patients. Tumor reduction was associated with the development of a partial empty sella in fourteen cases. In seventeen cases the pituitary became visible. Diminished visual acuity (six patients), bitemporal hemianopia (nine patients), unilateral and bilateral central scotomas (three patients) and oculomotor palsy (two patients) improved or normalized in all cases. Hypogonadism (all patients), hypothyroidism (nine patients) and hypocorticism (four patients) improved or normalized in most cases. It is concluded that in the medical treatment of macroprolactinomas 10-20 mg bromocriptine in four divided doses effectively reduces both plasma prolactin level and tumor size. The good results in this study may be related to the continued use of a fixed dose regimen of bromocriptine regardless of the plasma prolactin lowering effect.
Pituitary Adenomas in Childhood and Adolescence
Authors: Maira G. , Anile C.
Published: July 2016

Pituitary adenomas are considered rare tumors in the pediatric age group. The natural history in this age group is not well known, although many authors have suggested that pituitary adenomas in children tend to be extrasellar and invasive and that a subfrontal approach is mandatory in the majority. Our patients were part of a surgical series of 356 adenomas of all ages. We have divided these cases into three groups: the first consists of patients operated on before the age of 21 years (52 cases, 15%); the second, patients treated after the age of 20 but in whom the clinical history started before 21 years (52 cases, 15%); the third, patients with surgery and symptoms after 20 years (252 cases, 70%). Transsphenoidal removal of the adenomas was the initial procedure in all but one of these cases; a few required a second intracranial operation. In our series, pituitary adenomas in children were not more invasive than in other age groups.
Clinical Differences Between Metoclopramide- and Antipsychotic-Induced Tardive Dyskinesias
Authors: Lang Anthony E.
Published: July 2016

A patient with tardive dyskinesia dominated by pelvic thrusting movements is described. A retrospective review of the clinical features documented in patients with metoclopramide- and antipsychotic-induced tardive dyskinesias seen over a six year period demonstrated that the occurrence of pronounced pelvic thrusting and respiratory dyskinesias were significantly more common in the metoclopramide treated group. The occurrence of bucco-linguomasticatory movements, limb stereotypies or chorea, and mild truncal or abdominal rocking were not significantly different between the two groups. None of the tardive dystonia patients had metoclopramide as the causative agent. These findings will require confirmation in larger, better matched patient populations. Whether the differences relate to different pharmacologic profiles of drug action, patient populations exposed, or other factors, remains to be elucidated.
Drug-Induced Parkinsonism and Other Movement Disorders
Authors: Ross R.T.
Published: July 2016

This is a review of reserpine, haloperidol, and various phenothiazines that produce parkinsonism and other movement disorders. The by-products of illicit meperidine synthesis, MPTP and its more sinister companion, MPP, are also discussed. Movement disorders, transient or fixed, frank parkinsonism and/or dyskinesia, due to a variety of other medications and toxic agents are included. These are methanol, lithium, methyldopa, antimetabolites, antidepressants, sympathomimetic anorexiants, some types of antihistamines, and various combinations of agricultural chemicals.
Comparisons of the Clinical and Radiological Features and Surgical Management of Posterior Fossa Meningiomas and Acoustic Neuromas
Authors: Tator C.H. , Duncan E.G. , Charles D.
Published: July 2016

Next to acoustic neuromas, meningiomas are the most common benign tumour of the posterior fossa. This paper reviews the personal experience of one of the authors with the management of 26 posterior fossa meningiomas and 212 acoustic neuromas. The aim was to compare the clinical and radiological features of these two types of posterior fossa tumours to determine whether they can be differentiated preoperatively. The study also aimed at analysing the surgical treatment of posterior fossa meningiomas. The results showed that posterior fossa meningiomas can usually be differentiated from acoustic neuromas on the basis of clinical and radiological features. Preoperative differentiation aids surgical management, especially for selection of surgical approach. In most instances, posterior fossa meningiomas can be totally resected with minimal morbidity and mortality.
Photodynamic Therapy of Malignant Brain Tumours
Authors: Muller Paul J. , Wilson Brian C.
Published: July 2016

Fifty patients with malignant supratentorial tumours were treated with intra-operative photodynamic therapy (PDT); in 33 cases the tumour was recurrent. In 45 patients the tumour was a cerebral glioma and in 5 cases a solitary cerebral metastasis. All patients received a porphyrin photosensitizer 18-24 hours pre-operatively. Photoillumination was carried out at 630 nm to a tumour cavity created by radical tumour resection and/or tumour cyst drainage. The light energy density ranged from 8 to 175 J/cm2. In 8 patients additional interstitial light was administered. The operative mortality was 4%. Follow up has ranged from 1 to 30 months. The median survival for the 45 primary malignant tumours was 8.6 months with a 1 and 2 year actuarial survival rate of 32% and 18%, respectively. In 12 patients a complete or near complete CT scan response was identified post PDT. These patients tended to have a tumour geometry (eg. cystic) that allowed complete or near complete light distribution to the tumour. The median survival for this group was 17.1 months with a 1 and 2 year actuarial survival of 62% and 38%, respectively. In the 33 cases who did not have a complete response the median survival was 6.5 months with a 1 and 2 year actuarial survival of 22% and 11%, respectively. Photodynamic therapy of malignant brain tumours can be carried out with acceptable risk. Good responses appear to be related to adequate light delivery to the tumour.
Manual Tracking Performance in Patients with Cerebellar Incoordination: Effects of Mechanical Loading
Authors: Morrice Betty-Lynn , Becker Werner J. , Hoffer J.A. , Lee Robert G.
Published: July 2016

Manual tracking performance was studied in five patients with cerebellar incoordination due to unilateral cerebellar hemisphere lesions. The subjects were required to track a target on an oscilloscope screen by moving a cursor controlled by flexion-extension movements of the wrist. In comparison to normal subjects, the cerebellar patients, using their clinically affected arm, demonstrated irregular tracking patterns with inappropriate accelerations and decelerations, numerous high velocity peaks of movement, and an increased time lag between the cursor and the target.

The addition of a viscous load provided by feeding back wrist velocity to a torque motor coupled to the apparatus resulted in significant improvement in tracking performance and suppression of the high velocity peaks. Increasing elastic stiffness by feeding back wrist position or inertial load by adding weights to the hand did not improve performance on this task. It is proposed that a hypotonic cerebellar limb behaves like an underdamped mechanical system. The addition of viscous loads helps restore more normal damping during voluntary movements of the arm.
Clinical Recovery and Sleep Architecture Degradation
Authors: Hachinski Vladimir C. , Mamelak Mortimer , Norris John W.
Published: July 2016

We achieved a unique and timely recording of cerebral activity in a 70 year old woman immediately pre- and post-stroke, while studying the effect of acute cerebral infarction on sleep-electroencephalogram (EEG) patterns. Normal patterns, except for increased wakefulness, were recorded during two pre-infarct polysomnograms. Immediately following cerebral infarction increased delta activity was recorded from the infarcted hemisphere only. Initially, REM sleep could not be recorded from either side; however, on the third post infarct day REM sleep returned. Background EEG levels from both hemispheres became progressively slower, flatter and simpler. In addition, sleep spindles and the distinctive saw-tooth wave forms of sleep almost disappeared. At one year post-stroke sleep-EEG rhythm recordings from both hemispheres became more similar except for persisting delta activity from the left hemisphere. Unexpected deterioration of sleep-EEG pattern recordings from the undamaged hemisphere taken during the patient's clinical recovery remains unexplained. Serial sleep recording may facilitate the study of brain recovery, activity and reorganization following stroke.
CQA 206-291 in Parkinson's Disease: An Acute Single Escalating Dosage Study
Authors: Lang Anthony E. , Riley David E. , Vachon Luc , Lataste Xavier
Published: July 2016

CQA 206-291, a new ergot derivative with a “biphasic” dopaminergic profile, was studied in 6 patients with longstanding Parkinson's disease suffering from pronounced fluctuations in hourly mobility. On alternate days, up to seven single doses, escalating from 0.2 to 20 mg, were given as replacement for the usual first morning dose of levodopa. At the most effective dosage, four of the six patients obtained as good a peak response to CQA (8-20 mg) as to L-dopa. Side effects were common and similar to other ergot derivatives, suggesting that the initial weak dopamine antagonist properties of the parent compound, documented in animals, may be of little clinical significance. However, comparative studies will be needed to confirm this suspicion. The addition of domperidone successfully reduced the incidence and severity of side effects. CQA 206-291 has potent anti-parkinsonian properties; further longer-term treatment trials are indicated.
Visual Hallucinations on Eye Closure Associated with Atropine Toxicity. A Neurological Analysis and Comparison with other Visual Hallucinations
Authors: Fisher C. Miller
Published: July 2016

Visual hallucinations of remarkable intensity began shortly after intravenous atropine and persisted for 11 days. They were present only when the eyes were closed and were associated with heightened dreaming and disturbed sleep. The patient remained lucid and described his experiences to his attendants. Our patient's hallucinations bore some resemblance to hypnagogic hallucinations and this became the basis for the hypothesis that the hallucinations originated in the sleep-dream system of the brain stem. It is speculated that a similar site — a metabolic locus minoris resistentiae may play a part in other types of visual hallucinations and in delirium.
Organization of the Mouse Motor Cortex Studied by Retrograde Tracing and Intracortical Microstimulation (ICMS) Mapping
Authors: Li Cheng-Xiang , Waters Robert S.
Published: July 2016

The motor representation of the body musculature was studied in 11 adult mice by using ICMS. The motor responses elicited from both granular and agranular cortical fields showed that the mouse motor cortex is topographically organized; however, within the representation of individual body-parts the movements are multiply represented. In addition, several sites were encountered where more than one movement was elicited at the same stimulus threshold. The locations of pyramidal cells contributing axons to the pyramidal tract were examined by means of retrograde tracing with HRP injected into the cervical enlargement. This procedure labeled neurons only in lamina V in granular and agranular cortical fields. The similarities between the organization of motor cortex demonstrated in this study and the organization in the rat suggest that the rat and mouse share a common plan of rodent motor cortical organization.
Cephalic Axial Skeletal-Neural Dysraphic Disorders: Embryology and Pathology
Authors: Marín-Padilla Miguel
Published: July 2016

Three fundamental types of cephalic axial skeletal-neural dysrapic disorders are analyzed, including: cranioschisis aperta with encephaloschisis (anencephaly and/or exencephaly), cranioschisis occulta with occipital encephalocele, and the Chiari malformation (occipital bone hypoplasia) with compression, deformation and displacement of hindbrain, cerebellum, and medulla. Both clinical and experimental (vitamin A induced) examples of these malformations are used. The study establishes that these are not simple neurological (neural tube defects) disorders as it has been generally assumed, but complex developmental malformations affecting primarily the formation of the axial basicranium (causing skeletal defects) and the elevation of the neural folds and neurocranium (causing neural defects), and, secondarily, the topography of the facial skeleton or viscerocranium (causing oropharyngeal defects). The pathology of these skeletal, neural, and oropharyngeal defects is analyzed, their embryonic origin explored, and their developmental interrelationships discussed. The study proposes that an early paraxial mesodermal insufficiency may be the original anomaly common to all the different malformations that constitutes this heterogeneous group of dysraphic disorders. At any time during the segmental formation of the embryonic skeletal-neural axis, a simple reduction in the number of paraxial mesodermal cells produced by the Hensen node/primitive streak complex, could impair the formation of the axial skeleton as well as the elevation of the neural folds thus interfering with their closure. The final type of malformation is determined by variations of the degree, time of occurrence, and duration of the paraxial mesodermal insufficiency.
Cerebral Dysplasias as Expressions of Altered Maturational Processes
Authors: Sarnat Harvey B.
Published: July 2016

This review identifies the fundamental anatomical and physiological processes that provide the substrates of maturation in the developing nervous system. Cerebral malformations may be viewed from the perspective of aberrations in one or more of these processes. The maturational processes are generally sequential but with considerable temporal precision and overlap: 1) neuronogenesis and gliogenesis, including neural induction by the notochord and primary segmentation of the nervous system; 2) programmed cell death of excess neuroblasts and glioblasts; 3) neuroblast migration; 4) formation and growth of neurites; 5) development of membrane polarity and excitability; 6) synaptogenesis; 7) biosynthesis of neurotransmitters and other secretory products; and 8) myelination of axons. The anatomical and synaptic organization of the fetal brain differs greatly from the mature state. An excess of axonal collaterals, dendritic branches, spines and synapses are formed and later selectively deleted. Transitory neurons, specialized glial cells, synaptic circuits and transient neurotransmitter systems serve as functional elements for a limited time. The clinical expression of these transitory features of the fetal brain are incompletely understood, particularly in the context of embryonic cerebral plasticity.
A Movement-Associated Fast Rolandic Rhythm
Authors: McLachlan Richard S. , Leung Lai Wo S.
Published: July 2016

A stereotyped 32 Hz rolandic rhythm maximal over the mesial cortex and occurring only with voluntary movement is described in a patient with tonic postural seizures. This abnormal but nonictal rhythm, which is probably generated subcortically, is expressed synchronously at the level of the cortex and peripherally in the EMG.
Dementia with Leukoencephalopathy in Systemic Lupus Erythematosus
Authors: Kirk Andrew , Kertesz Andrew , Polk Marsha J.
Published: July 2016

Neurologic manifestations, afflicting up to 70% of SLE patients, include psychosis, seizures, chorea, neuropathies, and stroke. MRI is useful in evaluation of lupus patients and several reports have documented cerebral atrophy or focal hyperintensities. We report an unusual MRI appearance in a 56-year-old woman with SLE, diagnosed on the basis of pleuritis, lymphopenia, anti-DNA antibodies, and neurologic involvement. She reported recent onset of Raynaud's phenomenon and generalized macular rash. She presented after two months of gradual deterioration with memory loss, flattened affect, dysphagia, dysarthria, anomia, and somnolence, without focal neurologic signs. Investigations included elevated ESR, reduced complement, normal CSF without oligoclonal bands, negative viral serology, normal hormone and vitamin levels, normal renal and hepatic function. Neuropsychologic testing showed widespread impairment (WAIS-R: FSIQ-63; WMS-69; DRS-98; RCPM-14; WAB AQ-78.8). CT was normal but MRI showed strikingly symmetric, confluent hyperintensities extensively involving cerebral and cerebellar white matter on Tl and T2 weighted scans. Basal ganglia and subependymal and subcortical white matter were spared. Treated with prednisone, the patient made a gradual, but incomplete, recovery. These MRI findings may reflect widespread vasculopathy or direct immunologic brain insult with or without imunologic blood-brain barrier disruption.
Pupillary Areactivity in Hydrocephalus of Recent Onset
Authors: Boghen Dan , Aroichane Maryam
Published: July 2016

A patient who presented with bilateral loss of all pupillary reactions and normal ocular motor function is reported. Investigation revealed the presence of massive hydrocephalus. The syndrome developed shortly after transsphenoidal surgery for a suprasellar craniopharyngioma. Pupillary function returned to normal following the insertion of a ventriculo-peritoneal shunt. It is suggested that the syndrome was due to compression of the visceral oculomotor nuclei by a dilated sylvian aqueduct.
Effects of Dopamine Denervation on Striatal Peptide Expression in Parkinsonian Monkeys
Authors: Lavoie Brigitte , Parent André , Bédard Paul J.
Published: July 2016

In cynomolgus monkeys rendered parkinsonian by systemic injection of MPTP, severe cell losses were noted in the ventrolateral portion of the substantia nigra pars compacta (SNc), compared to a relative sparing of neurons in the ventral tegmental area (VTA) and dorsomedial portion of SNc. Most spared neurons in the SNc-VTA complex were found to contain the calcium binding protein calbindin (CaBP). At striatal levels the dopaminergic (DA) innervation, as visualized by tyrosine hydroxylase immunoreactivity, was markedly reduced in the ‘sensorimotor’ territory, variably affected in the ‘associative’ territory, and relatively well preserved in the ‘limbic’ territory. The immunoreactivity for enkephalin was enhanced and that for substance P was decreased in the sensorimotor territory, whereas the inverse was observed in the limbic territory. The distribution of the two peptides was highly heterogeneous in the associative territory. These findings suggest that the influence of the DA input on peptide expression varies from one striatal territory to the other, and that CaBP may protect midbrain DA neurons from MPTP toxicity.
Reactions of the Immune System in Chronic Degenerative Neurological Diseases
Authors: McGeer P.L. , McGeer E.G. , Kawamata T. , Yamada T. , Akiyama H.
Published: July 2016

Elements consistent with a cell mediated immune response were identified immunohistochemically in amyotrophic lateral sclerosis (ALS) spinal cord and Alzheimer disease (AD) hippocampus. T helper/inducer and cytotoxic/suppressor lymphocytes were detected in affected tissues in both diseases. In addition, abundant reactive microglia were found expressing the major histocompatibility glycoproteins HLA-A,B,C and HLA-DR, as well as receptors for the Fc chain (Fcyγl), for complement 3 and 4, and for vitronectin. In AD, the complement proteins Clq, C4d, C3d and C5b-9 were found on dystrophic neurites, neuropile threads and some neurofibrillary tangles. In ALS, the only complement proteins identified were C4d and C3d. The integrin ligands vitronectin and ICAM-1 were also identified in affected tissues in both diseases.
Growth Factors and Lymphokines: Modulators of Cholinergic Neuronal Activity
Authors: Quirion Rémi , Araujo Dalia M. , Lapchak Paul A. , Seto David , Chabot Jean-Guy
Published: July 2016

It is well known that various markers of the cholinergic synapse are altered in Alzheimer's Disease. Much interest is currently focussing on the evaluation of the possible efficacy of certain growth factors, especially nerve growth factor (NGF), to reduce or reverse cholinergic neuronal losses. Here we report that other growth factors (epidermal growth factor and insulin-like growth factor I) and a lymphokine, interleukin-2, are able to block acetylcholine release in the rat hippocampus. This suggests that while certain growth factors like NGF may have positive effects on the cholinergic neuron, others may act as “negative” factors on this neuronal population.
Exogenous NGF Affects Cholinergic Transmitter Function and Y-Maze Behavior in Aged Fischer 344 Male Rats
Authors: Williams Lawrence R. , Rylett R. Jane , Moises Hylan C. , Tang Andrew H.
Published: July 2016

Chronic ICV administration of NGF stimulates the activity of the cholinergic neuronal markers, HACU and ChAT, as well as the evoked release of both endogenous and newly synthesized acetylcholine in the brain of aging Fischer 344 male rats. However, the pattern of cholinergic phenotype stimulation indicates an age-related differential regulation of ChAT, HACU, and ACh release between specific brain areas, with the largest.effects found in the striatum. NGF treatment also increases the effectiveness of neurotransmission between basal forebrain cholinergic neurons and postsynaptic amygdaloid target neurons. The stimulation of central cholinergic transmitter function after NGF treatment affects behavior in a Y-maze brightness discrimination paradigm. NGF treatment does not affect the cognitive measure of brightness discrimination, but reduces the number of avoidance attempts, a measure of motor function.
Neuronal Migration Disorders: A Contribution of Modern Neuroimaging to the Etiologic Diagnosis of Epilepsy
Authors: Palmini André , Andermann Frederick , Olivier André , Tampieri Donatella , Robitaille Yvon , Melanson Denis , Ethier Romeo
Published: July 2016

Computed tomography and magnetic resonance imaging enable the identification of neuronal migration disorders during life. Several specific syndromes have been identified and early diagnosis of previously unrecognized entities is now possible. We report 51 patients with imaging. Thirty-two had a single widespread cortical dysplastic lesion. Twenty-eight had focal corticectomies. From a pathological standpoint, these encompassed focal cortical dysplasia (14 cases) and forme fruste of tuberous sclerosis (10 cases). These two groups of patients were indistinguishable from the clinical and radiological standpoint. In only two was the MRI examination normal. In addition, there were 10 with bilateral perisylvian dysplasia, four with diffuse cortical dysplasia or the "double cortex" syndrome, three with hemimegalencephaly, one with megalencephaly, and one with nodular neuronal heterotopia. The electroclinical and imaging findings led to the development of specific surgical strategies for the alleviation of the intractable seizures in each of these radiologically-defined syndromes.
Temporal Lobectomy with Amygdalectomy and Minimal Hippocampal Resection: Review of 100 Cases
Authors: Rasmussen Theodore , Feindel William
Published: July 2016

The evidence for the role of the amygdala in temporal lobe seizures is supported by this follow-up (2-20 years) of 100 patients who were treated surgically by excision of the antero-lateral temporal cortex, most or all of the amygdala and minimal resection of the hippocampus. The findings showed 53 patients seizure-free or with rare or occasional seizures, 10 patients with marked seizure reduction and 37 with moderate or less reduction of seizures. The results are the same as in another 100 patients where, in addition, half or more of the hippocampus was resected.
Selective Amygdalohippocampectomy: Indications and Follow-up
Authors: Wieser Heinz Gregor
Published: July 2016

Selective amygdalohippocampectomy (AHE) offers a real chance of cure only in patients with welldefined, precisely localized “epileptogenic area”, i.e. seizure focus. Therefore, a priori only a small proportion of all patients with epilepsy can meet the criteria for selective surgical interventions. From the evidence in patients meeting the criteria for AHE, we conclude that this technique is to be preferred to the “standard” anterior temporal lobectomy and represents a more selective but still effective surgical treatment of epilepsy.
Biochemical Markers of Excitability in Human Neocortex
Authors: Sherwin A.L. , Vernet O. , Dubeau F. , Olivier A.
Published: July 2016

We measured biochemical markers of excitability in brain excised for neurosurgical therapy of epilepsy. Intraoperative electrocorticography was used to identify and compare samples from regions of persistent interictal spike discharges and areas of the cerebral convexity which were free of interictal piking. We found that interictal spiking was associated with elevated tissue levels of the excitatory amino acids glutamic acid (26%, p < 0.001) and aspartic acid (25%, p < 0.05). There was also a significant increase in the activity of the enzymes glutamic acid dehydrogenase (20%, p < 0.01) and aspartate acid aminotransferase (18%, p < 0.01) which are involved in their formation. There was no change in the levels of the inhibitory neurotransmitters GABA or taurine. We also found a significant increase in the activity of tyrosine hydroxylase (52%, p < 0.001), the rate controlling enzyme in catecholamine biosynthesis. There was a reduction in the density (Bmax) of cortical alpha-1 adrenoceptors (26%, p < 0.01) and a concommitant diminution of receptor coupled phosphatidylinositide metabolism (21%, p < 0.01). This blunting of inhibitory noradrenergic transmembrane signaling may contribute to a relative imbalance between excitatory and inhibitory mechanisms in epileptogenic neocortex.
Surface Ictal Electroencephalographic Patterns in Frontal vs Temporal Lobe Epilepsy
Authors: Swartz Barbara E. , Walsh Gregory O. , Delgado-Escueta Antonio V. , Zolo Paolo
Published: July 2016

The effectiveness of long term EEG monitoring in the localization of the epileptic focus was studied in 37 patients with temporal lobe epilepsy comprising 190 recorded seizures, in 19 frontal lobe epileptic patients with 172 recorded seizures and in 12 additional patients which were classified as fronto-temporal. In the temporal lobe group, 49/190 seizures began focally (26%) and 20/190 seizures exhibited a regional onset (10%). In the frontal lobe group, only 21 out of 172 seizures (12%) had a focal ictal onset. 41/172 seizures began regionally (24%). In the fronto-temporal group, 31/55 seizures disclosed a focal EEG onset (57%). This study demonstrates that there is a two-fold increase in seizures beginning focally in the temporal lobe epilepsy group versus the frontal lobe group.
Evaluation of foot deformity using a three-dimensional geometric model
Authors: Sirois J.P. , Allard P. , Duhaime M. , Geoffroy G. , Kofman J.
Published: July 2016

A three-dimensional geometric model of the foot's bony structure based on the use of a stereoradiographic technique is presented. To illustrate the potential of such a model it has been applied here to the study of cavus foot in a patient having Friedreich's ataxia. This model permits an accurate evaluation of this complex spatial foot deformity.
Differential Aspects of Sleep Epilepsy
Authors: Young G. Bryan , Blume Warren T. , Wells George A. , Mertens Wilson C. , Eder Suzanne
Published: July 2016

A review of patients with epilepsy showed that 5.9% had seizures exclusively in sleep (ES) and 4.7% had seizures predominantly but not exclusively in sleep (PS). These groups were compared with a group (W) with seizures mainly in wakefulness. The following significant differences were obtained: 1) generalized convulsions predominated in the ES while partial seizures were more common among PS and W patients, 2) seizures occurred less frequently in the ES group, and 3) more W patients had EEGs with generalized epileptiform activity and positive family histories for epilepsy.

We suggest the lower frequency of seizures in the ES group and the declining prevalence of sleep epilepsy are due to: 1) the high proportion of generalized as opposed to partial seizures in sleep and 2) more effective control of generalized seizures compared to partial seizures by modern anti-epileptic drug management.
Hemangioma Calcificans of the Spinal Cord
Authors: Tyndel Felix J. , Bilbao Juan M. , Hudson Alan R. , Colapinto Edward V.
Published: July 2016

A 27-year-old woman presented with hemotomyelia during pregnancy. Needle drainage of the spinal cord was followed by relief of symptoms. Symptoms recurred during a second pregnancy 4 years later and she presented with an avascular mass in the midcervical spinal cord. This lesion proved to be hemangioma calcificans, a densely calcified and ossified variant of cavernous angioma.
Super-Selective Angiography and Embolization of Skull Base Tumours
Authors: Brugge K.G. Ter , Lasjaunias P. , Chiu M.C.
Published: July 2016

Treatment planning for patients with tumours of the skull requires a true multidisciplinary approach. Super-selective angiography, using a protocol approach, is necessary in building a proper strategy plan for treatment of these lesions. Embolization done by a well trained team has proven to be a valuable adjunct to the various treatment methods and it may eliminate the need for radiation of benign lesions of the base of the skull.
Surgical Management of Tumours Invading the Skull Base
Authors: Derome P.J.
Published: July 2016

The author describes four different approaches to clival tumours, and reviews his experience with 33 chordomas of the clivus. He also reviews his large experience with tumours invading the floor of the anterior and middle cranial fossae, and describes various surgical approaches for the removal of these tumours, followed by reconstruction of the bony floor with bone grafts, and dural reconstruction.
Acoustic Neuromas: Management of 204 Cases
Authors: Tator Charles H.
Published: July 2016

This paper reviews the author's personal experience with the management of 204 patients with the clinical diagnosis of acoustic neuroma. Craniotomy was performed in 181 of these cases, all of whom were proven to have acoustic neuromas. The remaining 23 cases with the clinical diagnosis of acoustic neuroma did not have tumour surgery, mainly because of advanced age or concurrent disease, although some required shunts for hydrocephalus. The management of acoustic neuromas including the selection of surgical approach depends upon the patient's age and general health, the size and growth direction of the tumour, previous surgical attempts at removal, and the presence of hydrocephalus, a contralateral tumour, and serviceable hearing. Twenty-nine other cases with the clinical diagnosis of acoustic neuroma underwent craniotomy: 20 had petrous or tentorial meningiomas encroaching on the porus acousticus or growing in the internal auditory canal, six had neuromas of the seventh cranial nerve and three had arachnoiditis occluding the internal auditory canal.