bluuebunny/crossref_metadata_2025_split huggingface dataset:
Abnormal Electroretinograms and Visual Evoked Potentials in Chronic Papilledema Using Time-Difference Analysis
Authors: Kirkham T.H. , Coupland S.G. 
Published: July 2016

SUMMARY:Visual function is usually considered to be normal in papilledema. We report previously undescribed abnormalities in the electroretinograms (ERG) and visual evoked potentials (VEP) of two patients with chronic papilledema. The steady-state latencies of the ERG and VEP were calculated using time-difference analysis. The ERG was abnormal under both scotopic (slow) and photopic (fast) flicker conditions, but the VEP was abnormal only to fast flicker photopic stimulation. This may indicate a relative vulnerability of a high frequency signal system under conditions of raised intracranial pressure. The abnormalities resolved rapidly once the intracranial pressure had been normalised. The cause of the V EP and particularly the ERG abnormalities remains unknown.
Heterotopic Growth of Dysplastic Cerebellum in Frontal Encephalocele in an Infant of a Diabetic Mother
Authors: Sarnat Harvey B. , deMello Daphne E. , Blair John D. , Siddiqui Saeeda Y. 
Published: July 2016

SUMMARY:An infant of a diabetic mother lived 13 days after birth. She had a small dysplastic 12.6 gm. brain, partly forming a frontal encephalocele. The uncleaved forebrain contained a mass of poorly organized heterotopic cerebellar cortex. The cerebellum itself had normal lamination, but was small and continuous with the dysplastic tissue. The ventricular system was absent except for a few midline ependymal rosettes, and the cerebral cortex was not developed. The cerebellar dysplasia resembled a proliferative and invasive lesion by its rostral extension.
A Case of Charcot-Marie-Tooth Disease Mimicking Friedreich’s Ataxia: is there any association between Friedreich’s ataxia and Charcot-Marie-Tooth disease?
Authors: Salisachs P. , Findley L.J. , Codina M. , Torre P. La , Martinez-Lage J.M. 
Published: July 2016

SUMMARY:The authors report a case of Charcot-Marie-Tooth disease that mimicked Friedreich’s ataxia and featured impaired tendon reflexes in the limbs, incoordination mimicking cerebellar disease in the extremities, extensor plantar responses on both sides, bilateral foot deformity, impaired position sense in the toes, absent vibratory sense in the distal parts of the legs and minimal distal weakness with wasting. Motor conduction velocity in the upper limbs was substantially reduced.Other cases similar in nature reported in the literature resemble spino-cerebellar degeneration in general, and Friedreich’s ataxia, in particular. It is emphasized that the natural history, EMG, motor conduction velocity studies and examination of other affected members of the family permit the correct diagnosis to be made in such cases. It is also emphasized that patients similar to the one reported here may also resemble, and should be differentiated from, cases of familial dorsal column ataxia (Biemond type). Stress is put upon the fact that when Charcot-Marie-Tooth disease mimicks spino-cerebellar degeneration, substantial slowing of motor conduction in the upper limbs is generally sufficient to establish the diagnosis.The relation between Friedreich’s ataxia and Charcot-Marie-Tooth disease is reviewed and it is concluded that these two disorders are distinct clinical and pathological entities.
Kinematics of the Foot
Authors: Allard P. , Thiry P.S. , Duhaime M. , Geoffroy G. 
Published: July 2016

SUMMARY:Orthogonal stereoradiographs are frequently utilized in determining three-dimensional geometrical parameters of human body segments. They have been applied here in the estimation of the length and elongation of the ligaments of the normal foot. Three small spherical metallic markers were respectively encrusted into the tibia and fibula, the seven bones of the tarsus and into the five metatarsals of an amputed lower limb to identify uniquely their spatial location. The foot was then positioned on a rotating platform. Standardized antero-posterior and lateral radiographs were taken. Afterwards the foot was dissected and the proximal and distal insertions of most of its ligaments were located by means of spherical markers. A second series of orthogonal radiographs were taken of each of the fourteen bones. The radiographs were digitized. The length of each ligament and elongation for a simple and complex movements were calculated by means of a computer program. The results of a simple movement of rotation representing a normal 20° dorsiflexion at the talocrural joint and of complex movements of rotation simulating an abnormal high arch such as encountered in Friedreich’s ataxia are presented and discussed.
Oral Lecithin and Linoleic Acid in Friedreich’s Ataxia: III. Biochemical Results
Authors: Melancon S.B. , Dallaire L. , Potier M. , Cousineau J. , Vanasse M. , Geoffroy G. , Fontaine G. , Grignon B. 
Published: July 2016

SUMMARY:Lecithin and sqfflower oil brought about the same changes in serum LAD activity and kinetics in patients with Friedreich’s Ataxia as in controls when results of this double-blind crossover study were analyzed according to group assignation. According to functional stages, pretrial LAD activity decreased with advancing severity while Km for lipoamide increased. Lecithin and sqfflower oil supplements corrected the elevated Km for lipoamide but produced a further reduction in LAD activity. These changes may have been due to the increased intake of linoleic acid, a precursor of lipoic acid, which is present in high percentage in both lecithin and safflower oil. Results of the biochemical study thus agreed with the clinical data gathered during the course of the one-year trial in suggesting that linoleic acid may well have been the active factor through which biochemical and clinical improvement was previously observed in patients with Friedreich’s Ataxia supplemented with lecithin.
Amino Acid Changes in the Mouse Mutant Dystonia Musculorum Similar to Those in Friedreich’s Ataxia
Authors: Messer A. 
Published: July 2016

SUMMARY:An autosomal recessive mutant strain of mouse with a progressive neurological disorder is described. Histopathology is dramatic in the sensory afferents and in the red nucleus. In the cerebellar vermis the concentrations of glutamate, aspartate, glycine and GABA are significantly reduced, and in the cerebellar hemispheres the taurine/glutamate ratio is elevated. These mice may provide a useful experimental model of Friedreich’s ataxia.
Increased Plasma Catecholamines in Patients with Friedreich’s Ataxia
Authors: Pasternac A. , Wagniart P. , Olivenstein R. , Petitclerc R. , Krol R. , Andermann E. , Melancon S. , Geoffroy G. , Champlain J. de , Barbeau A. 
Published: July 2016

SUMMARY:We studied free plasma catecholamines in 23 patients with Friedreich’s ataxia, having a mean age of 22 ± 9.6 (SD) years. Conjugated catecholamines were also studied in 10 patients. Mean plasma norepinephrine and epinephrine were significantly higher than controls both in the supine and standing positions. In total 15 out of 23 patients (65%) had increased free and/or conjugated plasma catecholamines. The increase in plasma catecholamines was more marked in patients with severe neuromotor impairment. Among the patients with left ventricular concentric hypertrophy (wall thickness >12 mm), only 3 had no demonstrable sympathetic hyperfunction.Since the high local concentrations of norepinephrine at the site of release from sympathetic nerve terminals may serve as a trigger for the hypertrophic response of the myocardial cell, it is suggested that early pharmacological intervention could prevent or limit the cardiomyopathic process or its clinical consequences.
Effect of a Valine Load Test on Plasma Alpha-Keto Acids in Friedreich Ataxia
Authors: Barbeau A. , Bertrand M. , Bouchard R. , Gauthier G.L. , Bouchard J.P. 
Published: July 2016

SUMMARY:To test the physiological significance in vivo of our previous in vitro finding of reduced valine dehydrogenase (VDH) activity inpatients with Friedreich’s Ataxia, we subjected ataxic patients and controls to an oral valine load test (1.0g) and measured the levels of branched chain α-keto acids in the plasma for 24 hours. We demonstrated a significantly higher peak for α-keto isovaleric acid in Friedreich’s Ataxia and a general trend towards higher than control values in all other α-keto acids measured, and at all times in the experiment. These changes are compatible with the postulated defect in regulation of the activity of VDH in this illness, but because of their small amplitude, they also indicate that a VDH – deficiency is not the genetic defect in Friedreich’s A taxia.
Respiratory Arrest and Cervical Spinal Cord Infarction Following Lumbar Puncture in Meningitis
Authors: Norman Margaret G. 
Published: July 2016

SUMMARY:A 6-year-old child with meningitis had a respiratory arrest 20 minutes after a lumbar puncture. Thereafter she required maintenance on a ventilator, had a flaccid quadriplegia, and died 12 days later. Necropsy showed infarction of the central portion of the cord at the level of the decussation of the pyramids. The suggested mechanism of damage is compression of the arterial supply to the cord at the level of the foramen magnum by herniated cerebellar tonsils; concomitant hypotension may have contributed to production of the damage. Four similar cases, who survived with residual deficit, have also been reported. Other separate mechanisms by which the cord can be damaged in meningitis are vasculitis, thrombosis and arachnoiditis.
Results of Burr Hole and Open or Closed Suction Drainage for Chronic Subdural Hematomas in Adults
Authors: Weir B.K.A. 
Published: July 2016

SUMMARY:Chronic subdural hematomas in adults are not technically difficult to treat. They probably constitute one of the most common potentially curable lesions with which the average neurosurgeon deals. Despite this (or perhaps because of it) numerous treatments, radically differing from one another, have been advocated. Fine judgement is called for in deciding whether or not to operate in the first instance, make a flap or one or more burr or twist drill holes, use a drain, employ suction, inflate the brain, leave the bone flap out. In a personal series of 71 chronic subdural hematomas, 46 were treated with a red rubber catheter drain through a burr hole and 23 with a closed system suction drain. The results of this series suggest that closed system suction drainage through a single burr hole is the better of the two techniques. It has important theoretical advantages and the procedure is simple and fast. The complication rate is low and patients have a shortened postoperative hospital stay.
Endartériectomie carotidienne: histoire médicale préopératoire et devenir à long terme de 82 patients
Authors: Trudel L. , Fabia J. , Bouchard J.P. 
Published: July 2016

SUMMARY:Using medical files, death certificates and an interview at home for the survivors, a long term retrospective follow-up study was done for 82 subjects operated for carotid stenosis. Causes of death, survival rates and medical history of survivors were studied. Subjects were classified in two preoperative risk categories according to their medical history prior to angiography: low risk (36 subjects) and medium risk (46 subjects). None of the subjects were lost during the follow-up period which varies from 81 to 105 months.At seven years, observed survival rate is significantly lower for the medium risk patients than for the low risk patients (43.0 ±8.6 versus 80.6 ± 6.6; X2 M-H, p = 0.006). The difference between the survival rates is markedly noticeable after the fifth year of follow-up. Besides, the percentage of survivors who have developed cerebrovascular problems after the operation is significantly higher in the medium risk group than the low risk group (54.5% versus 25.0%, X2 p ≃ 0,04).Does this mean that the preoperative risk classification separates two different stages or two different forms of atherosclerosis? Can. J. Neurol. Sci. 1983; 10:27–31
Facilitation of Kindled Seizures in Rats Fed Choline-Supplemented Diets
Authors: McCann Kevin , Cain Donald P. , Philbrick Diana J. 
Published: July 2016

SUMMARY:Different groups of rats were maintained on choline-deprived (CD), choline-supplemented (CS) or standard rat chow diets beginning at a body weight of approximately 100 g, and electrically kindled in the amygdala beginning not less than 40 days later. The CS group kindled significantly faster than the CD group, whereas the CD and chow-fed groups did not differ in their rate of kindling. This result is consistent with both the known effects of the dietary manipulation of choline on brain acetylcholine level, and the idea that acetylcholine has a role in amygdaloid kindling.
Acute Hemorrhagic Leukoencephalopathy
Authors: Suchowersky O. , Sweeney V.P. , Berry K. , Bratty P.J.A. 
Published: July 2016

SUMMARY:Two patients with acute hemorrhagic leukoencephalopathy, one of which was pathologically proven, were serially studied with CT scanning. Both patients showed marked distinctive low density white matter changes throughout both hemispheres, which correlated with clinically involved areas. One patient recovered from the disease, perhaps due to steroid treatment, and showed slow but complete resolution of CT scan changes. We feel that CT scan findings significantly help in the diagnosis of this disease, which may be amenable to early treatment with steroids.
Hemispherectomy for Seizures Revisited
Authors: Rasmussen Theodore 
Published: July 2016

SUMMARY:The serious, late complication of superficial cerebral hemosiderosis, which appears after several years in 1/4–1/3 of patients who have undergone hemispherectomy, has resulted in recent years in a considerable reluctance to carry out this operation despite the fact it has proved to be highly effective in patients with medically refractory seizures associated with hemiplegia. Preservation of a small portion of the hemisphere, usually the frontal or occipital pole, has proved to be effective in preventing this late complication, but at the cost of a significant reduction in the effectiveness of the operation in reducing the patients’ seizure tendency. Preserving the frontal and occipital poles but disconnecting them from the rest of the brain, resulting in a functional complete but anatomical subtotal hemispherectomy, retains the therapeutic effectiveness of a complete hemispherectomy while still protecting adequately against the serious late postoperative complication of superficial cerebral hemosiderosis and its associated neurologic deterioration, hydrocephalus and sometimes death.
Febrile Seizures in Epileptic Chicks: The effects of Phenobarbital, Phenytoin and Valproate
Authors: Johnson D.D. , Crawford K.D.A. , Crawford R.D. 
Published: July 2016

SUMMARY:Epileptic seizures can be evoked in chicks homozygous for the epileptic seizure gene (epi, epi) by elevating their body temperature using microwave diathermy. These seizures precede and differ in motor seizure pattern from a second clonic-tonic seizure produced by hyperthermia in both epileptic and carrier (heterozygote, Epi, epi) chicks. Hyperthermia did not evoke seizures in adult epileptic chickens. Phenobarbital delayed the onset of epileptiform seizures whereas phenytoin and valproate had no effect. These data suggest that epileptic chicks may provide a suitable model for studies on febrile convulsions.
Neurology and Neurosurgery in Nepal
Authors: Lee Robert G. , Gongal Dinesh N. 
Published: July 2016

During early 1982 one of the authors (RGL) was privileged to spend two months in Nepal as part of an exchange program between the University of Calgary, Faculty of Medicine and the new medical school at Tribhuvan University in Kathmandu. A short visit of this type does not qualify a Canadian neurologist to comment on the neurological-neurosurgical problems of a country such as Nepal with any degree of expertise. Nevertheless, as a result of a number of discussions between the two authors and interviews with many other physicians, both Nepalese and Western, it was possible to form some general impressions and draw some comparisons between neurologic illnesses in Nepal and Canada.Disorders affecting the nervous system are common in Nepal. Some of the problems which are seen regularly would be considered rare and exotic in North America. However, despite the vast differences in culture, geography, dietary habits, and socio-economic conditions, there are some surprising similarities between the patterns of neurologic illness in Nepal and Canada. Disorders such as migraine, stroke, epilepsy and meningitis are well known to Nepali physicians. To appreciate the magnitude of health care problems in Nepal and to view neurologic illness in its proper perspective, it is helpful to have a few basic facts and figures concerning the geography and population of the country.
Evaluation of Radiculopathies by Segmental Stimulation and Somatosensory Evoked Potentials
Authors: Eisen Andrew , Hoirch Maureen , Moll Alex 
Published: July 2016

SUMMARY:Thirty-six patients with suspected or myelographically proven radiculopathies were investigated with motor and sensory conductions, F-waves, needle electromyography, and somatosensory evoked potentials (SEPs). SEPs were elicited by cutaneous nerve stimulation representative of input from individual cervical and lumbosacral dorsal roots. A myelographic defect was present in 83% of 30 patients who had myelograms. Overall 78% of patients had one or more abnormal electrophysiologic tests, the needle EMG giving the best diagnostic yield (75%). F-waves and SEPs were abnormal in 43% and 57% of cases respectively. Motor deficit correlated best with abnormal EMGs, whilst abnormal SEPs occurred most frequently when sensory deficit predominated. Prolonged latency of the SEP occurred rarely, reduced amplitude or abnormal morphology being the most useful characteristics. SEPs evoked by cutaneous nerve stimulation are a useful addition to conventionally available electrophysiological methods of evaluating radiculopathies, especially in the absence of motor deficit.
Intradural Rupture of Lumbar Intervertebral Disc
Authors: Lee Shih-Tseng , Fairholm David 
Published: July 2016

SUMMARY:Two cases of intradural disc rupture with cauda equina compression are reported. Myelography demonstrated complete block and at surgery intradural fragments of sequestrated disc material were found. The pathogenesis of this condition is reviewed and our recommendation for management is presented.
Paraneoplastic Subacute Necrotic Myelopathy
Authors: Handforth Adrian , Nag Sukriti , Sharp Daniel , Robertson David M. 
Published: July 2016

SUMMARY:A 79 year old male acutely developed a Brown-Sequard syndrome two weeks following resection of renal cell carcinoma. The subsequent clinical course was marked by subacute progression of spinal cord signs. Pathologic studies showed extensive multifocal spinal cord necrosis that could not be attributed to vascular occlusion or to vasculitis. This case appears to be a rare example of paraneoplastic subacute necrotic myelopathy.
CSF Myelin Basic Protein Levels in Acute Optic Neuritis and Multiple Sclerosis
Authors: Warren K.G. , Catz Ingrid , McPherson T.A. 
Published: July 2016

SUMMARY:Normal CSF-MBP levels as determined by a RIA were <6.2 ng/ml CSF (mean 3.9). Eighty percent of patients with acute optic neuritis have CSF-MBP levels greater than 6.2 ng/ml (mean 7.6 ng/ml CSF). Five of 7 patients with acute internuclear ophthalmoplegia due to an initial exacerbation of demyelination have CSF-MBP levels above 6.2 ng/ml (mean 6.8 ng/ml). Fifty percent of MS patients with chronic progressive disease have CSF-MBP levels above 6.2 ng/ml (mean 6.7 ng/ml). MS patients experiencing monosymptomatic exacerbations show elevated CSF-MBP levels in 75% of cases (mean 8.2 ng/ml). MS patients experiencing polysymptomatic exacerbations show significantly higher levels of CSF-MBP (mean 22.3 ng/ml) than the patients with monosymptomatic exacerbations. Ninety-five percent of MS patients experiencing polysymptomatic exacerbations have elevated levels of CSF-MBP.
Spinal Injuries Due To Hockey
Authors: Tator Charles H. , Ekong Chris E.U. , Rowed David W. , Schwartz Michael L. , Edmonds Virginia E. , Cooper Perry W. 
Published: July 2016

SUMMARYAlthough many types of sports and recreational activities have been identified as common causes of acute spinal cord injury, hockey has been a rare cause of acute cord injury in Canada or elsewhere. For example, from 1948 to 1973 there were no patients with cord injuries due to hockey in a series of 55 patients with acute cord injuries due to sports or other recreational activities admitted to two Toronto hospitals. In contrast, between 1974 and 1981, the Acute Spinal Cord Injury Unit, Sunnybrook Medical Centre treated six patients with cervical spinal injury due to hockey, five of whom were seen during a 13 month period from September, 1980 to October, 1981. Five of the six sustained a severe acute cervical spinal cord injury, and one a cervical root injury. The cord injury was complete in two cases, while three had complete motor loss but incomplete sensory loss below the level of the lesion. All were males aged 15 to 26 years. Of the players with cord injury, four struck the boards with the neck flexed, and one struck another player with the neck flexed. The one player without cord injury struck the boards with his neck extended. The commonest bony injury was a burst fracture of C5 or C6. One of the patients with a complete cord injury died three months later of a pulmonary embolus, and the other patients with cord injury showed some recovery of root function, but little or no cord recovery. The reasons for the increase in spinal injuries in hockey are unknown.
Pharmacokinetic Interactions of Antiepileptic Drugs
Authors: Albright Penny S. , Bruni J. 
Published: July 2016

ABSTRACT:The problem of antiepileptic drug interactions is significant in that many epileptic patients are treated with multiple drug therapy. Moreover, patients may also be receiving additional medication for other concurrent disorders. Most drug interactions are pharmacokinetic, involving changes in absorption, protein binding, metabolism, or excretion. As a result, plasma levels of the antiepileptic drug may decrease leading to exacerbation of seizures. Alternatively, plasma levels may rise resulting in toxic side effects. Similar changes may also occur with drugs given for other disorders. In this paper, possible mechanisms of drug interactions are discussed. This is followed by a description of clinically significant interactions involving phenytoin, carbamazepine, barbiturates, valproic acid, benzodiazepines, and succinimides. Potentially serious drug interactions may be minimized by using as few medications as possible and by regularly monitoring plasma levels of antiepileptic drugs.
Occlusive Cerebrovascular Disease in Young Adults
Authors: Klein Gary M. , Seland T. Peter 
Published: July 2016

ABSTRACT:This retrospective study documents the experience of two large Canadian teaching hospitals with occlusive cerebrovascular disease in young adults. Chart review disclosed 76 patients aged 15-40 years during a recent six year period. An apparent cause, or significant coincident risk factors were found in 51 patients (67%). The most prevalent recognized causes were atherosclerosis, emboli from cardiac sources or intracranial aneurysms, and complicated migraine. Pregnancies or use of oral contraceptives were apparent coincident risk factors.