bluuebunny/crossref_metadata_2025_split huggingface dataset:
Roentgenographs Study of Cavus Foot Deformity in Friedreich Ataxia Patients: Preliminary Report
Authors: Allard P. , Sirois J.P. , Thiry P.S. , Geoffroy G. , Duhaime M. 
Published: July 2016

SUMMARY:The preliminary results based on a three year retrospective study in cavus foot deformity of forty-four Friedreich ataxia patients regularly seen at the Neuromuscular Disease Clinic of Sainte-Justine Hospital have been presented. An accurate “weight-bearing” foot stereoradiographic technique has been recently developed by our group. Since the follow-up period with this device is not sufficient to provide statistical information, the conventional non-weight bearing technique has been utilized in this study to enable a possible comparison between the radiographs of ambulant and non-ambulant patients. Due to the present technique, the results of this study must be interpreted with caution.For 132 pairs of radiographs, 28 parameters have been analyzed. Four of these, namely the calcaneal inclination angle, the first metatarsal inclination angle, the inferior cortex of calcaneus-first metatarsal angle and the first-fifth metatarsals angle, were of particular interest. From these parameters, a preliminary quantitative description of cavus foot deformity in Friedreich’s ataxia has been attempted. Three stages of evolution have been tentatively identified for this type of neurological disorder.
Pilot Study of Threonine Supplementation in Human Spasticity
Authors: Barbeau A. , Roy M. , Chouza C. 
Published: July 2016

ABSTRACT:Threonine supplementation (500 mg/day) was given to 6 patients with genetic spasticity syndromes for a period of 12 months, followed by a 4-month observation period without medication. All 6 patients showed partial improvement of spasticity, intensity of knee jerks and muscle spasms without changes in true pyramidal tract signs. The improvement in motor performance, objectively measured, averaged 29% (19% in upper limbs and 42% in lower limbs). The range of overall improvement was 19–35% (7–30% for upper limbs; 25–67% for lower limbs). No toxic clinical or biochemical side effects were encountered. Thus threonine, a precursor of glycine, produced the same effect on spasticity than that previously observed with glycine. It is concluded that threonine supplementation is feasible and safe and that it deserves a controlled trial in well defined (preferably genetic) cases of spasticity.
Oral Lecithin and Linoleic Acid in Friedreich’s Ataxia: II. Clinical Results
Authors: Melancon S.B. , Vanasse M. , Geoffroy G. , Barabe L. , Proulx A. , Fontaine G. , Dallaire L. , Potier M. 
Published: July 2016

SUMMARY:Twenty-two patients with Friedreich’s Ataxia and ten normal controls were followed for one year and assessed as to their clinical performance after two successive six-month periods of lecithin or safflower oil. Results demonstrated no significant difference in performance scores according to group assignation, neither in patients nor in controls. According to stages, two patients in stage I and to a lesser degree, one patient in stage IV showed better scores for muscle strength and some motor accuracy and coordination tests with lecithin. Controls as groups maintained positive scores in all tests. Patients as groups showed negative mean values in nine out of eleven tests. Again as groups, patients receiving safflower oil demonstrated a mean 8% less deterioration than patients receiving lecithin. This study demonstrates that objective clinical tests and the participation of normal controls are a must in a therapeutic trial implicating patients with a progressive disorder such as Friedreich’s Ataxia. The possible role of linoleic acid as the active factor from which clinical improvement proceeded in some specific patients and with early functional stages of the disease, has to be considered and reevaluated in the near future.
Percutaneous Localization of Conduction Abnormalities in Human Entrapment Neuropathies
Authors: Brown W.F. , Yates S.K. 
Published: July 2016

SUMMARY:In human entrapment neuropathies the characteristic abnormalities in conduction are frequently limited to a short segment of the nerve. Recognition and precise localization of these discrete conduction abnormalities may require measurement of conduction over shorter lengths of the nerves than those lengths commonly employed in the clinical laboratory. Techniques are described for the more precise location of the primary conduction abnormalities in median, ulnar and peroneal nerve entrapments. Distinctive or atypical locations of the major conduction abnormalities may point towards different mechanisms in the pathogenesis of these localized neuropathies.
Childhood Cerebral Cysticercosis: Clinical Features and Computed Tomographic Findings in 89 Mexican Children
Authors: López-Hernández Arturo , Garaizar Carmen 
Published: July 2016

SUMMARY:Cerebral cysticercosis was studied in 89 Mexican children. These patients were divided into two groups; those with proven diagnosis and those in whom the diagnosis was strongly suspected. The frequency of childhood cysticercosis at autopsy is significantly less than reported in adults from the same environment. Symptoms of initial cerebral invasion by the parasite include headache, fever, vomiting, and seizures. Cerebral edema is observed more frequently in children than in adults, and is the most common cause of intracranial hypertension. The cerebral edema generally is associated with an encephalitic syndrome, either a single bout or a chronic course with multiple relapses. Arterial hypertension, not previously described, was found in two probable cases. The variability of clinical symptomatology thus is great. Symptoms in childhood cerebral cysticercosis may be totally absent, moderate, or severe and ultimately fatal. Computed tomography of the brain is the most useful diagnostic aid, and determines the dynamic neuroradiologic sequence of this disease, since different images appear at each stage to permit its diagnosis. Uncalcified cysticerci may be demonstrated by contrast enhancement if associated with focal cerebral edema; images thus may disappear and reappear in the clinical course of the disease.
Valproic Acid Producing a Reye-Like Syndrome
Authors: Keene D. L. , Humphreys P. , Carpenter B. , Fletcher J.P. 
Published: July 2016

SUMMARY:Although several cases of acute hepatic failure associated with the administration of valproic acid (VPA) have been reported, the pathogenesis of this problem remains unclear. We report the case of a 40 month old male with a chronic seizure disorder treated with VPA and phenytoin for two years, who developed sudden progressive neurological deterioration with evidence of increased intracranial pressure and hepatic failure. The clinical features closely resembled those of Reye’s syndrome suggesting the possibility of a common pathogenesis.
Spinal Arachnoiditis
Authors: Esses Stephen I. , Morley T.P. 
Published: July 2016

SUMMARY:A review of the literature points to the many causes of arachnoiditis and the failure of treatment to arrest or reverse its effects. The true incidence cannot be determined, although it is probably lower than might at first appear from the published articles. In the radiological literature the diagnosis seems to derive from an examination of the films alone, often without reference to the clinical findings or appearance at operation. While attempts at treatment are usually unsuccessful, some iatrogenic cases can be prevented by the avoidance of intrathecal steroid injections or unduly rough or repeated surgical exploration of the lumbar vertebral canal.
Epilepsy from Cerebral Arteriovenous Malformations
Authors: Leblanc Richard , Feindel William , Ethier Roméo 
Published: July 2016

SUMMARY:Twenty-seven patients with epilepsy as the only manifestation of a cerebral AVM were seen at the MNI/MNH from 1973 to 1981. The nine females and eighteen males between the ages of fourteen and fifty-four years (Mean 25.2 years) had epilepsy for an average of 3.5 years prior to the diagnosis of AVM.Seven patients had primarily generalized seizures, ten patients had partial seizures with complex symptoms, fourteen patients had partial seizures with elementary symptoms, and thirteen patients had secondarily generalized seizures. Although there was no history of intracranial hemorrhage, seven patients had negative hemispheric signs (hemiatrophy, hemianopia, dysphasia, hemiparesis), two had impaired mentation, and two were ataxic. The EEG, normal in ten cases, showed non-epileptiform activity in six cases, and focal epileptiform activity in eleven cases. Angiographic and/or histological examination revealed eight small (less than 2 cm in diameter) and nineteen large lesions. Fifteen AVMs involved the frontal lobe, ten the temporal lobe, five the parietal lobe, and one the occipital lobe. Angiography failed to demonstrate three frontal and three temporal AVMs.Plain CT scanning demonstrated a high or low density lesion without a mass effect in sixteen cases, enlargement of the ipsilateral ventricle or Sylvian fissure in seven cases, and diffuse ventricular enlargement in three cases. CT scanning was performed after the intravenous injection of contrast material in nineteen cases and demonstrated vascular enhancement in fifteen cases and an abnormal blood vessel in six cases. Two angiographically occult AVMs demonstrated vascular enhancement with infusion CT scanning, thereby demonstrating the vascular nature of the lesion where angiography had failed to do so.
Life Effects of Narcolepsy: Relationships to Geographic Origin (North American, Asian or European) and to Other Patient and Illness Variables
Authors: Broughton Roger , Ghanem Quais , Hishikawa Yasuo , Sugita Yoshiro , Nevsimalova Sonia , Roth Bedrich 
Published: July 2016

SUMMARY:A recent questionnaire survey of the life-effects of narcolepsy in 180 patients, 60 each from North American, Asian and European populations, compared to similarly distributed age and sex matched controls, documented multiple and marked effects on work, education, driving, accidents, recreation, personality, memory and other parameters. The data have now been further analysed according to the patients’ geographic (culturo-genetic) origin and to a number of other patient and illness variables. The three different geographic populations showed few significant differences for the some 160 life-effects items in the questionnaire. This strongly indicates that these are an integral part of the disease or of the human reaction to it. Most of the few significant population differences appeared cultural in origin (e.g., concerning driving records, personality changes), although a few may possibly reflect genetic differences (e.g., visual problems). Analysis of the pooled data according to respondees’ age, sex, age at illness onset, duration of illness and treatment led again to relatively few significant findings. It is concluded that, in general, once the disease has been diagnosed, all the major life effects are present and remain so. The results strongly support the contention that most life-effects are not related to the diagnostic ‘tetrad’ symptoms themselves but rather to excessive daytime sleepiness, the symptom most resistant to anti-narcoleptic treatment.
Hemifacial Spasm: Results of Microvascular Relocation
Authors: Fairholm David , Wu Jiunn-Ming , Liu Kan-Nan 
Published: July 2016

SUMMARY:Twenty patients with hemifacial spasm were treated between February 1980 and June 1981. All presented with typical disabling unilateral contractions of the face. Computerized Tomographic Scan and angiograms ruled out structural mass lesions. All patients underwent posterior fossa microsurgical relocation of a vessel from the root entry zone of the facial nerve. An offending arterial loop was found in each case. Nineteen patients are free of symptoms and the remaining one is improved. One patient has permanent loss of hearing and two developed mild facial weakness. There has been no recurrence in 18 months follow up. These results give further support to the theory that hemifacial spasm is an affliction of the seventh nerve in the cerebello-pontine angle, most commonly caused by vascular cross compression at the root entry zone. Surgical relocation of the offending vessel relieves the symptoms and there appears to be no recurrence. This procedure is not difficult and carries acceptable risk for the patient with this disabling condition.
Correlation of Computed Tomography with Surgical Diagnosis in Lumbar Disc Disease
Authors: Griebel Robert , Tchang Stanley , Khan Moe , Varughese George 
Published: July 2016

SUMMARY:The lumbar spines of one hundred patients with suspected lumbar disc herniations were analysed with computed tomography. The results are compared with the patients’ surgical diagnosis and with myelography. CT scanning is with few exceptions the diagnostic procedure of choice in investigating lumbar disc disease.
Etiology of Parkinson’s Disease: A Research Strategy
Authors: Barbeau André 
Published: July 2016

SUMMARYIn this essay I present a new “global approach hypothesis” to explain the pathophysiology of Parkinson’s disease: “Susceptibility to Parkinsonism is genetically determined and is reflected in all cells. I propose that idiopathic Parkinson’s disease is the combined result of a generalized cell aging process accelerated, in susceptible individuals, by a variety of often repetitive trigger factors. These factors have in common the fact that they cause a transient increase in turnover within catecholamine producing neurons, centrally as well as peripherally. This results in accumulation within these neurons of free radicals. When the level of the toxic substances, in quantity or in time of exposure, exceeds the scavenging capacity of the cell, damage to organelles and to membranes results, leading to the formation of Lewy bodies through an autoimmune reaction to damaged filaments and to cell death, particularly in the pigmented neurons of the brainstem. The progressive cell depletion leads to a compensatory increase in catecholamine turnover in the remaining pigmented cells, and an ever-accelerating degenerative process. The resulting neurotransmitter imbalance in the basal ganglia explains the symptoms of Parkinson’s disease”. In the light of this hypothesis, our research objectives should be (1) to delineate the limits of true Parkinson’s disease from all phenocopies; (2) to identify individuals susceptible to parkinsonism and the most common trigger factors; (3) to reduce the metabolic effects of unavoidable trigger factors and (4) to protect susceptible individuals by increasing the functional availability of free radical trapping agents.
Fulminating Haemophilus Influenzae b Meningitis
Authors: MacDonald N.E. , Keene D.L. , Mackenzie A.M.R. , Humphreys P. , Jefferies A.L. , Ivan L.P. 
Published: July 2016

SUMMARYHaemophilus influenzae type b (HIb) is the most common cause of bacterial meningitis in children with a mortality rate ranging from 1.6% to 14%. Most patients have a 2-3 day history of symptoms prior to admission. A few have fulminating disease with rapid neurological deterioration. Review of 191 cases of HIb meningitis revealed a mortality rate of 2.1% but all who died had fulminating meningitis (FM). Four of six patients with FM died. FM patients had symptoms for less than 24 hours before rapid neurological deterioration with increased ICP, seizures, coma and/or respiratory arrest. Review of 10 FM cases revealed that on admission, 5 had hypotension, 3 had thrombocytopenia, and 8 had coma. Typical CSF changes were seen in only 7. All fatal cases died within 24 hours. Brain swelling and tonsillar herniation were found at autopsy. SDS-PAGE outer membrane protein subtyping did not show one “killer strain”. Animal and autopsy data suggest that diminished CSF outflow and cerebral edema contribute to increased ICP. To improve survival of FM patients, initial treatment must (1) decrease ICP below levels impairing cerebral perfusion, (2) maintain adequate ventilation and blood pressure, and include (3) LP when stable, (4) antibiotics, and (5) close monitoring. Utilizing these principles, two FM patients survived without major sequelae.
Dependence of EMG Responses Evoked by Imposed Wrist Displacements on Pre-existing Activity in the Stretched Muscles
Authors: Bedingham W. , Tatton W.G. 
Published: July 2016

ABSTRACT:The relationship between the segmented EMG activity in flexor carpi radialis evoked by imposed angular wrist displacement was studied with respect to the level of pre-existing background activity in 30 normal human subjects. Input-output response planes demonstrate that the magnitude of the Ml & M2-3 segments is dependent on both the displacement parameters and the level of pre-existing EMG activity in the stretched muscle. If the level of background activity exceeded 4-5% of the maximum voluntary contraction, the onset latency of the M1 segment and duration of the Ml and the M2-3 segments remained constant (within ± 2 msec) for different magnitudes of step load displacements, despite marked variation in the range of the displacement’s amplitude, duration, velocity, and acceleration. We propose that the dependency of the relationship between reflex magnitude and imposed movement parameters on tonic motoneuron activity, as represented by pre-existing EMG levels, may reflect an automatic adjustment mechanism that could be utilized in servo compensation of movements requiring markedly different force levels.
Characteristic Alterations in Responses to Imposed Wrist Displacements in Parkinsonian Rigidity and Dystonia Musculorum Deformans
Authors: Tatton W.G. , Bedingham W. , Verrier M.C. , Blair R.D.G. 
Published: July 2016

ABSTRACT:The amplitude and temporal modulation of the segmented EMG activity in flexor carpi radialis, evoked by imposed angular wrist extension, was studied with respect to the level of pre-existing background activity in rigid parkinsonian (PK) and dystonia musculorum deformans (DMD) patients. The interdependence of the evoked M1 and M2-3 segments on pre-existing background EMG activity and initial velocity of imposed displacement was established previously for a normal population. Individual responses of 21 parkinsonian and 12 dystonic patients were compared to the established normal “response volume”. The augmented magnitude of the M2-3 segment in rigid PK patients, which correlates to the measure of rigidity, could not be accounted for by the low level of pre-existing EMG activity. Therefore, increased descending facilitation does not impinge directly on alpha motoneurons. Paradoxical excitation in the shortened muscle and resetting of tonic tremor of the stretched muscle by the imposed wrist extension are two other demonstrated abnormalities which may also contribute to PK rigidity. In contrast, DMD patients demonstrated normal amplitude modulation of the M1 and M2-3 segments, but exhibited a disturbance of normal temporal mechanisms that result in constant duration of the M1 and M2-3 responses with imposed force step loads.
Astrocytoma Following Scalp Radiotherapy in Infancy
Authors: Zochodne Douglas W. , Cairncross J. Gregory , Arce Felix P. , MacDonald John C.F. , Blume Warren T. , Girvin John P. , Kaufmann John C.E. 
Published: July 2016

ABSTRACTA 24 year old woman with a 15 year history of intractable seizures underwent resection of a small low grade astrocytoma of the right mesial frontal lobe. The tumor arose beneath a benign scalp nevus that had been treated in infancy with radium patches and focal x-irradiation. Neuropathological changes within the tumor were compatible with radiation injury. Meningiomas and sarcomas complicate cranial irradiation but the evidence that gliomas do so is less convincing. Our observations support an association between radiation exposure and the subsequent development of glial tumors.
Cerebral Glucose Metabolism in Parkinson’s Disease
Authors: Martin W.R.W. , Beckman J.H. , Calne D.B. , Adam M.J. , Harrop R. , Rogers J.G. , Ruth T.J. , Sayre C.I. , Pate B.D. 
Published: July 2016

ABSTRACTLocal cerebral glucose utilization was measured in patients with predominantly unilateral Parkinson’s disease using 18F-2-fluoro-deoxyglucose and positron emission tomography. Preliminary results indicate the presence of asymmetric metabolic rates in the inferior basal ganglia. The structure comprising the largest portion of basal ganglia at this level is globus pallidus. These findings are consistent with metabolic studies on animals with unilateral nigrostriatal lesions in which pallidal hypermetabolism on the lesioned side has been demonstrated. Increased pallidal activity is likely secondary to a loss of inhibitory dopaminergic input to the striatum from substantia nigra.
Are Levodopa “Drug Holidays” Justified?
Authors: Kofman Oscar S. 
Published: July 2016

ABSTRACTComplete withdrawal of all levodopa compounds “drug holiday” for a period of at least one week in severely disabled, unresponsive Parkinson patients may influence receptor blockade by resensitizing and can alter denervation hypersensitivity.Twenty four patients with 31 holidays were evaluated. Transient improvement was noted in most patients, however significant long term benefit of over 1 years duration occurred in only 6 individuals.In view of the unpredictability and limited responsiveness, drug holiday has not yet been established as a satisfactory treatment for the severe, intractable problems associated with chronic levodopa administration in Parkinson patients. Further trials appear to be warranted.
Cough Syncope Mimicking Epilepsy in Asthmatic Children
Authors: Haslam Robert H. A. , Freigang Bruno 
Published: July 2016

ABSTRACT:Cough syncope is a more common complication of childhood asthma than formerly recognized. We report twelve children with typical cough syncope who were identified in a pediatric clinic over a period spanning seven years. The condition may be confused with epilepsy because of frequently associated brief clonic convulsive movements during the height of the cerebral anoxia. Cough syncope is readily distinguished from epilepsy by a thorough history. The management and prevention of cough syncope is directed at the aggressive control of bronchospasm in children with asthma.
The Effects of Local Cooling on Canine Spinal Cord Blood Flow
Authors: Hansebout Robert R. , Lamont R. Neil , Kamath M. Venkatraya 
Published: July 2016

ABSTRACT:The internal spinal cord blood flow was measured in dogs at the site of local cooling using hydrogen polarography. Blood flow decreased to 50% of the normothermic values during cooling of the cord to a central temperature of 16 degrees Celsius. Upon cessation of cooling internal blood flow rapidly returned to normal values. Implications of this finding for the treatment of spinal cord injury are discussed.
Osier and Neurology
Authors: Ebers George C. 
Published: July 2016

William Osier’s bibliography includes over 1400 papers, monographs, and notes touching on almost every subspecialty of medicine (Abbott 1939). He has been claimed by gastroen-terologists (Cunha 1948), pediatricians (McGovern et al 1970; Robbinsetal 1963), medical librarians, (Bett 1949), veterinarians (Giltner 1926–27) (Murphy 1960) obstetricians (Rucker 1952) and members of other disciplines as one of their own.Osier published close to 200 papers, reviews, editorials and monographs dealing with neurology, but it is not widely recognized that his contributions to neurology exceed those to many of the other fields. For example, Osier’s interest in neuropathology was entirely omitted from the recent excellent monograph “An Oslerian Pathology” (Rodin 1981). The purpose of this paper is to bring into focus his impressive neurological contributions and to document his substantial interest in the neurosciences.Osier’s activities in neurology constituted a microcosm of his overall medical contributions. His interests in the neurosciences began early. In 1884 he wrote on the comparative anatomy of the brain of the seal (Osier 1884a), having given a lecture on “the brain as a thinking organ” in the previous year (Osier 1883a).