bluuebunny/crossref_metadata_2025 huggingface dataset:
Dopamine Uptake Capacity of Platelets From People At Risk For Huntington’s Chorea
Authors: McLean Donald R. , Nihei Taichi
Published: July 2016

Dopamine (DA) uptake by platelets from 41 patients at risk to develop Huntington’s chorea was assayed and compared to controls. The DA uptake was significantly higher in the at risk group. However, the at risk group did not separate into two distinct subgroups. which reduces the usefulness of the assay as a disease prognosticator.
Anoxic Tonic Seizures Due to Asthma; A Serious Complication in Adults
Authors: Keene D.L. , Melmed C.A. , Andermann F. , Baxter D.W.
Published: July 2016

Anoxic tonic seizures are reported as a complication of severe asthma in two adults. The nature of these attacks can be misinterpreted. They do not indicate the presence of a recurrent seizure problem unconnected to the episodes of anoxia caused by asthma. These attacks do not respond to antiepileptic medication, but cease if the asthma can be controlled. One of our patients developed an anoxic encephalopathy and the other died. The need for prompt diagnosis and appropriate treatment is emphasized.
Trigeminal Neuralgia: A Comparison of the Results of Percutaneous Rhizotomy and Microvascular Decompression
Authors: Ferguson G.G. , Brett D.C. , Peerless S.J. , Barr H.W.K. , Girvin J.P.
Published: July 2016

Seventy-five patients were treated between March 1976 and June 1980 for classical idiopathic tic douloureux. Fifty-five patients underwent percutaneous trigeminal rhizotomy (PTR) and twenty-four had posterior fossa microvascular decompression (M VD) of the trigeminal nerve. Four patients had both procedures. In the PTR group. 4% were immediate failures, 42% had a delayed recurrence of pain, while 54% remained totally pain free with an average follow-up of 30 months. In the MVD group, 12% were immediate failures, 17% had a delayed recurrence of pain, and 71% have remained free of pain with an average follow-up of 28 months. Neither procedure can be regarded as ideal surgical treatment for patients with pain refractory to medical treatment. Percutaneous rhizotomy has an established place because of its safety, particularly in elderly patients. A high rate of recurrent pain is to be expected. Microvascular decompression has appeal in younger patients because of its non-destructive nature but the long term efficacy of the procedure is not known.
Occult Fifth Nerve Dysfunction In Multiple Sclerosis
Authors: Eisen Andrew , Paty Donald , Purves Sherrill , Hoirch Maureen
Published: July 2016

Somatosensory evoked potentials (SEPs) were recorded following trigeminal nerve stimulation in 25 normal subjects. Mucosal stimulation of the lip resulted in a reduced stimulus artefact. The three initial peaks, N13, P19, and N30, measured respectively 12.8 ± 0.9 S−3, 19.3 ± 1.4 S−3 and 28.6 ± 1.7 S−3. Blink reflex studies were also performed in most of these subjects. In 41.4% of 29 patients with established or suspected multiple sclerosis, the trigeminal SEP was abnormal. Additional use of the blink reflex raised the overall incidence of trigeminal nerve dysfunction to 51.7%. None of the patients had clinical evidence of fifth nerve involvement either historically or on examination. Four of seven patients with progressive spinal MS and two patients whose only deficit was that of optic neuritis, had abnormal trigeminal SEPs. It is concluded that occult involvement of the pontine fifth nerve structures occurs frequently in MS despite the rarity of corresponding clinical findings. The trigeminal SEP is a useful additional neurophysiological method.
Abnormal Adaptation of Visual Contrast Sensitivity In Multiple Sclerosis Patients
Authors: Raymond J. , Regan D. , Murray T.J.
Published: July 2016

Some multiple sclerosis patients with 20/20 acuity complain of poor vision. In a previous report we accounted for this in our patient group by showing that multiple sclerosis had caused a depression of contrast sensitivity while sparing visual acuity. In this study we investigated whether some of this measured depression might be due to abnormally rapid or severe adaptation during the test procedure rather than a true permanent loss. Our finding was opposite to this supposition: adaptation was abnormally slight and/ or slow.

Depressed contrast sensitivity was not well correlated with abnormal adaptation to contrast. In patients whose contrast sensitivity losses were restricted to a band of spatial frequencies, we found no evidence that abnormalities of contrast adaptation were restricted to this same spatial frequency band. Further evidence of dissociation between abnormal contrast sensitivity and abnormal contrast adaptation is that some patients with normal contrast sensitivity showed abnormally small adaptation.

Our finding of abnormally slow or abnormally small contrast adaptation in MS patients seems to conflict with Enoch et al’s (1978) report of abnormally great adaptation after inspecting a bright stimulus. This apparent disagreement may be due to their use of prior dark adaptation, very bright adapting stimuli and patients in the acute stage of retrobulbar neuritis.
Transient Choreiform Dyskinesias During Alcohol Withdrawal
Authors: Fornazzari L. , Carlen P. L.
Published: July 2016

Three chronic alcoholics developed choreiform dyskinesias involving the face, lips, tongue and, in one case, all limbs; 2 patients for the first time, 9 to 10 days after alcohol withdrawal. These abnormalities improved spontaneously with maintained abstinence from alcohol for 2 to 7 weeks. None had a family history of movement disorder, there was no history of other psychoactive drug use or abuse, and there was no evidence of portal-systemic encephalopathy.
A Tentative Classification of Recessively Inherited Ataxias
Authors: Barbeau André
Published: July 2016

We present a working classification of recessively inherited ataxic syndromes based on the use of simple tools available to every clinician: a good history (particularly pinpointing the age of onset) and a good neurological examination (simplified to the verification of the presence of ataxia, deep tendon reflexes in the knee, optic nerve, retinal and/or 8th nerve signs). In the three groups of disorders (non progressive, intermittent or progressive) patients can be hyper/normo reflexic, or they can be hypolareflexic. Six principal types of progressive ataxic disorders are further delineated by the age of onset. Sub-types depend on the presence or absence of eye and ear signs, whereas eponymic or regional denominations are used only for simplicity while awaiting exact delineation of the biochemical defects.
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

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

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

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

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

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

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

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

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

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

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

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.
Myoglobinuria, 1984
Authors: Rowland Lewis P.
Published: July 2016

The year is included in the title of this review because the subject is the diversity of conditions that result in myoglobinuria, and new causes keep appearing as conditions in society change or new drugs are introduced. The syndrome is often linked to seamier aspects of society or medicine : war; sadistic drill sergeants; drug abuse; attempted suicide; self-medication or inadequate supervision of drug therapy. On the other hand, study of myoglobinuric syndromes has informed us about new hereditable biochemical causes and we have learned more about the action of viruses on muscle.
Etiology of Parkinson’s Disease: A Research Strategy
Authors: Barbeau André
Published: July 2016

In 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

Haemophilus 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

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

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

A 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

Local 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

Complete 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

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

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).
Coexistence of Idiopathic Parkinson’s Disease and Creutzfeldt-Jakob Disease
Authors: Ezrin-Waters C. , Resch L. , Lang A.E.
Published: July 2016

The association of idiopathic Parkinson’s disease and Creutzfeldt-Jakob disease has not been reported previously. We describe the clinical findings in a patient with both disorders, confirmed at autopsy. The differential diagnosis of parkinsonism with cognitive and psychiatric disturbances is reviewed with emphasis on conditions which may complicate the course of idiopathic Parkinson’s disease. The etiological implications of this combination are considered.
Occipital Bone Cyst Causing Lower Cranial Nerve Palsies
Authors: Banna M. , Bradley W. G. , Kalbag R. M. , Tomlinson B. E.
Published: July 2016

A 20-year-old girl presented with neck and occipital pain for six weeks, which was found to be due to a unicameral bone cyst of the left occipital condylar region.

Although there have been very few reports of simple bone cysts in bones than the long tubular bones, it appears that the present case is probably the first report of such a lesion occurring in the skull.
The Incorporation of 3H (G) L-Leucine into Single Muscle Fibers in Duchenne Dystrophy and Charcot-Marie Tooth Disease
Authors: Monckton George , Marusyk Halyna
Published: July 2016

The results are reported of single muscle fiber uptake of tritiated leucine in muscle biopsy material from Duchenne dystrophy and Charcot-Marie Tooth disease. The uptake in the two conditions is compared and suggests that the previously reported increase in synthesis of cytoplasmic proteins in muscular dystrophy are probably related to regenerative efforts by the muscle fibers.
Degenerative Progressive Myoclonic Epilepsy Electrokymographic Observations
Authors: Derakhshan Iraj , Lotfi Jamshid , Abbsssioun Kazem
Published: July 2016

Clinical and pathological findings in two cases of degenerative progressive myoclonic epilepsy (PME) are described. The clinically difficult task of differentiating a “cerebellar” tremor from an action myoclonus is emphasized. Simultaneous elect roencephalography and electrokymography was done, using capacity to ground transients for recording hand movements. This method was found useful in corroborating the cerebellar nature of the remaining disorder, after successful treatment of the myoclonic element with anticonvulsants.
Regulation of Respiration in Friedreich's Ataxia
Authors: Begin R. , Lupien L. , Bureau M. A. , Labbe J. , Lemieux B.
Published: July 2016

Friedreich's Ataxia (F.A.) is a degenerative disease which commonly leads to premature death of cardio-respiratory origin. To explain the early death of these patients, previous investigations have established the existence of I) a cardiomyopathy in nearly 100% of cases, 2) a restrictive pulmonary syndrome of scoliotic origin and 3) a mild hypoxemia associated with slight respiratory alkalosis and a normal oxyhemoglobin dissociation curve. To further assess the cause of early death in patients with such neuromyo-pathy, we evaluated, in eleven FA. patients, the sensitivity of the respiratory centers to hypercapnia, hypoxia, and hyperoxia.

Ventilatory (VE, VT, F, VT/Ti) and occlusion pressure (P0.1) responses were taken as indices of the respiratory centers output during progressive hypercapnia (Read's method) and isocarhic hypoxia (Weil's method). We studied lì Friedreich's Ataxia patients and II age, sex, and arm-span matched controls. The responses of patients to hypercapnia were significantly greater than controls hut their responses to hypoxia were similar to controls.

Our study establishes that the respiratory centers are functioning adequately in early Friedreich's Ataxia and do not contribute to cardio-respiratory insufficiency in such neuromyopathy.
Oculomotor Abnormalities in Friedreich's Ataxia
Authors: Kirkham T.H. , Guitton D. , Katsarkas A. , Kline L.B. , Andermann E.
Published: July 2016

A clinical neuro-opthalmo-logical and electro-oculographic study was made on fourteen patients with Friedreich's ataxia. None had evidence of optic nerve dysfunction. No patient complained of oscillopsia although all had ocular motor deficits of varying degrees, which appeared to be related to the severity of the general manifestations of the disease. The defects comprised square wave jerks, jerky pursuit with inability to maintain eccentric gaze resulting in gaze paretic nystagmus and rebound nystagmus. There was failure to suppress by fixation the vestíbulo-ocular reflex. The slow phase velocity of caloric nystagmus was always of reduced velocity. There was inability to augment the slow phase velocity of optokinetic nystagmus with increasing stimulus velocity. Abnormalities of the saccadic system were manifest particularly as hypermetria. These signs in combination are suggestive of disease involving the cere be I lar flocculus and vermis or their brain stem connections. No abnormalities were found in 17 parents or siblings.
Electromyography and Nerve Conduction Studies in Friedreich's Ataxia and Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS)
Authors: Bouchard J.P. , Barbeau A. , Bouchard R. , Bouchard R.W.
Published: July 2016

Twenty four ataxie patients were investigated with electromyography and nerve conduction studies. They were divided in two groups according to the area they came from, the evolution of the disease, and the clinical signs. Group I patients from the Rimouski area displayed all the clinical and electrophysiological signs of Friedreich's ataxia. Group II comprised patients who presented with a new syndrome known as the autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Although the clinical evolution was better in the latter, there were more electromyographic signs of denervation and the motor conduction velocities were slower. Both groups showed identical and important abnormalities in sensory nerve conduction.

The results of electrophysiological studies in spastic ataxia have not been reported to our knowledge. They underline the place of spastic ataxia as distinct f rom Friedreich's ataxia, spastic paraplegia, and the known familial neuropathies.
Electroencephalographic Findings in Friedreich's Ataxia and Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS)
Authors: Bouchard R.W. , Bouchard J.P. , Bouchard R. , Barbeau A.
Published: July 2016

Electroencephalographie studies have heen done in two groups of hereditary ataxia: a group hearing the classical features of Friedreich's ataxia and a group clinically different described as autosomal recessive spastic ataxia of Charlevoix-Saguenay (A RSA CS). The qualitative anomalies observed in the two Ķroups were similar and were comparable with the data reported in the literature. However, the main difference between the two groups is the greater incidence of EEG abnormalities in the A RSA CS group, which suggests more involvement of the cortical and subcortical structures. This is reinforced by the lower I.Q. performance in the latter patients. Some comments are made about focal EEG findings, behavior and I.Q. In general, EEG was not considered a valuable instrument for diagnosis since no qualitative electric pattern could be identified. With regard to prognosis, EEG cannot be used as a criterion, since there is no relation between the degree of anomalies and the severity of the disease and since EEG does not worsen with the progression of the disease.
Hemagglutination by Lectins in Friedreich's Ataxia
Authors: Steinberg M.S. , Magnani J. , Czarkowski N. , Coccia M.B. , Barbeau A.
Published: July 2016

Coded erythrocyte samples from ten individuals with Friedreich*š ataxia, from parents of five of these individuals, and from five unrelated control individuals were subjected to lecţin agglutination tests at three temperatures; before and after trypsinization; and before and after treatment with echinocyte-producing sodium salicylate and stornato^ cyte-pröducing tetracaine followed by shape-fixation with glutaraldehyde. The aġglutinins tested were the polycationic poly-L-lysirie (PLL) and four lectins with different saccharide specificities: soybean agglutinin, wheat germ agglutinin, Ufex europeus agglutinin (VEA) and concana-valin A. Altogether, over 45,000 individual test wells were scored, the status of each blood donor with respect to diagnosis being disclosed to the experimenters only after all results were tabulated.

The majority of these tests revealed no significant difference among the three groups of blood samples. A few tests did reveal statistically valid (p<0.0l) differences between groups, the most significant of which were the following: Trypsin ized control RBC were more sensitive, on average, to agglutination by VEA (fucose-inhibited) than were RBC of ataxies or their parents. Non-trypsinized control “stomatocytes” were less sensitive, on average, to agglutination by PLL than were those of ataxies or their parents. Trypsinization appeared, on average, to sensitive control but not ataxia or parent RBC to PLL-agglutination. Other differences of borderline (p-0.01-0.025) or near borderline (p = 0.025-0.05) significance were also noted. None of the statistically significant, Friedreich's ataxia-telated differences in median agglutination titers were large, the greatest being about threefold, and in every case the ranges of individual titers within the differing groups overlapped. Thus, none of these tests at present offers a method of pre clinic al diagnosis or carrier detection, and only further tests can establish whether even the differences observed in the present series of tests are reproducible.
The Youth Version of the Positive and Negative Affect Schedule: A Psychometric Validation
Authors: Wilson Kay , Gullone Eleonora , Moss Simon
Published: July 2016

This study investigated the psychometric properties of the revised Positive and Negative Affect Schedule for Children (PANAS-C; Joiner, Catanzaro & Laurent, 1996) in 228 nonclinical children and adolescents aged between 8 and 15 years. The results revealed that the PANAS-C possesses high internal consistency and encouraging convergent validity, as demonstrated by correlations with the theoretically related constructs of Neuroticism and Extraversion. Construct validity was supported through confirmatory factor analysis, which revealed a two dimensional structure comprising Negative and Positive Affect. Divergent validity was confirmed by the nonsignificant correlation between positive and negative affect. Thus, the PANAS-C was demonstrated to have good reliability and validity, subject to minor changes in item content.
Pharmacotherapy of School Refusal
Authors: Tonge Bruce J.
Published: July 2016

This paper reviews the role and indications for pharmacotherapy of children with school refusal. The psychopharmacological treatment of school refusal is based mainly on evidence of the effectiveness of certain drugs in the treatment of adult disorders. There is some evidence that tricyclic antidepressants and benzobiazepines may be specifically useful in the treatment of school refusal, but further research is warranted. Potentially serious side effects mean that drugs are best confined to cases where psychological treatments have not been effective or where drugs are used briefly as an adjunct to a broader psychological treatment plan. Drugs may also have a role in the specific treatment of comorbid conditions associated with school refusal. Any use of drugs should involve regular reviews to monitor response, compliance, and side effects.
Antecedents of Problem Behaviour of People with Intellectual Disability
Authors: Tustin R. Don , Bond Malcolm J. , Forsaith Paivi
Published: July 2016

The study examined associations reported by carers between problem behaviour of 92 adults with intellectual disability and 169 antecedent events, which were either environmental situations or prior behaviours of the client. Factor analyses were used to identify groupings of antecedents, providing a basis for constructing 19 sets of antecedents of problem behaviours. Assessments were made of the psychometric properties of these sets of items or scales. Of the 19 scales, 18 were associated with an increased likelihood of problem behaviour, showing that carers reported that the problem behaviours of this sample occurred in a wide range of defined situations. The preliminary analysis suggests that the scales may be useful for inquiring more systematically about antecedents, for developing descriptions of situations where problems occur, and for identifying situations that warrant further analysis in individual cases.
Increases in the Communicative Behaviours of Students with Cerebral Palsy as a Result of Feedback to, and the Selection of Goals by, Paraprofessionals
Authors: Hall Laura J. , Macvean Michelle L.
Published: July 2016

The employment of untrained paraprofessionals to support children with disabilities in educational settings is not uncommon. Although urgently needed, research on effective methods of increasing the skills of these paraprofessionals is scarce. This study, which was conducted over 3 academic years, assessed the effectiveness of incorporating naturalistic prompting strategies by paraprofessionals for increasing target communicative behaviours of students with severe spastic quadriplegic cerebral palsy. A concurrent multiple-baseline design replicated across 3 paraprofessionals was used to determine the effectiveness of verbal and written feedback regarding their attainment of self-selected goals for prompting target communicative behaviours. The results indicate that, after the introduction of the intervention, the prompting behaviours of all paraprofessionals increased, as did the target behaviours of all students. Increases maintained 5 months after intervention for all students observed and for 2 of the 3 paraprofessionals observed. The implications of designing a program to include specific performance feedback and active involvement by staff are discussed.
Family Environment and Psychiatric History in Women with Binge-eating Disorder and Obese Controls
Authors: Fowler Sandra J. , Bulik Cynthia M.
Published: July 2016

We examined the prevalence of comorbid psychopathology, family psychiatric history, and perceptions of family environment in 20 women with binge-eating disorder (BED) and 20 non-binge-eating obese controls. Women with BED were significantly more likely to report current or lifetime affective and anxiety disorders as well as family histories of BED. Family histories of substance abuse did not differ across groups. Significantly more women with BED rated their parents' parenting style as consistent with “affectionless control” on the Parental Bonding Instrument, and reported significantly lower cohesion, expressiveness, conflict, independence, intellectual-cultural orientation, and active-recreational orientation on the Family Environment Scale.
Applying Behavioural Psychology in Education: Contributions and Barriers to the Implementation of Effective Instruction
Authors: Leach David J.
Published: July 2016

Between 10% and 25% of children in Australian schools have significant difficulty in mastering basic educational skills. Failure to achieve “tool” competencies in literacy (and numeracy) expected for independent adult functioning can have cumulatively negative consequences for individuals and their communities. The contribution of behavioural approaches to the instruction in basic literacy skills in schools is outlined. Empirical evidence of their effectiveness is presented and two of the most comprehensive (and most successful) models are described: theMorningside model of generative instructionanddirect instruction. Despite strong research support, however, behavioural approaches have not been widely adopted in general education. It is suggested that the main barriers to their adoption are the currently dominant paradigms of structuralism and constructivism, neither of which have translated into effective teaching practices for children most at risk of failure. Constructivist epistemology, in particular, is seen as oppositional to behaviour-based instruction and scientist–practitioner intervention, and its disregard for empiricism is cause for concern that ineffective practices will be maintained. It is concluded that behaviour-based practitioners (and researchers) need to become more effectively involved in system-level change strategies if they are to make an impact on the wider problems of educational failure.