bluuebunny/crossref_metadata_2025 huggingface dataset:
Quantitative evaluation of the effects of acetazolamide in Friedreich's ataxia: A pilot study
Authors: Richards C.L. , Bouchard J.P. , Dumas F. , Tardif D.
Published: July 2016

We evaluated the effects of acetazolamide in 4 young patients with Friedreich's ataxia by clinical and quantitative laboratory methods. Dynamic muscle function of the lower extremity was measured during isokinetic knee movements and gait. The acetazolamide trial was terminated at 7 to 11 weeks because of reported side effects or increased ataxia in 3 of the patients. The quantitative evaluations revealed lower dynamic strength values and alterations in the gait movement pattern in all patients. These changes, which were interpreted as deterioration, were partially reversible with cessation of acetazolamide. The advantages of such quantitative evaluations of dynamic muscle function in the evaluation of therapy in Friedreich's ataxia are discussed.
A progressive neurological syndrome associated with an isolated Vitamin E deficiency
Authors: Laplante P. , Vanasse M. , Michaud J. , Geoffroy G. , Brochu P.
Published: July 2016

Several authors have recently reported a neurological disorder associated with chronic vitamin E deficiency in man. Except in one patient, this deficiency has always been secondary to an underlying disease resulting in lipid malabsorption. We report a second case of such a neurological syndrome in a patient in whom vitamin E deficiency was an isolated finding.

The clinical picture in our patient was characterized by a diffuse muscle weakness most prominent distally and in the lower limbs, generalized areflexia, a decrease in proprioception and vibration sense and slight limb and gait ataxia. His condition improved on alpha tocopherol therapy so that it is very likely that vitamin E deficiency is responsible for his neurological deficit.

Since in our patient as well as in several other reported cases this condition has been treatable, it is important that this syndrome be recognized in children presenting a suggestive clinical picture even if they do not have lipid malabsorption.
Scoliosis assessment in friedreich's ataxia by means of intrinsic parameters
Authors: Allard P. , Dansereau J. , Duhaime M. , Geoffroy G.
Published: July 2016

Spinal deformities have been evaluated in a five year retrospective study where 28 boys and 25 girls all having Friedreich's ataxia were on the average assessed once a year in a multidisciplinary clinic. Scoliosis seems somewhat more progressive in girls than in boys and more severe in non-ambulatory than ambulatory patients. Some of those scolioses are very progressive reaching 60° to 100° Cobb angle values requiring spinal surgery while other progress less rapidly and do well on their own. In addition it was shown that the intrinsic geometric spine curve parameters namely that of curvature and torsion are a powerful diagnostic tool in the assessment of evolutive scoliosis.
Platelet taurine content in Friedreich's disease
Authors: Rodrigue F. , Bélanger F. , van Gelder N.M. , Barbeau A.
Published: July 2016

We have studied the concentrations of taurine and of 6 other amino acids in platelets from 12 patients with Friedreich's disease and 12 age sex-matched normal control subjects. No significant differences could be demonstrated between the two groups. The glycine/serine ratio was lower in all the patients but this change did not reach statistical significance. From these and other data, we conclude that the taurine retention deficit observed previously in Friedreich's disease cannot be the primary causal defect.
The Quebec Cooperative Study of Friedreich's Ataxia: 1974-1984 — 10 Years of Research
Authors: Barbeau A.
Published: July 2016

In this paper the author reviews the progress accomplished in the understanding of Friedreich's disease since the start of the “Quebec Cooperative Study of Friedreich's Ataxia” in 1974. The last ten years have indeed seen important strides taken in the definition and nosography of the hereditary ataxias and the characterization of a number of new entities. Biochemically, the principal leads uncovered during the initial prospective survey, have been pursued to great detail. Unfortunately no clear-cut constant and severe enzyme block in the principal metabolic pathways has yet been identified, despite intensive studies. It is postulated that the defect may instead be a regulatory one and involve a decreased availability or utilization of one of the vitamin cofactors that are known experimentally, or clinically, to produce central nervous system damage with ataxia: Vitamin E, Biotin or Pantothenic Acid. Studies in that direction and in molecular genetics to localize the Friedreich's disease gene are being untertaken for the next phase of the Cooperative Study.
Vein of Galen Malformation with Cerebral Calcification: A Reversible Cause of Neurodegenerative Disease
Authors: Phillips Stephen J. , Dooley Joseph M. , Camfield Peter R.
Published: July 2016

This paper reports two children who had mental retardation as the predominant manifestation of vein of Galen malformations. C.T. scans demonstrated extensive intracerebral calcification. Surgery reversed the progressive downhill course in one patient and may have arrested further deterioration in the other. The pathophysiological mechanisms underlying the clinical and radiographic features are discussed.
Numbness of Half of the Tongue
Authors: Sadler R.M. , Curran T. , Pryse-Phillips W.E.M.
Published: July 2016

We report a case of intermittent compression of the lingual nerve due to sialolithiasis and presenting with episodic unilateral numbness of the tongue. Removal of the obstruction has relieved the patient of symptoms over a 21 month period to date.
Chiari Malformation in Adults: A Review of 40 Cases
Authors: Eisenstat David D.R. , Bernstein Mark , Fleming J.F. Ross , Vanderlinden R.G. , Schutz Hart
Published: July 2016

Forty adult patients (average age 40 years), with the clinical and radiological features of the Chiari malformations, were seen at the Toronto Western Hospital between 1967 and 1984. Surgical confirmation of the diagnosis was obtained in 32 cases; of these, 23 were classified as Chiari I malformation while 9 fulfilled the anatomic criteria of Chiari II. The patient population consisted of 22 males and 18 females. Common presenting symptoms included head and neck pain (60%), sensory complaints (60%), upper extremity weakness (42%), and gait disturbance (40%). Neurological findings included signs of central cord dysfunction (73%), long-tract motor and/or sensory findings (58%), brainstem signs (38%), cerebellar dysfunction (18%), and increased intracranial pressure (15%). The majority of patients underwent myelography with or without computed tomography of the cervical-medullary junction. Two recent patients had 0.15T MRI scans which helped demonstrate an intramedullary syrinx. Thirty-three patients underwent 47 operative procedures (discounting spinal fusion and CSF shunt revisions). Open surgical management was performed in 32 patients, with CSF shunting along in one patient. Five patients (15%) incurred surgical complications within a six week postoperative period. Follow-up to date, ranges from one month to 11 years. In the 33 surgically treated patients, 18 are improved (55%), 10 are neurologically stable (30%), and five have worsened clinically (15%), including one death. Based on this study it appears that the Chiari II malformation maybe more common in adults than previously recognized. Surgical intervention has a favourable outcome in the majority of patients but a significant proportion continue to deteriorate.
Comparative Neuropsychology of Cortical and Subcortical Dementia
Authors: Freedman Morris , Oscar-Berman Marlene
Published: July 2016

The terms “cortical” and “subcortical” dementia are controversial; however, the clinical distinction between them is real. For example, although Alzheimer's and Parkinson's disease (prototypical of cortical and subcortical dementia, respectively) share clinical features, they differ in the presence of aphasia, apraxia, and agnosia in Alzheimer's disease but not in Parkinson's dementia. We review our studies aimed at clarifying the mechanisms underlying the differences between these neurological disorders. Experimental paradigms adopted from animal models were used to study the functional anatomy and neuropsychological characteristics of Alzheimer's and Parkinson's disease. The tasks administered include delayed alternation (DA) and delayed response (DR), which are sensitive to frontal system damage, and tactile discrimination learning (TOL) and reversal (TRL) paradigms sensitive to parietal system damage. Alzheimer's patients were significantly impaired on all tasks whereas Parkinsonians with dementia were impaired only on DR and TRL. Consideration of neuroanatomical and neuropsychological mechanisms involved in DA, DR, TOL, and TRL appears to have sharpened the distinction between Alzheimer's and Parkinson's dementia. Dementia in Alzheimer's disease may involve dorsolateral frontal, orbitofrontal and parietal systems. In contrast, dementia in Parkinson's disease may involve prominent dorsolateral frontal system damage.
The Correlation of Vascular Capacity with the Parenchymal Lesions of Alzheimer's Disease
Authors: Bell M.A. , Ball M.J.
Published: July 2016

Hippocampal capillary and arteriolar measurements showed a significant reduction in capacity with normal aging. Alzheimer's dementia was not associated with any further reduction; in fact regional variations suggested that the zones of Amnion's horn most severely affected by tangles and granulovacuoles retained the best vascular capacity.

The arterial system supplying the hippocampus, from the posterior cerebral artery to the series of small hippocampal arteries, was also assessed. Its capacity, as judged by arterial diameters, was similarly found to decrease significantly with age; the arterial diameters of Alzheimer cases were (insignificantly) greater than those of the normal old.

Calcarine capillary and arteriolar measurements also indicated a marked reduction of capacity with normal aging, and, as before, in Alzheimer's dementia there was no further significant change. All three phases of the study thus suggest that cerebrovascular capacity in Alzheimer's dementia is at least as good as in the normal old, if not better.

Senile (neuritic) plaques with amyloid cores and their relationship to the microvasculature were examined in the calcarine cortex. In both normal old and Alzheimer cases the plaques tended to congregate where capillaries were densest. Their correlation with capillary density was however better in the normal old, raising the question of whether their pathogenesis might differ in the two conditions.
Primary Degenerative Dementia Without Alzheimer Pathology
Authors: Clark Arthur W. , White Charles L. , Manz Herbert J. , Parhad Irma M. , Curry Bernadette , Whitehouse Peter J. , Lehmann John , Coyle Joseph T.
Published: July 2016

To define the pathology in cases of non-Alzheimer primary degenerative dementia (non-AD PDD), we have studied autopsies from four medical centres accessioned in consecutive years since 1976. Neurochemical studies of the basal forebrain-cortical (BF-C) cholinergic system have been conducted in cases from which frozen tissue was available. Twenty-two cases (mean age 70 years, range 47-86) in which the history was consistent with PDD, but which did not meet anatomic criteria for AD, were selected. Approximately 70 cases of PDD, which were accessioned in the same years and met the anatomic criteria for AD, were excluded. The pathologic findings permitted a classification into six groups: Lewy body disease (LBD), 4 cases; Pick's disease, 6 cases; cortical degeneration with motor neuron disease (CDmnd), 2 cases; hippocampal and temporal lobe sclerosis, 3 cases; few or nonspecific abnormalities, 5 cases; other disorders, 2 cases. Our findings suggest that LBD and Pick's disease account for a large proportion of cases of non-AD PDD in the presenile age group, but that a large number of other disorders occasionally present as PDD. Careful examination of the motor systems, as well as cerebral structures relate' to cognitive function, is important in the neuropathologic evaluation. Lesions of the BF-C cholinergic system have been most consistent and severe in LBD, and have not been identified in CDmnd.
Comparison of Counts of Neurons in the Locus Coeruleus Made From Serial Sections and From a Single Section at the Centre of the Nucleus
Authors: Mountjoy C.Q. , Bondareff W.
Published: July 2016

This paper describes the comparability of counts in the locus coeruleus made from serial sections and those made from a single section at the point of greatest density of neurons. Samples of the locus coeruleus neuronal population derived from single section counts though not exactly comparable, are of comparable utility to those obtained from more laborious total neuronal counts. The simpler method was used to examine the hypothesis that there are subtypes of senile dementia of the Alzheimer type. Separation into two groups was achieved when independent variables of cortical neuronal counts and tangle estimates were used. This finding adds to the growing evidence that Alzheimer's disease of the senile type is not a unitary disorder.
Learning and Memory Deficits After Ibotenate Lesions of the Nucleus Basalis: Reversal by Cholinomimetics
Authors: Fibiger H.C.
Published: July 2016

The effects of bilateral ibotenic acid-induced lesions of the nucleus basalis magnocellularis (nBM) on the acquisition and retention of several spatial memory tasks were studied in the rat. Maintenance of spatial memory in a food search task was impaired following nBM lesions. Acquisition of spontaneous alternation and reinforced alternation in a T-maze was also significantly impaired in animals with these lesions. In contrast, the animals with nBM lesions were not impaired in the acquisition of a position habit in a T-maze. In several of the tasks there was evidence of some learning in the lesioned animals after substantial training, although they were significantly deficient when compared with the controls. Administration of the cholinergic agonists physostigmine sulfate or pilocarpine nitrate prior to behavioral testing resulted in a rapid and significant improvement in the performance of the lesion animals. The ibotenate-induced lesions significantly reduced the activity of choline acetyltransferase (CAT) in the anterior and the posterior neocortex. Hippocampal CAT activity was not changed. The results indicate that the cholinergic projections originating in the nBM are involved in the learning and memory of spatial tasks. They also suggest that degeneration of cholinergic neurons in the basal forebrain contributes significantly to the learning and memory deficits observed in Alzheimer's disease.
Cholinergic Receptors in Cognitive Disorders
Authors: Perry E.K. , Perry R.H. , Smith C.J. , Purohit D. , Bonham J. , Dick D.J. , Candy J.M. , Edwardson J.A. , Fairbairn A.
Published: July 2016

Cholinergic receptors (muscarinic subtypes M1 and M2, and putative nicotinic binding) have been examined in the hippocampus obtained at autopsy from a variety of patients with cognitive disorders (Alzheimer's, Parkinson's, and Huntington's diseases, Down's Syndrome and alcoholic dementia) and compared with neurologically normal controls and cases of Motor Neuron disease. In all of the disorders associated with a pre-synaptic cortical cholinergic deficit reflected by an extensive loss of choline acetyltransferase (Alzheimer's disease, Parkinson's disease and Down's Syndrome) there was a substantial reduction in the binding of (3H) nicotine to the nicotinic receptor. By contrast reductions in both muscarinic subtypes (M1 and M2) were apparent to only a moderate extent in Alzheimer's disease, whereas in Parkinson's disease binding was significantly increased (apparently not in relation to anti-cholinergic drug treatment) in the non-demented but not demented cases. A further abnormality detected in Alzheimer's disease but not the other disorders investigated was a decrease in an endogenous inhibitor of nicotinic binding, the identity of which is as yet unknown but which may be a candidate for a possible endogenous modulator of the nicotinic receptor. These observations suggest that in Alzheimer's disease not only muscarinic but also nicotinic receptor function should be considered in relation both to future therapeutic strategies and, in the search for a clinical marker which might be of diagnostic value, to potential probes of the cortical cholinergic system.
Natural History of the Vascular Dementias: A Prospective Study of Seven Cases
Authors: Hershey Linda A. , Modic Michael T. , Jaffe David F. , Greenough P. Gregg
Published: July 2016

The clinical course of vascular dementia has been described as a stepwise deterioration over time. We studied the chronologic course of cognitive performance over 1-4 years in seven patients with known ischemic cerebrovascular disease whose dementia subtype was assigned according to the clinical history and the pattern of white matter lesions seen on magnetic resonance imaging (MRI). One patient had the lacunar state, three had subcortical arteriosclerotic encephalopathy and three had multiple cortical and subcortical strokes. All were being treated with an antiplatelet agent and six received antihypertensive therapy. Four of the seven vascular dementia patients improved cognitively over the first year. A fluctuating course was eventually seen in all patients. None showed stepwise deterioration of cognitive function over time. The MRI was useful in subclassifying vascular dementia patients, but the clinical course did not appear to vary as a function of the dementia subtype.
Guam ALS/Parkinsonism-Dementia: A Long-Latency Neurotoxic Disorder Caused by “Slow Toxin(s)” in Food?
Authors: Spencer Peter S.
Published: July 2016

Parkinsonism (P) with progressive dementia (D) of the Alzheimer type is recognized as a clinical variant of a form of amyotrophic lateral sclerosis (ALS) that has occurred in high incidence among the Chamorro people of the islands of Guam and Rota in the Marianas chain of Micronesia. The declining annual incidence, upward shifting of the age of onset, narrowing of the sex ratio, and occurrence of the disease among non-Chamorros, point to a disappearing environmental causation peculiar to the traditional culture of these islands. Evidence is presented in support of the proposal that heavy use of certain toxic plants, notably cycads, a traditional source of food and medicine for the Chamorro people, plays an important etiological role. Clinical and epidemiological approaches are offered to test for a relationship between ALS/P-D and long-latency plant toxicity.
Clinical Importance of D-1 and D-2 Receptors
Authors: Beaulieu Michèle
Published: July 2016

The existence of subtypes of dopamine receptors as defined by Kebabian and Calne is now well accepted. The Dl receptor is typically associated with stimulation of adenylate cyclase, while the D2 receptor is either independent of adenylate cyclase or mediates its inhibition. Considerable interest has been generated by the potential physiological and clinical roles of these receptor subtypes. Availability of agonists and antagonists specifically acting at the D1 or D2 receptor site has stimulated research to characterize the functional effects of each receptor subtype. This might facilitate the development of effective compounds to control the signs and symptoms of Parkinson's disease and perhaps prevent the induction of debilitating side effects. Recent evidence indicates that Dl receptor stimulation is required to obtain full expression of the D2 receptor site, which has typically been associated with the clinical benefits of dopaminergic therapy. Both pre- and postsynaptic location of the receptors must also be taken into consideration as well as involvement of other neuronal systems. It appears that in PD, progressive involvement of the dopaminergic pathways is the principal pathological course, however, noradrenergic and serotonergic pathways are likewise involved. This multineuronal involvement suggests that drugs acting specifically at receptor sites, located on both pre- and postsynaptic neurons, might be required at different times during the course of the disease process to control its symptoms and/or the complications occurring after long-term treatment with a given drug.
Systemic Therapy of Dystonia
Authors: Fahn Stanley
Published: July 2016

A number of pharmacologic agents have been found to be effective for the dystonias. Anticholinergic drugs have been shown to be the most effective in terms of percentage of subjects who receive moderate to marked benefit. About 50% of children and 40% of adults obtain such improvement. Peripheral adverse effects are usually overcome by pyridostigmine. It may be necessary to utilize pilocarpine eyedrops for blurred vision. Central adverse effects, such as forgetfulness, can be reduced only by a reduction in dosage of the anticholinergic. In comparing trihexyphenidyl and ethopropazine, we found that children tend to have better tolerance of the former and adults tend to have better tolerance of the latter. The antidopaminergics are the group of drugs that were found to be the next most effective agents in terms of percentage of patients who respond. However, these drugs, particularly the dopamine receptor blockers, have the capacity to induce tardive dyskinesia and tardive dystonia. Tardive syndromes are difficult to treat and can persist indefinitely. Other agents that have shown usefulness in controlling dystonia are levodopa, baclofen, carbamazepine, and the benzodiazepines, either alone or in combination with each other and with the anticholinergics. Stereotactic thalamotomy is particularly useful in contralateral hemidystonia. The risk of adverse effects is less than with bilateral thalamotomy, which may need to be employed when generalized dystonia is severely disabling and not responsive to pharmacotherapy.
Task Dependent Variations of Ocular Lateropulsion in Wallenberg’s Syndrome
Authors: Kirkham T.H. , Guitton D. , Gans M.
Published: July 2016

The eye movements of a patient with a left lateral medullary infarct (Wallenberg’s syndrome) were recorded using the scleral search coil in magnetic field technique. When asked to look at spontaneously appearing targets, saccades to the left were generally accurate hut those to the right reached the target by multiple step refixation saccades. Large amplitude rightward saccades were possible between two continuously visible targets or when making voluntary saccades in the dark.

Vertical saccades, up or down, between spontaneously appearing targets were always associated with a leftward eye movement (lateropulsion). Voluntary vertical saccades between continuously visible targets showed that upward movements had left lateropulsion but downward movements were normal. Vertical voluntary saccades in the dark were oblique, upward saccades showing left lateropulsion and downward saccades showing rightward deviation. The aberrant horizontal components of vertical saccades had amplitude and velocity characteristics for components of normal oblique saccades. Possibly impaired assessment of vertically with incorrect eye position information produced by the infarct accounts for the lateropulsion of saccades in Wallenberg’s syndrome.
Effect of Therapy on Cerebral Blood Flow Following Aneurysm Surgery
Authors: Weir Bryce , Deleo Roberto , Menon Devidas
Published: July 2016

The authors report on a patient with a ruptured anterior communicating aneurysm treated surgically on the day following her subarachnoid hemorrhage (SAH). Repeated measurements of cerebral blood flow (rCBF) were carried out using the inhalational Xenon 133 method. Dramatic acute increases in rCBF occurred, coincident with clinical improvement, following mannitol, CSF drainage and dopamine. Later, further elevation to normal levels occurred following ventriculo-peritoneal shunting.
The Computerized Tomographic Assessment of Brain Infarcts
Authors: Tubman D.E. , Ethier R. , Melançon D. , Bélanger G. , Taylor S.
Published: July 2016

The computed tomographic (CT) examinations of one hundred and sixty patients with a clinical diagnosis of cerebral infarction were reviewed. A characteristic CT pattern was noted. Infused scans and follow-up studies allowed for considerable accuracy in diagnosis.
Neuropsychological Changes Following Carotid Endarterectomy
Authors: Bornstein Robert A. , Benoit Brien G. , Trites Ronald L.
Published: July 2016

Fifty-five subjects undergoing carotid endarterectomy were grouped according to side of operation, i.e. right, left and bilateral. These subjects were administered a comprehensive neuropsychological battery prior to and six months following surgery. The performance of these subjects was compared with a control group undergoing surgical procedures that did not in volve the brain or cerebral vasculature, and a second control group composed of patients with cerebrovascular symptoms who were not operated. It was found that as a group the endarterectomy subjects improved on a greater percentage of measures. When various subgroups were examined, it was found that the right operated stroke patients improved on significantly more measures than any other group. The difference between right and left stroke patients was significant, but there was no difference between right and left TIA patients in the extent of improvement. These findings were discussed in terms of possible underlying mechanisms.
Pitfalls in Posterior Fossa CT Scanning: An Illustrative Case Report
Authors: Saunders Fraser W. , Tranmer Bruce I.
Published: July 2016

A case is described thai illustrates the difficulties involved with the interpretation of postoperative posterior fossa CT scans. The reasons for the difficulties are discussed and the pertinent literature reviewed.
The Effect of Plasmapheresis on Post-Thymectomy Ocular Dysfunction
Authors: Olanow C.W. , Roses A.D. , Fay J.W.
Published: July 2016

Five myasthenia gravis patients with post-thymectomy residual ocular signs were treated with plasmapheresis. Despite a significant reduction in AChR antibody titer, there was no clinical improvement. Subsequently, there was a dramatic response to prednisone. The AChR antibody titer did not correlate with the clinical state of the individual patient. It is suggested that plasmapheresis may operate by removing a thymic factor and that prednisone acts by a different mechanism.
Brain Stem Glioma in Childhood: Acute Hemiplegic Onset
Authors: Rothman S.J. , Olanow C.W.
Published: July 2016

Two children, age seven and 16 years, are described with the abrupt onset of a pure motor hemiplegia as the initial manifestation of a brain stem neoplasm. Subsequent rapid neurological deterioration localized the lesion to the brain stem and glioblastoma multiforme was diagnosed by surgical biopsy. It is suggested that brain stem gliomas with this unusual presentation are likely to be highly malignant and prone to rapid bulbar deterioration.
Life Effects of Narcolepsy in 180 Patients from North America, Asia and Europe Compared to Matched Controls
Authors: Broughton Roger , Ghanem Quais , Hishikawa Yasuo , Sugita Yoshiro , Nevsimalova Sonia , Roth Bedrich
Published: July 2016

A questionnaire survey has been made of the life effects of narcolepsy in 180 patients, 60 each from North American, Asian and European populations, with 180 similarly distributed age and sex matched controls. Life-effects were attributed by the patients to the primary symptoms of excessive daytime drowsiness, sleep attacks, cataplexy, vivid hypnagogic hallucinations and sleep paralysis, and also to other frequent symptoms such as visual problems (blurring, diplopia) and memory impairment. Occupational problems were prevalent (over 75%) and included statistically significant deleterious effects upon performance, promotion, earning capacity, fear of or actual job loss and increased disability insurance. Driving was greatly affected and patients fell asleep at the wheel more frequently (66%), had near or actual accidents from drowsiness or falling asleep at the wheel (67%), and could experience cataplexy (29%) or sleep paralysis (12%) while driving. Work or home accidents attributed to sleepiness or sleep (49%) or related to smoking (49%) were much more common in patients. There were also deleterious effects on education, recreation and personality related to the disease. Narcolepsy can produce a variety of life-effects probably more serious and pervasive than, for instance, those of epilepsy, therefore emphasizing the importance of early diagnosis and treatment.
Ultrastructural Observations on Spinal Ganglion Biopsy in Friedreich’s Ataxia: A Preliminary Report
Authors: Lamarche J. , Luneau C. , Lemieux B.
Published: July 2016

We report the preliminary study of a dorsal root ganglion obtained at biopsy during a surgical intervention in a patient with Friedreich’s ataxia. There was an apparent decrease in the number of large myelinated fibers without necrosis, but with numerous axonal swellings consisting mainly of dense accumulated neurofilaments. Large amounts of lipofuscin were also found as well as some onion bulb formations suggesting a process of axonal atrophy.
Oral Lecithin and Linoleic Acid in Friedreich’s Ataxia: I. Design of the Study, Material and Methods
Authors: Melancon S.B. , Dallaire L. , Potier M. , Vanasse M. , Marois P. , Geoffroy G. , Barbeau A.
Published: July 2016

A clinical and biochemical evaluation of twenty-two patients with Friedreich’s Ataxia and ten normal controls was undertaken in 1980 to assess the effect of lecithin and linoleic acid supplements on the course of the disease. The trial consisted of two consecutive six months periods on either supplements in a double-blind crossover fashion. Clinical appraisal was performed with regards to the following parameters: joints mobility, muscle strength, equilibrium, coordination, motor accuracy, speech and numerous day to day activities. Blood samples were obtained at the beginning and in the course of the trial for enzymatic determinations. This paper describes the methodology of the study.
Tissue Lipids in Acute Acrylamide Intoxicated Rats
Authors: Huang Y.S. , Wong P. , Blache D. , Barbeau A. , Davignon J.
Published: July 2016

A preliminary survey of tissue lipid composition in acrylamide intoxicated rats is reported. The animals were injected intraperitoneally with acrylamide 50 mg/kg body weight per day for 10 days. Liver cholesterol, mainly in the ester fraction, was decreased in treated rats. When fatty acid composition of liver cholesterol esters was examined, the proportions of linoleate and stearate were found to be decreased and were compensated by the increase of palmitate. Atrophy of epididymal fat pad resulted in severe triglyceride depletion and a relative increase in the proportion of phospholipids and cholesterol. There was also a reduction of linoleate, palmitate and palmitoleate in triglycerides and phospholipids of this tissue. There were, however, only minor changes in the fatty acid profile of the sciatic nerve.
A Possible Genetic Pattern of Taurine Urinary Excretion in Friedreich’s Ataxia
Authors: Barbeau A. , Patenaude F. , Nadon G. , Charbonneau M. , Cloutier T.
Published: July 2016

The taurine urinary excretion pattern, before and after an oral load of 250 mg taurine, was studied in normal control subjects and in patients with typical Friedreich’s ataxia. It was demonstrated that in both situations the ataxic patients fell within the sub-types of “intermediate” and “high taurine excretors”, while none were “low taurine excretors”. It was also demonstrated that the excretion of taurine after a load in the obligate heterozygotes parents of the ataxic patients was intermediate between normal controls and patients. It is postulated that patients with Friedreich’s Ataxia lack normal regulation of the high affinity-low capacity uptake system for taurine (the TH system) in the brush border of kidney tubules. The low affinity-high capacity uptake system in the same membranes (the TL system) appears to be normal in Friedreich’s patients. The normal allele could be called THN and the variant THF and this trait would be inherited in an autosomal recessive fashion if it is linked to the Freidreich phenotype. Whether this finding is or is not the basic genetic defect in Friedreich’s Ataxia will require more studies to clarify, but it is of interest to note that a similar pattern appears to be present in the fibroblasts of these patients.
Pyruvate Dehydrogenase Activity in the Liver, Brain and Adipose-Tissue of Lipid-Deprived Developing Rats. Effect of Minute Amounts of Polyunsaturated Fatty Acids
Authors: Loriette C. , Launay M. , Lapous D. , Raulin J.
Published: July 2016

The present experiment was carried out using the following diets:FF, fat-free, andLPthe same diet with 0.7% sunflower oil - given to the progeny of females kept on theFFdiet since the mating. After 10 mM Mg2+ activation of the PDH phosphatase, the rate of [1-14C] pyruvate decarboxylation into acetyl-CoA ester units was determined in the liver, brain and adipose-tissue of the pair-fed developing rats.Results: In the male progeny, pyruvate dehydrogenase (PDH) activity was higher (61%) in theLPgroup livers than in theFFgroup livers, at the end of the 13 week experiment. Such a difference was not observed in the two group brains up to the 91 days postweaning, but was even larger (94%) between adipose-tissues of theLPandFFgroups. In the female progeny kept 12 weeks on the diets, PDH activity in theLPgroup tissues was also higher than in theFFgroup tissues: 63% in the liver, 43% in adipose-tissues, and less than 10% in the brain. Therefore, a minute amount of lipids high in linoleic acid appeared to increase PDH activity, and especially in the liver and adipose-tissues of animals kept on a strictly fat-free diet. This stimulation of the PDH activity seems closely related to the phospholipid rehabilitation in the tissues (decrease in the trienoic: tetraenoic acid ratio values).
Current Views on Parkinson’s Disease
Authors: Calne Donald B.
Published: July 2016

Since the introduction of levodopa therapy, there have been major advances in the understanding and treatment of Parkinson’s disease. However, the problems and limitations associated with this form of treatment are now becoming increasingly apparent. This article reviews some of the current knowledge concerning the etiology and pathophysiology of Parkinson’s disease and discusses some-practical approaches to drug therapy.
Ventricular Dilation Following Intraventricular Hemorrhage in the Premature Infant
Authors: Hill Alan
Published: July 2016

Periventricular/intraventricular hemorrhage occurs commonly in the premature newborn. Recent studies indicate an incidence of 35–45%. Following PVH/IVH, the likelihood of developing hydrocephalus is related to the severity of the hemorrhagic lesion. Ventricular dilation may be due to an obliterative arachnoiditis affecting principally the posterior fossa or, less commonly, due to obstruction of flow of cerebrospinal fluid within the ventricular system by clots or other debris. With moderate to severe hemorrhagic lesions, ventricular dilation may occur at the time of PVH/IVH. More commonly, progressive dilation begins one to three weeks following PVH/IVH. The classical clinical signs of hydrocephalus, ie. bulging of anterior fontanelle and inappropriate increase in head circumference, may not appear for days to weeks following onset of ventricular dilation. The precise significance of such normal-pressure hydrocephalus in the genesis of brain injury in the newborn is unknown. Following diagnosis of PVH/IVH, close surveillance of ventricular size with ultrasound scans is indicated. When there is slowly progressive ventricular dilation with normal intracranial pressure, the choice of therapy is made difficult because of frequent spontaneous arrest in such cases. Several modes of therapy have been reported including drugs to decrease the formation of cerebrospinal fluid and the use of serial lumbar punctures. When ventricular dilation is rapid with intracranial hypertension, ventricular drainage is indicated.
University of Toronto Combined Neurosciences Rounds — Rhabdomyosarcoma of the Head and Neck in Children
Authors: Humphreys R.P. , McGreal D. , Fitz C.R. , Becker L.E. , Gribbin M.A. , Morley T.P.
Published: July 2016

Rhabdomyosarcoma of the head and neck region in children frequently presents with otolaryngologic symptoms. But tumours which arise along the cranial base and involve the middle ear, mastoid or nasopharynx may be associated with specific neurological phenomena (Fleischer et al., 1975). The following case illustrates these points. It also records the unusual development of remote, intradural metastatic disease. Rhabdomyosarcoma rarely gains access to the subarachnoid space. When it does, the route of penetration is usually directly from ear through internal auditory meatus to cerebellopontine angle (Russell and Rubinstein, 1970). Our case developed a metastatic intradural spinal lesion without interposed disease.
The Pattern Electroretinogram in Optic Nerve Demyelination
Authors: Kirkham Trevor H. , Coupland Stuart G.
Published: July 2016

The pattern electroretinogram (PERG) is probably generated by the ganglion cell layer of the retina whose axons constitute the conductive fibres of the optic nerves. A study was undertaken of the PERG in a group of patients with clinically definite multiple sclerosis who had suffered optic nerve demyelination to assess the value of recording the PERG as a method for electrophysiological determination of optic nerve dysfunction. Pattern visual evoked potentials (PVEP) were simultaneously recorded, each eye being stimulated separately, and four different check sizes were used. Although individual patients showed PERG abnormalities the results as a group showed no significant difference from the normal population so far as latency or amplitude of the PERG was concerned. Thus we conclude that the PERG, while an interesting measurement, does not satisfactorily reflect optic nerve dysfunction. Further, our results indicate that it is not such a useful measure as the pattern visual evoked response for detecting optic nerve disease.
Hypothesis: Phylogenetic Diseases of the Nervous System
Authors: Sarnat Harvey B. , Netsky Martin G
Published: July 2016

A few human diseases may be viewed from a phylogenetic perspective. Some metabolic or degenerative diseases selectively affect recently evolved or exclusively mammalian structures of the brain and spare the older structures. Examples include Krabbe’s leukodystrophy, olivopontocerebellar atrophy, Friedreich’s ataxia, Pick’s disease, and Leber’s optic atrophy. Some pathologic conditions in man are similar to normal anatomy in other species, although the mechanisms may differ. Congenital muscle fiber-type disproportion in rodents, Dandy-Walker cyst in birds, and agenesis of the corpus callosum in marsupials are representative of this category. Loss of basal dendritic spines from pyramidal cells in Pick’s disease is reminiscent of certain large neurons normally found in the cortex of reptiles. Changes in metabolism in the evolution of mammals in general and of man in particular may explain some aspects of’phylogenetic diseases’. Some potential examples are the shift from predominantly phospholipids to galactolipids in myelin composition as mammals evolved, and the greater toxicity of cyanide and other poisons of oxidative metabolism in mammals than in other vertebrates because of less reliance on anaerobic metabolism as an alternative energy source.
Serial Pattern Shift Visual Evoked Potentials in Multiple Sclerosis
Authors: Becker Werner J. , Richards Irene M.
Published: July 2016

Forty patients with MS initially tested in our laboratory were recalled for repeat PSVEP testing approximately two years later. Twelve normal controls were tested in a similar manner approximately two years apart. The PSVEP positive peak latency changed little in the 24 control eyes (mean 1.4 msec, range 0-6) over the study interval. Most MS patient eyes also showed little change in PSVEP latency over the two year study interval. Fifty-eight eyes changed 8 msec or less. Eighteen eyes showed a PSVEP latency increase of 10 msec or more. Six of these eighteen eyes were symptomatic (attack of clinical optic neuritis), twelve asymptomatic during the study interval. Symptomatic eyes tended to have greater latency increases during the study interval than asymptomatic eyes.

Significant latency increases occurred with equal frequency in previously normal eyes (normal PSVEP on first test) and abnormal eyes (abnormal PSVEP on first test or previous clinical optic neuritis). Significant latency increases occurred with greater frequency in patients with a mixed or progressive course than in patients with a remitting-relapsing course, and in patients with greater disability ratings (Kurtzke 3-7) than in patients with lower disability ratings (Kurtzke 0-2). Bilateral latency increases occurred during the study interval more frequently than expected by chance. Patient age and disease duration did not significantly influence the number of PSVEP latency increases seen during the study interval.

Four eyes decreased in latency by 10 msec or more during the study interval. All these eyes had had an episode of acute optic neuritis which began in the 5 weeks immediately preceding the 1st PSVEP test.

In our MS patients, 13% of eyes per year developed latency increases of 10 msec or more. These may represent new demyelinating lesions. If so, one-third of these lesions were clincally symptomatic.
Retrograde Amnesia in Parkinson’s Disease
Authors: Freedman Morris , Rivoira Peter , Butters Nelson , Sax Daniel S. , Feldman Robert G.
Published: July 2016

Retrograde amnesia was assessed in demented and non-demented Parkinson’s patients using a test of remote memory spanning the years from 1920-1979. Results indicated that the demented patients 1) scored significantly below normal controls and 2) had equal impairment for all time periods. This pattern was like that seen in other dementing illnesses (i.e., Huntington’s and Alzheimer’s diseases), but different from that in amnesic disorders, such as Korsakoff s syndrome. The data, therefore, suggest qualitative differences in pattern of remote memory loss between the dementias and amnesic syndromes.
The University of Toronto Head Injury Treatment Study: A Prospective, Randomized Comparison of Pentobarbital and Mannitol
Authors: Schwartz Michael L. , Tator Charles H. , Rowed David W. , Reid S. Ross , Meguro Kotoo , Andrews David F.
Published: July 2016

Fifty-nine patients were treated in a prospective, randomized comparison of pentobarbital and mannitol for the control of intracranial hypertension resulting from head injury. Patients with elevated intracranial pressure (ICP) after evacuation of intracranial hematomas were randomized to one of two treatment groups; mannitol initially or pentobarbital initially, followed by the second drug as required by further elevation of ICP. Similarly, patients with raised ICP but without hematomas requiring evacuation were randomly assigned to two treatment groups in an identical paradigm.

Those with ICP elevation and no hematoma treated with pentobarbital as initial therapy had a 77% mortality compared to a 41% mortality for those with mannitol as initial treatment. Patients with evacuated hematomas had mortalities of 40% and 43% (no significant difference) for pentobarbital and mannitol respectively. In both no-hematoma and hematoma streams pentobarbital was less effective than mannitol for control of raised ICP.

Multivariate statistical analysis indicates that pentobarbital coma is not better than mannitol for the treatment of intracranial hypertension and may be harmful in no-hematoma patients with intracranial hypertension after head injury.
Aneurysmal Bone Cyst of the Skull
Authors: Ameli N.O. , Abbassioun K. , Azod A. , Saleh H.
Published: July 2016

Aneurysmal bone cysts rarely affect the skull. Thirty-six cases including four reported in this paper are reviewed. The condition usually affects children and young adults and progresses rapidly. It may result in raised intracranial or intraorbital pressure. Complete excision of the mass is the treatment of choice, but if not feasible curettage followed by low dose radiotherapy is effective. As the prognosis in this condition is good, prompt diagnosis and early treatment are of utmost importance.
New Intraventricular Catheter for Volume Pressure Response Measurements
Authors: Gorecki John , Saunders Fraser
Published: July 2016

Measurements of the volume-pressure response (VPR) to determine the relative position of a patient on the graphic volume-pressure curve have been used to derive clinically useful information. One reason that these measurements have not been used more frequently has been the fear of introducing infection into the ventricular system. We have designed an intraventricular catheter that allows repeated VPR measurements and reduces the risk of infection.
Behaviour Correlates of Neurotransmitter Activity
Authors: Poirier Louis J. , Bédard Paul J.
Published: July 2016

Most disorders of motor activity including disturbances of muscle tone and of locomotor activity observed in patients with neurological disorders have been reproduced experimentally in animals. Most motor disorders of the extrapyramidal type including those associated with Parkinson’s disease and choreiform and athetoid involuntary movements, have been reproduced exclusively in primates. This is most likely related to the highly complex organization of the extrapyramidal and related nervous mechanisms subserving the corresponding peculiar type of motor control in the primate brain. Other types of motor disturbances including cervical and trunkal dystonias, ataxia, hypotonicity, spasticity and intention tremor, however, have been successfully induced in various mammalian species. The latter types of motor disorders are related to disturbances of central nervous mechanisms which show similar patterns in the brains of different animal species. Histopathological and neurochemical changes associated with extrapyramidal disorders have been discovered and more precisely determined as a consequence of the development of new technical approaches. Therefore numerous morphological, physiological and neurochemical data concerning the extrapyramidal system are now available but a better knowledge of their precise and subtle interrelationship is greatly needed in order to develop more efficient therapeutic procedures.
Pharmacological and Biochemical Evidence for the Existence of Two Categories of Dopamine Receptor
Authors: Kebabian J.W. , Beaulieu M. , Itoh Y.
Published: July 2016

Evidence supporting the validity of the ‘two dopamine receptor’ hypothesis is presented. The availability of the ‘first generation’ of selective agonists and antagonists of the D-1 and the D-2 dopamine receptors provides pharmacological support for the hypothesis. The demonstration that stimulation of the D-2 receptor either inhibits or has no effect upon adenylate cyclase activity while stimulation of the D-1 receptor enhances cAMP formation provides biochemical support for the hypothesis. Finally, binding assays demonstrating two affinity states for the D-1 and the D-2 receptors are briefly discussed.