bluuebunny/crossref_metadata_2025 huggingface dataset:
Peripheral Neuropathy in Oxalosis. A Case Report with Electron Microscopic Observations
Authors: Bilbao Juan M. , Berry Henry , Marotta Joseph , Ross Roderick C.
Published: July 2016

A 61 year old man had chronic renal failure because of oxaluria and renal calculi. Two years before death, while on hemodialysis, he developed severe progressive peripheral neuropathy. At autopsy calcium oxalate crystals were found in the peripheral nerves and other tissues. Nerve lesions included segmental demyelination, axonal degeneration and crystalline deposits within the myelin sheath. Ultrastructurally there were foci of osmiophilic granular material within myelin lamellae and endoneurium, and pleomorphic lamellar bodies in the perinuclear Schwann cell cytoplasm.

It is probable that chronic hemodialysis favors the deposition of oxalate in the Schwann cells and the development of neuropathy in patients with primary hyperoxaluria and renal failure.
Neuronal Growth Factors from Tumours of Von Recklinghausen Neurofibromatosis
Authors: Riopelle Richard J. , Riccardi Vincent M.
Published: July 2016

Explants of 21 neurofibromas from 16 patients with Von Recklinghausen neurofibromatosis (NF-I) plus tumour tissue from 5 comparison patients and normal tissue from one of the NF-I patients were assayed for neuronal growth factor(s) using dissociated embryonic sensory neurons from chick embryo. Twenty-one of 21 neurofibroma explants released detectable quantities of neuronal growth factors, but only 2 of 8 non-neurofibroma tissue explants released activity.

While antiserum to mouse nerve growth factor (NGF) fractionally inhibited neurite outgrowth induced by some of the neurofibromas, overall differences between assays containing antibody and controls reached statistical significance in 3 cases; in one case, explants of a separate tumourfrom the same patient had no detectable NGF-like activity.

These data support the hypothesis that local release of neuronal growth factors in neurofibromas are responsible for neurites observed within these tumours. Further evidence that endoneurial tissue of peripheral nerve is a rich source of heterogeneous neuronal growth facts has been provided by these studies.
Sleep Apnea Following Percutaneous Cervical Cordotomy
Authors: Tranmer Bruce I. , Tucker William S. , Bilbao Juan M.
Published: July 2016

Sleep apnea (Ondine's Curse) is an uncommon but potentially fatal complication of percutaneous cervical cordotomy. The authors have had the opportunity to review the case histories of 6 patients who have died of sleep apnea following this procedure. In a series of 112 patients, 144 cervical percutaneous cordotomies were performed from 1977 to 1985 — 80 unilateral cordotomies and 32 staged bilateral cordotomies. Six (5%) patients died as the result of sleep apnea. Five patients (16%) died following bilateral cordotomy and one (1%) patient with pulmonary disease died following unilateral cordotomy. The clinical data of these 6 patients are presented and unique spinal cord pathology is described in this report. The pathophysiology of the sleep apnea syndrome is also discussed.
Congenital Malformations Associated with Maternal Use of Valproic Acid
Authors: Huot Celine , Gauthier Marie , Lebel Marc , Larbrisseau Albert
Published: July 2016

Two children born with birth defects after intrauterine exposure to valproic acid are reported. The mothers took the drug throughout pregnancy as sole treatment for primary generalized epilepsy. The first baby showed facial dysmorphism, arachnodactyly and triphalangeal thumbs. The second had facial dysmorphism, severe laryngeal hypoplasia, tracheomalacia and an aberrant innominate artery that caused tracheal compression. A left superior vena cava, abnormal pulmonary lobulation, and unilateral hydronephrosis were also found at autopsy. Valproic acid has probable teratogenic potential in humans but the number of reported cases is few and the spectrum of anomalies is broad so it is not possible to delineate a definite fetal valproate syndrome.
Hand Wasting Due to Mid-Cervical Spinal Cord Compression
Authors: Goodridge A.E. , Feasby T.E. , Ebers G.C. , Brown W.F. , Rice G.P.A.
Published: July 2016

Three patients presented with hand wasting and weakness secondary to mid-cervical spinal cord compression. This was due to cervical spondylosis in two patients and a meningioma in one case. This phenomenon is probably similar to that seen with foramen magnum lesions and may be due to spinal cord ischemia distal to the compression, secondary to venous stasis.
A Syndrome of Brain, Inner Ear and Retinal Microangiopathy
Authors: MacFadyen D.J. , Schneider R.J. , Chisholm I.A.
Published: July 2016

We report the seventh case of a self-limiting syndrome of brain, inner ear and retinal microangiopathy which affects adult women producing mild to moderate mental status and personality changes, mild pyramidal and cerebellar dysfunction, patchy and asymmetrical visual loss secondary to non-inflammatory retinal infarctions, and asymmetrical neurosensory hearing loss. There is no clinical or laboratory evidence of involvement beyond the brain, the eyes and the ears. Magnetic resonance imaging or brain biopsy in previously reported cases best outline the brain pathology: multiple microinfarcts. All cases have been treated with steroids or cyclophosphamide. The etiology is unknown but may be related to systemic lupus erythematosus.
Comparison of Surgical and Conservative Management in 208 Patients with Acute Spinal Cord Injury
Authors: Tator C.H. , Duncan E.G. , Edmonds V.E. , Lapczak L.I. , Andrews D.F.
Published: July 2016

The role of surgery in the management of acute spinal cord or cauda equina injuries remains controversial. The present study analyzed ten admission features and three outcome variables in 208 patients treated in an Acute Spinal Cord Injury Unit, 116 (56%) of whom underwent at least one spinal operation. The surgical and non-surgical groups showed no significant differences in the following seven clinical features: age, sex, distance travelled to the Unit, time interval between trauma and admission, type of accident, severity of injuries to the spinal cord, and severity of associated injuries. However, the two groups showed significant differences in level and type of vertebral column injury, and in the frequency of pre-existing spinal abnormalities. These differences were due to management policies which selected certain injuries for surgical or non-surgical treatment. One-third of the operative procedures were performed primarily for neural decompression, one-third primarily for reduction of bony structures and one-third for fusion. However, 95% of the operative patients had a fusion at the initial operation. Operative treatment was associated with a lower overall mortality rate (6.1%) than non-operative (15.2%), despite a higher frequency of thrombo-embolic complications in the surgical group. Overall, there was no difference between operated and non-operated patients in length of stay or neurological recovery. Surgical management of patients with acute spinal cord injury appears safe in terms of mortality rate and neurological recovery, but it has not been proven to improve the latter.
Microsurgical Discectomy: A Prospective Study of 200 Patients
Authors: Schutz H. , Watson C.P.N.
Published: July 2016

This report describes the results of microsurgical discectomy in a prospective study of 200 consecutive patients with intractable sciatica, followed independently by a pain clinic. Post operative morbidity was minimal, the complication rate very low, and patients were discharged home early. Leg pain was relieved in almost all patients, but back pain was relieved in only 72% of patients. The rate of return to work at one, three and twelve months post-operatively was 50%, 83% and 95% respectively. Microsurgical discectomy is an effective and safe procedure.
Pathology of Spontaneous Dissection of Intracranial Arteries
Authors: Deck John H.N.
Published: July 2016

Spontaneous dissection of a major subarachnoid artery is an uncommon cause of stroke in young and apparently healthy individuals. Such dissection does not correlate well with systemic conditions such as emboli, thrombotic or hemorrhagic tendencies or hypertension, nor with disease of blood vessels such as atherosclerosis, fibromuscular dysplasia, cystic medial necrosis or Moya-Moya disease. Unusual exertion has been implicated as a factor in some cases. Gap defects were found in the internal elastic lamina near the site of dissection in three of four cases of spontaneous dissection reported here. It is suggested that such defects, because of their unusual size or number, may be responsible for initiating dissection in susceptible individuals. A detailed study of the major cerebral arteries in cases of dissection and control cases to document the size and frequency of such defects could shed light on the pathogenesis of spontaneous dissection. The subsequent course of events tends to differ in the internal carotid and vertebro-basilar systems. In most cases, the dissection in the internal carotid system is sub-intimal leading to thrombosis and cerebral infarction, while in the vertebrobasilar system dissection lies between the media and adventitia leading to subarachnoid hemorrhage. It is not known what structural differences of the two arterial systems may underlie this difference in the pattern of arterial dissection.
Treatment of Dopaminomimetic Psychosis in Parkinson's Disease with Electroconvulsive Therapy
Authors: Hurwitz T.A. , Calne D.B. , Waterman K.
Published: July 2016

Two Parkinsonian patients with chronic nonconfusional dopaminomimetic psychosis were treated with a course of electroconvulsive therapy. In both cases sustained remission of psychosis was obtained in the face of ongoing administration of dopaminomimetics.
Current Status of Artificial Vision by Electrocortical Stimulation
Authors: Girvin John P.
Published: July 2016

The history of the provision of artificial vision by electrocortical stimulation has its origin in observations over 50 years ago. However, the realistic modern stimulation of human visual cortex is only 20-years-old. No useful device has been devised as yet and it therefore remains purely experimental. The primary objective of such a prosthetic device is that of independent mobility. This would require the appreciation of half-tone pictures, the detection of depth and discontinuities in outlines, and a significant quality of resolution. Some of the problems which threaten resolution in such a device and the factors which must be overcome in order to achieve a useful visual prosthesis are discussed.
Jakob-Creutzfeldt Disease Associated with Wernicke Encephalopathy
Authors: Gaytan-Garcia S. , Gilbert J.J. , Deck J.H.N. , Kaufmann J.C.E.
Published: July 2016

Wernicke disease (WD) is a complication of alcoholism and malnutrition and usually presents acutely and is characterized by disturbances of consciousness, paralysis of the external ocular muscles, and ataxia. The disease results from deficiency of vitamin B 1, or thiamine, an essential coenzyme in intermediate carbohydrate metabolism. On the other hand, Jakob-Creutzfeldt disease (J-C) results from infection with an unconventional agent with a long incubation period and is characterized by a rapidly progressive dementia and histologically by a spongiform encephalopathy associated with neuronal destruction and pronounced astrogliosis. Combination of both diseases has not been reported in the literature previously and their relationship is uncertain. We present 3 cases with this interesting association and consider their relationship.
Cognitive Enhancing Agents: Current Status in the Treatment of Alzheimer's Disease
Authors: Waters Cheryl
Published: July 2016

Extensive recent literature on drugs used to enhance cognitive functioning, reflects the growing social problem of dementia. Many clinical trials have been undertaken with variable success. In most cases the disorder studied has been Alzheimer's disease. The pharmacological approach has been designed to rectify the presumed pathophysiological processes characteristic of the condition. Agents tested include cerebral vasodilators, cerebral metabolic enhancers, nootropics, psychostimulants, neuropeptides and neurotransmitters with a special emphasis on drugs used to enhance cholinergic function. Ethical and practical issues concerning clinical drug trials in dementia will be discussed.
Cerebellar Atrophy in Epileptic Patients
Authors: Botez M.I. , Attig Ezzedine , Vézina Jean Lorrain
Published: July 2016

High-resolution CT scans of the brain and posterior fossa were performed on 106 phenytoin (PHT)- treated epileptics, 28 de novo epileptics and 43 control subjects. A higher incidence of cerebellar and brainstem (CBS) atrophy was observed in chronic PHT- or PHT+ phenobarbital-treated epileptics compared to the two other groups. Some control subjects and de novo epileptics presented mild CBS atrophy, whereas moderate to severe atrophy was noted exclusively in chronically-treated patients. In attempting to delineate the etiology of CBS atrophy, epileptic patients were divided in three groups: 55 subjects with normal CT scans, 30 with both cerebral and CBS atrophy, and 49 with pure CBS atrophy. Their ages, length of illness, number of generalized seizures, number of other seizures, and amount of PHT received during their lifetime were assessed. Statistical analysis revealed that posterior fossa atrophy in epileptics was significantly correlated with both the length of the illness and the amount of PHT ingested during the patient's lifetime. The number of seizures appears to not be related to CBS atrophy.
Origin and Diffusion of the Myotonic Dystrophy Gene in the Saguenay Region (Quebec)
Authors: Bouchard Gérard , Roy Raymond , Declos Manon , Mathieu Jean , Kouladjian Kevork
Published: July 2016

A very high prevalence (approximately 1/475 in 1985) of myotonic dystrophy (Steinert disease) is observed in the Saguenay region, which is located in the north-east part of the Province of Quebec. For various reasons, however, the literature on the subject generally associates a high degree of selective disadvantage with this gene, which seems to contradict the Saguenay data. Using a computerized regional population register, we have reconstituted patients' genealogies and family biographies. We have thus been able to study the origin of the gene and to compare the demographic behavior of patients and controls. On the whole, patients seem to be very little disadvantaged compared to controls, in terms of reproduction as well as of geographical and occupational mobility.
Computed Tomography, Magnetic Resonance Imaging and Pathological Correlations in a Case of Binswanger's Disease
Authors: Mascalchi M. , Inzitari D. , Dal Pozzo G. , Taverni N. , Abbamondi A.L.
Published: July 2016

The results of 3 computed tomography (CT) examinations carried out over a 7 year period and of a post-mortem magnetic resonance (MR) study showed aspects of a white matter disease in a hypertensive patient suffering from vascular dementia. Histopathology revealed the primary cause of dementia to be a white matter degeneration sparing the U fibers. Rarefaction of both the myelin sheaths and the axons was present together with severe thickening of the medullary arteries. These findings support the existence of Binswanger's disease (BD) as a distinct variety of arteriosclerotic dementia. CT and MR imaging are valuable aids for diagnosis. However, since there are many other causes of CT and MR demonstrated diffuse white matter degeneration in the elderly, a conclusive diagnosis of BD requires pathological confirmation.
Trends in Blood Pressure, Osmolality and Electrolytes after Subarachnoid Hemorrhage from Aneurysms
Authors: Disney Lew , Weir Bryce , Grace Michael , Roberts Paul
Published: July 2016

Daily trends in blood pressure, osmolality and electrolytes were analyzed in a series of 173 operated aneurysm cases who had subarachnoid hemorrhage (SAH) and were admitted within 4 days of the ictus. High blood pressure was associated with a greater risk of mortality and the development of clinically significant vasospasm (VSP). High osmolality shortly after admission was related to mortality but not VSP. Changes in sodium and potassium had no obvious relationship to mortality or VSP.
Multiple Intra-Cranial Hemorrhages After Carotid Endarterectomy
Authors: Shuaib Ashfaq , Hunter K. Michael , Anderson Melvin A.
Published: July 2016

Intra-cerebral hemorrhage is an unusual and often fatal complication of endarterectomy (CEA). Previous cerebral infarction, intra-operative or post operative hypertension, and the use of anti-coagulants are some of the defined major risk factors. Hemorrhage usually occurs early after CEA, and a second hemorrhage has only once been reported. We wish to report a patient who developed three separate hemorrhages following CEA. This patient had a long history of poorly controlled hypertension. We believe that poorly controlled hypertension, in addition to other reported factors, may be an important separate risk factor for post-CEA hemorrhage.
Routine Screening of Blood and Urine for Severe Anticonvulsant Reactions in Asymptomatic Patients is of Doubtful Value
Authors: Camfield Peter , Camfield Carol , Dooley Joseph , Farrell Kevin , Langevin Pierre , Humphreys Peter
Published: July 2016

On rare occasions anticonvulsant medication may cause severe or fatal toxic reactions. This position paper, approved by the Canadian Association for Child Neurology, examines the value of routine blood and urine screening to prevent catastrophic reactions in asymptomatic patients. The basic assumptions of such a screening program are faulty or unproven. Although not definitive, available studies of screening do not appear to support its value. It is recommended that routine blood and urine screening be discontinued in asymptomatic patients. Until such time as further research is able to identify patients at special risk, it is suggested that patients be carefully informed of the early symptoms of severe toxic reactions and report these immediately to a physician.
Clustering of Parkinson's Disease in Southern Israel
Authors: Herishanu Y.O. , Goldsmith J.R. , Abarbanel J.M. , Weinbaum Z.
Published: July 2016

On three adjacent kibbutzim (collective rural communities) in the Negev (Southern Israel) 13 parkinsonian patients were found among a population of 592 persons 40 years or older. The clinical picture was not different from that of patients from other areas with idiopathic parkinsonism. Long term residence in the kibbutzim is characteristic of this population. In the past most of the drinking water has been supplied by wells from a common aquifer. From other patients with Parkinson's disease in the Negev, we estimated the age-specific incidence for the region. The incidence is about five times greater in each of these kibbutzim than in the remainder of the Negev. Although associations with rural residence and well water use have been reported elsewhere, clusters of this sort have not been reported. They strongly suggest that a common environmental factor exists.
Growth Hormone Rebound After Cessation of SMS 201-995 Treatment in Acromegaly
Authors: Charest L. , Comtois R. , Beauregard H. , Serri O.
Published: July 2016

We studied a 42-year-old woman who had persistent active acromegaly despite conventional therapies. She was treated for 6 months with SMS 201-995. Her mean plasma growth hormone GH values decreased during treatment from 9.1 ± 1.2 to 6.6 ± 1.2 µg/L. One month after the withdrawal of SMS 201-995, the plasma GH level increased to 24.4 µg/L (P < 0.001). This elevation was clinically silent and transitory, as GH levels decreased 8 months later to 6.9 ± 1.3 µg/L. Furthermore, at the beginning of therapy, her intractable headache was completely relieved; however, it progressively resumed under therapy. In conclusion, cessation of SMS 201-995 may be followed in some acromegalic patients by a rebound of plasma GH levels. This rebound suggests that SMS 201-995 decreases GH levels by an inhibition of its release from the pituitary. Furthermore, SMS 201-995 may relieve intractable headache in some acromegalic patients, but tolerance to the analgesic effect may develop.
Cortical Glutaminase, β-Glucuronidase and Glucose Utilization in Alzheimer's Disease
Authors: McGeer E.G. , McGeer P.L. , Akiyama H. , Harrop R.
Published: July 2016

Large pyramidal neurons of rat and human neocortex stain immunohistochemically for phosphate-activated glutaminase (PAG). In a limited number of postmortem brains, we find large reductions in cortical PAG activity in Alzheimer's disease (AD). This finding is consistent with histological evidence that pyramidal neurons are affected in AD. The reductions are greater than those found in the same samples in choline acetyltransferase (ChAT) but the possible deleterious effects of coma and similar premortem factors on human PAG activity have yet to be assessed. The activity of (β-glucuronidase, a lysosomal enzyme which occurs in reactive astrocytes, is elevated in the same samples. Positron emission tomography (PET) studies, using18F-fluorodeoxyglucose (FDG), have demonstrated significant deficiencies in glucose metabolism in the cortex in AD, with the parietal, temporal and some frontal areas being particularly affected. We found in serial scans of 13 AD cases, including one relatively young (44-46 year old) familial case, an exacerbation of the defect over time in most cases. We have found a negative correlation between the regional metabolic rates for glucose (LCMR(s)) measured premortem and the (β-glucuronidase activities measured postmortem on a few AD cases that have come to autopsy. The correlations between LCMR(s) and PAG and ChAT activities tend to be positive. The results are consistent with previous suggestions that decreased LCMR(s) in AD reflect local neuronal loss and gliosis.
Ultrastructural Studies of the Cells Forming Amyloid Fibers in Classical Plaques
Authors: Wisniewski H.M. , Wegiel J. , Wang K.C. , Kujawa M. , Lach B.
Published: July 2016

Three-dimensional reconstruction and ultrastructural studies of classical plaques from the cortex of patients with Alzheimer's disease showed that microglial cells of the plaques are the amyloid-forming cells. The amyloid star of the single plaque represents the product of five or six microglial cells covering about 80% of the amyloid star surface. The amyloid fibers appear to be formed within altered cisterns of the endoplasmic reticulum. Distended cisterns form channels filled with amyloid fibers. Numerous vesicles derived from the Golgi apparatus appear to be attached to or fused with the amyloid-filled channels. Reconstruction of the amyloid star and the microglia cell pole that forms the amyloid star reveals three different zones of distribution of cytoplasmic organelles and amyloid deposits. The peripheral zone comprises channels filled with loosely packed amyloid fibers arranged in a parallel manner. The transient zone consists of a mixture of fusing amyloid channels and products of disintegration of cytoplasmic pockets, dense bodies and fragments of cellular membranes. The core of the amyloid star is composed of condensed, densely packed amyloid fibers that are free of cellular debris. Formation of the three zones supports the idea that the microglia/macrophages are not phagocytes but instead are the cells manufacturing the amyloid fibers.
Surgical Management of Giant Pituitary Adenomas
Authors: Mohr Gérard , Hardy Jules , Comtois Ronald , Beauregard Hughes
Published: July 2016

During the past 25 years, 77 giant pituitary adenomas have been treated surgically, including suprasellar extensions of type C in 66 cases and of type D in 11 cases. Non-secreting adenomas were present in 53 and secreting adenomas in 24 cases. All patients except 3 presented with significant visual field defects; including bitemporal hemianopia, superior quadranopia or unilateral temporal hemianopia, contralateral blindness in 73% of the cases, and one case with sudden bilateral blindness due to acute pituitary apoplexy. A single transsphenoidal procedure was carried out in 74% of the patients while 11 patients (7%) required re-operations for recurrent or residual tumor. Only 3 patients required a subsequent transcranial procedure. Complications included 1 CSF-leak, 1 empty-sella syndrome and 4 fatal post-operative hematomas. We prefer the transsphenoidal route even in very large or giant pituitary adenomas, since it allows rapid and adequate decompression of the optic nerves and chiasm, avoids major pituitary insufficiency in 60% of the cases and is associated with low morbidity-mortality rates.
Differential Response to Aminergic Stimuli and Biological Behavior of Growth Hormone Secreting Pituitary Adenomas
Authors: Fanghanel Guillermo , Larraza Oscar , Villalobos Martha , Fanghanel Leticia , Velasco Marcos , Velasco Francisco
Published: July 2016

Growth hormone (GH) serum levels in response to the administration of aminergic drugs and thyroliberine (TRH) were determined in a group of 34 acromegalics. Administration of bromocriptine (10 mg single oral dose) was followed by a decrease in GH below 60% control values in 35% of the cases. Administration of diazepam (10 mg single oral dose) to those cases not responding to bromocriptine induced a decrease in GH in 58% of the cases and an increase in GH in 42%. Administration of cyproheptadine (24 mg/day for one month) to those cases not responding to bromocriptine or with increased GH after the administration of diazepam, decreased GH in 75%, while increased GH in 25% of the cases. TRH 200 (Xg single I.V. dose induced increase of 128% GH basal level in 65% of cases (TRH positive) which correlated with more benign clinical course, decreased GH levels in response to bromocriptine, increased PRL levels, PRL-GH mixed secreting adenomas in immunohistochemistry studies, presence of granulated cells in electron microscopy studies and normalization of GH in the majority of surgically treated cases. By contrast, TRH negative cells correlated with aggressive tumor growth, lack of response to bromocriptine, normal PRL levels, pure GH secreting adenomas by immunohistochemistry, poorly granulated cells and lack of response to surgical treatment. Results suggest that there is more than one type of acromegaly that might be distinguished by the aminergic control on GH secretion.
Visual Recovery After Blindness From Pituitary Apoplexy
Authors: Parent Andrew D.
Published: July 2016

In a series of 11 patients with classic pituitary apoplexy, two patients had the acute onset of bilateral blindness. After transsphenoidal decompressive surgery both patients had useful recovery of vision. Although impairment of neurologic function appears irreversible when central nervous tissue has been compressed to the point of total loss of function it would appear that the optic chiasm may be an exception to this experience. Our review of the literature and our experience suggests that decompression can be worthwhile even late in the course of this disease and visual loss should not be treated expectantly.
Myotubular Myopathy: Arrest of Morphogenesis of Myofibres Associated with Persistence of Fetal Vimentin and Desmin Four cases compared with fetal and neonatal muscle
Authors: Sarnat Harvey B.
Published: July 2016

Vastus lateralis muscle biopsies of four unrelated male neonates showing myotubular (i.e. centronuclear) myopathy (MM) were compared with muscle from four human fetuses in the myotubular stage of development, a 31 week preterm infant and four term neonates. The perimysium, blood vessels, spindles, myelinated intramuscular nerves, and motor end-plates in MM are as well developed as in term neonatal muscle. The cytoarchitecture of myofibres in MM is more mature than that of fetal myotubes in the spacing of central nuclei, Z-band registry, development of the sarcotubular system, and in the condensation of nuclear chromatin and nucleoli. Triads in MM may retain an immature oblique or longitudinal orientation. Myofibrillar ATPase shows normal differentiation of fibre types, consistent with nonnal innervation. Spinal motor neurons are nonnal in number and in RNA fluorescence. Immunoreactivity for vimentin and desmin in myofibres of MM is uniformly strong, as in fetal myotubes and unlike mature neonatal muscle. Maternal muscle biopsies of two cases also showed scattered small centronuclear myofibres reactive for vimentin and desmin. The arrest in morphogenesis of fibre architecture in MM is not a general arrest in muscle development. Persistence of fetal cytoskeletal proteins that preserve the immature central positions of nuclei and mitochondria may be important in pathogenesis. Vimentin/desmin studies of the infant and maternal muscle biopsies are useful in establishing the diagnosis.
Intermediate Filaments in the Human Pituitary Gland: An Immunohistochemical Study
Authors: Halliday W.C. , Asa S.L. , Kovacs K. , Scheithauer B.W.
Published: July 2016

Normal pituitaries, pituitaries showing CHC and selected adenomas were assessed for their possible content of intermediate filaments (IF), using immunohistochemical techniques. Normal pituitaries were stained for each of the five known IF (Cytokeratins (CK), glial fibrillary acidic protein (GFAP), desmin, vimentin, and neurofilaments (NF)). Desmin could not be demonstrated, and NF staining was limited to processes in the posterior gland. In serial sections, CK was demonstrated in some corticotrophs, somatotrophs, and lactotrophs. Vimentin was limited to processes in the posterior gland. Folliculo-stellate cells (FSC), demonstrated by staining for SlOO protein, exhibited a small subpopulation staining for CK, and another subpopulation staining for GFAP. GFAP was also demonstrated in glial processes in the posterior gland. Cells showing Crooke's hyaline change stained intensely for CK. Null cell adenomas did not stain for CK. Corticotroph adenomas, somatotroph adenomas, with or without fibrous bodies, and lactotroph adenomas contain CK-immunoreactive cells. We conclude that CK is the major IF present in the anterior pituitary gland, within some normal corticotrophs, somatotrophs and lactotrophs, and also in adenomas derived from these cells.
Mechanisms of Tumor-Associated Edema: A Review
Authors: Maestro Rolando F. Del , Megyesi Joseph F. , Farrell Catherine L.
Published: July 2016

An understanding of the mechanisms responsible for tumor-associated edema involves the elucidation of the role played by a number of intra-related processes. These include (i) the permeability of new tumor microvessels that are associated with tumor angiogenesis; (ii) alterations in microvascular permeability due to factors secreted by tumor cells; (iii) immunological mechanisms and (iv) increased microvessel permeability associated with inflammation. The rationale for a role for inflammatory processes in tumor-associated edema has been outlined and the role of non-steroidal anti-inflammatory drugs in modulating experimental and human tumor-associated edema has been explored.
Experience with Ommaya Reservoir in 120 Consecutive Patients with Meningeal Malignancy
Authors: Perrin R.G. , Lishner M. , Guha A. , Curtis J. , Feld R. , Messner H.
Published: July 2016

The Ommaya Reservoir plays an important role in a select group of neuro-oncology patients with meningeal malignancy. The benefits derived must be balanced against potential complications associated with insertion and use of the apparatus. Side effects may be minimized by careful attention to patient selection, pre-operative CAT scan (or MRI), precise surgical technique, perioperative prophylactic antibiotics and meticulous procedure during use of the reservoir.
Facteurs environnementaux dans l'étiologie de la maladie de Parkinson
Authors: Zayed J. , Ducic S. , Campanella G. , Panisset J.C. , André P. , Masson H. , Roy M.
Published: July 2016

We examined the role of the environment in the development of Parkinson's disease (PD). A group of 42 parkinsonians have been compared with a group of 84 matched controls. The epidemiological study (1987-1989) covered the territory of the Community Health Department of Valleyfield, in southern Quebec (Canada). Odds ratio adjusted for age and sex were calculated for seven environmental factors. A decreased risk for PD was associated with residence in rural areas (OR: 0.31; p ≤ 0.05) and residence near industry or mining (OR: 0.15; p ≤ 0.05). An increased risk for PD seems to be associated with occupational exposure to the three metals Mn, Fe and Al (OR: 2.28; p = 0.07) especially when the duration of exposure is longer than 30 years (OR: 13.64; p ≤ 0.05). Other environmental factors not found to be associated with PD were: pesticides manipulation, farm work, industrial work and well water consumption.
Primary CNS Lymphoma: Lymphomatous Meningitis Presenting as a Cauda Equina Lesion in an AIDS Patient
Authors: Klein Pavel , Zientek Gary , VandenBerg Scott R. , Lothman Eric
Published: July 2016

Neurologic complications of the acquired immune deficiency syndrome (AIDS) most often present as brain dysfunction and/or a polyneuropathy. We describe a unique neurological problem of a woman with AIDS who presented with a Cauda equina mass due to primary CNS lymphoma. She subsequently developed a fulminant lymphomatous meningitis and died. Although previously rare, primary spinal cord lymphoma is expected to be encountered more often with the current AIDS epidemic and may be difficult to distinguish from infectious mass lesions.
Neural Maturational Delay as a Link in the Chain of Events Leading to SIDS
Authors: Becker Laurence E.
Published: July 2016

Although numerous etiological or triggering factors have been suggested in sudden infant death syndrome (SIDS), the underlying mechanism of death is ultimately cardiac and/or respiratory in nature. As there is no evidence of lung or heart abnormalities, attention has focussed on the neural control of respiration and cardiac function. It is important to appreciate the methodological limitations involved in utilizing autopsy material and the criteria for selection of appropriate controls. This report reviews the pathological evidence for developmental delay in SIDS emphasizing delay of neural maturation of both myelination and synapses. Other abnormalities of the nervous system apparently associated with hypoxia-ischemia such as brainstem astrogliosis are also discussed. The occurrence of SIDS at a precise age together with our preliminary studies indicate that neural developmental delay is an important link in the chain of events leading to SIDS.
Encephalopathy in Liver Transplantation: Neuropathology and CMV Infection
Authors: Power C. , Poland S.D. , Kassim K.H. , Kaufmann J.C.E. , Rice G.P.A.
Published: July 2016

The clinical histories and pathological findings of 27 autopsied cases of orthotopic liver transplantation (OLT) were reviewed. Fatal OLT was complicated in 93% of cases by neurological dysfunction, usually manifested by encephalopathy, with or without seizures. The etiology of the encephalopathy was largely multifactorial (44%) or undetermined (20%). Subarachnoid hemorrhage, central pontine myelinolysis, meningitis, brain infarction, polyclonal B cell lymphoma and spinal cord necrosis were common neuropathological findings. These diagnoses were often masked by other systemic illnesses. The role of cytomegalovirus (CMV) in neurologic dysfunction was explored with in situ hybridization and immunohistochemical techniques. OLT cases showed a significantly higher (89%) frequency of CMV genomic material in brain tissue compared to age-matched non immunocompromised (NIC) patients (23%). All OLT cases with encephalopathy of undetermined cause demonstrated unusually prominent hybridization to the CMV probe. CMV may be an important cause of encephalopathy in such patients.
Heterogeneity in Multiple Sclerosis: Comparison of Clinical Manifestations in Relatives
Authors: Sadovnick A.D. , Hashimoto L.L. , Hashimoto S.A.
Published: July 2016

Once diagnosed to have MS, relatives of persons who have been previously diagnosed frequently ask whether their disease course will follow that of their relative(s) with MS. The present study compared the following clinical manifestations of MS among 43 index cases and 47 of their relatives, all of whom were diagnosed to have MS and regularly attended the MS Clinic in Vancouver, British Columbia: (i) age of onset of MS, (ii) clinical course, (iii) lesion site(s) and (iv) initial symptom(s) of MS. The results from the present study are preliminary because of the small size of the study group. However, these data suggest that apart from possibly age of onset between sibling pairs, the clinical manifestations of MS are not correlated among relatives who are assessed according to the same methodology. This is significant for counselling newly diagnosed relatives of longstanding MS patients.
Petrous Meningiomas: A Review of Seventeen Cases
Authors: Macdonald R.L. , Muller P.J. , Tucker W.S. , Moulton R.J. , Hudson A.R.
Published: July 2016

Seventeen patients with petrous meningiomas managed at St. Michael's Hospital, during the years 1973-1987, were retrospectively reviewed. There were 15 females and 2 males; their ages ranged from 42 to 68 years (mean age: 53 years). The clinical presentation most commonly included headache and eighth cranial nerve dysfunction; the average duration of symptoms was 6 years (3 month - 27 years). Computed tomography was performed in 15 cases. The mean tumour size was 2.5 centimeters (0.5 - 4 cm). The most common site of tumour origin was at or medial to the poms acousticus. Meningioma was suspected preoperatively in 10 of the 15 patients who had preoperative CT scans. Complete excision was obtained in 12 cases. There were no operative deaths after initial resections. Postoperative morbidity included worsening of pre-existing hearing loss in six patients, transient facial nerve palsies in six, permanent facial nerve palsies in four and new facial or corneal hypesthesia in three. Two patients developed cerebrospinal fluid fistulae. Tumour recurrence occurred into two patients in whom a complete resection was anticipated. Also, in two patients with incompletely resected tumours second operations were required. Fourteen patients are alive, 13 of whom care for themselves independently. The average follow-up was 5 years (6 months - 9 years). It seems appropriate to recommend initial radical surgical excision of these benign tumours, where possible, in order to prevent tumour recurrence.
Positron Emission Tomography Study of Brain Benzodiazepine Receptors in Friedreich's Ataxia
Authors: Chavoix C. , Samson Y. , Pappata S. , Prenant C. , Mazière M. , Seck A. , Agid Y.
Published: July 2016

Central type benzodiazepine receptors were studied in 9 patients with Friedreich's ataxia and 12 healthy subjects using positron emission tomography (PET) and [11C]Ro 15-1788, a specific antagonist of the central type benzodiazepine receptors, as radioligand. A standard PET procedure was used in 5 patients and 8 controls to obtain brain kinetics of the total binding of the radioligand. The remaining subjects were intravenously injected with a saturating dose of unlabeled Ro 15-1788, 30 minutes after the tracer injection, to determine the nondisplaceable binding of [11C]Ro 15-1788. A semi-quantitative method was used to quantify the [11C]Ro 15-1788 data. None of the quantification indices in the cerebellar hemispheres, or in the other brain areas investigated, was significantly modified in patients with Friedreich's ataxia. These findings suggest that brain benzodiazepine receptors are unaffected in Friedreich's ataxia.
Effect of an Inhibitor of Lipid Peroxidation, U78517F, on C6 Astrocytoma Growth
Authors: Del Maestro Rolando F. , Farrell Christine R. , Stroude Eric , McDonald Warren
Published: July 2016

A potentin vitroinhibitor of lipid peroxidation, U78517F, has been employed to assess its influence on C6 astrocytoma growth in monolayer culture and tumor growth and protein extravasation in the rat C6 astrocytoma spheroid implantation model. Results demonstrate that 1 μM concentration of U78517F inhibits cell division, but is not tumoricidal to C6 astrocytoma cells in monolayer culture. Concentrations of 5 μM and above significantly decreased astrocytoma cell viability. Following spheroid implantation, rats were treated with one of these U785I7F regimes: 12 mg/kg/day for 13 days post-implantation, 4 mg/kg/day for 13 days post-implantation or 12 mg/kg/day commencing seven days post-implantation until 13 days post-implantation. Tumor wet and dry weights were lower in all treatment groups, but these decreases were not statistically significant. Protein extravasation as measured by Evans blue extravasation was not significantly reduced by any treatment regime used. It is concluded that U78517F inhibits C6 astrocytoma growth in monolayer culture at and above 5 μM concentrations, but the treatment regimes utilized did not significantly decrease tumor growth or permeability in the C6 astrocytoma spheroid implantation model.
Vernix Caseo Granulomatous Meningitis (Vernicomyelia)
Authors: Midha Rajiv , Becker Laurence E.
Published: July 2016

An apneic and tetraplegic infant with an open lumbosacral myelomeningocele and an Arnold-Chiari malformation is reviewed. An exuberant chronic aseptic meningitis with foreign body giant cells and immunoreactive keratin was present around the spinal cord and brainstem. This paper discusses the recognition and role of granulomatous meningitis in the clinical course and in the pathogenesis of the unusual cerebellar abnormalities found in this infant.
Perioperative Management and Outcome after Surgical Treatment of Anterior Cerebral Artery Aneurysms
Authors: Awad Issam A. , Little John R.
Published: July 2016

The authors present clinical experience with 28 cases of ruptured anterior cerebral artery (ACA) aneurysms managed personally during a 36 month period, and 10 unruptured ACA aneurysms. The cases included five giant aneurysms and four distal ACA aneurysms. Management strategy was uniform and included early operative intervention (except in the setting of deteriorating neurologic deficit, not attributable to hydrocephalus or hematoma), and vasospasm prophylaxis including calcium channel blockers and hypervolemic hemodilution and arterial hypertension. Modern diagnostic adjuncts including transcranial doppler were used as they became available. Good outcome (outcome grade 1 or 2) was observed at 6 months in 71% (20/28) of ruptured cases and in 90% (9/10) of unruptured cases; fair outcome (outcome grade 3) was observed in 14% (4/28) of ruptured cases and in 10% of unruptured cases; bad outcome (outcome grade 4 or 5) was observed in 14% (4/28) of ruptured cases. There were no instances of rebleeding after admission to the hospital. There was a single mortality in a patient moribund on admission. Delayed ischemic deterioration (DID) was documented in 46% (13 of 28) of the ruptured cases, and was a major source of morbidity in 7 of the 9 instances of fair or poor outcome in the series. Management outcome, including the occurrence of subtle neuropsychological difficulties commonly described in cases with ACA aneurysms, is discussed with relation to the incidence of DID, the clinical course of DID, and the possible impact of various therapeutic strategies.
Parkinson's Disease and Exposure to Rural Environmental Factors: A Population Based Case-Control Study
Authors: Semchuk Karen M. , Love Edgar J. , Lee Robert G.
Published: July 2016

To determine whether a history of exposure to rural environmental factors leads to an increased likelihood of developing idiopathic Parkinson's disease, we conducted a case-control study of 130 cases and 260 randomly selected community controls (matched with the cases by sex and age ± 2.5 years at a ratio of 2 controls: 1 case) in the city of Calgary. The data were collected by personal interviews and were analyzed using conditional logistic regression for matched sets. The ages of the cases ranged from 36.5 to 90.7 years (mean = 68.5 ± 11.3 years). The mean age at diagnosis was 61.1 ± 12.4 years. The mean duration of disease was 7.8 ± 0.6 years. Eleven (9.1%) cases were diagnosed before age 40. In this sample from the Province of Alberta, Canada, no significant increase in risk for Parkinson's disease was associated with a history of rural living, farm living, or well water drinking in early childhood or at any time during the first 45 years of life.
Benign Brainstem Hemorrhage
Authors: Shuaib Ashfaq
Published: July 2016

Most patients with brainstem hemorrhage have a poor prognosis. There are occasional reports of a favorable outcome with hematomas restricted to the mid-brain region. We report two patients with hematomas within the lower brainstem (pons and medulla) who made a good recovery. Diagnosis was unsuspected until the cranial CT scan was done. Angiography was normal in both cases. Recovery was excellent in one and fair in the second patient. Patients with medullary hemorrhage are at risk for aspiration and may suddenly develop respiratory arrest and therefore should be carefully observed. Our review of the literature suggests that with some small brain-stem hemorrhages recovery is good to excellent and recurrences are rare.
Pneumocephalus following treatment of Esthesioneuroblastoma
Authors: Klimek E.H. , Macdonald R.L. , Deck J.H.N.
Published: July 2016

The course of a patient with an olfactory neuroblastoma treated with conventional doses of chemotherapeutic agents and radiotherapy is presented. The patient survived 34 months after tumour necrosis with resultant pneumocephalus requiring surgical intervention.
A Footnote to Medical History: David Alexander Shirres on Spinal Cord Regeneration
Authors: Robb Preston
Published: July 2016

Little attention has been paid to an early Canadian experiment in neuronal regeneration and what may have been the world's first attempt to replace a damaged spinal cord with a transplant. In 1905, a paper entitled “Regeneration of the Axones of Spinal Neurones in Man” was published in the Montreal Medical Journal. It had been read at the Panamerican Congress in Panama. The author was David Alexander Shirres, a Scot who had trained in Aberdeen in neurology and neuropathology. He came to Canada in 1902 to assume the position of neurologist at the Montreal General Hospital, with the responsibility of establishing clinics and teaching undergraduates about the mysteries of the nervous system. To my knowledge, he was the first man in Canada to be appointed as a neurologist. (There were others, notably James Stewart, who devoted most of their time and writing to diseases of the nervous system but considered themselves to be internists. Stewart, for example, left the MGH to become the first chief of medicine at the Royal Victoria Hospital in Montreal.)
Intraparenchymal and Intraventricular Hemorrhage without Mass Effect in Traumatic Coma
Authors: Lee Jen-Pei
Published: July 2016

A group of 57-head injured patients showing computerized tomographic (CT) findings compatible with “diffuse brain injury” or of the so called “diffuse axonal injury” is analyzed. Thirty-four patients showed intraparenchymal hemorrhage in the CT scan study, 8 intraventricular hemorrhage and 15 patients had both intraparenchymal and intraventricular hemorrhage. Forty percent (23/57) of those with these findings had associated intracranial focal lesions. The deep nuclei are the most common location of intraparenchymal hemorrhage. Signs of brain stem hemorrhage were seen in 9 patients. Forty-nine percent of patients in this series had a good outcome. Old age (>60), abnormal motor response, abnormal eye signs, associated with focal lesions, and evidence of brain stem hemorrhage are reliable prognostic parameters for a grave outcome.