bluuebunny/crossref_metadata_2025_split huggingface dataset:
Prevalence of Multiple Sclerosis in British Columbia
Authors: Sweeney Vincent P. , Sadovnick Adele D. , Brandejs Vilma 
Published: July 2016

ABSTRACT:A province wide prevalence study on multiple sclerosis (MS) was conducted in British Columbia (B.C.). The prevalence date was July 1, 1982. The major portion of this study was a review of all the files of neurologists practicing in B.C. as this was judged to be the most accurate source for identifying MS patients. 239,412 neurologists' files were hand searched by one researcher using modified Schumacher criteria for classification. Other sources used during the study for identifying MS patients were the MS Clinic, general practitioners, ophthalmologists, urologists, specialized facilities such as long term care facilities and rehabilitation centres, and patient self-referrals.A total of 4,620 non-duplicated cases were identified and classified. 4,112 of these (89%) were classified according to information contained in neurologists' records.The prevalence estimate for definite/probable MS in B.C. was 93.3/100,000 population. This increased to 130.5/100,000 population if possible MS and optic neuritis were also included. These rates are among the highest reported in Canada or elsewhere. The cooperation of B.C. neurologists made this study unique in its scope and accuracy of diagnosis.
Nitrazepam for Periodic Movements in Sleep (Sleep-related Myoclonus)
Authors: Moldofsky Harvey , Tullis Carole , Quance Grace , Lue Franklin A. 
Published: July 2016

ABSTRACT:In a follow-up study (mean, approximately six months), nitrazepam was helpful in suppressing periodic movements in sleep (sleep-related myoclonus) and improving disturbed sleep physiology and daytime symptoms of 13 patients (mean age - 53 yr).
Rapid Reversal of Ergotamine-induced Vasospasm
Authors: Lewis P. Jeffrey , Noseworthy Thomas W. , Fitzgerald Avril A. , Andrews George C. , Geeraert Albert J. 
Published: July 2016

ABSTRACT:Peripheral ischemia, secondary to ergotamine tartrate and caffeine suppositories is reported in a 48-year-old female. Lower extremity pre-gangrenous changes were unresponsive to surgical sympathectomy, calcium channel blockade, intra-arterial vasodilators and systemic anticoagulation. A dramatic clinical and radiological reversal of the vasospasm was obtained with intravenous sodium nitroprusside when surgical amputation appeared inevitable.
Tourette-like Syndrome Following Low Dose Short-term Neuroleptic Treatment
Authors: Lal Samarthji , AlAnsari Esam 
Published: July 2016

ABSTRACT:A Tourette-like syndrome (TLS) may occur after long-term neuroleptic treatment. A review of 11 cases reported in the literature is given. We describe the onset of a TLS in a 13-year old boy with childhood schizophrenia after short term, low-dose treatment with thioridazine. The syndrome resolved 5 months after neuroleptic withdrawal. Subsequent exposure to neuroleptics (mainly perphenazine) induced a recurrence of motor tics and involuntary vocalizations which resolved on drug discontinuation. Awareness that neuroleptics may induce a TLS may lead to prompt recognition and avoidance of labelling the manifestations as symptoms of the underlying psychosis or attention-seeking behaviour.
“Whiplash” - Injury of the 2nd Cervical Ganglion and Nerve
Authors: Keith William S. 
Published: July 2016

ABSTRACT:Amongst the many patients with persisting neck pain and headache following cervical injuries are a small number in whom the mechanism is compression of the second cervical nerve root and ganglion. This paper describes the clinical features in 14 patients seen by the author. The main features are unilateral pain in the upper cervical and occipital region, tenderness in the suboccipital region, and diminished sensation in the C2 dermatome. The anatomical basis for this syndrome is discussed and illustrated with dissections from a cadaver.
Swallow Syncope
Authors: Guberman A. , Catching J. 
Published: July 2016

ABSTRACT:Swallow syncope is an often misdiagnosed rare disorder due to enhanced vagal tone during eating in patients with underlying esophageal and/or cardiac abnormalities. We present three cases of this disorder, one related to digitalis toxicity and the other two with diffuse esophageal spasm. The investigation, differential diagnosis, prognosis and management of swallow syncope are discussed.
A Controlled Study of Oral Vigabatrin (ɣ-Vinyl GABA) in Patients with Cerebellar Ataxia
Authors: Bonnet A.M. , Esteguy M. , Tell G. , Schechter P.J. , Hardenberg J. , Agid Y. 
Published: July 2016

ABSTRACT:Vigabatrin (ɣ-vinyl GABA; GVG), an irreversible inhibitor of GABA-transaminase, at a daily dose of 2-4 g, and a placebo were each administered orally for 4 months to 14 patients with cerebellar ataxia (9 with Friedreich's ataxia, 5 with olivopontocerebellar atrophy), in a double-blind, placebo-controlled crossover study. For the group as a whole, there was no significant difference between the GVG and placebo periods in any of the parameters of cerebellar symptomatology measured. Individually, one patient showed some improvement after 3 months of treatment with 2 g/day GVG. Tolerance to 4 g/day GVG was poor, whereas 2 g/day was well tolerated. The results suggest that agents which increase central GABA concentrations are not likely to be of benefit to patients with Friedreich's ataxia or olivopontocerebellar atrophy.
Speculations on the Role of Transmissible Agents in the Pathogenesis of Alzheimer's Disease
Authors: Haase Ashley T. , Lewis Elizabeth , Wietgrefe Stephen , Zupancic Mary , Jane Diedrich , Minnigan Hal , Ball Melvyn J. 
Published: July 2016

Abstract:Unconventional agents and conventional viruses provide model systems to investigate the pathogenesis of Alzheimer's disease (AD). The essay which follows examines the hypothetical role of herpes simplex in AD and presents some generally applicable experimental approaches to detecting genes in brain tissues. The concluding section, on parallels between AD and diseases of the brain caused by unconventional viruses, defines strategies for isolating genes related to pathology.
Lewy Body Dementia Without Alzheimer Changes
Authors: Sima A.A.F. , Clark A.W. , Sternberger N.A. , Sternberger L.A. 
Published: July 2016

Abstract:Three patients with clinical Alzheimer's disease were found at postmortem examination to have Lewy-bodies and Lewy-like bodies in the cerebral cortex and the pigmented brainstem nuclei. Neuritic plaques were found in neocortical areas but no neurofibrillary tangles. The distribution of cortical neuronal inclusions correlated with the proposed projection of dopamine terminals. Neuronal cell loss was marked in the ventral tegmental area (paranigral nucleus) and the basal nucleus of Meynert, suggesting a defect in dopaminergic and cholinergic innervation of the cerebral cortex. Immunohistochemical investigations revealed positive staining of cortical Lewy- and Lewy-like bodies for monoclonal antibodies to phosphorylated neurofilaments (03-44, 06-17, 04-7). Also cerebral neurons containing no inclusions showed positivity, suggesting an early neurofilament abnormality, preceding the formation of Lewy-type inclusions.
Leuko-Araiosis: An Ancient Term for a New Problem
Authors: Hachinski V.C. , Potter P. , Merskey H. 
Published: July 2016

Abstract:Recent research with computerized tomography (CT) and magnetic resonance imaging (MR) of the brain has revealed a type of tissue change for which no fitting term exists. The change appears as areas of decreased density on CT and changed signals of the white matter in MR images. Because neither a definite pathological change nor a specific clinical deficit has been linked with the CT and MR changes, a designation is required that limits itself to describing the changes themselves.We propose “leuko-araiosis”. The Greek root leuko-, signifying “white”, has found wide usage in modern medical terminology: e.g. leucine, leukocyte, leukorrhoea. Also several precedents exist for its application to the white matter of the central nervous system: e.g. leukoencephalitis, leukomyelitis, leukotomy. Araios is an adjective meaning “rarefied”, and the suffix -osis converts the adjective to a noun meaning “rarefaction, diminution of density”, a word used in the Hippocratic Collection to describe a state of excessive porosity of the lung.A descriptive term precludes premature presuppositions and encourages the search for causes.
Optimum Symptomatic Control of Parkinson's Disease with Dopaminergic Therapy
Authors: Bouchard Sylvie 
Published: July 2016

ABSTRACT:This paper presents a review of the literature on the therapeutic action and the side effects of the two main dopaminergic agents: L-DOPA/decarboxylase inhibitor (L-DOPA/DI) and bromocriptine (Parlodel ®) used either as monotherapy or in combination in patients with Parkinson's disease. The combination of L-DOPA/DI and bromocriptine gives the best therapeutic efficacy (49% improvement) in the total score (bradykinesia, rigidity and tremor). However, treatment by monotherapy or combination gives the same pattern of activity: greatest improvement in tremor, followed by rigidity and bradykinesia. Improvement observed in the short term is not sustained over longer periods of time for monotherapy with either drug. The short-term side effects are similar for each treatment, whereas long-term complications (dyskinesia, end-of-dose deterioration and on-off phenomenon) appear only when levodopa is used, alone (high incidence) or in combination with bromocriptine (low incidence). The overall optimum treatment is obtained with a combination of L-DOPA/DI and bromocriptine.
Antioxidant Therapy in Parkinson's Disease
Authors: Grimes J. David , Hassan Mohamed N. , Thakar Jay 
Published: July 2016

ABSTRACT:It is postulated that endogenous oxidative mechanisms are a major factor in the continuing death of dopaminergic neurons and the progression of Parkinson's disease. Scientific evidence in support of, and negating, the free radical auto-toxicity and dopamine toxicity concepts is reviewed. There is conflicting evidence whether free radicals are involved in the toxicity of l-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) and attempts to prevent the toxicity of MPTP with antioxidant therapy have had variable results. The oxidation of dopamine by monoamine oxidase produces toxic metabolites however animal studies with high dose longterm levodopa and MPTP have failed to show clear evidence for autoxidation. Firm supportive evidence is obtained from the monoamine oxidase B inhibitor experience which demonstrated a block of the toxicity of MPTP in animals and probable prolongation of the course of human Parkinson's disease.The scientific data available is inconclusive but there is significant hope of retarding progressive catecholaminergic neuron degenerative changes by augmenting the free radical scavenging system with antioxidants (such as Vitamin E) and slowing catecholamine oxidation by monoamine oxidase B inhibition. Careful clinical trials with these agents must be performed.
Continuous Dopaminergic Stimulation in Parkinson's Disease
Authors: Obeso J.A. , Luquin M.R. , Vaamonde J. , Grandas F. , Martinez Lage J.M. 
Published: July 2016

ABSTRACT:Complex motor fluctuations and dyskinesias (“on-off” phenomenon) in Parkinson's disease can be corrected by parenteral administration of levodopa, levodopa-methyl-ester, lisuride and apomorphine. Levodopa and levodopa-methyl-ester may only be administered intravenously because of their low solubility. Lisuride and apomorphine are readily absorbed after subcutaneous administration. Repeated or continuous intravenous infusions of levodopa have been given for a few days, using a wearable “jacket-like” pump, with good results. So far, lisuride is the only dopamine agonist used for chronic treatment by continuous infusion. The “on-off” effect is adequately controlled in most patients by subcutaneous lisuride administration (plus oral levodopa). However, adverse effects, particularly psychiatric complications, constitute a major limiting factor for routine applications of this form of treatment. Subcutaneous apomorphine infusion is unlikely to become a standard therapeutic procedure in Parkinson's disease, but it is a very valuable research method to gain further insight into the pathophysiology of motor fluctuations in Parkinson's disease.
Electromyography in Disorders of Muscle Tone
Authors: Eisen Andrew 
Published: July 2016

ABSTRACT:No single clinical electrophysiological test can evaluate disorders of muscle tone. These disorders, symptomatic of a variety of diseases have a multifactorial physiological basis. The several tests used are complimentary each aiming to study different aspects of spinal and supraspinal reflexes which become deranged. The H reflex and F wave (H max/M max and F max/M max ratios) measure motoneuron pool excitability in general. The tendon reflex includes spindle mechanisms bypassed by the H reflex and, with limitations, comparison of H max/M max and T max/M max yields information about the -y system. Tonic vibration of a tendon inhibits the H reflex from the same muscle. The TVR measures autogenous presynaptic inhibition exerted by the la afferents of the muscle. Recurrent inhibition via Renshaw cells is evaluated by studying the effect of collision on the H reflex. Reciprocal inhibition of the la afferents can be assessed by measuring H reflex change induced by stimulating la afferents from antagonists. Changes in the H reflex recovery cycle measure polysynaptic influences on spinal motoneuron excitability. Cutaneomuscular (flexor) reflexes measure poly- and oligosynaptic excitatory drive to spinal motoneurons and the blink reflex evaluates the excitatory drive to brainstem motoneurons. Long loop (segmental) responses can be evaluated by limb pertubation using a torque motor or electrical stimulation applied during voluntary muscle contraction. Finally needle electromyography is a more relevant test in several disorders of muscle tone such as the stiff-man syndrome and Isaacs' syndrome.
Motor, Behavioral and Pharmacologic Findings in Tourette's Syndrome
Authors: Jankovic Joseph , Rohaidy Haydee 
Published: July 2016

ABSTRACT:We studied 112 patients with Tourette's syndrome (TS); the male-to-female ratio was 3.8, the mean age of onset was 7.3 years, and the average duration of symptoms prior to the initial evaluation was 15.2 years. Seventy-nine percent of the patients had at least one family member with motor or vocal tics, and an additional 10 percent had a family member with marked obsessive-compulsive behavior. Simple motor tics occurred as the presenting symptom in about one-third of patients; one-third had multiple motor tics at the onset, and another third started with vocal tics. During the course of the illness all patients developed multifocal motor tics and 86 percent had vocal tics. Verbal and mental coprolalia was present in 44 percent of the patients. Copropraxia was seen in 19 percent of patients, and both coprolalia and copropraxia were more frequent among the males than expected. Attentional deficit disorder was diagnosed in 36 percent of the patients and 32 percent had obsessive-compulsive personality. Sleep disturbances were reported by 62 percent of the patients and polysomnography in 34 patients showed motor and vocal tics during all stages of sleep, sleep apnea, abnormal arousal pattern, and other sleep disturbances. Patients with mild symptoms improved with clonidine or clonazepam, but those with more advanced disorder required fluphenazine, pimozide, haloperidol or tetrabenazine.
Global Arteriovenous Malformation of the Cervical Region
Authors: Holgate R.C. , Lougheed W.M. 
Published: July 2016

This case was presented as an interesting example of a global arteriovenous malformation (AVM) involving muscle, bone, extradural and intradural spaces of the cervical region.A twenty-eight year old male in October, 1976, fell at work sustaining a blow to his lower back. The following day he complained of cervical pain. Physical examination was negative and he had a full range of movement of his cervical spine.Plain films and tomograms of the cervical spine showed a lytic honeycomblesion of C2 and C3, and a 4 mm. subluxation of C3 on C4 (Fig. 1). The bone scan showed increased flow and decreased static activity in the upper cervical region. The radiological diagnosis was vertebral hemangioma; tumor, infection and histiocytosis were unlikely but important differential considerations.
Structural Lesions of the Brain in Young Schizophrenics
Authors: Averback P. 
Published: July 2016

SUMMARYSwollen, degenerate and fragmented neurons of the septal nuclei and the nucleus ansae peduncularis are commonly found in chronic neurological diseases such as Alzheimer’s or Huntington’s disease, and are rarely found in neurologically normal control patients without mental symptoms. These pathological lipid and pigment laden neurons appear to be a common feature of schizophrenia and have been consistently found in a series of unselected young (mean age 38) schizophrenics.
Delayed Encephalopathy Following Carbon Monoxide Intoxication
Authors: Sawa G.M. , Watson C.P.N. , Terbrugge K. , Chiu M. 
Published: July 2016

SUMMARYThe case history and CT scan of a patient with delayed encephalopathy following carbon monoxide intoxication are described. The patient recovered.The CT scan revealed symmetrical areas of decreased density at the level of the globus pallidus. This case differs in several ways from other cases of carbon monoxide intoxication in which abnormalities of the CT scan were found.
Dopamine Uptake Capacity of Platelets From People At Risk For Huntington’s Chorea
Authors: McLean Donald R. , Nihei Taichi 
Published: July 2016

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

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

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

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

SUMMARY:Some multiple sclerosis patients with 20/20 acuity complain of poor vision. In a previous report we accounted for this in our patient group by showing that multiple sclerosis had caused a depression of contrast sensitivity while sparing visual acuity. In this study we investigated whether some of this measured depression might be due to abnormally rapid or severe adaptation during the test procedure rather than a true permanent loss. Our finding was opposite to this supposition: adaptation was abnormally slight and/ or slow.Depressed contrast sensitivity was not well correlated with abnormal adaptation to contrast. In patients whose contrast sensitivity losses were restricted to a band of spatial frequencies, we found no evidence that abnormalities of contrast adaptation were restricted to this same spatial frequency band. Further evidence of dissociation between abnormal contrast sensitivity and abnormal contrast adaptation is that some patients with normal contrast sensitivity showed abnormally small adaptation.Our finding of abnormally slow or abnormally small contrast adaptation in MS patients seems to conflict with Enoch et al’s (1978) report of abnormally great adaptation after inspecting a bright stimulus. This apparent disagreement may be due to their use of prior dark adaptation, very bright adapting stimuli and patients in the acute stage of retrobulbar neuritis.
Transient Choreiform Dyskinesias During Alcohol Withdrawal
Authors: Fornazzari L. , Carlen P. L. 
Published: July 2016

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

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