bluuebunny/crossref_metadata_2025 huggingface dataset:
A Randomized, Controlled Trial of Linopirdine in the Treatment of Alzheimer's Disease
Authors: Rockwood Kenneth , Beattie B. Lynn , Eastwood M. Robin , Feldman Howard , Mohr Erich , Pryse-Phillips William , Gauthier Serge
Published: February 2016

Objectives:

We tested the efficacy and safety of linopirdine, a novel phenylindolinone, in the treatment of Alzheimer's disease.

Methods:

A multicentre, randomized, double-blind, parallel group, placebo-controlled trial of linopirdine (30 mg three times per day or placebo). Patients (n = 382, 55% male, 98% Caucasian, age range 51-95 years) with mild or moderate Alzheimer's disease, of whom 375 received at least one treatment dose were analysed. There were no important differences between the groups at baseline.

Results:

No difference was seen in Clinical Global Impression scores between patients receiving placebo and those receiving linopirdine (n = 189). Small differences in the Alzheimer's Disease Assessment Scale-Cognitive Subscale (ADAS-Cog) scores were seen throughout the study favouring linopirdine; at 6 months the ADAS-Cog scores were 20.2 (linopirdine) and 22.1 (placebo) p = 0.01.

Conclusions:

This trial did not detect clinically meaningful differences in patients receiving linopirdine for 6 months, despite evidence of a small degree of improved cognitive function. Further studies may benefit from more sensitive tests of treatment effects in Alzheimer's disease.
Parkinsonian Syndrome as a Neurological Manifestation of Behcet's Disease
Authors: Bogdanova D. , Milanov I. , Georgiev D.
Published: February 2016

Background:

The central nervous system is often involved in Behcet's disease. Most common are meningoencephalitic and brain stem syndromes. Although basal ganglia involvement is not an uncommon finding on necropsy, there are only single reports on extrapyramidal syndromes-dyskinesia, chorea and Parkinsonism in patients with Behcet's disease.

Case study:

We report a patient fulfilling the criteria of the International Study Group for Behcet's disease. He had recurrent oral ulcerations, bilateral posterior uveitis and retinal vasculitis, skin papules and pustules, and recurrent monoarthritis. Neurologic examination revealed pseudobulbar palsy, slight and asymmetric bilateral pyramidal syndrome, muscle rigidity involving the four limbs, bradykinesia, masked face, and impaired postural reflexes. There was postural tremor in the extremities and myoclonic jerks involving the tongue and face muscles. Magnetic resonance imaging demonstrated small bilateral multifocal hyperintense lesions, with right predilection, involving the periventricular white matter, brain stem and basal ganglia.

Conclusions:

The Parkinsonian syndrome found in our patient might be due to involvement of both substantia nigra and basal ganglia. This case further emphasizes the wide spectrum of the neurological manifestations of Behcet's disease
A 21 Point Unifying Hypothesis on the Etiology and Treatment of Multiple Sclerosis
Authors: Weiner Howard L.
Published: February 2016

Multiple sclerosis (MS) is postulated to be a cell mediated autoimmune disease directed against central nervous system myelin components. Our understanding of the disease has been enhanced by a number of factors: 1) advances in our understanding of the immune system; 2) clinical trials which are beginning to identify treatments which can affect MS; 3) a better understanding of the clinical features of MS; and 4) advances in MRI imaging of the brain. Based on the current state of knowledge, this paper proposes a 21 point unifying hypothesis on the etiology and treatment of the disease. This hypothesis makes a series of assumptions, many of which are unproven, and is presented as a framework from which to investigate and treat the disease, not as a established biology. It is hypothesized that the underlying pathogenesis of MS is related to an inappropriate class of immune response against myelin antigens favoring proinflammatory Th1 versus anti-inflammatory Th2 or Th3 type responses. Environmental and genetic factors predispose toward MS by affecting the class of response and effectiveness of treatment is also related to how it impacts on this common final pathway. Because of epitope spreading, there is not one autoantigen involved in MS and the progressive form of MS differs immunologically from the relapsing remitting form. Viruses trigger and perpetuate MS, although MS is not related to a persistent viral infection. Because MS is a multifactorial disease, there are clinical and perhaps immunological subtypes of MS and a single type of treatment is unlikely to control the disease in all patients. Thus, there will be responders and non-responders to each effective therapy and ultimately combination therapy will be required to cure the disease.
Hemorrhagic Necrotic Schwannoma Presenting as Purulent Meningitis
Authors: Onyiuke H.C. , McLean A. , Schwartz M. , Louie M. , Ang L.C.
Published: February 2016

Background:

Intracranial schwannoma involving the XIIth cranial nerve is rare. We report an unusual clinical presentation and pathological verification of a schwannoma, which had become haemorrhagic and necrotic, simulating acute purulent meningitis.

Methods:

A literature review of intracranial tumors presenting as acute purulent meningitis, with emphasis on schwannomas, was undertaken.

Results:

Few cases of hypoglossal schwannoma have been reported; the association with purulent meningitis has not been previously documented.

Conclusion:

In patients presenting with sterile purulent meningitis, a necrotic tumor should be considered as a possible etiology.
Congenital Entrapment of the Lateral Cutaneous Nerve of the Calf Presenting as a Peroneal Sensory Neuropathy
Authors: Hackam Daniel G. , Zwimpfer Thomas J.
Published: February 2016

Objective:

Presentation of an unusual case of congenital entrapment of the lateral cutaneous nerve of the calf (LCNC) mimicking a peroneal sensory neuropathy.

Methods:

We report the case of a 16-year-old girl with a 3 year history of progressive tingling, numbness and pain in her right calf precipitated by athletic activities involving repeated flexion and extension of the knee. A Tinel's sign was present over the common peroneal nerve in the distal popliteal fossa but absent at the fibular neck. Motor and sensory examination of the common peroneal nerve was normal as were electrophysiological studies and MRI.

Results:

At surgery, the LCNC, a sensory branch of the common peroneal nerve, was entrapped at a point where it pierced the tendon of the biceps femoris muscle. Transection of the part of the tendon overlying the LCNC resulted in complete and permanent relief of symptoms.

Significance and Conclusion:

The proximal location of the Tinel's sign, absence of motor or sensory deficits and normal electrophysiology suggested, preoperatively, that this was not an entrapment of the common peroneal nerve at the fibular neck but rather a more proximal abnormality likely involving only a part of the peroneal nerve or one of its sensory branches. As a result, more proximal exposure of the peroneal nerve within the popliteal fossa revealed entrapment of the LCNC due to its anomalous course through the biceps femoris tendon. This case has two noteworthy features. It is a unique example of an entrapment neuropathy of the common peroneal nerve or one of its branches, due to a normal nerve piercing an otherwise normal tendon. Secondly, there are no previously reported cases of surgically documented compression of the LCNC in an otherwise normal patient (i.e., non-diabetic).
Stereotactic Radiosurgery for Acoustic Neuroma: A Canadian Perspective
Authors: Ross Ian B. , Tator Charles H.
Published: February 2016

Stereotactically delivered radiation is now an accepted treatment for patients with acoustic neuroma. In some cases, patient preference may be the reason for its selection, while in others neurosurgeons may select it for patients who are elderly or have significant risk factors for conventional surgery. The majority of patients with acoustic neuroma treatment with stereotactic radiosurgery have been treated with the Gamma Knife, with follow ups of over 25 years in some instances. Other radiosurgical modalities utilizing the linear accelerator have been developed and appear promising, but there is no long-term follow up. Canada does not possess a Gamma Knife facility, and its government-funded hospital and medical insurance agencies have made it difficult for patients to obtain reimbursement for Gamma Knife treatments in other countries. We review the literature to date on the various forms of radiation treatment for acoustic neuroma and discuss the current issues facing physicians and patients in Canada who wish to obtain their treatment of choice.
Summary: Electrophysiology in the ICU
Authors: Bolton C.F.
Published: February 2016

Dr. Michael Aminoff, guest of the Canadian Society of Clinical Neurophysiologists, discussed the Management of Status Epilepticus. The EEG is essential for the recognition of status epilepticus where there are minor or no motor manifestations, and also in clarifying the nature of certain motor and behavioural disturbances that do not relate to seizures. It also is important in evaluating the adequacy of treatment, such as the response to barbiturate or midazolam induced anaesthesia.
Efficacy of Topiramate
Authors: Bruni J.
Published: February 2016

In controlled clinical trials, topiramate (TopamaxR) has demonstrated efficacy in refractory patients with complex partial seizures and secondarily generalized tonic-clonic seizures. Approximately 45 percent of 534 patients had a ≥ 50 percent reduction in seizure frequency. Limited open label trials have shown that topiramate has broad spectrum activity and may be effective in patients with primary generalized epilepsies. The efficacy of topiramate compares very favourably with the efficacy of other new antiepileptic drugs recently introduced.
Using Topiramate in Patients with Epilepsy: Practical Aspects
Authors: Sander J.W.A.S.
Published: February 2016

Clinical trials are important in determining the relative efficacy and safety of a new antiepileptic drug (AED); however, experience acquired in clinical practice will eventually determine its position in the antiepileptic armamentarium. Topiramate (TPM), a new AED has been available in the United Kingdom since mid-1995 and a considerable number of patients have being treated. As a result of this experience, a number of changes have being made in the way TPM is used, particularly in the starting doses and titration rates. This seems to have improved patients' tolerability of treatment, an important consideration if a drug is to be used to its optimum level. In this article, practical tips for the use of TPM are given and these include starting doses, titration rates, options for managing side effects occurring early in treatment, advice concerning the withdrawal of concomitant AEDs and indications for discontinuation of TPM. The need for adequate patient counseling regarding potential side effects and expectations of treatment is also reviewed.
Monotherapy or Polytherapy for Epilepsy?
Authors: Guberman Alan
Published: February 2016

Monotherapy has been promoted as the ideal in epilepsy treatment because of reduced side effects, absence of drug interactions, better compliance, lower cost and, in many cases, improved seizure control compared to polytherapy. The question of monotherapy vs. polytherapy has assumed increasing importance with the availability of multiple new antiepileptic drugs (AEDs), initially tested as add-on agents. The new drugs clobazam, lamotrigine, vigabatrin, gabapentin and topiramate, have also been shown to be effective as monotherapy. These data bring up the possibility of using them as first-line agents. However, a high percentage of patients with resistant epilepsy are treated with polytherapy, which probably benefits only a minority of them. The availability of multiple drugs with different mechanisms of action favours the possibility of "rational polytherapy", taking advantage of possible synergism, a yet unproven concept. This article reviews the theoretical advantages of monotherapy and monotherapy with traditional and newer AEDs.
The Efficacy of Retrograde Infusion with LY231617 in a Rat Middle Cerebral Artery Occlusion Model
Authors: Inoue Nobuhiro , Yamamoto Y. Lucas , Ito Yasushi , Clemens James A. , Panetta Jill K. , Diksic Mirko
Published: February 2016

Background and Purpose: We examined the efficacy of the antioxidant LY231617 administered five hours following middle cerebral artery (MCA) occlusion in rats. Methods: The treatment was contrived for a two hour interval. Group A (n=16) was left untreated. Group B (n=16) received an intravenous infusion of LY231617. Group C (n=6) received saline (86 μI/min) by retrograde infusion of the cerebral vein (RICV). Group D (n=22) was administered LY231617 (10mg/kg/2hr) in saline (86μI/min) by RICV. Local cerebral blood flow with [14C]-iodoantipyrine and blood-brain transfer constant with 14C-α-amino-isobutyric acid were examined. Early ischemic damage was histologically examined with cresyl violet and Luxol fast blue and with triphenyl-tetrazolium chloride. Results: The results revealed a marked increase in local cerebral blood flow (over 600%, p < 0.01) after RICV with LY231617, with a significant improvement of BBB permeability in rats from group D. Ischemic brain damage measured with Luxol fast blue and triphenyl-tetrazolium chloride methods showed a significant improvement (50-91 %) of ischemic damage in group D, as compared to groups B and C. Conclusion: Retrograde infusion of the cerebral vein with LY231617 resulted in a significant amelioration at seven hours post MCA occlusion.
Periodontoid Synovial Cyst Causing Cervico-medullary Compression
Authors: Kaufmann Anthony M. , Halliday William C. , West Michael , Fewer Derek , Ross Ian
Published: February 2016

Background: Periodontoid synovial cysts are rare lesions which may produce symptomatic cervico-medullary compression. Method: We report such a patient, whose progressive neurological deterioration required surgical treatment by transoral odontoidectomy and decompression. Results: The diagnostic and theraputic interventions are described, including a lumbar puncture which precipitated a transient loss of consciousness and respiratory arrest. Surgery achieved clinical improvement, without complications or need for operative stablization. Detailed neuropathology is presented, as well as a literature review. Conclusion: Appropriate neuroradiological assessment is required in patients with suspected cervico-medullary compression, and symptomatic periodontoid synovial cysts may respond well to transoral surgical decompression.
Epidemiology of Childhood Brain Tumors
Authors: Miltenburg Dartene , Louw Deon F. , Sutherland Garnette R.
Published: February 2016

Background: Brain tumors comprise more than 20% of all childhood malignancies, and constitute the greatest number of solid pediatric cancers. Incidence rates reported have varied from 2.4 to 3.5/100, 000 children, reflecting the impact of modern imaging techniques, the application of diverse investigative methodologies, and the accessibility of the community to health care. Methods: Material from patients < 18 years of age was collated from the Manitoba Cancer Foundation Tumor Registry, the personal records of Winnipeg pediatric neurologists, and autopsy data. Patient data were also obtained from hospital charts and operating room log books. Histological sections were examined and classified according to the American Cancer Society by a single neuropathologist. The chi-square test was used for statistical evaluation. Results: During the seven-year study period, the diagnosis of brain tumor was made in 89 pediatric patients, of which 88 were diagnosed premortem. The overall average annual incidence rate for both sexes was 4.03/100, 000 child-years, higher than that previously reported. The male and female average annual incidence rates were 4.2 and 3.7/100, 000 child-years, respectively. Tumor type and location were relatively unremarkable, with an expected peak of medulloblastoma occurring in young males. The yearly incidence of tumor occurrence was fairly stable, and the geographic distribution of cases within Manitoba, homogeneous. Conclusion: The highest incidence rates of pediatric brain tumors have been recorded in countries possessing sophisticated universal health care systems, possibly reflecting their efficacy in disease surveillance.
Kinematics of Initiating a Two-Joint Arm Movement in Patients with Cerebellar Ataxia
Authors: Massaquoi Steve , Hallett Mark
Published: February 2016

Objective:

To characterize kinematically any systematic aberration in multi-joint movements in cerebellar ataxia.

Methods:

Nine patients with cerebellar degeneration and nine normal subjects, mobile only at the shoulder and elbow of the right arm, were required to produce left-to-right cross-body linear hand trajectories on the horizontal surface of a digitizing tablet. Nonlinearity indicated failure of precise coordination of the two joints. A wide range of hand speeds was studied. Data analysis was restricted primarily to the first 130 ms of movement.

Results:

As hand velocities increased, normal subjects and, especially, patients produced misdirected, curved paths. Normal subjects had significant curvature when peak speeds exceeded 100 cm/s and a trend toward significant bi-directional angular deviation at velocities greater than 300 cm/s. In patients, peak path curvature was significantly greater than normal at peak velocities of 50 to 200 cm/s. By 3.3 cm, their paths deviated significantly outward at all but the slowest speeds. Overall, patients’ maximal hand velocities and shoulder angular velocities, as well as maximal angular accelerations at both joints, were significantly lower than normal.

Conclusions:

The patients’ trajectory aberrations were attributed to a deficient rate of rotation at the shoulder relative to that at the elbow. Relative to task requirements, their rate of torque development was apparently deficient at both joints, but to a greater degree at the shoulder. Joint torque-rate impairment may contribute to the ataxia in both multi- and single-joint movements of patients with cerebellar disorders. A similar, but smaller impairment may produce milder nonlinearity in high-velocity movements of normal subjects.
Prior Intra-operative Hypotension is not a Risk Factor for Development of Alzheimer’s Disease
Authors: Bohnen N.I. , Wijdicks E.F.M. , Kokmen E. , Warner M.A. , Kurland L.T.
Published: February 2016

Objective:

A retrospective, population-based, case-control study was carried out to evaluate episodes of prior intra-operative hypotension as a potential risk factor for Alzheimer’s disease (AD).

Methods:

Patients were all incident cases of AD from 1975–1984 who resided for 40 years or more in Olmsted County prior to their onset of dementia (N = 252). One age and gender-matched control for each case was selected from all registrations for care at Mayo Clinic during the year of onset in the incident case. Each case and control group had 252 individuals.

Results:

Of these, 27 cases and 32 controls had at least one ten minute or longer episode of intra-operative hypotension of a systolic blood pressure of less than 90 mm Hg prior to the year of onset of dementia in the matched AD patient. We did not find a significantly increased risk of AD for hypotensive episodes of less than 75 or 90 mm Hg.

Conclusions:

It is unlikely that intra-operative hypotensive events of this degree increase the risk of AD.
Protein Kinase C and Growth Regulation of Malignant Gliomas
Authors: Baltuch Gordon H. , Dooley Nora P. , Villemure Jean-Guy , Yong Voon Wee
Published: February 2016

This article reviews the role of the signal transduction enzyme protein kinase C in the regulation of growth of malignant gliomas, and describes how targetting this enzyme clinically can provide a novel approach to glioma therapy.
Long Term Exposure to Manganese in Rural Well Water Has No Neurological Effects
Authors: Vieregge P. , Heinzow B. , Korf G. , Teichert H.-M. , Schleifenbaum P. , Mösinger H.-U.
Published: February 2016

Background

There is debate on the neurological impact of chronic exposure to Manganese (MN).

Methods

MN burden from rural well water was studied cross-sectionally in two proband cohorts from rural dwellings located in northern Germany. Both cohorts had exposure times for up to 40 years and were separated on the basis of well water MN content. Group A (41 subjects; mean age 57.5 years) was exposed to MN water contents of at least 0.300 mg/l (range 0.300 to 2.160), while group B (74 subjects; mean age 56.9 years) was exposed to concentrations of less than 0.050 mg/1. Both proband groups were homogenous with regard to age, sex, nutritional habits, and drug intake. Neurological assessments by clinical investigators blinded for proband’s exposure status was done using structured questionnaires, standardized neurological examination with assessment of possible Parkinsonian signs by the Columbia University Rating Scale, and instrumental tests of fine motor coordination.

Results

No significant difference in any neurological measure was found between groups. Results were not confounded by demographic and dietary features.

Conclusion

Exposure to high body burden of MN does not result in detectable neurological impairment. Exposure to MN in drinking water does not seem to be a risk factor for idiopathic Parkinson’s disease.
Experimental Delayed Postischemic Spinal Cord Hypoperfusion After Aortic Cross-clamping
Authors: Follis F. , Miller K. , Scremin O.U. , Pett S. , Kessler R. , Temes T. , Wernly J.A.
Published: February 2016

Background

As in the brain, recent evidence has suggested a defect in the microcirculation during the reperfusion period after spinal cord ischemia. This investigation was undertaken in order to delineate blood flow dynamics in the postischemic spinal cord of the rat.

Methods

Male Sprague-Dawley rats underwent cross-clamping of the aorta and subclavian arteries (XC) for 11 minutes. Spinal cord blood flow (SCBF) was measured by autoradiography in the gray and white matter of cervical (Ce), thoracic (Th) and lumbar (Lu) regions during XC, 1 h, 6 h and 24 h (XC n = 8, 1 h n = 9, 6 h n = 9, and 24 h n = 11, groups) after XC. Control groups underwent surgical manipulations and SCBF measurement but no XC (Sham 1, n = 8), or clamping of the subclavian arteries only (Sham 2, n = 8).

Results

In Ce cord, there was no difference between SCBF of 1 h, 6 h, 24 h and Sham 1. In Th cord, SCBF was reduced during XC (P < 0.003 vs. Sham 2), 1 h, 6 h (P < 0.04 and P < 0.01 vs. Sham 1). In Lu cord, SCBF was not detectable in XC, and depressed in 1 h (P < 0.003) and 6 h (P < 0.003). There was no difference between 24 h and Sham 1 in Ce, Th, and Lu cords.

Conclusions

The study demonstrated a period of delayed postischemic hypoperfusion in the white and gray matter of Th and Lu cord segments lasting 6 h after XC. The phenomenon may play an important role in the ultimate fate of neural elements with borderline viability after ischemic injury.
The Excitability of Human Corticospinal Neurons is Depressed by Thiopental
Authors: Kong K. , Ukachoke C. , McGuire G. , Wong D. , Ashby P.
Published: February 2016

Background

We tested the effect of thiopental on the excitability of the corticospinal-motoneuron axis in normal human subjects.

Methods

Magnetic stimulation was used to excite the neurons in the motor cortex which give rise to the fast conducting corticospinal pathway. The characteristics of the composite excitatory post-synaptic potentials (EPSPs) produced in individual spinal motoneurons by cortical stimulation were derived from changes in the firing probability of voluntarily activated motor units of the first dorsal interosseous muscle.

Results

In 5 normal subjects, we found that thiopental, in incremental doses sufficient to sustain drowsiness (total dose 75 to 175 mg), significantly reduced the amplitude of these composite EPSPs.

Conclusions

Thiopental reduced the facilitation of motoneurons from the cortex most likely by depressing cortical neurons.
Stereotactic Insertion of an Ommaya Reservoir: Technical Note
Authors: McDermott Michael W. , Ciricillo Samuel F. , Gutin Philip H. , Edwards Michael S.B.
Published: February 2016

Background

Stereotactic insertion of catherters into deep-seated tumors or developmental cysts is easily accomplished, but connecting the catheter to an Ommaya reservoir while maintaining catheter position can be difficult. We describe a technique for easy placement of a catheter-Ommaya reservoir construct with one pass.

Methods

Standard stereotactic imaging is performed. The distance from the outer table of the skull to the target point is measured. A catheter-Ommaya reservoir construct is assembled to this length and directed to the target position with a standard Cosman-Robert-Wells (CRW) stereotactic frame.

Results

Use of this technique placed catheters into tumor or developmental cysts accurately and with no surgical complications in 12 patients.

Conclusions

This technique is simple, safe, reliable, and requires no special equipment. It avoids the risk of dislodging the catheter when it is being connected to the Ommaya reservoir, reducing the chances of cyst leakage and collapse.
“Unexplained” Delayed Death From Fungal Meningitis After Meningioma Resection
Authors: John Hurlbert R. , Bilbao Juan M. , Tucker William S.
Published: February 2016

Background

Progressive deterioration and ensuing death following a neurosurgical procedure often represents a diagnostic challenge to the team responsible for patient care. Many, but not all, causes are treatable if a diagnosis is made early.

Methods

A 69-year-old woman who died 6 weeks post-operatively following a meningioma resection is reported. An initial routine post-operative course became complicated by progressive neurological deterioration 3–4 weeks later. Despite extensive investigation she died 6 weeks post-operatively without a diagnosis.

Results

Autopsy demonstrated extensive Candida meningitis. A review of the literature demonstrates this to be a reported complication in high risk patients, difficult to diagnose, but treatable when identified.

Conclusions

Fungal meningitis should be high in the differential diagnosis in the post-operative patient with delayed, unexplained neurological deterioration, especially when associated with negative CSF cultures.
HIV-1 Associated Dementia: Clinical Features and Pathogenesis
Authors: Power Christopher , Johnson Richard T.
Published: February 2016

HIV-1 infection is characterized by multiple neurological syndromes occuring at all stages of infection. HIV-1-associated dementia, however, is the most devastating CNS consequence of AIDS because of its poor prognosis and functional impairment. A clinical triad of progressive cognitive decline, motor dysfunction, and behavioural abnormalities typifies this subcortical dementia which eventually affects 15 to 20% of AIDS patients. Neuroimaging, CSF studies and neuropsychological testing are frequently required in diagnosing HIV-associated dementia, to exclude other conditions including psychiatric illnesses, opportunistic diseases and systemic disorders. The pathogenesis of HIV dementia is uncertain and there is evidence that multiple mechanisms of neurological injury occur. These mechanisms include: the role of neurovirulent strains of HIV; the potential neurotoxicity of HIV gp120, nitric oxide and quinolinic acid; immunologically mediated CNS injury through the action of cytokines and arachidonic acid metabolites; and altered blood-brain barrier permeability. A collective approach involving clinical studies,in vitroassays and animal models will provide greater insight into the pathogenesis and the rational development of therapy for HIV dementia.
“Hysteria” in Clinical Neurology
Authors: Mai François M.
Published: February 2016

Hysteria is an ancient word for a common clinical condition. Although it no longer appears in official diagnostic classifications, “hysteria” is used here as a generic term to cover both “somatoform” and “dissociative” disorders as these are related psychopathological states. This paper reviews the clinical features of four hysterical syndromes known to occur in a neurologist’s practice, viz conversion, somatization and pain disorders, and psychogenic amnesia. The presence in the clinical history of a multiplicity of symptoms, prodromal stress, a “model” for the symptom(s), and secondary reinforcement all suggest the diagnosis, and minimise the need for extensive investigations to rule out organic disease. Psychodynamic, behavioral, psychophysiologic and genetic factors have been proffered to explain etiology. Appropriate treatment involves psychotherapeutic, behavioral and pharmacological techniques. A basic requirement is to avoid errors of commission such as multiple specialist referrals and invasive diagnostic and treatment procedures. Hysteria is a remediable condition if identified early and managed appropriately.
Psychogenic Dystonia: a Review of 18 Cases
Authors: Lang Anthony E.
Published: February 2016

Objective:

To review the clinical characteristics and associated features found in patients with psychogenic dystonia.

Methods:

A 10 year retrospective chart review of all patients diagnosed by the author as having psychogenic dystonia.

Results:

Eighteen patients fulfilled diagnostic criteria for “Documented” or “Clinically Established” psychogenic dystonia. Clinical characteristics of the dystonia were inconsistent or incongruous with established forms of organic dystonia. Fourteen of the 18 patients had a known precipitant. In most, the onset was abrupt and progression occurred rapidly, often to fixed dystonic postures. In contrast to idiopathic dystonia, involvement of the legs was common (12 patients), despite onset in adult life. Although cases of isolated paroxysmal dystonia were excluded in the review, 10 patients had paroxysmal worsening of dystonia or other abnormal movements. Pain was a prominent feature in 14 of 16 patients with the complaint and 1 patient with documented psychogenic dystonia also had well established reflex sympathetic dystrophy (RSD). Other psychogenic movement disorders, psychogenic neurological signs and multiple somatizations were common. Long-term follow up was available for less than one-half of the patients. Outcome varied considerably; some patients had complete resolution of symptoms (including 1 who had undergone 2 previous thalamotomies) and others remained disabled by persistent dystonia.

Conclusions:

Dystonia is uncommonly due to primary psychological factors. At times this is an extremely difficult diagnosis to make and even when the diagnosis is confirmed, management remains very challenging. Future studies are required in hopes of providing more efficient means of distinguishing psychogenic dystonia from other dystonic syndromes especially those which rarely follow peripheral injury or accompany RSD/causalgia syndromes.
Acute and Chronic Cerebral White Matter Damage in Neonatal Hydrocephalus
Authors: Del Bigio Marc R. , da Silva Marcia C. , Drake James M. , Tuor Ursula I.
Published: February 2016

The neonatal cat model of kaolin-induced hydrocephalus is associated with progressive and severe ventriculomegaly. In this experiment we studied the evolution of the histopathological changes in hydrocephalic (n = 23) cats from 5–168 days after the induction of hydrocephalus along with age-matched controls (n = 10). In the periventricular white matter, extracellular edema and axonal damage were present within days of the onset of hydrocephalus. This was followed by reactive gliosis, white matter atrophy, and in some animals gross cavitation of the white matter. Even in the chronic, apparently compensated state there was ongoing glial cell death. Six cats were shunted an average of 23.6 ± 6.5 days after the induction of hydrocephalus because they were no longer able to feed independently. In spite of clinical improvement the white matter changes persisted. Overt cortical changes were minimal except where areas of white matter destruction encroached upon the deep layers. The white matter changes are very similar to those seen in periventricular leukomalacia and suggest that ischemia plays a role in neonatal brain injury caused by hydrocephalus.
Proliferating Cell Nuclear Antigen Expression in the Survival of Astrocytoma Patients
Authors: Ang L.C. , Plewes M. , Tan L. , Begley H. , Agranovich A. , Shul D.
Published: February 2016

The PC 10, a monoclonal antibody against proliferating cell nuclear antigen (PCNA), is known to show immunoreactivity in routinely processed paraffin embedded tissue. This antibody was applied to 72 astrocytic tumours from surgical biopsy material obtained in a ten year period. The PCNA labelling index (LI) obtained by image analysis was compared with patient’s survival, age at diagnosis, and Karnofsky score as well as the histological grade of tumour. The survival analysis shows that patients with tumour PCNA LI of more than 6% have significantly poorer prognosis than those with 6% and below. In addition, there is also good correlation between PCNA LI with age, Karnofsky and tumour grade. This study suggests that although the PCNA expression of astrocytoma could be a useful predictor of patient’s outcome, it is not an independent prognostic factor but has derived its statistical association with survival secondarily through its relationship with tumour grade, age and Karnofsky score.
Frameless Stereotaxy for Pre-treatment Planning and Post-treatment Evaluation of Radiosurgery
Authors: Schwartz M.L. , Ramami R. , O’Brien P.F. , Young C.S. , Davey P. , Hudoba P.
Published: February 2016

In our centre, 111 patients have been treated with linear accelerator stereotactic radiosurgery. Angiographic, CT and MRI images are generated and the target coordinates calculated in 3 dimensions. For CT scanning, cross sections of perpendicular and oblique fiducial markers are seen. For follow-up CT scans done without the frame, a virtual frame is generated by means of a computer program that places fiducial markers on each CT scan cut, as if the patient had been wearing the OBT frame and the scan produced with the gantry parallel to the base of the frame. The position of the oblique marker may be calculated by knowing the thickness and position of each CT cut. Various natural fiducial markers (bony landmarks) are identified by coordinates in the scan with the patient wearing the real frame and in the scan with the virtual frame applied. A transformation matrix is utilized to establish the equivalence between the original CT scan with the real frame applied and subsequent scans without the real frame but with the virtual frame applied. In effect, the virtual frame is re-applied in exactly the same position as the real frame. Lesion measurements may then be duplicated and growth or regression accurately established. The uncertainty in this system of re-application resides in possible patient movement, CT scan slice thickness and inter-observer error in the identification of natural fiducial markers.
Anterior Interosseous Neuropathy in the Postpartum Period
Authors: Carmant Lionel , Veilleux Martin
Published: February 2016

The anterior interosseous neuropathy is a rare focal neuropathy with typical clinical and electromyographic features. Most commonly reported etiologies include lesion of the median nerve following fracture of the radius and ulna, acute or repeated trauma or prolonged pressure on the forearm. In some cases, no predisposing factors can be elicited. Over a one-year period, two young women in their late twenties were evaluated for weakness of the flexor pollicis longus, flexor digitorum profundus of the 2nd and 3rd fingers, and pronator quadratus muscles that occurred within a month following parturition. Nerve conduction studies and concentric needle electrode examination of the upper extremities performed respectively 3 and 10 months after the onset of symptoms confirmed a severe anterior interosseous neuropathy and excluded more common conditions such as carpal tunnel syndrome, cervical radiculopathy or brachial plexopathy. The prognosis was unfavorable in both cases.
Glutamate Dehydrogenase Deficiency in Machado-Joseph Disease
Authors: Gonçalves A. , Oliveira C. , Ferro M.A. , Dinis M. , Cunha L.
Published: February 2016

We studied the activity of glutamate dehydrogenase (GDH) in leukocytes from 23 patients with domi-nantly inherited ataxia. All the patients were assessed with a rating scale for ataxias and met the clinical criteria for the diagnosis of Machado-Joseph disease. The mean age of onset of symptoms was 37.8, SD 13.4 years and the duration of the disease was 7.4, SD 4.9. Leukocyte GDH activity was significantly decreased (p < 0.001) when compared to 20 normal controls. These data extend previous reports indicating that a GDH deficiency is present in peripheral tissues from some patients with spinocerebellar degenerations. Furthermore, this study suggests that a genetic deficiency of GDH may underlie some forms of dominant ataxias; this deficiency may be marked in patients with Machado-Joseph disease and is not specific for any type of multiple system atrophy.
Cardioembolic Caudate Infarction as a Cause of Hemichorea in Lupus Anticoagulant Syndrome
Authors: Kirk Andrew , Harding Sheila Rutledge
Published: February 2016

An association exists between antiphospholipid antibodies and chorea. As these antibodies are associated with thrombosis, it has been suggested that cerebral infarction might cause chorea. However, CT and MRI typically do not demonstrate focal basal ganglionic lesions in such patients and an autoimmune mechanism for chorea has also been proposed. We report a young woman with left hemichorea and dyspnea. She was found to have lupus anticoagulant, large aortic and tricuspid vegetations, and pulmonary emboli. CT and MRI showed a small lesion in the head of the right caudate. In the presence of a definite cardiac source for emboli (valvular vegetations) with embolic activity (pulmonary emboli), it is likely that this patient's hemichorea was caused by cardioembolic caudate infarction.
Nuclear Magnetic Resonance Study of Cerebrospinal Fluid From Patients With Multiple Sclerosis
Authors: Joanna Lynch , James Peeling , Anthony Auty , Garnette R. Sutherland
Published: February 2016

Proton nuclear magnetic resonance (NMR) spectroscopy was used to examine cerebrospinal fluid (CSF) from patients (n = 30) with actively progressive multiple sclerosis (MS). Metabolite concentrations obtained from the spectra were compared to those determined from the spectra of CSF from control patients (n = 27) with benign spinal disorders. No significant difference was found between the 2 groups for most constituents, including lactate, glutamine, citrate, creatine and creatinine, and glucose. Acetate levels were significantly higher in MS patients, while formate levels were significantly lower, than the controls. There were no significant differences in metabolite concentrations in CSF from early and longstanding MS patients. A peak due to an unidentified compound was found at 2.82 ppm in the spectra of CSF from patients with actively progressive MS, but not in the spectra of CSF from the controls. The peak was not found in spectra of CSF from patients with AIDS dementia complex (n = 9) or Parkinson's disease (n = 5), but it did appear in spectra of CSF from 1 patient with Jakob-Creutzfeldt disease (out of 3 examined) and from 1 patient (out of 7) with Guillan-Barré disease. The unidentified compound is volatile and, from the chemical shift of the observed NMR peak, is probably an N-methyl compound. As such, it may be an intermediate in the cholino-glycine cycle, in which an abnormality has been proposed to exist in MS patients.
Contact Thermography in the Diagnosis of Childhood Migraine
Authors: Jörg R. Wolstein , Martin H. Reed , Shashi S. Seshia , Kubrakovich P. , Linsey B. , Samuel A.
Published: February 2016

The objective of our prospective study was to assess the role of contact thermography in children with migraine. Contact thermograms were done in 54 children aged 4.2 - 16.5 years (median 10.5 years), who were seen for headache and on 10 age-matched controls, between July and December 1991. Thermograms were interpreted as definitely normal, equivocally normal, equivocally abnormal, and definitely abnormal by a radiologist who was blinded to clinical information. Forty-eight children had the test between headaches; of these, four out of 26 patients (15%) who had migraine without aura and 3 out of 14 children (21%) who had migraine with aura had definitely abnormal thermograms. Nine out of 10 normal controls had definitely normal thermograms. The sensitivity of contact thermography in the diagnosis of childhood migraine, when done between headaches, was low in our study.
Inflammatory Myopathy and Walker-Warburg Syndrome: Etiologic Implications
Authors: Michael I. Shevell , Bernard Rosenblatt , Kenneth Silver
Published: February 2016

Walker-Warburg syndrome is a well delineated clinical entity with characteristic brain and eye anomalies. Recent diagnostic surveys have revealed that muscular dystrophy is an obligatory feature of this syndome. We report a patient with an inflammatory myopathy that preceded dystrophic changes. While reports of parental consanguinity and multiple affected sibships strongly suggest an autosomal recessive genetic basis for this syndrome, previous pathological analyses of the CNS have suggested an inflammatory process. Our case supports both the notion of an aberrant inflammatory process that is likely under genetic control or etiologic heterogeneity (phenocopies) underlying this syndrome.
Concerning Mind
Authors: Miller Fisher C.
Published: February 2016

The nature of the human mind is addressed from a neurological viewpoint. First from reported observations on subjects born blind with congenital cataract, who acquired vision after surgery, the concept of the primacy of somatosensation is developed. Therefrom some principles of the organization of higher sensory functions in the dominant parietal, occipital and temporal lobes are deduced. Sentience is traced to somatosensation. Some characteristics of the mind and of thinking are described. The non-existence of the unconscious is inferred. The question of free will is approached. Using neurological experience it is demonstrated that a good case for operation of a free will is difficull to establish. Positing the absence of a free will may be beneficial in the analysis and management of behavior. Finally the opportunity to study the nature and mechanism of the emotions in clinical neurology is outlined.
Cervical Myelopathy Secondary to Ossification of the Posterior Longitudinal Ligament in a Caucasian Patient
Authors: Falah B. Maroun , Akira P. Makino , Tommy R.S. Tong , Philip G. Perkins , Rudolph Arts , Jacob C. Jacob , R. Reddy
Published: February 2016

Cervical myelopathy consequent on ossification of posterior longitudinal ligament (OPLL) is very rare in Caucasians. A 65-year-old Anglo-Saxon woman developed progressive gait disturbance, paresthesia in both legs and urinary urge incontinence. Radiological examination showed OPLL from fifth to seventh cervical vertebral level; the dense OPLL was graphically displayed by three-dimensional computerized tomography. Medial corpectomy, C5 to C7, and removal of OPLL, with subsequent fusion C4 to Tl using a free fibula graft resulted in clinical improvement. Three dimensional computerized tomographic imaging is a valuable diagnostic procedure in OPLL.
Organization of Excitatory Inputs from the Cerebral Cortex to the Cerebellar Dentate Nucleus
Authors: Yoshikazu Shinoda , Yuriko Sugiuchi , Takahiro Futami
Published: February 2016

Intracellular recording was made from dentate nucleus neurons (DNNs) in anesthetized cats, to investigate cerebral inputs to DNNs and their responsible pathways. Stimulation of the medial portion of the contralateral pericruciate cortex most effectively produced EPSPs followed by long-lasting IPSPs in DNNs. Stimulation of the pontine nucleus (PN), the nucleus reticularis tegmenti pontis (NRTP) and the inferior olive (IO) produced monosynaptic EPSPs and polysynaptic IPSPs in DNNs. The results indicate that the excitatory input from the cerebral cortex to DNNs is at least partly relayed via the PN, the NRTP and the 10. Intraaxonal injection of HRP visualized the morphology of mossy fibers from the PN to the DN and the cerebellar cortex. The functional significance of the excitatory inputs from the PN and the NRTP to the DN is discussed in relation to the motor control mechanisms of the cerebellum.
Paraneoplastic Cerebellar Degeneration
Authors: Jerome B. Posner
Published: February 2016

Paraneoplastic cerebellar degeneration is a rare complication of a number of cancers, particularly small cell lung cancer, gynecologic cancers and Hodgkin’s disease. The disorder is clinically characterized by rapid development of pancerebellar dysfunction, which usually does not improve, and pathologically characterized by loss of Purkinje cells with or without inflammatory infiltrates. In some but not all patients, an autoantibody that reacts with the tumor and Purkinje cells can be found in the serum and spinal fluid of patients with paraneoplastic cerebellar degeneration. The presence of the autoantibody suggests, but does not prove, that the disorder has an autoimmune mechanism for its pathogenesis.
A Segmental Chronic Pain Syndrome in Rats Associated with Intrathecal Infusion of NMDA: evidence for selective action in the dorsal horn
Authors: Zochodne Douglas W. , Murray Marilyn , Nag Sukriti , Riopelle Richard J.
Published: February 2016

We explored the effects of chronic lumbar intrathecal NMDA infusion (mini-osmotic pumps) in Sprague-Dawley rats on motor and sensory axon integrity. Several different infusion protocols, each given over a 4 week period were examined: 0.15 M NMDA in phosphate buffered saline; phosphate buffered saline without NMDA; and 0.20 M magnesium sulfate plus 0.15 M NMDA; 0.35 M NMDA. In two additional protocols, 0.15 M NMDA or phosphate buffered saline were infused for a total of 8 weeks. Within 1-2 weeks of the onset of NMDA, but not phosphate buffered saline infusions, the rats exhibited irritability, circling, biting and excessive grooming resulting in loss of hair, and skin ulcerations from autotomy localized to lumbar and sacral innervated dermatomes. Co-infusion of NMDA with magnesium sulfate almost completely prevented these findings. The behavioural changes were not associated with abnormalities of sensory or motor conduction. Intrathecal infusion of NMDA induces a chronic “central” experimental pain disorder in rats, localized to the cord segment with the greatest exposure to the infusion, without involvement of peripheral sensory axons and sparing the axonal integrity of anterior horn cells.
Influence of Bolus Volume on Hyoid Movements in Normal Individuals and Patients with Parkinson's Disease
Authors: Wintzen Axel R. , Badrising Umesh A. , Roos Raymund A.C. , Vielvoye Jan , Liauw Lishya
Published: February 2016

The swallowing movements of 22 ambulant patients with Parkinson's disease were examined by videofluoroscopy, 15 spouses serving as controls. The relation between bolus volume and movement of the hyoid bone was studied in the lateral view to assess the adaptation of the pharynx musculature. Although movement characteristics of the hyoid varied considerably both intra- and interindividually, mean values followed typical patterns. In controls, the position of the hyoid before the start of the swallow tended to be lower as the bolus volume increased, apparently to create space in the oral cavity by lowering the floor of the mouth. This trend was not seen in the group of Parkinson patients. We suppose that this specific lack of adaptation represents hypokinesia. The amplitude of the hyoid movement did not change with increasing bolus volumes, either in patients or in controls. Moreover, Parkinson patients had many more aborted swallowing movements (“hesitations”) than controls, probably representing defective switching from the voluntary phase of swallowing to the involuntary, automatic phase.