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Gabreyes A.A.,Royal Infirmary | Abbasi H.N.,Institute of Neurological science | Forbes K.P.,Institute of Neurological science | Mcquaker G.,Royal Infirmary | And 2 more authors.
European Journal of Haematology | Year: 2013

Copper is an essential trace element that is involved in a number of important enzymatic processes throughout the body. Recent single case reports and small studies have shown that deficiency of copper can cause reversible haematological changes and irreversible neurological injury. We chose to undertake a national study, looking at all cases of copper deficiency in Scotland over a 5-yr period using information from a national reference laboratory. From 16 identified patients, we determined that 86% had both haematological and neurological features of copper deficiency, while 18% had haematological features only at presentation. Twelve of the sixteen patients had high serum zinc concentrations (>18 μm/L) nine patients were using zinc-containing dental fixatives at time of diagnosis. 94% of patients had haematological features as an initial manifestation of copper deficiency, which included anaemia, thrombocytopenia and neutropenia. Patients who underwent later bone marrow testing had appearances in keeping with refractory cytopenia with multilineage dysplasia, refractory anaemia with excess of blasts, unclassified marrow dysplasia or probable myelodysplasia (MDS). 75% of patients had neurological symptoms or signs, including progressive walking difficulties and paraesthesia, or gait difficulties without sensory signs. Clinical examination was in keeping with spastic paraparesis (either with or without sensory neuropathy). Magnetic resonance imaging (MRI) showed multifocal T2 hyper intense foci in the subcortical white matter, and atrophy of the cerebrum and cerebellum was also seen on computerised tomography (CT). MRI of the spinal cord showed signal change in the dorsal columns in either the cervical or thoracic cord. 93% of cytopenias responded to copper replacement and addressing the original cause of the copper deficiency, but only 25% of patients had improvement in their neurological function, while 33% deteriorated and 42% remained unchanged. Our study demonstrates that copper deficiency is an under-recognised cause of several types of cytopenia, which are reversible but can progress to significant neurological injury if left untreated. We illustrate the importance of identifying these patients early to prevent irreversible neurological injury. © 2012 John Wiley & Sons A/S.


Morrison I.,Institute of Neurological science | Buskova J.,Charles University | Nevsimalova S.,Charles University | Douglas N.J.,Royal Infirmary | Riha R.L.,Royal Infirmary
European Journal of Neurology | Year: 2011

Background and purpose: The second version of the International Classification of Sleep Disorders suggests narcolepsy with cataplexy can be diagnosed on history alone. Patients: Five patients with a history supportive of narcolepsy/cataplexy. Method: Case review following clinical investigation. Results: None of the five patients had a diagnosis of narcolepsy/cataplexy on the basis of objective testing using polysomnography (PSG) and multiple sleep latency testing (MSLT). Conclusion: PSG and MSLT should always be used in conjunction with a comprehensive history taken by an experienced sleep physician to support a diagnosis of narcolepsy with cataplexy and to exclude other conditions that may mimic narcolepsy. © 2010 The Author(s). European Journal of Neurology © 2010 EFNS.


Hunter M.A.,Royal Alexandra Hospital | Santosh C.,Institute of Neurological science | Teasdale E.,Institute of Neurological science | Forbes K.P.,Institute of Neurological science
American Journal of Neuroradiology | Year: 2012

We performed high-resolution DIR-BBI of the cervical arteries at 3T in 19 subjects with cervical dissection. It offered excellent visualization of both the lumen and arterial wall, allowing detection of the primary and secondary features of dissection. We suggest that this is a highly useful technique for diagnosis of cervical dissection, either routinely or in equivocal cases of suspected dissection. It also offers further insight into the pathogenesis of this disorder.


Condon B.,Institute of Neurological science
EPMA Journal | Year: 2011

MRI/MRS can produce information on over 40 physico-chemical parameters regarded as biomarkers of structural, functional or metabolic significance. Though of undisputed worth in the detection of macroscopic lesions or of metabolic derangements, MRI's use in prognosis and prediction has not been so extensively studied. Serial studies can be performed to show early pre-clinical changes in biomarkers caused by disease progression or therapy, such as the adverse effect on heart function of certain cancer therapies. It can utilise various haemodynamic measures to predict the evolution of stroke and so help justify certain interventions. Changes in cerebral metabolite concentrations or the volumes of brain sub-structures can be used as objective measures of drug response in psychiatric conditions. However care must be exercised as MR can sometimes be considered 'too sensitive' as it often detects real abnormalities even in asymptomatic volunteers, the actual predictive significance of which have yet to be fully assessed. © 2011 European Association for Predictive, Preventive and Personalised Medicine.


Shaw M.,Institute of Neurological science
Acta neurochirurgica. Supplement | Year: 2012

Cerebral pressure autoregulation (AR) is a process by which blood flow is kept constant over a specific cerebral perfusion pressure (CPP) range. There have been a number of advances in recent years in the monitoring and modelling of this physiological variable; however, there has been very little work done on the comparison or optimisation of some of the existing models in clinical use today: pressure reactivity index, highest modal frequency techniques and compartmental modelling. Presented here is a methodology for the comparison and optimisation results for these main AR models. By simple mathematical manipulation of the original modelling end points each model can be converted into a form that is directly comparable to the others. Using a standardised data set with known gold standard AR status indications, the models can then be readily assessed. As a consequence each of the models can then be optimised to maximise specificity and sensitivity.


Teo M.,Institute of Neurological Science | St George E.J.,Institute of Neurological Science
World Neurosurgery | Year: 2016

Background The management of untreated unruptured intracranial aneurysms remains controversial. The natural history is still not well understood and many patients are not routinely followed up. We present a single surgeon's data on radiologic surveillance of these lesions. Methods A total of 94 patients with 152 unruptured intracranial aneurysms, with a mean follow-up time of 3.4 years from the time of diagnosis, underwent surveillance using computer tomogram angiography, magnetic resonance angiography, or digital subtraction angiography. Aneurysm growth was defined as an increase in ≥1 dimensions above the measurement error. Statistical analysis was performed. Results Of 152 aneurysms, 126 (83%) were <7 mm, 25 (16%) were 7-12 mm, and 1 aneurysm was 13-24 mm. Eighteen of 152 (12%) cerebral aneurysms in 17 patients grew larger; 7% (9/126) of the aneurysms were <7 mm and 36% (9/25) of the aneurysms were 7-12 mm enlarged. Spontaneous aneurysmal rupture occurred in 4 of 152 aneurysms (2.6%) (i.e., 4/94 patients [4%]), with an average initial aneurysm size of 5.7 mm. The risk of aneurysm rupture per patient-year was 5% with growth, 0.2% without growth, and there was a 24-fold increase in aneurysmal rupture risk for growing aneurysm (P = 0.005). Of aneurysms in group 1 patients (no previous subarachnoid hemorrhage [SAH]) 15% (16/109) compared with 5% (2/43) of group 2 (previous SAH) aneurysms showed evidence of growth during the study period (P = 0.0424). Conclusions These results support imaging follow-up of patients with untreated unruptured intracranial aneurysms, including those with aneurysms smaller than the current treatment threshold of 7 mm. Apart from the initial size, aneurysm growth is associated with an increased risk of SAH and therefore growing aneurysms warrant treatment. The data also demonstrated that incidental aneurysms, in patients without previous SAH, do not behave less aggressively, contrary to current opinion. © 2016 Elsevier Inc. All rights reserved.


Teo M.,Institute of Neurological Science | Bhattacharya J.,Institute of Neurological Science | Suttner N.,Institute of Neurological Science
British Journal of Neurosurgery | Year: 2012

A 53-year-old hypertensive female smoker presented with a WFNS grade II subarachnoid haemorrhage. Angiography showed a left persistent hypoglossal artery with multiple intracranial aneurysms. She underwent successful coil embolisation of the ruptured aneurysm. We reviewed the literature on persistent hypoglossal artery and found 26% of them have associated intracranial aneurysms. This controversial topic is discussed. © 2012 The Neurosurgical Foundation.


Grosset D.,Institute of Neurological science
Journal of the Neurological Sciences | Year: 2010

The response to dopamine replacement therapy in patients with degenerative parkinsonism is variable. Reasons for a poor therapy response include the type of parkinsonism, comorbidities, and differential effects on clinical features. An additional explanation, which has received much less attention, is sub-optimal therapy compliance. Single and multicentre studies of therapy compliance report significant under- and overuse of dopamine replacement therapy resulting in poor symptomatic control, or features of the dopamine dysregulation syndrome or other signs of dopaminergic excess (dyskinesia, confusion, visual hallucinations). In this article, the evidence for sub-optical adherence in Parkinson's disease (PD) is reviewed, and factors associated with sub-optimal compliance were examined, with two case vignettes to illustrate clinical consequences of deviation from the prescribed therapy regimen. © 2009.


Vijayaraghavan L.,Institute of Neurological science | Natarajan S.,Institute of Neurological science | Krishnamoorthy E.S.,Institute of Neurological science
Behavioural Neurology | Year: 2011

Disturbances in cognitive function, particularly memory, are a common complaint of patients with epilepsy. Factors contributing to cognitive dysfunction are the type of epilepsy, type and frequency of seizures, anti-epileptic drugs and the location of underlying brain lesions. Whilst a great deal of attention has been paid to permanent cognitive impairment, the nature and underlying mechanisms of ictal and peri-ictal cognitive changes are poorly understood. In-depth investigation of seizure related cognitive dysfunction is of great clinical relevance, as these changes are potentially reversible and treatable, thus reducing the cumulative effect of frequent seizures Greater knowledge of peri-ictal and ictal cognitive dysfunction would improve seizure prediction, localization of seizure focus and assessment of treatment effectiveness, greatly reducing distress and disability. This paper will review current understanding of peri-ictal and ictal cognitive dysfunction and discuss future directions for research.


Handique S.K.,Institute of Neurological science
Neuroimaging Clinics of North America | Year: 2011

Viral infections of the central nervous system in the tropical countries of Asia and the Indian subcontinent are different from those of the Western and developed world. Many of the endemic and epidemic encephalitides that are prevalent in these regions, such as Japanese encephalitis, have characteristic findings on imaging, especially on magnetic resonance imaging, allowing a rapid diagnosis and differentiation from clinically similar syndromes. Other emerging viral infections in the region in recent years have posed new challenges. The contribution of neuroimaging to the management of these emerging infections is also discussed. © 2011 Elsevier Inc.

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