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3681. A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series. Full Text available with Trip Pro

with apathy as the dominant feature. However, many patients had language output impairment that was either a progressive non-fluent aphasia or decreased speech output consistent with a dynamic aphasia. Neurological and neuropsychological examination also suggests that parietal lobe dysfunction is a characteristic feature of GRN mutation and differentiates this group from other patients with FTLD. MR imaging showed evidence of strikingly asymmetrical atrophy with the frontal, temporal and parietal lobes

2008 Brain

3682. Phenotype variability in progranulin mutation carriers: a clinical, neuropsychological, imaging and genetic study. Full Text available with Trip Pro

with motoneuron disease (FTD-MND), primary progressive aphasia (PPA) and corticobasal degeneration syndrome (CBDS). We studied the clinical, neuropsychological and brain perfusion characteristics of mutation carriers. Eighteen mutations, seven novel were found in 24 families including 32 symptomatic mutation carriers. No copy number variation was found. The phenotypes associated with GRN mutations vary greatly: 20/32 (63%) carriers had fvFTD, the other (12/32, 37%) had clinical diagnoses of PPA, CBDS, Lewy

2008 Brain

3683. Natural course and pathogenesis of transient focal neurologic symptoms during pregnancy. Full Text available with Trip Pro

(range, 24-41) years and mean gestational age at symptom onset was 28 (range, 17-44) weeks. No controls reported transient focal neurologic symptoms, migraine aura, or headache. Presenting symptoms included dysphasia (6 patients) and hemisensory (5) and hemimotor (7) syndrome. In 4 patients, these symptoms were preceded by scintillating scotoma; in 9 patients, focal symptoms were followed by a first-ever, throbbing, migraine-like headache. Only 1 patient had evidence of frank infarction on magnetic

2008 Archives of Neurology

3684. Application of full-scale three-dimensional models in patients with rheumatoid cervical spine. Full Text available with Trip Pro

models as a template, appropriate shape of the plate-rod construct could be created in advance. No troublesome Halo-vests were needed for preoperative adjustment of occipitocervical angle. No patients complained of dysphasia following surgery. Screw entry points and trajectories were simultaneously determined with full-scale dimensions and perspective, proving particularly valuable in cases involving high-riding vertebral artery. Full-scale stereoscopic recognition has never been achieved with any

2008 European Spine Journal

3685. Long-term cognitive outcome in teenage survivors of arrhythmic cardiac arrest. (Abstract)

memory, basic auditory-visual functions, praxis and speech, short-term memory, ability to learn new verbal and visual material were assessed. These tests were repeated at about 6 months.Eight patients had an initial assessment; one boy remained in a coma and one was making simple emotional contact, revealing intensified mixed aphasia and dyskinesia. Six patients had severe disturbances of memory, motor functions and praxis. After 6 months, four patients had no neurocognitive disturbance. Four

2008 Resuscitation

3686. Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: comparison with patients with MAPT and no known mutations. Full Text available with Trip Pro

in a single patient, with no family history, who showed a mixed FTLD/MND picture and A324T change in exon 9 was found in two cases; one case of frontotemporal dementia (FTD) with a sister with FTD+MND and the other in a case of progressive non-fluent aphasia (PNFA) without any apparent family history. MAPT mutations were found in 17 (8%) patients. One patient bore exon 10 + 13 splice mutation, and 16 patients bore exon 10 + 16 splice mutation. When PGRN and MAPT mutation carriers were excluded, there were

2008 Brain

3687. The neurotoxicity and safety of treatment with cefepime in patients with renal failure. Full Text available with Trip Pro

conscience, confusion, agitation, global aphasia, myoclonus, chorea-athetosis, convulsions and coma. The latency, the period between the start of treatment and neurological deterioration, was 4,75 +/- 2,55 days (range: 1-10 days). All patients died 17 +/- 14,7 days (range: 1-42 days) after becoming symptomatic. Three of them died shortly after neurological deterioration. Five patients developed a neurological "tableau" with global aphasia. Three patients showed clinical improvement after (...) the discontinuation of cefepime. Electroencephalography revealed diffuse slow-wave activity (delta) and triphasic sharp wave activity. These findings confirm the possible neurotoxicity of treatment with cefepime in patients with renal failure. In none of the deceased patients have we been able to directly demonstrate a causal relationship between neurotoxicity and mortality. However, when a patient treated with cefepime develops neurological deterioration or aphasia, one must be aware of cefepime's potential

2008 Transplantation

3688. Risk factors for chest infection in acute stroke: a prospective cohort study. Full Text available with Trip Pro

disease, lower Abbreviated Mental Test scores, and a higher oral cavity score, and a greater proportion tested positive for bacterial cultures from oral swabs. In binary logistic-regression analysis, independent predictors (P<0.05) of pneumonia were age >65 years, dysarthria or no speech due to aphasia, a modified Rankin Scale score > or =4, an Abbreviated Mental Test score <8, and failure on the water swallow test. The presence of 2 or more of these risk factors carried 90.9% sensitivity and 75.6 (...) % specificity for the development of pneumonia.Pneumonia after stroke is associated with older age, dysarthria/no speech due to aphasia, severity of poststroke disability, cognitive impairment, and an abnormal water swallow test result. Simple assessment of these variables could be used to identify patients at high risk of developing pneumonia after stroke.

2007 Stroke

3689. Interobserver agreement for the bedside clinical assessment of suspected stroke. Full Text available with Trip Pro

. Pairs of observers independently assessed suspected stroke patients. Findings from history, neurological examination, and the diagnosis of stroke or mimic, were recorded on a standard form. Reliability was measured by the kappa statistic. We assessed the impact of observer experience and confidence, time of assessment, and patient-related factors of age, confusion, and aphasia on inter-rater reliability.Ninety-eight patients were recruited. Most items of the history and the diagnosis of stroke were (...) found to have moderate to good inter-rater reliability. There was agreement for the hour and minute of symptom onset in only 45% of cases. Observer experience and confidence improved reliability; patient-related factors of confusion and aphasia made the assessment more difficult. There was a trend for worse inter-rater reliability among patients assessed very early and very late after symptom onset.Clinicians should be aware that inter-rater reliability of the clinical assessment is affected

2006 Stroke

3690. Neurological complications of Schistosoma infection. (Abstract)

, dysphasia, visual field impairment, focal motor deficits and ataxia. Cerebral and cerebellar tumour-like neuroschistosomiasis can present with increased intracranial pressure, headache, nausea and vomiting, and seizures. Myelopathy (acute transverse myelitis and subacute myeloradiculopathy) is the most common neurological complication of Schistosoma mansoni infection. Schistosomal myelopathy tends to occur early after infection and is more likely to be symptomatic than cerebral schistosomiasis

2007 Transactions of the Royal Society of Tropical Medicine & Hygiene

3691. Prominent phenotypic variability associated with mutations in Progranulin. Full Text available with Trip Pro

and disease duration ranged from 1 to 14 years. Clinical diagnoses included frontotemporal dementia (FTD), primary progressive aphasia, FTD with parkinsonism, parkinsonism, corticobasal syndrome, Alzheimer's disease, amnestic mild cognitive impairment, and others. One kindred exhibited maximal right cerebral hemispheric atrophy in all four affected individuals, while another had maximal left hemisphere involvement in all three of the affected. Neuropathologic examination of 13 subjects revealed

2007 Neurobiology of Aging

3692. Thrombolytic therapy for acute stroke in late pregnancy with intra-arterial recombinant tissue plasminogen activator. (Abstract)

function.The patient presented with left hemiplegia, left-sided neglect, and aphasia. Right internal carotid artery cerebral angiogram showed occlusion of the mid-M1 segment of the middle cerebral artery (MCA). After 15 mg of tissue plasminogen activator was administered via intra-arterial route, there was greatly improved retrograde flow through the posterior communication artery and the MCA territory.A reduction in size of the MCA occlusion was noted with improvement of antegrade flow through the MCA

2005 Stroke

3693. Anteromedian, central, and posterolateral infarcts of the thalamus: three variant types. Full Text available with Trip Pro

, anterograde amnesia, and aphasia in left-sided or bilateral lesions. The most frequent stroke mechanism was cardiac embolism. (2) Central territory (4 patients [6%]), with lesions on the central part of the thalamus, resulting in a variety of neurological and neuropsychological signs, reflecting the involvement of several adjacent structures. Microangiopathy was the most frequent etiology. (3) Posterolateral territory (8 patients [11%]), involving inferolateral and posterior territories (...) , with hemihypesthesia as the most frequent manifestation, followed by hemiataxia, executive dysfunction, and aphasia in left-sided lesions. Artery-to-artery embolism and microangiopathy were the main stroke mechanisms.We describe 3 variant topographic patterns of thalamic infarction with distinct manifestations and etiologies. We postulate that these infarcts are the result of a variation in thalamic arterial supply or reflect borderzone ischemia.

2004 Stroke

3694. Informed consent for thrombolytic therapy for patients with acute ischemic stroke treated in routine clinical practice. Full Text available with Trip Pro

in 10 Connecticut hospitals (1996-1998). Consent was defined as any documentation of discussion about risks and benefits of tPA. Patients had adequate decision-making capacity if they were alert, oriented, and without aphasia or neglect (patient was appropriate decision-maker). Patients with any of these deficits were considered to have diminished capacity (surrogate was appropriate decision-maker).Among 63 patients who received tPA, 53 (84%) had informed consent documented; 16/53 (30%) gave

2004 Stroke

3695. Evaluating atypical dementia syndromes using positron emission tomography with carbon 11 labeled Pittsburgh Compound B. Full Text available with Trip Pro

Pittsburgh Compound B ((11)C-PiB) in patients with atypical presentations of dementia.At a tertiary referral center for memory disorders, 15 healthy controls, 10 patients with Alzheimer disease, a patient with primary progressive aphasia (PPA), and a patient with posterior cortical atrophy (PCA) underwent (11)C-PiB positron emission tomographic studies. Retention of (11)C-PiB was compared between different groups using statistical parametric mapping.The topography of cortical (11)C-PiB binding

2007 Archives of Neurology

3696. Focal cortical presentations of Alzheimer's disease. Full Text available with Trip Pro

Focal cortical presentations of Alzheimer's disease. To determine the frequency of Alzheimer's disease (AD) pathology in patients presenting with progressive focal cortical syndromes, notably posterior cortical atrophy (PCA), corticobasal syndrome (CBS), behavioural variant frontotemporal dementia (bvFTD), progressive non-fluent aphasia (PNFA) (or a mixed aphasia) and semantic dementia (SD); and to compare the age of onset, evolution and prognosis in patients with focal cortical presentations (...) with CBS (50%); 2 of 28 with bvFTD (7.1%); 12 of 26 with PNFA (44.1%); 5 of 7 with mixed aphasia (71.4%) and 2 of 20 with SD (10%). Of 20 with clinically typical AD, 19 had pathological AD. Age at both onset and death was greater in the atypical AD cases than those with non-AD pathology, although survival was equivalent. AD is a much commoner cause of focal cortical syndromes than previously recognised, particularly in PCA, PNFA and CBS, but rarely causes SD or bvFTD. The focal syndrome may remain pure

2007 Brain

3697. Clinical and radiological characteristics of methotrexate-induced acute encephalopathy in pediatric patients with cancer. Full Text available with Trip Pro

(4.5%) with bone sarcoma experienced acute encephalopathy within 2 weeks (median, 7.5 days) after receiving high-dose i.v. and/or intrathecal MTX. The signs and symptoms varied at presentation and during episodes: hemiparesis (eight patients, alternating from side to side in four), dysphasia (six), confusion/emotionality (six), headache (three), choreoathetosis (two), and seizure (two). All patients recovered after 1-7 days (median, 5.5 days). DWI revealed restricted diffusion in anatomic brain

2007 Annals of Oncology

3698. Acute intracranial subdural hematoma following a lumbar CSF leak caused by spine surgery. (Abstract)

described only twice in the literature following spine surgery.Retrospective review of the patient's medical record and head CT imaging.A 55-year-old woman underwent lumbar surgery for failed back syndrome. Intraoperatively, a dural tear was noted and repaired. One week later, she developed expressive aphasia, and CSF drainage from her lumbar wound was noted. A head CT revealed an acute intracranial subdural hematoma with mass effect. Evacuation of the hematoma occurred via craniotomy, and the lumbar

2005 Spine

3699. Post-craniotomy analgesia: current practices in British neurosurgical centres--a survey of post-craniotomy analgesic practices. (Abstract)

of centres (82%) used balanced analgesia. Pain assessments were only carried out in 57% of centres and no centre used a validated pain assessment tool specifically for dysphasic patients.Codeine phosphate continues to be the mainstay of post-craniotomy analgesia, however, it is proposed that patient controlled analgesia with morphine is an efficacious and safe alternative.

2005 European Journal of Anaesthesiology

3700. Binge eating is associated with right orbitofrontal-insular-striatal atrophy in frontotemporal dementia. Full Text available with Trip Pro

eating, while the nonovereaters were diagnosed with FTD, semantic dementia, progressive aphasia, progressive supranuclear palsy, and Alzheimer disease. VBM revealed that binge-eating patients had significantly greater atrophy in the right ventral insula, striatum, and orbitofrontal cortex.Binge eating can occur despite reported satiety and is associated with damage to a right-sided orbitofrontal-insular-striatal circuit in humans. These findings support a model in which ventral insular

2007 Neurology

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