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Total 27 results found since Jan 2013.

164 Dementia, a Familial Affair
ConclusionThis case highlights the importance of genetics shaping phenotypes and that consideration should always be given to a full relevant family history.  It also shows a rare case of rapidly progressive dementia confirmed due to CJD with a likely underlying familial predisposition.
Source: Age and Ageing - September 16, 2019 Category: Geriatrics Source Type: research

Effects of Neurotrophic Factors in Glial Cells in the Central Nervous System: Expression and Properties in Neurodegeneration and Injury
Conclusion and Future Aspects This review summarizes available NTF expression data, compiles existing evidence on the effects of glial NTF signaling in healthy conditions and in disease models (Figure 1), and highlights the importance of this topic for future studies. The relationship between NTFs and glia is crucial for both the developing and adult brain. While some of these factors, such as NT-3 and CNTF, have highly potent effects on gliogenesis, others like BDNF and GDNF, are important for glia-mediated synapse formation. Neurotrophic factors play significant roles during neurodegenerative disorders. In many cases, ...
Source: Frontiers in Physiology - April 26, 2019 Category: Physiology Source Type: research

Renewed assessment of the risk of emergent advanced cell therapies to transmit neuroproteinopathies
AbstractThe inadvertent transmission of long incubating, untreatable and fatal neurodegenerative prionopathies, notably iatrogenic Creutzfeldt –Jakob disease, following transplantation of cadaver-derived corneas, pituitary growth, hormones and dura mater, constitutes a historical precedent which has underpinned the application of precautionary principles to modern day advanced cell therapies. To date these have been reflected by geograph ic or medical history risk-based deferral of tissue donors. Emergent understanding of other prion-like proteinopathies, their potential independence from prions as a transmissible agent ...
Source: Acta Neuropathologica - November 27, 2018 Category: Neurology Source Type: research

Crossed cerebellar diaschisis in Creutzfeldt-Jakob disease evaluated through single photon emission computed tomography
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of human prion disease; it is characterized as a transmissible spongiform encephalopathy. Previously, only two case reports have discussed crossed cerebellar diaschisis (CCD) and sCJD. Herein, we detected CCD in five patients with sCJD and four patients with genetic CJD through SPECT. The combined data of the nine patients further supported CCD when analyzed by 3D-SSP. CCD can occur in several disorders, including, stroke, epilepsy, and encephalitis.
Source: Journal of the Neurological Sciences - September 24, 2018 Category: Neurology Authors: Kenya Nishioka, Michimasa Suzuki, Katsuya Satoh, Nobutaka Hattori Tags: Letter to the Editor Source Type: research

Rapidly Progressing Sporadic Creutzfeldt-Jakob Disease Presenting as a Stroke
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, fatal human prion disease that is characterized by progressive dementia and neurologic degeneration. It can mimic multiple other neurological disorders, and a high index of clinical suspicion is necessary to make a diagnosis. A 74-year-old woman with a 3-month history of a stroke and progressive neurologic deterioration was found to have sCJD. She expired within a week of her diagnosis. Autopsy revealed spongiform encephalopathy consistent with prion disease, and genetic analysis revealed 129 polymorphism and no pathologic mutation, confirming the diagnosis of nonfamilia...
Source: Case Reports in Neurology - September 17, 2018 Category: Neurology Source Type: research

Rectal administration of baclofen at the end of life
Spasticity can be a complicating symptom of a variety of neurological conditions such as multiple sclerosis, motor neuron disease, Creutzfeldt-Jakob disease, or post-stroke. Several pharmacological treatment options are available, including baclofen, tizanidine, gabapentin, botulinum toxin A and tetrahydrocannabinol/canabidiol (1, 2). However, treatment feasibility decreases as the end of life approaches, e.g. when patients are no longer able to tolerate oral medications, the onset of action would be too long, or the initiation of parenteral drug therapy (intravenous, intrathecal) is no longer indicated or appropriate.
Source: Journal of Pain and Symptom Management - August 7, 2018 Category: Palliative Care Authors: Charlotte Selge, Claudia Bausewein, Constanze Remi Source Type: research

Operationalization of the Transition to Comfort Measures Only in the Neurocritical Care Unit: A Quality Improvement Project.
CONCLUSIONS: Implementation of a multidisciplinary huddle in the neuro-intensive care unit before transition to CMO may improve clinician's experience of the end-of-life process through enhanced nursing inclusion and involvement and organized communication with the neurocritical care team. PMID: 30041532 [PubMed - as supplied by publisher]
Source: The American Journal of Hospice and Palliative Care - July 24, 2018 Category: Palliative Care Authors: Lele A, Cheever C, Healey L, Hurley K, Kim LJ, Creutzfeldt CJ Tags: Am J Hosp Palliat Care Source Type: research

Rapid progressive visual decline and visual field defects in two patients with the Heidenhain variant of Creutzfeld-Jakob disease
Conclusions Loss of visual acuity and a decline in visual fields without corresponding MRI findings and marked changes in behaviour should lead to a diagnosis of HvCJD. Corresponding diagnostic tests should be performed for confirmation. The prognosis for survival is poor and should be immediately communicated to affected patients and their relatives.
Source: Journal of Clinical Neuroscience - February 21, 2018 Category: Neuroscience Source Type: research

P 164 Parietal stroke mimicking the Heidenhain variant of Creutzfeldt-Jakob Disease
The Heidenhain variant of Creutzfeldt-Jakob disease (CJD) is a rapidly progressive neurodegenerative disease with an occipito-parietal focus.It initially presents with visual disturbances such as visual agnosia or cortical blindness (Kropp et al., 1999).After presentation of early visual symptoms, rapid progression of dementia and death follows.While the Heidenhain variant matches neuropathological criteria of CJD, differential diagnosis from other neurological disorders using biomarkers from electroencephalography (EEG), magnetic resonance imaging (MRI), and cerebrospinal fluid (CSF) reveal low sensitivity and specificity...
Source: Clinical Neurophysiology - September 8, 2017 Category: Neuroscience Authors: D. Becker, D. Kramer, W. M üllges, K. Boelmans Tags: Poster Source Type: research

Palliative Care for Hospitalized Patients With Stroke Clinical Sciences
This study explores patterns of palliative care utilization and death in hospitalized patients with stroke across the United States.Methods—Using the 2010 to 2012 nationwide inpatient sample databases, we included all patients discharged with stroke identified by International Classification of Diseases-Ninth Revision codes. Strokes were subclassified as ischemic, intracerebral, and subarachnoid hemorrhage. We compared demographics, comorbidities, procedures, and outcomes between patients with and without a palliative care encounter (PCE) as defined by the International Classification of Diseases-Ninth Revision code V66....
Source: Stroke - August 28, 2017 Category: Neurology Authors: Tarvinder Singh, Steven R. Peters, David L. Tirschwell, Claire J. Creutzfeldt Tags: Race and Ethnicity, Quality and Outcomes, Cerebrovascular Disease/Stroke, Intracranial Hemorrhage, Ischemic Stroke Original Contributions Source Type: research

Two older drugs could be 'repurposed' to fight dementia
Conclusion This early stage experimental research has demonstrated a beneficial neurological effect of trazodone and dibenzoylmethane on mice with diseases mimicking neurodegenerative diseases. It is important to acknowledge that this is animal research and therefore the drugs might not have the same effect when they are trialled on humans. That being said, trazodone is already an approved drug for depression and sleep problems and has therefore already passed safety tests. If the mechanisms of neurodegeneration in humans and mice are similar, it is possible trazodone could be used in the future in treating Alzheimer's and...
Source: NHS News Feed - April 20, 2017 Category: Consumer Health News Tags: Older people Neurology Medication Source Type: news

Elevated cerebrospinal fluid total Tau levels reflects predominant cortical involvement in Alzheimers disease (P1.093)
Conclusions:Elevated CSF Tau levels in AD patients could be surrogate biomarker for AD subtypes with predominant early cortical symptoms and atrophy. There is a need for a systematic prospective unbiased follow-up to see if rapid clinical progression in AD is related to elevated T-Tau and distinct AD subtypes.Disclosure: Dr. Pillai has nothing to disclose. Dr. Khrestian has nothing to disclose. Dr. Bekris has nothing to disclose. Dr. Safar has nothing to disclose. Dr. Leverenz has received personal compensation for activities with Axovant, GE Healthcare and Navidea Biopharmaceuticals as a consultant.
Source: Neurology - April 17, 2017 Category: Neurology Authors: Pillai, J., Khrestian, M., Bekris, L., Safar, J., Leverenz, J. Tags: Aging and Dementia: Biomarkers Source Type: research

Creutzfeldt-Jakob Disease Presenting as Stroke: A Case Report and Systematic Literature Review
Conclusions: CJD develops primarily within a population in whom stroke risk factors are common and represents a rare but important stroke mimic. Negative vascular imaging in elderly patients with apparent acute stroke syndromes should prompt diagnostic review including consideration of prion diseases.
Source: The Neurologist - March 1, 2017 Category: Neurology Tags: Review Article Source Type: research

Sporadic Creutzfeldt-Jakob Disease Presenting as Non-Convulsive Status Epilepticus: A Case Report (P1.333)
CONCLUSIONS: Non-convulsive status epilepticus is an extremely rare presentation of prion disease. To date, there have only been three reported cases in the literature. This case highlights the need to consider prion disease as an etiology in NCSE and NORSE.Disclosure: Dr. Alobaidy has nothing to disclose. Dr. Parker has nothing to disclose. Dr. Bershad has nothing to disclose.
Source: Neurology - April 4, 2016 Category: Neurology Authors: Alobaidy, A., Parker, A., Bershad, E. Tags: Fungal and Other Infectious Disorders Source Type: research

A toxic byproduct of hemoglobin could provide treatments for Creutzfeldt-Jakob disease
Scientists at Case Western Reserve University School of Medicine have identified a novel mechanism that could be used to protect the brain from damage due to stroke and a variety of...
Source: Parkinson's Disease News From Medical News Today - March 8, 2016 Category: Neurology Tags: Stroke Source Type: news

Neurologists as primary palliative care providers: Communication and practice approaches
Purpose of review: To present current knowledge and recommendations regarding communication tasks and practice approaches for neurologists as they practice primary palliative care, including discussing serious news, managing symptoms, aligning treatment with patient preferences, introducing hospice/terminal care, and using the multiprofessional approach. Recent findings: Neurologists receive little formal palliative care training yet often need to discuss prognosis in serious illness, manage intractable symptoms in chronic progressive disease, and alleviate suffering for patients and their families. Because patients with ...
Source: Neurology Clinical Practice - February 15, 2016 Category: Neurology Authors: Creutzfeldt, C. J., Robinson, M. T., Holloway, R. G. Tags: All Clinical Neurology, Prognosis, All Cerebrovascular disease/Stroke, Palliative care Review Source Type: research

Neuron-Specific Enolase as a Biomarker: Biochemical and Clinical Aspects.
Authors: Isgrò MA, Bottoni P, Scatena R Abstract Neuron-specific enolase (NSE) is known to be a cell specific isoenzyme of the glycolytic enzyme enolase. In vertebrate organisms three isozymes of enolase, expressed by different genes, are present: enolase α is ubiquitous; enolase β is muscle-specific and enolase γ is neuron-specific. The expression of NSE, which occurs as γγ- and αγ-dimer, is a late event in neural differentiation, thus making it a useful index of neural maturation.NSE is a highly specific marker for neurons and peripheral neuroendocrine cells. As a result of the findings of NSE in specific...
Source: Advances in Experimental Medicine and Biology - November 6, 2015 Category: Research Tags: Adv Exp Med Biol Source Type: research

Palliative Care: A Core Competency for Stroke Neurologists Topical Reviews
Source: Stroke - August 24, 2015 Category: Neurology Authors: Creutzfeldt, C. J., Holloway, R. G., Curtis, J. R. Tags: Other Ethics and Policy, Emergency treatment of Stroke, Other Stroke Treatment - Medical Topical Reviews Source Type: research

Rethinking of doxycycline therapy in Creutzfeldt-Jakob disease
Compassionate use of doxycycline, a tetracycline antibiotic, in patients with Creutzfeldt-Jakob disease (CJD) revealed an increased survival of 4–7 months as compared with historical controls, a result not confirmed by a randomised, double blind, placebo-controlled trial.1 Is then therapy with doxycycline for patients with CJD over? The report of Assar et al2 on a single patient with variably protease-sensitive prionopathy (VPSPr),3 a rare subtype form of sporadic CJD, who received 4-year treatment with doxycycline at a relatively early stage of disease, suggests it is not and encourages novel studies on the use...
Source: Journal of Neurology, Neurosurgery and Psychiatry - June 12, 2015 Category: Neurosurgery Authors: Pocchiari, M., Ladogana, A. Tags: Dementia, Infection (neurology), Sleep disorders (neurology), Stroke, Variant Creutzfeld-Jakob Disease, Child and adolescent psychiatry, Memory disorders (psychiatry), Sleep disorders Editorial commentaries Source Type: research

Paradoxical herniation after decompressive craniectomy provoked by lumbar puncture or ventriculoperitoneal shunting.
CONCLUSIONS Lumbar puncture and ventriculoperitoneal shunting carry substantial risk when performed in a patient after decompressive craniectomy and before cranioplasty. When the condition that prompts decompression (such as brain swelling associated with stroke or trauma) requires time to resolve, risk is associated with lumbar puncture performed ≥ 1 month after decompressive craniectomy. PMID: 26067613 [PubMed - as supplied by publisher]
Source: Journal of Neurosurgery - June 12, 2015 Category: Neurosurgery Authors: Creutzfeldt CJ, Vilela MD, Longstreth WT Tags: J Neurosurg Source Type: research

Creutzfeldt-Jakob Disease Presenting as Stroke: Case Report and Literature Review (P1.022)
CONCLUSIONS CJD develops primarily within a population in whom stroke risk factors are common and represents a rare but important stroke mimic. Negative sensitive imaging in elderly patients with apparent acute vascular syndromes should prompt diagnostic review including consideration of prion diseases.Disclosure: Dr. Van Heuven has nothing to disclose. Dr. Boggild has nothing to disclose. Dr. White has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Van Heuven, A., Boggild, M., White, R. Tags: Cerebrovascular Disease and Interventional Neurology: Case Reports Source Type: research

An Unusual Stroke-like Clinical Presentation of Creutzfeldt-Jakob Disease: Acute Vestibular Syndrome
Conclusions: To our knowledge, this is the first report of an initial presentation of Creutzfeldt-Jacob disease closely mimicking vestibular neuritis, expanding the known clinical spectrum of prion disease presentations. Despite the initial absence of neurological signs, the central lesion location was differentiated from a benign peripheral vestibulopathy at the first visit using simple bedside vestibular tests. Familiarity with these tests could help providers prevent initial misdiagnosis of important central disorders in patients presenting vertigo or dizziness.
Source: The Neurologist - April 1, 2015 Category: Neurology Tags: Case Report/Case Series Source Type: research

Creutzfeldt-Jakob Disease
(CJD) information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
Source: NINDS Disorders: National Institute of Neurological Disorders and Stroke - October 26, 2014 Category: Neurology Source Type: research

Periodic Lateralized Epileptiform Discharges (PLEDs) in Patients With Neurosyphilis and HIV Infection.
Abstract Periodic lateralized epileptiform discharges (PLEDs) are an electroencephalographic pattern recorded in the setting of a variety of brain abnormalities. It is best recognized for its association with acute viral encephalitis, stroke, tumor, or latestatus epilepticus. However, there are other conditions that have been recognized as the underlying pathology for PLEDs such as alcohol withdrawal, Creutzfeldt-Jacob disease, anoxic brain injury, and hemiplegic migraine. However, there are only rare case reports of PLEDs in patients with neurosyphilis. Here, we report 2 patients presenting with encephalopathy an...
Source: Clinical EEG and Neuroscience - October 17, 2014 Category: Neuroscience Authors: Takagaki K, Morales MK, Vitantonio D, Berkowitz F, Bell WL, Kumar PN, Motamedi GK Tags: Clin EEG Neurosci Source Type: research

Seizures after craniectomy: an under-recognised complication?
Decompressive craniectomy for ischaemic stroke is being increasingly used, at least in the USA.1 This has likely been stimulated by three randomised clinical trials reporting improved survival in appropriately selected patients.2 As the number of patients so treated, and surviving, increases, a better picture of the complications and outcomes is emerging, with recognition of the relatively high risk of infection when the cranium is reconstructed after the craniectomy, as well as the occurrence of postoperative extra-axial haemorrhages and delayed hydrocephalus.3 Creutzfeldt et al4 highlight another surprisingly frequent se...
Source: Journal of Neurology, Neurosurgery and Psychiatry - June 10, 2014 Category: Neurosurgery Authors: Macdonald, R. L. Tags: Epilepsy and seizures, Hydrocephalus, Neurooncology, Stroke, CNS cancer, Drugs: psychiatry, Drugs: musculoskeletal and joint diseases, Surgical oncology Editorial commentaries Source Type: research

Seizures after decompressive hemicraniectomy for ischaemic stroke
Conclusions In this case series, patients were at high risk of developing seizures after malignant MCA stroke with DHC, especially after cranioplasty. Assuming these findings are replicated, means should be sought to reduce the occurrence of this complication.
Source: Journal of Neurology, Neurosurgery and Psychiatry - June 10, 2014 Category: Neurosurgery Authors: Creutzfeldt, C. J., Tirschwell, D. L., Kim, L. J., Schubert, G. B., Longstreth, W. T., Becker, K. J. Tags: Epilepsy and seizures, Stroke Cerebrovascular disease Source Type: research

Palliative and End-of-Life Care in Stroke: A Statement for Healthcare Professionals From the American Heart Association/American Stroke Association AHA/ASA Scientific Statement
Conclusions— Addressing the palliative care needs of patients and families throughout the course of illness can complement existing practices and improve the quality of life of stroke patients, their families, and their care providers. There is an urgent need for further research in this area.
Source: Stroke - May 27, 2014 Category: Neurology Authors: Holloway, R. G., Arnold, R. M., Creutzfeldt, C. J., Lewis, E. F., Lutz, B. J., McCann, R. M., Rabinstein, A. A., Saposnik, G., Sheth, K. N., Zahuranec, D. B., Zipfel, G. J., Zorowitz, R. D., on behalf of the American Heart Association Stroke Council, Coun Tags: AHA Statements and Guidelines AHA/ASA Scientific Statement Source Type: research