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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

Corticobasal syndrome associated with antiphospholipid syndrome without cerebral infarction
Antiphospholipid syndrome (APS) is a rare autoimmune coagulopathy with various systemic and neurologic symptoms. Ischemic stroke and TIA are common neurologic manifestations of APS, while migraine, epilepsy, chorea, and multiple sclerosis–like syndromes have also been reported.1,2 Dementia is an unusual manifestation of APS, and the presentation of APS with corticobasal syndrome (CBS) has rarely been reported.3,4 CBS is a progressive neurologic syndrome characterized by asymmetric cortical dysfunction, such as apraxia, alien limb phenomenon, or cortical sensory changes accompanied by akineto-rigid syndrome or dystoni...
Source: Neurology - February 24, 2014 Category: Neurology Authors: Lee, D.-W., Eum, S.-W., Moon, C. O., Ma, H.-I., Kim, Y. J. Tags: All Medical/Systemic disease, Corticobasal degeneration CLINICAL/SCIENTIFIC NOTES Source Type: research

Voltage Gated Calcium Channels Antibody-Associated Autoimmune Encephalitis (P5.228)
ConclusionThis case demonstrates a correlation between a high titer of VGCC antibodies and autoimmune encephalitis. Our patient's initial improvement with steroids and plasma exchange in the face of a negative CT and PET suggests a primary autoimmune process rather than a paraneoplastic etiology, though we admit that it is premature to rule out remission and we will continue to follow herDisclosure: Dr. Alwaki has nothing to disclose. Dr. Lugo has nothing to disclose. Dr. Goshgarian has nothing to disclose. Dr. Ahmad has nothing to disclose. Dr. Hefzy has nothing to disclose. Dr. Mitsias has nothing to disclose.
Source: Neurology - April 9, 2014 Category: Neurology Authors: Alwaki, A., Lugo, A., Goshgarian, C., Ahmad, O., Hefzy, H., Mitsias, P. Tags: Aging, Dementia, and Cognitive and Behavioral Neurology: Creutzfeldt-Jakob Disease and Subacute Encephalopathies 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

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

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

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

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

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 3, 2016 Category: Neurology Authors: Alobaidy, A., Parker, A., Bershad, E. Tags: Fungal and Other Infectious Disorders 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

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

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

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

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