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Absence of susceptibility vessel sign and hyperdense vessel sign in patients with cancer-related stroke
ConclusionThe absence of SVS but not HVS could help to identify paraneoplastic hypercoagulability in stroke patients with active cancer and guide patient care.
Source: Frontiers in Neurology - March 20, 2023 Category: Neurology Source Type: research

Case report: Acute vestibular syndrome and cerebellitis in anti-Yo paraneoplastic syndrome
We report three patients evaluated over the past decade with an acute AVS along with subtle downbeat nystagmus (DBN), followed by dysarthria and progressive truncal and limb ataxia, as well as increasing DBN intensity.MethodsAll patients underwent neurologic examination, video-oculography, MRI, serum cancer markers, spinal fluid examination, paraneoplastic panel testing, and oncologic workup. With a consolidated diagnosis of cancer/paraneoplastic syndrome, we treated with plasma exchange (PLEX), high-dose steroids, surgery, and oncologic investigation. We additionally provided oncotherapy in one out of three patients.Resul...
Source: Frontiers in Neurology - August 26, 2022 Category: Neurology Source Type: research

A Novel Variant in Non-coding Region of GJB1 Is Associated With X-Linked Charcot-Marie-Tooth Disease Type 1 and Transient CNS Symptoms
Conclusion A novel GJB1 variant of c.-170T>G in non-coding region was found in this big Chinese CMTX1 pedigree. This is the first report of variant in non-coding DNA sequence associated with transient CNS symptoms. Thyroid malfunction may contribute to the CNS symptoms in this case. Ethics Statement This study has been reviewed and approved by the Ethics Committee of the China-Japan Union Hospital of Jilin University. Each member of the family provided written informed consent to the participation in the study, the genetic test, and authorized to publish the study including the photos in accordance with the Decl...
Source: Frontiers in Neurology - April 23, 2019 Category: Neurology Source Type: research

Fatal Atypical Progressive Multifocal Leukoencephalopathy (PML) in an African American Female with Sarcoidosis Associated Lymphopenia (SAL). A Rare Case Report and Literature Review. (P2.317)
Conclusions:This is the first case of SAL presenting with medullary PML in an African-American patient not on immunosuppressive therapy. The role of SAL in PML pathogenesis or Sarcoidosis Associated Immunodeficiency is unclear. No guidelines for CD4/CD8 monitoring are available and needs further investigation. We suggest an aggressive diagnostic approach in evaluating Sarcoidosis with atypical neurologic manifestations and neuroimaging to reduce morbidity-mortality.Study Supported by: Not applicable.Disclosure: Dr. Rathore has nothing to disclose. Dr. Sweiss has nothing to disclose. Dr. Campanella has nothing to disclose. ...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Rathore, J., Sweiss, N., Campanella, F., Andreoni, J., Wichter, M. Tags: HIV, HTLV-I, PML, Other Viral Infections, and CJD Source Type: research

Paraneoplastic Progressive Vasculopathy as the Presenting Manifestation of Polyneuropathy, Organomegaly, Endocrinopathy or Edema, M-Spike, and Skin Manifestations: A Case Report
We report a patient with ischemic stroke due to an underlying vasculopathy as the initial presentation of POEMS. This case highlights the importance of recognizing ischemic stroke as a presenting sign for POEMS as early diagnosis and treatment in patients with POEMS may prevent progression of multisystem involvement and may potentially stabilize the vasculopathy associated with it.
Source: Journal of Stroke and Cerebrovascular Diseases - May 9, 2016 Category: Neurology Authors: Shadi Yaghi, Mitchell S.V. Elkind Tags: Case Studies Source Type: research

The Evaluation and Management of Alternative Diagnoses in Amyotrophic Lateral Sclerosis (P5.091)
Conclusions: Treatment with intravenous steroids, immunoglobulin and surgical interventions prior to diagnosis of ALS is associated with a significantly prolonged mean ODI.Disclosure: Dr. Kannan has nothing to disclose. Dr. Glass has nothing to disclose.
Source: Neurology - April 3, 2016 Category: Neurology Authors: Kannan, M., Glass, J. Tags: Neuroepidemiology: Neuro-oncology and ALS Source Type: research

Limbic Encephalitis Exacerbations and Remissions Charted by Microglial PET Scan: A Case Study (P4.104)
CONCLUSIONS: C-11-PK11195 PET imaging can be useful in the diagnosis of limbic encephalitis and for monitoring response to treatment. MALT lymphoma may also be associated with autoimmune or paraneoplastic encephalitis, especially in individuals with other predisposing factors to autoimmune disorders, such as Tourette’s and autoimmune thyroid disease. The reduction in microglial activity with both IVIG and anti-psychotics supports previous findings that anti-psychotics may act through anti-inflammatory pathways.Disclosure: Dr. Shatz has received research support from Janssen Pharmaceutica. Dr. Chugani has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Shatz, R., Chugani, H. Tags: Aging, Dementia, Cognitive, and Behavioral Neurology: Rapidly Progressive and Inflammatory Dementias Source Type: research

Limbic encephalitis: Clinical spectrum and long-term outcome from a developing country perspective
Conclusion: Early recognition of LE is important based upon clinical, MRI data in the absence of antineuronal surface antibody screen in developing nations. Early institution of immunotherapy will help in improvement in outcome of these patients in long-term.
Source: Annals of Indian Academy of Neurology - May 17, 2014 Category: Neurology Authors: Sujit Abajirao JagtapGopal Krishna DasHarsha J. KambaleAshalatha RadhakrishnanM.D. Nair Source Type: research

Progressive limbic encephalopathy: Problems and prospects
Conclusion: All patients who present with new onset neuropsychiatric symptoms need to be evaluated for sub-acute infections, inflammation, autoimmune limbic encephalitis and paraneoplastic syndrome. A repeated 20 minute EEG is a very effective screening tool to detect organicity.
Source: Annals of Indian Academy of Neurology - May 17, 2014 Category: Neurology Authors: Sadanandavalli Retnaswami ChandraRoopa SeshadriYasha ChikabasaviahThomas Gregor Issac 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

N-type calcium channel antibody-mediated paraneoplastic limbic encephalitis: A diagnostic challenge
Conclusion: This case highlights limbic encephalitis as an atypical presentation of neuroendocrine cancer. It also illustrates how treatment of the underlying cancer can reverse limbic encephalitis and Lambert–Eaton myasthenic syndrome in a neuroendocrine carcinoma patient even before the paraneoplastic panel becomes negative.
Source: Journal of the Neurological Sciences - January 27, 2014 Category: Neurology Authors: Carlos Kamiya-Matsuoka, David Blas-Boria, Michelle D. Williams, Pedro Garciarena, Sudhakar Tummala, Ivo W. Tremont-Lukats Tags: Original Articles Source Type: research