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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

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

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

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

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