We report the case of a 70-year-old man who developed probable unilateral Creutzfeldt-Jakob disease. Clinically, he presented with right hemiparesis, progressive aphasia, temporospatial disorientation and cerebellar ataxia and later on, myoclonia. The MRI showed a hypersignal from the left caudate in DWI with decreased ADC. Repeated electroencephalograms showed a slow background rhythm in the left hemisphere with superimposed periodic, biphasic and triphasic sharp-wave complexes in the left temporal region. Death occurred after 5weeks. Although exceptional, unilateral Creutzfeldt-Jakob disease was retained as possible.
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http://dx.doi.org/10.1016/j.neurol.2012.07.019 | DOI Listing |
J Neurol
February 2023
Neurology Department, Hospital Ruber Internacional, Madrid, Spain.
Neuropsychiatr Dis Treat
November 2021
Department of Ophthalmology, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, People's Republic of China.
Visual snow syndrome (VSS) is a clinical disorder characterized by pan-field visual disturbance. It is a diagnosis of exclusion since its pathophysiology remains unknown. Excluding other mimics is of great significance since some serious pathologies can have secondary visual snow (VS) as an initial presentation.
View Article and Find Full Text PDFNeurophysiol Clin
June 2021
Department of Neurology, The Sagol Neuroscience Center, and Chaim Sheba Medical Center, 52621, Israel; Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv 6997801, Israel.
Objectives: Electroencephalogram (EEG) pattern in Creutzfeldt-Jakob disease (CJD) is characterized by diffuse abnormal activity, although lateralization to one hemisphere has been described in the first stages of the disease. This study aimed to determine whether abnormal EEG activity predominantly occurs in anterior versus posterior brain regions.
Methods: As part of a prospective study, the demographics, clinical features and MRI findings of genetic E200K CJD patients were collected.
Int Med Case Rep J
February 2021
Department of Medicine, Aga Khan University Hospital, Aga Khan University Medical College of East Africa, Nairobi, Kenya.
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare prion disease that causes rapidly progressive fatal neurodegeneration. The rarer Heidenhain variant of sCJD presents with visual symptoms and is rarely reported in the literature from sub-Saharan Africa. We report the case of a 57-year-old male with a three-week history of losing direction when driving home and visual hallucinations described as seeing rainbows.
View Article and Find Full Text PDFCreutzfeldt-Jakob disease typically presents as rapidly progressive dementia. We describe the case of a 59-year-old male patient presenting with sudden onset of central facial palsy and dysarthria, followed by myoclonus of his left upper and lower limbs. Initial brain magnetic resonance showed hyperintensity of the right caudate and putamen on diffusion-weighted imaging and T2 sequences.
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