Objective: To investigate the correlation between the EEG findings and the clinical signs of Creutzfeldt-Jakob disease (CJD).
Methods: Serial standard recording during different stage of disease was conducted among 13 patients with CJD, 10 males and 3 females, with the year of onset of 58.2 (49 approximately 65). The EEG findings were analyzed in combination of the clinical symptoms and signs.
Results: 12 of the 13 patients showed typical periodic sharp wave complexes (PSWCs), occurring after the onset of myoclonus and akinetic mutism in 8 patients, both after myoconus and before akinetic mutism in 2 cases, and just at the onset of akinetic mutism before or after the onset of myoclonus respectively in 2 cases. Different EEG features were observed in different stages of disease. Frontal intermittent rhythmic delta activity (FIRDA) and frontal intermittent rhythmic triphasic slow wave, which was replaced by PSWC later, were seen in 4 cases.
Conclusion: PSWC has a close association with myoclonus and akinetic mutism. The best time of EEG recording is when myoclonus and/or akinetic mutism happen. FIRDA and frontal intermittent rhythmic triphasic slow wave may be hint of the onset of PSWC.
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Eur Radiol
December 2024
Department of Neurosurgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China.
Background: This study aimed to establish a voxel-based map to predict the occurrence of cerebellar mutism syndrome (CMS) and investigate the relationship between CMS and motor dysfunction.
Method: This multicenter study cohort included 224 patients diagnosed with medulloblastoma at Beijing Children's Hospital (n = 88) and Beijing Tiantan Hospital (n = 136). The dataset was randomly divided into training (n = 95), test (n = 41), and validation (n = 88) datasets.
J Assoc Physicians India
November 2024
Associate Professor, Department of General Medicine, Medical College Baroda, Vadodara, Gujarat, India.
J Med Invest
October 2024
Department of Neurosurgery, Tokyo Women's Medical University Adachi Medical Center, Tokyo, Japan.
Intermittent clinical course of akinetic mutism is very unusual. We describe a 74-year-old man who started to demonstrate episodes of altered mental state with stopped moving and talking, poor response to commands, and muscle stiffness in both upper limbs approximately 1.5 months after cardioembolic bilateral paramedian thalamic infarction.
View Article and Find Full Text PDFColomb Med (Cali)
October 2024
Hospital Nacional Edgardo Rebagliati Martins, Departamento de Neurología, Lima, Peru.
Description Of The Cases: A series of 6 cases with a probable diagnosis of sporadic CJD, treated in a Peruvian national reference hospital, are presented.
Clinical Findings: The relevant clinical signs were rapidly progressive dementia and myoclonus, followed by akinetic mutism and pyramidal signs.
Treatment And Results: Of the cases presented, 80% were men, with an average age of presentation of 65 years and duration from diagnosis to death of 6.
Neurologist
January 2025
Department of Neurology, University of Utah.
Introduction: The C9orf72 mutation can manifest in diverse clinical ways, including rapid cognitive decline, parkinsonism, or late-life neuropsychiatric symptoms, sometimes mimicking autoimmune encephalitis.
Case Report: A 64-year-old female presented to the autoimmune neurology clinic with rapidly progressive dementia (RPD) associated with episodes of headache, confusion, auditory hallucinations, and abnormal electroencephalogram. She was treated empirically at an outside hospital for possible autoimmune encephalitis with intravenous methylprednisolone, but there was no improvement, and rapid cognitive decline continued.
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