[The correlation between the electroencephalogram findings and clinical manifestations of Creutzfeldt-Jakob disease].

Zhonghua Yi Xue Za Zhi

Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100730, China.

Published: September 2003

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.

Download full-text PDF

Source

Publication Analysis

Top Keywords

akinetic mutism
20
frontal intermittent
12
intermittent rhythmic
12
findings clinical
8
eeg findings
8
onset myoclonus
8
myoclonus akinetic
8
firda frontal
8
rhythmic triphasic
8
triphasic slow
8

Similar Publications

A predictive model for cerebellar mutism syndrome based on lesion map in children with medulloblastoma.

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.

View Article and Find Full Text PDF
Article Synopsis
  • - Creutzfeldt-Jakob disease (CJD) is a rare and fatal brain disorder that typically affects people in their 60s and manifests as rapid cognitive decline due to the accumulation of abnormal prion proteins, leading to death within a year of diagnosis.
  • - Diagnosis of CJD is complicated by overlapping symptoms with other degenerative and infectious diseases, requiring a comprehensive approach that includes clinical evaluations and advanced imaging techniques.
  • - A case study of a 60-year-old man with probable sporadic CJD highlights symptoms such as dementia and rigidity, as well as MRI findings that indicate severe neurodegeneration, demonstrating the need for early identification for palliative care, since there is no effective treatment.
View Article and Find Full Text PDF

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 PDF

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.

View Article and Find Full Text PDF

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.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!