A rare case of Creutzfeldt-Jakob disease

Dr. Saumya is reporting a rare case of Creutzfeldt-Jakob disease. Share your views on the case & learn new things Follow us for more such updates!

76 Likes

LikeAnswersShare

A VERY RARE CASE OF Creutzfeldt-Jakob DISEASE IS VERY WELL HANDLED BY @Dr. Saumya Mittal WITH ELABORATIVE PRESENTATION ABOUT INTRODUCTION, CASE PROFILE & HISTORY, EXAMINATION FINDINGS, INVESTIGATIONS, DIFFERENTIAL DIAGNOSIS, MANAGEMENT & KEY LEARNINGS. THANKS FOR SHARING.

Thanks @Dr. Vijay Kumar Singh sir
0

Congratulations! Your case has been selected as Case of the day and you have been awarded 5 points for sharing the case. Keep posting your interesting cases, Happy Curofying!

Eating beef and beef products contaminated with the infectious agent of bovine spongiform encephalopathy (BSE) is the main cause of VCJD where as Classical CJD has no such connection . The incidence is 1 to 2 per million a rare disease . 1. The CJD has a very long history causing diffuse cortical neuronal atrophy manifesting as dementia . Here the history is just 3 months only. 2 .The family history is negative here . Affected parents have 50%chance of transmission of CJD . So it should be non genetic non varient type of CJD. 3. Diffuse atrophy of brain is common but here there is a band of cortical atrophy in MRIThe 4 .short history is suggestive of a Vasular dementia., than chronic CjD . 5. What is the clinching diagnostic feature to call it CJD , in this case presented , Sir ?

First and foremost there should be vascular events noted at least on mri to call it vascular dementia. No such changes. MRI has cortical ribboning, Typical to CJD. Cortical atrophy in band? Can you extend a DD and mechanism for that Dr @Asv Prasad ? Just because family history isn't present doesn't rule out CJD. It's called an index case. And every long history has a starting point. Typically infact CJD is a DD of rapidly progressive dementia. Criteria for Sporadic CJD- Definite: Diagnosed by standard neuropathological techniques; and/or immunocytochemically; and/or Western blot confirmed protease-resistant PrP; and /or presence of scrapie-associated fibrils.Probable:Neuropsychiatric disorder plus positive RT-QuIC in cerebrospinal fluid (CSF) or other tissues OR Rapidly progressive dementia; and at least two out of the following four clinical features:MyoclonusVisual or cerebellar signsPyramidal/extrapyramidal signsAkinetic mutism AND a positive result on at least one of the following laboratory tests a typical EEG (periodic sharp wave complexes) during an illness of any durationa positive 14-3-3 CSF assay in patients with a disease duration of less than 2 yearsHigh signal in caudate/putamen on magnetic resonance imaging (MRI) brain scan or at least two cortical regions (temporal, parietal, occipital) either on diffusion-weighted imaging (DWI) or fluid attenuated inversion recovery (FLAIR) AND without routine investigations indicating an alternative diagnosis. Possible:Progressive dementia; and at least two out of the following four clinical features:MyoclonusVisual or cerebellar signsPyramidal/extrapyramidal signsAkinetic mutism AND the absence of a positive result for any of the four tests above that would classify a case as “probable” AND duration of illness less than two years AND without routine investigations indicating an alternative diagnosis.
1

View 1 other reply

Informative

Very informative and knowledge boosting educational presentation on Creutzfeldt -Jakob disease. Causes, symptoms, Case Profile & History, Vitals, Neurologic Examinations, Investigations, Differential Diagnosis and Managements are very informative. Thanks Dr Saumya H Mittal Sir sharing very informative and useful presentation with us.

Thanks @Narendra Kumar
1

View 7 other replies

Informative post

Excellent @Dr. Saumya Mittal 👍

Thanks@Vijay Kumar Singh
0

Helpful

Dr Saumya H.Mittal - Consulting neurologist have presented a rare case of Creutzfeldt - Jakob disease- a progressive & fatal disease with no treatment. But at least we think of how we can prevent transmission How can people avoid spreading the disease. To reduce the already very low risk of CJD transmission from one person to another, people should never donate blood, tissues, or organs if they have suspected or confirmed CJD, or if they are at increased risk because of a family history of the disease, a dura mater graft, or other factor. Normal sterilization procedures such as cooking, washing, and boiling do not destroy prions.  Although there is no evidence that caregivers, healthcare workers, and those who prepare bodies for funerals and cremation have increased risk of prion diseases when compared to general population, they should take the following precautions when they are working with a person with CJD: Cover cuts and abrasions with waterproof dressings. Wear surgical gloves when handling the person’s tissues and fluids or dressing any wounds. Avoid cutting or sticking themselves with instruments contaminated by the person’s blood or other tissues. Use disposable bedclothes and other cloth for contact with the person.If disposable materials are not available, regular cloth should be soaked in undiluted chlorine bleach for an hour or more, and then washed in a normal fashion after each use. Use face protection if there is a risk of splashing contaminated material such as blood or cerebrospinal fluid. Soak instruments that have come in contact with the person in undiluted chlorine bleach for an hour or more, then use an autoclave (pressure cooker) to sterilize them in distilled water for at least one hour . Congratulations- Sir for enlightening us Thanks

Nice input @Parveen Yograj . However all these apply to iatrogenic CJD. With the now common sterilization procedures, common to most standard hospitals, the risk of such transmission is minimal, if at all
0

View 2 other replies

Informative

Load more answers

Cases that would interest you