This article discusses Creutzfeldt-Jakob disease (CJD), a rare and fatal neurological disorder caused by misfolded prion proteins in the brain. It describes the clinical presentation of CJD, which includes rapidly progressive dementia, personality changes, involuntary movements, and death usually within 6 months of symptom onset. The article outlines different types of CJD, discusses the pathogenesis involving prion transmission and brain degeneration, and summarizes diagnostic studies and management approaches, which are largely supportive since there is no cure. Nursing care focuses on preventing injury, promoting comfort, and providing psychological support to patients and families.

1. Asian J. Nursing Edu. and Research 5(3): July- September 2015
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ISSN 2231-1149 (Print) www.anvpublication.org
2349-2996 (Online)
REVIEW ARTICLE
Creutzfedldt- Jakob Disease (CJD)
Mrs. Neethu Jose
Assistant Professor, Jubilee Mission College of Nursing, Thrissur, Kerala
*Corresponding Author Email: agnesfeb9@gmail.com
ABSTRACT:
Background: Creutzfeldt Jakob Disease (CJD) is a degenerative neurological disorder that is incurable and
invariably fatal. CJD is also called a human form of mad cow disease. CJD caused by an agent called a prion.
Objective: To describe the features and complications of CJD and discuss the current management
Methods: Detailed history, physical examination and laboratory investigations.
Conclusion: This report highlights the importance of detailed family history and physical examination in the
diagnosis of Creutzfeldt Jakob Disease. In addition, it gives a good insight about the pathogenesis and the
clinical presentation of Creutzfeldt Jakob Disease.
.
KEYWORDS: Creutzfeldt Jakob Disease, CJD
INTRODUCTION:
Creutzfeldt Jakob Disease (CJD) is a degenerative
neurological disorder that is incurable and invariably
fatal. CJD is also called a human form of mad cow
disease ( bovine spongiform encephalopathy/BSE)1
. CJD
was described by a German neurologist Hans Gerhard
Creutzfeldt and shortly afterwards Alfons Maria Jakob2
.
CJD is a rare rapidly developing form of dementia
caused by infectious proteins called prions. Prions are
proteins that occur naturally in the brain and are
normally harmless. In CJD Prions are misfolded proteins
that replicate by converting their properly folded
counterparts, in their host, to the same misfolded
structure they possess. The disease leads to rapid
neurodegeneration, causing brain tissue to develop holes
and take a more sponge like structure.CJD is occurring
one out of every one million people every year with in
the age group of 45-75 yr, in which variant CJD is the
common one2
.
TYPES:2
Variant CJD:
Caused by the consumption of food contaminated with
prions, which also cause BSE.
Received on 20.02.2015 Modified on 14.03.2015
Accepted on 24.04.2015 © A&V Publication all right reserved
Asian J. Nur. Edu. and Research 5(3): July- Sept.2015; Page156-161
DOI:
Sporadic CJD:
This accounts for 85% cases of CJD
Familial CJD:
This accounts the majority of the other 15% cases of
CJD
Iatrogenic CJD:
This form of CJD arises from contamination with tissue
from an infected person, usually as a result of a medical
procedure. Eg: blood transfusion from infected person,
use of human derived pituitary growth hormones,
gonadotrophin hormone therapy, cornea/ meningeal
transplants. People can also acquire CJD genetically
through mutation of gene that codes for the prion
protein. This type account 5-10%.
CASE REPORT
Mrs. Pushpam 72yr old female , was admitted to neuro-
medical ward at Jubilee Mission Medical College and
Research Institute, Thrissur as she was having blurring
of vision, decreased hearing and slurring of speech since
6weeks.nodding of head, generalized weakness ,
increased sleep and decreased bowel movements since 2
days.
On admission blood pressure was 140/80mmof Hg, the
pulse was 88beats per minute. The patient had a
temperature of 98.60
F with a respiratory rate of 20
breaths per minute and saturation was100% with GRBS
of 275mg/dl. On examination she was conscious and

2. Asian J. Nursing Edu. and Research 5(3): July- September 2015
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oriented muscle strength 5/5 in left upper and lower
extremity, with decreased muscle strength on right side
as she had an episode of CVA 6 years back. Reflexes
were diminished and muscle tone was absent.
Abdominal examination shows no organomegally and
soft abdomen.
Patient was previously admitted to JMMC with blurring
of vision, hyponatremia before one month. She was a
known case of CAD, CVA and DLP since 6years,
diabetic since 15 years and on treatment
She had undergone MRI scanning, results shows small
vessel ischemic changes in white matter.CSF analysis
shows no pus cells and bacteria. Lactate 62.17mg/dl,CSF
protein 0.89mg%, SGOT 59U/L ammonia 40-Umol/L,
HDL 34mg/dl, sodium 134meq/Land Hb 10.5%
According to the patient her mother had Diabetes
Mellitus. Mrs. Pushpam is married and having six
children. No history of any other medical surgical illness
in her family. As the symptoms suggesting CJD, the
patient and family members were not ready to accept the
diagnosis. As CJD was incurable the patient was on
symptomatic treatment.
DISCUSSION:
Pathophysiology and Etiology:2
Prions which lacks a nucleic acid that enables them to
withstand conventional means of sterilization. The
prions cross Blood Brain Barrier and deposited in brain
tissue and causes degeneration of brain tissue. Cell death
occurs, and spongiform changes are produced in brain
and they are surrounded by amyloid plaque.
Clinical Presentation
Typically patients with Creutzfeldt Jakob Disease
present with
 rapidly progressive dementia, leading top memory
loss, personality change and hallucination
 anxiety, psychosis
 paranoia, Depression, Obsessive-compulsive
symptoms
 accompanied by physical problems such as speech
impairment, jerky movement(myoclonus)
 balance and co-ordination dysfunction(ataxia)
 changes in gait, rigid posture and seizure
In some people symptom can continue for years. In most
patients these symptoms are followed by involuntary
movements and the appearance of an atypical diagnostic
EEG tracing most victims die 6months after initial
symptoms appear, often of pneumonia due to impaired
coughing reflex. Symptoms of CJD are caused by
progressive death of brain’s nerve cells, which is
associated with the buildup of abnormal prion proteins
forming amyloids. This patient presented with most of
the mentioned symptoms.
Diagnostic Studies:1,2
 Detailed medical history
 Computed Tomography
 EEG, shows a characteristic triphasic spikes
 Optical fibre immunoassay, specific antibodies
against prpsc
 MRI
 Lumbar puncture and CSF analysis shows 14-3-
3protein (Protien Kinase Inhibitor) indicates neuronal
cell death
 Blood test , to detect mutation in the person’s genes
and determines the genetic form of CJD
Management:
The major goal in management of patients with
Creutzfeldt Jakob Disease is prevention of injury related
to immobility and dementia, promotion of patient
comfort, supportive and palliative care, and provision of
support and education for the family. While there is no
cure for the disorder, careful management can improve
the prognosis and lengthen the life span. The treatment
focus on alleviation of symptom
Nursing Consideration:1
 Psychological and emotional support of patient and
family through the course of illness
 Prevention of disease transmission
 Use of standard precaution
 Destroy the equipments/ use disposable one and
should be incinerated, because conventional methods of
sterilization does not destroy prion
Complications:2
 Infection(pneumonia)
 Heart failure
 Respiratory failure
CONCLUSION:
CJD is rare rapidly developing form of dementia caused
by infectious proteins called prions. Prions are proteins
that occur naturally in the brain and are normally
harmless.CJD is transmitted through contaminated
harvested human brain products, immunoglobulin,
corneal graft, dural graft or electrode implant and
familial history. Prions may not be deactivated by means
of routine surgical instrument sterilization. The WHO
and United States Centre for Disease Control and
prevention recommends immediate destroy of
equipments after use.
REFERENCE:
1. Hikle J L, Kerry H Cheever, “Brunner and Suddarth’s Textbook
of medical surgical Nursing”, Volume2, 13th
edition
2. http//www.phac-aspc.gc.ca/hcai.iumss/pdf/cjd_prion