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.

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 98.6⁰F with a respiratory rate of 20 breaths per minute and saturation was100% with GRBS of 275mg/dl. On examination she was conscious and 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 prp^sc

· 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, 13^th edition

2. http//www.phac-aspc.gc.ca/hcai.iumss/pdf/cjd_prion

Received on 07.03.2015 Modified on 23.03.2015

Asian J. Nur. Edu. and Research 5(3): July- Sept.2015; Page 429-430

DOI: 10.5958/2349-2996.2015.00085.3