Journal of Gerontological Nursing

CREUTZFELDT-JAKOB DISEASE: Assessment and Management

Meredith Wallace, RN, MSN

Abstract

Creutzfeldt- Jakob Disease (OD) is a rare, slowly degenerating, viral disease that attacks the central nervous system. It affects primarily late middle-aged and elderly individuals, with a peak incidence occurring between the ages of 55 and 75 (Brown, 1989). GD was originally discussed by Jakob in 1921. Similar cases were documented by Creutzfeldt in the years 1920 through 1921 (Kirschbaum, 1968).

Abstract

Creutzfeldt- Jakob Disease (OD) is a rare, slowly degenerating, viral disease that attacks the central nervous system. It affects primarily late middle-aged and elderly individuals, with a peak incidence occurring between the ages of 55 and 75 (Brown, 1989). GD was originally discussed by Jakob in 1921. Similar cases were documented by Creutzfeldt in the years 1920 through 1921 (Kirschbaum, 1968).

Symptoms of the CreutzfeldtJakob disease are often compared with those of Alzheimer's disease and consequently, the nursing care given to CJD patients is similar to the care given to patients with Alzheimer's disease. However, the etiology, symptomatology, and prognosis vary markedly from Alzheimer's disease. Subsequently, nursing care should reflect such differences.

CASE STUDY

Mrs. S., a 57-year-old Jewish woman who had fallen and hit her head on the toilet bowl, was brought to the emergency department by her adult daughter. While taking her history, information was elicited indicating that for the past 6 months, Mrs. S. had been increasingly "forgetful." Her daughter stated that she rarely went out, stayed in her bathrobe most of the day, and spent most of her time napping or aimlessly wandering around her home.

An electroencephalogram showed triphasic slow wave periodicity. A neurologic examination indicated visual difficulties, and cerebellar and extrapyramidal deficits with myoclonus and other involuntary movements. Physical examination indicated a subdural hematoma. Mrs. S. was prepared for surgery.

Following surgery, however, Mrs. S. was found to have no reduction of her neurologic symptoms. The nursing staff became concerned as Mrs. S. began to deteriorate quickly. Within days, she changed from an ambulatory and verbal - albeit confused - woman to a comatose individual whose only movement consisted of periodic jerking of the extremities.

As the weeks progressed, Mrs. S. was no longer able to feed herself, and an endogastric feeding tube was inserted. Her family was consistently at her bedside encouraging her to return to her previous disposition, but to no avail. After 2 weeks on the medical-surgical unit, Mrs. S. was moved to the hospital's gerontological holding unit, where she would remain for the short duration of her life.

Although the diagnosis was not confirmed until after the death of Mrs. S, it is now known that she suffered from a disease known as Creutzfeldt-Jakob Disease. This disease affects approximately one in one million individuals annually (Wood, 1988). It is a viral disease that affects men and women equally (Wood, 1988). The transmission of the disease is not known.

PATHOLOGY

Research has shown that 5% to 15% of cases were familial (Brown, 1989). However, it is not known whether this relationship is genetic, or if there is a culturally related common source of infection (Wood, 1988). Kuroda and colleagues (1986) postulated that individuals with CJD have an inherited susceptibility to the disease. The disease has been found to be prevalent in Jewish immigrants to Israel and Libya (Wood, 1988). A study by Masters and associates (1979) on the worldwide occurrence of CJD indicated that, in addition to Israeli and Libyan Jews, the disease was found in a rural community in Czechoslovakia. The average was 1.4 cases per million in certain areas of Italy, and a high occurrence was noted in Santiago, Chile. In the United States, five cases per million were found in Burlington, Vermont, from 1974 to 1979.

Higher incidences of CJD have been found in individuals who received corneal transplants or intracerebral electrode implants, indicating the possibility of iatrogenic transmission (Bernoulli, 1977; Duffy, 1974). In addition, an elevated number of cases were found among individuals who took growth hormone generated from human pituitary glands (Brown, 1985). Although the disease predominantly occurs in middle and late-middle aged individuals, incidences of CJD have been noted in young people who have received growth hormone generated from human pituitary glands (Wood, 1988). Davanipour and co-workers (1985) postulated that the disease may be transmitted to humans through the consumption of infected sheep and sheep products.

Davanipour and co-workers (1985) suggested through their research that tests for intraocular pressure using a tonometer may be one risk factor for the disease. Their study also indicated statistically significant associations between the use of suturing, and trauma and surgery to head and neck to the onset of symptoms of CJD.

Masters and associates (1979) found that the onset of symptoms of CJD coincided with a medical illness or surgical procedure in 38 of 337 cases they reviewed, and that symptoms occurred within 4 months following an illness or surgical procedure in 12 of the 337. Thirteen of the surgical procedures included cranial or ocular surgery. The illnesses included cardiovascular disease, and neurologic or psychiatric illnesses.

Kondo and Kuroiwa (1982) performed a similar study in Japan. They studied 60 cases of CJD to associate the onset of the disease with physical injury. No associations were found between the onset of the disease and contact with animals, ingestion of raw meat or brains from four-footed animals, 18 diseases, allergies, immunization, tooth extraction, blood transfusion, or lumbar puncture. However, 25.9% of male subjects and 24.2% of female subjects had a surgical operation within 5 years before the onset of disease symptoms.

Mechanical injuries occurring within the same time span occurred in 33.3% of male subjects and 18.2% of female subjects in the study. It is not known whether the illnesses and injuries listed caused the disease or activated the CJD virus already acquired by the individual. Both options remain open for future research.

Research has found that the CJD virus is not easily destroyed. The CJD virus has been found to remain infectious even after extensive boiling in water and after exposure to some commonly used disinfecting agents (Wood, 1988). Fortunately, it is currently theorized that the only method of transmission among humans is percutaneous contact (Brown, 1990). After contact with a CJD patient, consultation with the infection control department of the hospital should be made to obtain appropriate cleaning agents and appropriate methods for sterilizing equipment. Use of an autoclave at a temperature of 132°C to 134°C is the most commonly accepted method for cleaning infected equipment; sodium hypochlorite (bleach) at full strength and sodium hydroxide (lye) at a concentration of 80 g/L are the most effective surface decontaminants (Brown, 1990).

PATHOPHYSIOLOGY OF A SPONGIFORM ENCEPHALOPATHY

CJD; a disease known as kuru; and two animal disorders - scrapie and transmissible mink encephalopathy - are categorized as subacute spongiform viral encephalopathies (Masters, 1979). It is labeled subacute because the time span of the disease is neither short nor long (Brown, 1990). Although no virus has been isolated in a CJD patient, the disease is generally accepted as viral in nature among medical professionals (Gajdusek, 1974). Encephalopathy implies brain degeneration without inflammation (Gajdusek, 1974).

Brown (1989) stated that other hypotheses are presently being investigated, indicating that the disease may not be viral at all. These other theories include the self-replicating host protein, an infecting nucleic acid cooperating with a host protein, and a nucleating agent of protein crystallization.

The disease is termed "spongiform" (resembling a sponge) because of the physiologic effects the virus has on the tissues of the brain. Although the actual mechanism is not entirely understood, it has been noted (through postmortem examination of brain tissue) that the virus causes the neurons and glia to swell and the nucleus to displace to the side of the cell (Gajdusek, 1974). The result of the spongiform process is known as vacuolation: The contents of the neuron deteriorate; all that remain are membrane-bound vacuoles containing membranous profiles of cellular structures (Figures 1A and 1B).

Figure 1A. Normal neuron with representative structures.

Figure 1A. Normal neuron with representative structures.

Figure 1B. Neuron after vacuolation. Contents of the neuron have deteriorated; all that remain are membrane-bound vacuoles containing membranous profiles of cellular structures.

Figure 1B. Neuron after vacuolation. Contents of the neuron have deteriorated; all that remain are membrane-bound vacuoles containing membranous profiles of cellular structures.

This intracellular swelling has been described as status spongiosus (Gajdusek, 1974). The range of neuron involvement is reportedly distributed throughout the nervous system. Some cases have shown spongiform changes primarily in the cortex. Other cases, however, have shown such changes deep in nuclei of primarily the occipital, temporal, and parietal lobes of the brain, and in the cerebellum (Karp, 1990). The location of involvement has been used to further classify the disease and to assist in identifying the symptoms (Table 1).

In addition to the spongiform changes found in patients with CJD, astrocytosis has been determined to coincide with the clinical course of the disease. Astrocytes are starshaped, neuroglial cells with many branches. These cells partially compose the tissue that forms the supporting cells and fibers of the nervous system. The astrocytes become infected by the virus and proliferate, branching out and interfering with neuronal transmission.

Amyloid plaques are proteins composed of linear nonbranched, grouped fibrils, occurring in approximately 9% of CJD cases (Karp, 1990; Masters, 1979). Researchers have postulated three theories on the formation of such plaques. The first theory postulates that the plaques are formed as the circulating virus is attacked by the macrophages of the immune system (Karp, 1990). The second theory states that local, intrinsic, and physiochemical factors in the brain of the diseased patient may provoke amyloid formation and deposition (Brown, 1989; Wood, 1988).

Gajdusek (1974) postulated the third theory, which states that the scrapie-associated fibrils found in the CJD victim's brain are neurofHamentlike, atypical viruses that may interfere with the transport of neurofilaments down the axon and occasionally lead to amyloid accumulations. These amyloid plaques should not be confused with the senile plaques found in dementia. However, although now recognized as morphologically different, the plaques are similar to the amyloid plaques found in Alzheimer's disease patients.

PHYSICAL FINDINGS

The time span from onset to inevitable death from CJD is 6 to 12 months (Wood, 1988). However, there is evidence indicating that extensive changes in the brain may be present prior to the onset of symptoms (Gajdusek, 1974). The disease is generally divided into three stages (Table 2). The disease may progress from stage 1 to stage 2 in a matter of several weeks (Kirschbaum, 1968). However, the transition from stage 2 to stage 3 is more gradual, lasting from 6 to 18 months. Because of the rapid onset and duration of the disease, symptoms from all stages may occur simultaneously.

Diagnosis of CJD is dependent on a thorough neurologic examination. Karp and Mirra (1990) reported that the advance of the disease may be measured day-to-day. The mental status findings of CJD are not easily distinguishable from findings of other dementias except in their onset and duration. Brown (1989) reported that the progress of CJD dementia occurs over 4 to 7 months; Alzheimer's disease progresses over 4 to 7 years.

However, accompanied by the neurologic and specific electroencephalograph findings, the disease is easily distinguished from other dementias. The range of involvement of the central nervous system includes the pyramidal, extrapyramidal, and cerebellar systems (Wood, 1988). Hypertonicity, hyperreflexia, and spastic paralysis are likely to occur as early as stage 1, indicating upper motor neuron disease (Brown, 1989; Karp, 1990; Kirschbaum, 1968; Wood, 1988). This may result in muscular atrophies in the later stages of the disease. Postural changes, such as rigid neck positions and rigidity of the limbs /fingers, are common signs of extrapyramidal system involvement. Less common are lower motor neuron disease signs and symptoms, including hyporeflexia, fasciculations, and fibrillations (Karp, 1990).

Table

TABLEClassification ot Creutzfeldt-Jakob Disease According to Area of Involvement

TABLE

Classification ot Creutzfeldt-Jakob Disease According to Area of Involvement

The cerebellar examination will show difficulty with rapid alternating movements and point-to-point examinations. As the cerebellum is a target area for this virus, these symptoms will be evident early in the disease (Brown, 1989; Karp, 1990; Kirschbaum, 1968; Wood, 1988). The patient may have marked gait disturbances and difficulty performing tandem walking and heel-to-toe walking. A Romberg's sign will most likely be positive as early as stage 1 (Kirschbaum, 1968). Altered muscular tonicity also has been noted (Kirschbaum, 1968).

Cranial nerve examination has revealed loss of sensation in some cases (Kirschbaum, 1968). The response to pain, temperature, and touch may be decreased or, at times, take the reaction of a startled response (Karp, 1990). Loss of olfactory and gustatory sensation, hemianopia, and disassociation of eye movements point to dysfunction of the cranial nerve pathway. Apparent imperceptibility and neglect of a limb or of one side of the body indicate parietal lobe dysfunction; optic agnosia may indicate occipital lobe disease. Overt sensory loss, however, is rarely seen (Karp, 1990).

An examination of the reflexes may reveal exaggerated deep tendon reflexes of the arms and legs, nonelicitable ankle reflexes and negative abdominal reflexes (Brown, 1989; Karp, 1990; Kirschbaum, 1968; Wood, 1988). Babinski's reflex will generally be present (Kirschbaum, 1968). There is no agreement on the pupillary reflexes; in some cases they have been present, and during other assessments they have been nonelicitable (Kirschbaum, 1968).

CT scan readings may reveal mild to moderate ventricular dilation and cortical atrophy (Karp, 1990). Electroencephalogram readings will always be abnormal and remain the most effective tool in diagnosing CJD. Slow wave activity will be seen, spreading as the condition worsens (Brown, 1989; Karp, 1990; Wood, 1988). Rhythmic, periodic bursts of high voltage diphasic or triphasic sharp wave complexes intervening with periods of electrical silence will be evident (Figure 2). The frequency of the spikes will be 20 to 40 per minute initially, but as the disease progresses, the waves may decrease to three to five spikes per minute (Karp, 1990). Cerebrospinal fluid examination has yielded elevated protein levels, but this is not consistently diagnostic (Wood, 1988).

Table

TABLE 2Symptoms of CreutzfeldtJakob Disease According to Stages

TABLE 2

Symptoms of CreutzfeldtJakob Disease According to Stages

Figure 2. The electroencephalogram in Creutzfeldt-Jakob disease.

Figure 2. The electroencephalogram in Creutzfeldt-Jakob disease.

DIAGNOSIS

Brain biopsies have been used to diagnose CJD; however, apprehension surrounding the possibility of transmissibility of the disease and the constraints of decontaminating the autopsy field have limited the use of this diagnostic tool (Brown, 1990). Recently though, an immunologic test comparing a section of a CJD~infected brain with that of a scrapie-infected hamster brain detected the CJD antigen. The technique has been successful in 81% of cases using the Western blot technique (Wood, 1988).

Intracerebral inoculation of brain tissue from an CJD-infected human brain to the brain of a primate has yielded growth of the virus in the infected primate in 90% of experimental cases within 2 years (Brown, 1989). This system remains the most accurate diagnosis of CJD in humans. However, the lengthy period of time required to grow the virus in the primates prevents early diagnosis of the disease in humans. Often, diagnosis is not made until the individual has died.

The final diagnosis of CJD is made during autopsy. Physiologic findings indicate cerebral atrophy, and neuronal loss of the cortex in the frontal, parietal, and occipital lobes of the brain and the cerebellum (Brown, 1989; Wood, 1988). Marked spongiform changes are apparent in the deeper cortical layers. The amyloid plaques that are found in Alzheimer's disease are found much less frequently in CJD.

TREATMENT AND PROGNOSIS

No treatment has been found for CJD. Furthermore, nothing to aide in the slowing of the progression of the disease has been documented. As in the case of Mrs. S., many patients are incorrectly diagnosed as having Alzheimer's disease or some other long-term organic illness. Consequently, these patients are transferred to long-term care facilities to live out the rest of their lives.

The treatment for CJD remains palliative. Among individuals with CJD, death from intercurrent infections, such as pneumonia, or from effects of seizures, occurs within 6 to 12 months of diagnosis (Kirschbaum, 1968; Wood, 1988).

NURSING ASSESSMENT AND CARE

Nurses may initially encounter the CJD patient during the nursing history and physical assessment. Questions should be asked regarding the patient's family, cultural, and geographical history. Medical and surgical history also should be obtained to aid in the differential diagnosis of CJD.

The nurse may begin the physical exam with a mental status examination to evaluate the following items: level of consciousness; attention span; short- and long-term memory; orientation to person, place and time; ability to process information; affect; mood; and judgment. Abnormal findings are listed in Table 2. This may be followed by a complete neurologic exam and an electroencephalogram.

When the diagnosis is made, consultation should be made with the infection control department of the institution to obtain appropriate precautions for the CJD patient. Universal precautions are currently being used to protect individuals from percutaneous contact with infected material. Teaching should be provided to the nursing staff, the patient, and the family on the nature of the disease and the required precautions.

The nurse should provide teaching and counseling to the family in collaboration with the patient's physician and the social service department. Unlike Alzheimer's disease and other organic brain diseases, with CJD death occurrs between 6 and 12 months from diagnosis. Teaching and therapeutic counseling should be provided to the family on the disease, and on the treatment and care options available. The family will need assistance in deciding when to limit life-sustaining interventions, and where and how their family member will be cared for.

Because the disease progresses quickly, frequent evaluations of the patient's mental and neurologic status should be made. The death of CJD patients is generally caused by intercurrent problems such as injuries, epileptic seizures, malnutrition, pneumonia, and sepsis. Nurses must realize that the patient will regress to the point at which they no longer are able to care for themselves or recognize when they are in environmental or physical danger. The nurse must implement interventions to protect the patient from further physical deterioration, injury, and infection. It is important to note that because the CJD patient has been known to have a heightened startled response to stimuli, low stimulus may assist to decrease hallucinations and delusions.

Finally, teaching should be provided to the family of the CJD patient on the need to be cautious for symptom development. The family should be taught the signs and symptoms of the disease and told to notify their physician if these symptoms arise. Teaching and follow-up evaluation also should be provided to patients undergoing high-risk procedures.

CONCLUSION

It is possible that CJD is rarely diagnosed and reported because medical professionals have little information about it. Health care providers must have increased awareness and knowledge about this disease, so that they can provide the care necessary to ensure the CJD patient has a peaceful end to his or her life. It is to be hoped that additional research on CJD will yield information that will aide in more effective care of the CJD patient - or even a cure.

REFERENCES

  • Bernoulli, C, Siegfried, ]., Baumgartner, G., Regli, F., Rabinowicz, X, Gajdusek, D.C., et al. Danger of accidental person-toperson transmission of Creutzfeldt-Jakob disease by surgery. Lancet 1977; 80(1):478479.
  • Brown, P. Central nervous system amyloidosis: A comparison of Alzheimer's disease and Creutzfeldt-Jakob disease. Neurology 1989; 39(8):1103-1105.
  • Brown, P. Guidelines for high risk autopsy cases: Special precautions for CreutzfeldtJakob disease. In Autopsy performance and reporting. Northfield, IL: College of American Pathologists, 1990, pp. 68-74.
  • Brown, P., Gajdusek, D.C., Gibbs, CJ., Jr., Asher, D.M. Potential epidemic of Creutzfeldt-Jakob disease from human growth hormone therapy. N Engl J Med 1985;313:728-731.
  • Davanipour, Z., Alter, M., Sobel, E., Asher, D., Gajdusek, D.C. Creutzfeldt-Jakob disease: Possible medical risk factors. Neurology 1985; 35(10):1483-1486.
  • Dufiy, P., Wolf, J., Collins, C, DeVoe, A.G., Streeten, B., Cowen, D. Possible person-toperson transmission of Creutzfeldt-Jakob disease. N Engl J Med 1974; 290(12):692693.
  • Gajdusek, D.C. Kuru, Creutzfeldt-Jakob, and scrapie. Lancet 1974; 78(96):1551-1552.
  • Karp, H.R., Mirra, S.S. Dementia in adults. In R.J. Joynl (Ed.), Clinical neurology. Philadelphia: J.B. Lippincott, 1990, pp. 24-25, 4144.
  • Kirschbaum, W.R. Jakob-Creutzfeldt disease. New York: American Elsevier, 1968.
  • Kondo, K., Kuroiwa, Y. A case control study of Creutzfeldt-Jakob disease: Association with physical injuries. Ann Neurol 1982; 11(4):377-381.
  • Kuroda, Y., Hidetoshi, K., Shibasaki, H., Kume, S., Yamaguchi, M. HLA study of Japanese patients with Creutzfeldt-Jakob disease: Significant associations with HLA-DQw3. Ann Neurol 1986; 20:356-359.
  • Masters, C.L., Harris, J.O., Gajdusek, D.C, Gibbs, C.J., Bernoulli, C., Asher, D.M. Creutzfeldt-Jakob disease: Patterns of worldwide occurrence and significance of familial and sporadic clustering. Ann Neurol 1979; 5(2):177-187.
  • Wood, M., Anderson, M. Infections caused by slow viruses. In M. Wood, M. Anderson (Ed.), Neurological infections. London: W.B. Saunders, 1988, pp. 574-577.

TABLE

Classification ot Creutzfeldt-Jakob Disease According to Area of Involvement

TABLE 2

Symptoms of CreutzfeldtJakob Disease According to Stages

10.3928/0098-9134-19931101-06

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