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Gerstmann syndrome

Gerstmann syndrome is a neurological disorder characterized by four primary symptoms: more...

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  1. Dysgraphia/agraphia
  2. Dyscalculia/acalculia
  3. Finger agnosia
  4. Left-right disorientation

In adults, the syndrome may occur after a stroke or in association with damage to the parietal lobe. In addition to exhibiting the above symptoms, many adults also experience aphasia, which is a difficulty in expressing oneself when speaking, in understanding speech, or in reading and writing.

There are few reports of the syndrome, sometimes called developmental Gerstmann syndrome, in children. The cause is not known. Most cases are identified when children reach school age, a time when they are challenged with writing and math exercises. Generally, children with the disorder exhibit poor handwriting and spelling skills, and difficulty with math functions, including adding, subtracting, multiplying, and dividing. An inability to differentiate right from left and to discriminate among individual fingers may also be apparent. In addition to the four primary symptoms, many children also suffer from constructional apraxia, an inability to copy simple drawings. Frequently, there is also an impairment in reading. Children with a high level of intellectual functioning as well as those with brain damage may be affected with the disorder.

There is no cure for Gerstmann syndrome. Treatment is symptomatic and supportive. Occupational and speech therapies may help diminish the dysgraphia and apraxia. In addition, calculators and word processors may help school children cope with the symptoms of the disorder.

In adults, many of the symptoms diminish over time. Although it has been suggested that in children symptoms may diminish over time, it appears likely that most children probably do not overcome their deficits, but learn to adjust to them.

The National Institute of Neurological Disorders and Stroke (NINDS) supports research on disorders that result from damage to the brain such as dysgraphia. The NINDS and other components of the National Institutes of Health also support research on learning disabilities. Current research avenues focus on developing techniques to diagnose and treat learning disabilities and increase understanding of the biological basis of them.

This disorder is often associated with brain lesions in the dominant (usually left) side of the angular and supramarginal gyri near the temporal and parietal lobe junction.

It should not be confused with Gerstmann-Straussler syndrome, which is a transmissible spongiform encephalopathy.


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Creutzfeldt-Jakob Disease; office-based surgery accreditation; new cancer probe; patients' jewelry
From AORN Journal, 8/1/01 by Dorothy M. Fogg

Question: I have been hearing a lot about CJD and its potential for spreading via surgical instruments. What is CJD, and how can it be spread by instruments that have been cleaned and sterilized?

Answer: The acronym CJD stands for Creutzfeldt-Jakob Disease, an infectious, degenerative, and fatal disease of the central nervous system.(1) Creutzfeldt-Jakob Disease is one of a group of encephalopathies known as transmissible spongiform encephalopathies (TSEs). Other human forms of TSEs are kuru, Gerstmann-Straussler-Sheinker syndrome, and familial insomnia syndrome. Recently, a fourth type of human TSE called variant CJD (vCJD) has been recognized.(2) Animal forms of TSEs are scrapie in sheep and goats; transmissible mink encephalopathy; exotic ungulate encephalopathy; chronic wasting disease of mule, deer, and elk; feline spongiform encephalopathy; and bovine spongiform encephalopathy (BSE), also known as Mad Cow Disease.

The pathogenic agent responsible for CJD is highly resistant to standard sterilization and disinfection methods. Unless special protocols are used, the CJD causative agent can survive routine sterilization and disinfection processes.

Creutzfeldt-Jakob Disease is considered a slow viral infection caused by an unconventional virus known as a prion.(3) Prions are a unique class of pathogen that have no detectable DNA or RNA. These small, proteinaceous agents are abnormal isoforms of normal cellular proteins. An isoform is a group of proteins that are produced by different genes and are specific to different tissues but have the same function.(4) The cause of this transformation is unknown; however, it is thought to be a mutation of the chromosome on which the protein resides.(5)

The incubation period for CJD can vary from months to years to decades. Symptoms include rapidly progressing dementia, psychiatric and behavioral abnormalities, and a distinctive electroencephalograph reading. Positive diagnosis can be made only by direct examination of affected brain tissue. Most cases occur in patients between 50 and 70 years of age. The duration of illness after patients become symptomatic is an average of six months, and the disease always ends in death.

In contrast, vCJD has an earlier onset (ie, between 16 and 48 years of age). Patients exhibit sensory and psychiatric symptoms differing from those of CJD, and the course of illness averages 14 months. Again, the disease is always fatal.(6) According to the Centers for Disease Control and Prevention, there is strong epidemiological and laboratory evidence of a causal association between vCJD and BSE.(7) As of September 2000, no cases of vCJD were reported in the United States.

Creutzfeldt-Jakob Disease can be familial (ie, inherited in the form of a mutant gene) or sporadic (ie, no family history and no known source of transmission). Approximately 90% of cases are sporadic. Approximately 1% of cases result from person-to-person transmission and are primarily the result of iatrogenic (ie, medically related) exposure. Exposures have occurred via transplantation of central nervous system tissue, such as dura mater or corneas; repeated injections of pituitary hormone extracts; and use of contaminated surgical instruments or stereotactic depth electrodes.(8)

Tissues vary in their degree of infectivity according to prion content as follows:

* high-infectivity tissue (eg, brain, dura mater, spinal cord, corneas);

* medium-infectivity tissue (eg, cerebrospinal fluid, liver, lymph nodes, lung, kidney, spleen); and

* low- or no-infectivity tissue (eg, heart, skeletal muscle, thyroid glands, adrenal glands, intestines, peripheral nerves, bone marrow, whole blood, leukocytes, serum, adipose tissue, prostate, testis, semen, placenta, vaginal secretions, milk, tears, gingiva, nasal mucus, saliva, sputum, urine, feces).

Creutzfeldt-Jakob Disease and other TSEs are unusually resistant to conventional chemical and physical decontamination methods. The causative prions are resistant to steam sterilization, dry heat, ethylene oxide gas, and chemical disinfection with either formaldehyde or glutaraldehyde, as normally used in the health care environment. Both formaldehyde and glutaraldehyde act as fixatives, causing the prions to become more stable and less susceptible to these normal sterilization and disinfection protocols. Special protocols for instrument care after exposure to prions should be followed.(9) Table 1 is a suggested protocol for caring for instruments exposed to the CJD prion.

Information about prions, prion infectivity, and decontamination is evolving. Updates are available online from the Centers for Disease Control and Prevention at World Health Organization at http:// Health Canada at

Question: I recently took a position as manager of a well-established office-based surgery center. With three ORs and four physicians in our practice, the rooms are busy the majority of the time. One reason I was hired was to obtain accreditation for the facility. Is it possible to obtain accreditation from the Joint Commission on Accreditation of Healthcare Organizations for my office-based center? Is my facility required to meet the same standards as inpatient facilities?

Answer: The Joint Commission does provide accreditation for office-based surgery centers. To become Joint Commission-accredited, your facility does not have to meet the same standards as inpatient facilities. It does, however, have to meet standards specifically developed for office-based surgery practices, as well as comply with applicable laws and regulations. The office-based surgery standards are published in the Joint Commission's Accreditation Manual for Office-based Surgery Practices, which was released in February 2001.(10) This new manual provides state-of-the-art, nationally recognized standards for office-based surgery and focuses on patient care and safety. The manual is presented in the familiar format of performance-focused standards, intent statements, and scoring questions. The manual addresses

* patient services, including patients' rights and practice ethics;

* patient care, including surgical and invasive procedures, sedation and anesthesia, post-anesthesia care, clinical support services, and patient and family member education;

* staff development, training, and competence;

* credentialing;

* patient safety, including the environment of care;

* leadership; and

* performance improvement.(11)

When surveyors visit your facility, they will assess your compliance with the applicable standards of the office-based manual. They will conduct interviews with staff members at various levels during the tour of the facility and patient-care areas. Surveyors then may choose to conduct more in-depth interviews with selected staff members to follow up on information obtained earlier in the process. Patients and their family members also may be interviewed to determine their perceptions of the services and care received. Surveyors will provide on-site education and consultation during the survey, offering suggestions to help you better meet the standards and improve patient care.

This accreditation process is designed primarily for single office sites that meet the following criteria:

* have no more than four licensed physicians or other licensed independent practitioners;

* are owned or operated by a physician-owned corporation, a private physician's office, or a group practice;

* permit licensed independent practitioners who are employees or partners to perform surgical or other invasive procedures; and

* use minimal sedation, moderate (ie, conscious) sedation, or general anesthesia for procedures.

Accreditation decisions are based on how well your facility meets the office-based surgery standards. Even though standards cannot directly measure quality, they are the most effective means of assessing and predicting performance. Facilities seeking a first accreditation must be able to demonstrate four months of compliance with the standards. For facilities seeking subsequent accreditation, 12 months of compliance are required.(12)

Question: I recently heard one of our surgeons discussing a probe that can be used to test whether tissue is benign or malignant without doing an open biopsy. What Is this probe, and where can I purchase it?

Answer: The probe actually was developed by a researcher at the National Aeronautics and Space Administration (NASA) research center who was developing a probe for a Mars lander. After describing the device to a neurosurgeon friend, the researcher developed the tissue-sensing probe. The probe measures optical and electrical responses that indicate differences between cancerous and normal cells. The probe must be inserted into ,the tissue to be diagnosed, thus, misleading data can be obtained when bleeding occurs. Researchers are developing software that will enable the probe to recalibrate itself as it moves through the normal tissue toward the suspicious lesion.(13)

Technology has been licensed from NASA to begin studying the effectiveness of using the tiny probe to determine whether tissue is benign or malignant. The probe is not available to the public at this time. Further studies are needed, and the device must be approved by the US Food and Drug Administration before it can enter the marketplace.

Question: Recently, an increasing number of patients are entering our surgery center wearing various pieces of jewelry in various parts of their bodies. Our policy calls for patients to remove all jewelry before surgery, but we are encountering a lot of resistance from these patients. They claim that the body piercing is permanent and the jewelry cannot be removed. Others claim it will be too painful to remove and later reinsert the jewelry. Should we continue our policy of removing all jewelry, or should we change policies to accommodate these patients?

Answer: Your policy for removing jewelry should be maintained. Body jewelry is removable. If the body jewelry has been recently inserted, holes can close quickly when the jewelry is removed. This can cause patients to resist removing the jewelry. The manner in which nurses explain the rationale for removal can affect patients' receptiveness. In addition to the possibility of patient injury if his or her jewelry becomes trapped in a drape or the OR bed, there also is the possibility of bum if an electrosurgery unit (ESU) is used. Electrosurgery units with ground-referenced generators may seek alternate paths to ground through a patient's jewelry. Newer models of ESUs have isolated generators that eliminate the occurrence of alternate-site tissue burns; however, many health care facilities still use ESUs with ground-referenced generators. Manufacturers of electrosurgery units continue to advocate for removal of all jewelry.

Many of the jewelry pieces work on f threading principle. In some cases, ring spreaders and closers are needed to remove the jewelry. You may want to purchase ring spreaders and closers in an assortment of sizes for your facility if you encounter this type of jewelry often. Using a cutting tool to sever the rings is not recommended. Consulting a body-piercing establishment and becoming familiar with the various types of jewelry can help prepare you for any jewelry removal problems you may encounter.

Some health care facilities have written policies that mandate the cancellation of surgical procedures if patients' jewelry is not removed. You may want to include a question about body piercing in your preoperative telephone interview. Inform the patient that he or she is responsible for having the jewelry removed before surgery. The patient then can return to the body-piercing establishment to have the jewelry temporarily removed. If you do not use preoperative telephone interviews, educate staff members at physicians' offices to discuss this issue with the patient before scheduling surgery. Then be sure to verify with the scheduling staff member that any jewelry issue is resolved before a procedure is scheduled.

If the patient is having emergency surgery in a hospital OR, the jewelry may be removed after the patient is under anesthesia and replaced before the patient wakes. Of course, tools for removal and reinsertion of jewelry will be necessary.


(1.) M S Favaro, W W Bond, "Chemical disinfection of medical and surgical materials," in Disinfection, Sterilization, and Preservation, fifth ed, S S Block, ed (Philadelphia: Lippincott Williams & Wilkins, 2001) 910.

(2.) W A Rutala, D J Weber, "Creutzfeldt-Jakob disease: Recommendations for disinfection and sterilization," Healthcare Epidemiology 32 (May 1, 2001) 1348.

(3.) Favaro, Bond, "Chemical disinfection of medical and surgical materials," 910, 911.

(4.) Dorland's Illustrated Medical Dictionary, 28th ed (Philadelphia: W B Saunders Co, 1994) 863.

(5.) Rutala, Weber, "Creutzfeldt-Jakob disease: Recommendations for disinfection and sterilization," 1349.

(6.) Ibid.

(7.) "Bovine Spongiform Encephalopathy and New Variant Creutzfeldt-Jakob Disease," Centers for Disease Control and Prevention, National Center for Infectious Diseases, /bse_cjd.htm (accessed 27 June 2001); "Research letter Creutzfeldt-Jakob Disease in the United States: 1779-1998," American Medical Association, (accessed 19 June 2001).

(8.) Favaro, Bond, "Chemical disinfection of medical and surgical materials," 911.

(9.) Ibid, 913.

(10.) Joint Commission on Accreditation of Healthcare Organizations, Accreditation Manual for Office-based Surgery (Oakbrook Terrace, Ill: Joint Commission on Accreditation of Healthcare Organizations, 2001).

(11.) Joint Commission on Accreditation of Healthcare Organizations, "Setting national quality standards for small office surgery practices," Joint Commission Perspectives 21 (April 2001) 3.

(12.) Ibid, 1-3.

(13.) Association for the Advancement of Medical Instrumentation, "Innovative technology developed by NASA being studied for breakthrough in biopsies" AAMI News 36 (March 2001) 6.

At various times throughout the year, the Recommended Practices Committee seeks review and comments on proposed recommended practices from members and other interested individuals. When available, these proposed recommended practices appear on AORN Online at http// The proposed recommended practices also con be obtained by calling the AORN fox-on-demand service at (800) 755-7980. Interested individuals without access to the Internet or to a fox machine may obtain copies of the proposed documents by coiling Joyce Davis, Center for Nursing Practice, at (800) 755-2676 x 334. Proposed recommended practice documents ore available for review and comment for a 30-day period offer being posted. A deadline for comments will be indicated with each document. Please check the above sources frequently to locate proposed recommended practices. All comments received are considered as the document is finalized. Thank you for your participation.


COPYRIGHT 2001 Association of Operating Room Nurses, Inc.
COPYRIGHT 2001 Gale Group

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