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GENERAL ARTICLES

Anesthetic Management of a Patient with Gerstmann-Sträussler-Scheinker Syndrome (Mutation of Prion Protein)

Department of Anesthesiology, University of Occupational and Environmental Health, School of Medicine, Fukuoka, Japan

Address correspondence and reprint requests to Masanori Ogata, MD, Department of Anesthesiology, University of Occupational and Environmental Health, School of Medicine. 1-1, Iseigaoka, Yahatanishiku, Kitakyushu, Fukuoka, 807-8555, Japan. Address e-mail to mogata{at}med.uoeh-u.ac.jp.

	Abstract

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Gerstmann-Sträussler-Scheinker Syndrome (GSS) is a rare, infectious syndrome related to a mutation in the prion protein. A 60-yr-old, 152-cm, 31-kg woman with GSS was scheduled for open gastrostomy. This is the first report about an anesthetic experience in a patient with GSS. We describe our experience and precautions in the anesthetic management of a GSS patient.

	Introduction

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Gerstmann-Sträussler-Scheinker Syndrome (GSS) is a rare, infectious syndrome related to a mutation in the prion protein. We describe our experience and precautions in the anesthetic management of a GSS patient.

	Case Report

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A 60-yr-old, 152-cm, 31-kg woman was scheduled for open gastrostomy. She had been healthy for 55 yr, when she began to develop ataxia. At 56 yr, she developed dysarthria. At 57 yr, she suffered from progressive cerebellar ataxia, pseudo-bulbar palsy, and absent tendon reflexes in the lower limbs. Her father and uncle had the same symptoms. After a mutation typical of the 102nd amino acid, a drastic change was found in the normal prion gene. She was then diagnosed with classical GSS. Her blood, urine, cerebrospinal fluid, electrocardiography, and chest radiograph were normal. Her Hasegawa Dementia Scale-Revised score was 15/30. The Hasegawa Dementia Scale-Revised is 90% sensitive and 82% specific in screening for dementia using an established cut-off of <20/30 points. (1) The patient was scheduled for open gastrostomy because her dysphagia was worsening gradually and she often had aspiration pneumonia. Surgeons did not choose percutaneous endoscopic gastrostomy under regional anesthesia because they thought that they could not get her cooperation under regional anesthesia because of her severe dementia and ataxia. We then planned general anesthesia with tracheal intubation.

No preoperative medication was given. In addition to disposable masks and hats, all medical staff wore gowns and gloves. We used disposable surgical instruments and anesthetic equipment, including a disposable laryngoscope (OPTI SCOPE®, South Africa).

In the operating room, routine ASA monitors were placed and a Train-Of-Four (TOF)-Guard® (Biometer, Denmark) was attached. Anesthesia was induced with propofol 50 mg. Vecuronium 0.1 mg · kg^–1 was administered after we had measured the control twitch height using the TOF-Guard®. Propofol 30 mg was administered just before tracheal intubation. The trachea was intubated when neither the T1 nor TOF ratio could be detected. After induction, her arterial blood pressure decreased to 65/27 mm Hg transiently and systolic blood pressure was maintained 80–100 mm Hg before operation with a total of 8 mg of ephedrine. Anesthesia was maintained with sevoflurane 1.5%–2.5% and nitrous oxide 66% in oxygen. The operation was completed uneventfully after 70 min. The TOF ratio recovered to 70% 3 h after vecuronium administration. The muscle relaxant was then reversed using neostigmine 1.0 mg and atropine 0.5 mg. After confirming full recovery of muscle strength by the TOF ratio, double-burst stimulation and spontaneous eye opening, the trachea was extubated. Immediately, unexpected upper airway obstruction occurred. We maintained her airway for 30 min until she could maintain her upper airway herself by chin-lift and head extension procedure. During this time, her Spo₂ remained more than 99%, and the end-tidal CO₂ was <45 mm Hg. After stopping to maintain her airway, we confirmed that the blood gas analysis, including the Paco₂, was within normal limits. She was admitted to the intensive care unit, where her vital signs were stable.

All disposable equipment, including the laryngoscope and surgical instruments, were discarded and incinerated.

The patient's postanesthetic course was uneventful. She returned to the ward the next day.

	Discussion

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Transmissible spongiform encephalopathy (TSE) agents, or prions, induce neurodegenerative fatal diseases in humans and in some other mammalian species. Human TSEs include Creutzfeldt-Jakob disease, GSS, kuru, and fatal familial insomnia. They are characterized by amyloid deposits (kuru plaques), spongiform changes in the brain, and transmissibility to experimental animals.

GSS is characterized by cerebellar ataxia, pyramidal and extra-pyramidal features, and progressive dementia, eventually leading to death. GSS is invariably fatal and there is no proven treatment or prophylaxis. The disease is transmitted in an autosomal dominant pattern. From 1993 to 2000, in 10 countries, the majority in Western Europe, 2774 TSE patients were reported, including 24 with GSS (2). There are a few reports of anesthesia in Creutzfeldt-Jakob disease (3,4), but this is the first report of anesthesia in a GSS patient.

GSS patients transmit the disease to mice in about one third of inoculated cases (5), suggesting that the prion protein of GSS is infectious. The World Health Organization guideline (6) says that persons with confirmed or suspected TSEs are the highest risk patients and must be managed using specific precautions. The prion protein is most common in the central nervous system, appendix, and lymphoreticular tissues and is resistant to inactivation by radiation, heat, or harsh chemical treatments. To prevent infection, our medical staff wore gowns and gloves and used only disposable equipment. In addition, in view of the existence of the prion protein in lymphoreticular tissues, we used a disposable laryngoscope.

We extubated the tracheal tube 40 min after sevoflurane was discontinued and confirmed that muscle strength had recovered to normal. Nevertheless, airway obstruction occurred unexpectedly. We consider that the patient's peculiar pseudobulbar palsy was exacerbated by a small residual amount of inhaled anesthetic and neuromuscular blocking drug, which may have caused the upper airway obstruction. This single case cannot determine whether the risk of airway obstruction after extubation is increased in GSS patients who had pseudobulbar palsy.

The authors conclude that important points in management of GSS patients are 1) to prevent infection, use disposable equipment whenever possible, and 2) upper airway obstruction may occur after tracheal extubation. These two points should be considered for the safe anesthetic management of GSS patients.

	Footnotes

 
Accepted for publication November 7, 2005.

Supported, in part, by Grant-in-Aid for Science Research (C) 16591575 from the Ministry of Education, Science, Sport and Culture of Japan.

	References

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1. Homma A. Assessment and treatment of patient with dementia of the Alzheimer type. Nippon Ronen Igakkai Zasshi 1992;4:264–70.
2. Pocchiari M, Puopolo M, Croes EA, et al. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain 2004;127:2348–59.[Abstract/Free Full Text]
3. MacMurdo SD, Jakymec AJ, Bleyaert AL. Precautions in the anesthetic management of a patient with Creutzfield-Jacob disease. Anesthesiology 1984;60:590–2.[Medline]
4. Hernandez-Palazon J, Martinez-Lage JF, Tortosa JA, Garcia-Cayuela JM. Anaesthetic management in patients suspected of, or at risk of, having Creutzfelt-Jakob disease. Br J Anaesth 1998;80:516–8.[Abstract/Free Full Text]
5. Tateishi J, Kitamoto T. Inherited prion diseases and transmission to rodents. Brain Pathology 1995;5:53–9.[Web of Science][Medline]
6. World Health Organization. WHO infection control guidelines for transmissible spongiform encephalopathies. March 23–23, 1999. Available at: http://whqlibdoc.who.int/hq/2000/WHO_CDS_CSR_APH_2000.3.pdf.

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