infection control and hospital epidemiology december 2007, vol. 28, no. 12 concise communication A Case of Creutzfeldt-Jakob Disease Associated With a Dura Mater Graft in the United States David B. Blossom, MD, MS; Ryan A. Maddox, MPH; Suzanne F. Beavers, MD; Kelly A. Church, MD; Doug A. Thoroughman, PhD; Lawrence B. Schonberger, MD, MPH; Ermias D. Belay, MD We describe a case of Creutzfeldt-Jakob disease associated with a dura mater graft (Lyodura brand) in a 26-year-old man who un- derwent several neurosurgical procedures as a child. Clinicians and infection control personnel should be aware that recipients of Lyod- ura brand dura mater grafts processed before May 1987 may remain at increased risk for Creutzfeldt-Jakob disease throughout their lives. Infect Control Hosp Epidemiol 2007; 28:1396-1397 Creutzfeldt-Jakob disease (CJD) is an invariably fatal neu- rodegenerative disorder believed to be caused by infectious protein agents (prions). The disease is characterized by rap- idly progressive dementia, ataxia, myoclonus, pyramidal and/ or extrapyramidal signs, akinetic mutism, and, ultimately, death. Confirmation of the diagnosis usually requires study of brain tissue obtained at biopsy or autopsy. Classic forms of CJD are etiologically separated into 3 categories: sporadic (approximately 85%-90% of cases), familial (approximately 10%-15% of cases), and iatrogenic (less than 1% of cases). The rate of sporadic forms of CJD peaks in the seventh decade of life. Both sporadic and familial CJD are rare in individuals younger than thirty years of age, and the occurrence of such a case may suggest a possible iatrogenic cause. Published sources of iatrogenic CJD include contaminated human pi- tuitary-derived hormone, 1 cornea transplants, 2 neurosurgical equipment, 3 and dura mater grafts. 4-7 We describe a 26-year- old white male patient who developed iatrogenic CJD 18.7 years after receiving a Lyodura brand dura mater graft (man- ufactured by B. Braun Melsungen). case report The case patient was born in February 1980 and had a Chiari Type II malformation resulting in myelomeningocele and hy- drocephalus. These malformations were treated surgically within 2 weeks after birth; treatment included placement of a ventriculoperitoneal (VP) shunt. The patient’s congenital abnormalities were complicated by a neurogenic bladder, bowel incontinence, right lower extremity paralysis with club foot, and kyphoscoliosis. In May 1985, the case patient underwent a procedure to add 12 inches of tubing to the distal end of the VP shunt. In April 1987, he underwent a tethered cord repair procedure to improve the neurologic function in his lower extremities, bowel, and bladder. As part of the tethered cord repair, a Lyodura brand dural graft was used to close the dural defect created by the procedure. Throughout the patient’s childhood, hydrocephalus re- mained static and without progression. Although radio- graphic imaging confirmed on several occasions that the VP shunt had become disconnected, no further neurosurgical procedures were performed until 3 months before his death. In December 2005, the case patient noted the gradual onset of facial and right arm paresthesias, accompanied by a con- stant headache. Walking with braces and crutches became more difficult. In February 2006, VP shunt revision surgery was performed, but it provided no benefit. By March 2006, the patient had become ataxic, with my- oclonus, dysarthria, bilateral nystagmus, and a large-ampli- tude tremor of his right arm. An electroencephalogram showed long stretches of posterior dominant high-voltage slowing, in the frequency of 1.5-2.0 Hz, suggesting marked diffuse posterior cerebral dysfunction. Brain magnetic reso- nance imaging demonstrated unchanged hydrocephalus. Chiari decompression with C1 laminectomy and craniectomy of the suboccipital bone was performed, followed 10 days later by reexploration surgery with extension of the lami- nectomy to C2-3. A diagnosis of CJD was considered when the case patient showed no neurologic improvement after these procedures. In April 2006, the diagnosis was substantiated by detecting an increased level of 14-3-3 protein in the cerebrospinal fluid. In mid-May 2006, the patient died. CJD was confirmed by examination of brain tissue obtained at autopsy; immuno- staining with 3F4, the monoclonal antibody to the prion pro- tein, yielded positive results. Because this tissue was fixed in formalin, no further prion protein analyses were performed. The case patient had no family history of neurologic or psychiatric diseases. He had never received growth hormone or traveled outside of the United States. discussion The unusually young age of the patient we describe and his receipt of a Lyodura graft, a brand of dura mater linked to an ongoing international outbreak of more than 180 reported cases of CJD, indicate that the dural graft was the most likely cause of CJD. 5 Despite the large international outbreak, this patient is only the third US patient with Lyodura-associated CJD and the first to be reported in more than 10 years. 6,7 It is likely that the United States has been spared a large number of cases, because the German manufacturer had no distrib- utor in this country. Nevertheless, the first case of Lyodura- associated CJD in the United States was the first such case