Human Growth Hormone–Related Iatrogenic Creutzfeldt-Jakob Disease With Abnormal Imaging

Lewis, Aaron M.; Yu, M.; DeArmond, Stephen J.; Dillon, William P.; Miller, Bruce L.; Geschwind, Michael D.

doi:10.1001/archneur.63.2.288

Cited by 25 publications

(13 citation statements)

References 17 publications

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“…Poon et al [29] reported one case with the Brownell-Oppenheimer (cerebellar) variant of CJD with clear DWI (and weaker FLAIR) cerebellar hyperintensity. Lewis et al [30] reported similar findings in one case of hGH-related iCJD, but two other cases revealed no cerebellar MRI findings, despite clinical cerebellar signs. In fact, this paradoxical pattern of negative cerebellar MRI with proven neuropathological degeneration postmortem is common in the CJD literature [31,32].…”

Section: Discussionmentioning

confidence: 69%

MRI Detection of the Cerebellar Syndrome in Creutzfeldt–Jakob Disease

et al. 2009

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Creutzfeldt-Jakob Disease (CJD) is characterized by bilateral basal ganglia hyperintensities on T2W and diffusion-weighted imaging (DWI) magnetic resonance imaging (MRI) scans, consistent with its extrapyramidal neurological manifestations. MRI is diagnostically uninformative about the cerebellar symptoms, equally prominent in CJD. This study was undertaken to explain this apparent paradox. Eleven CJD patients with definite cerebellar or brain stem symptoms were selected from a large prospective study, as well as 11 healthy controls matched for age and gender. All subjects participated in a standardized MRI protocol, including SPGR, fluid-attenuated inversion recovery (FLAIR), DWI and diffusion tensor imaging (DTI). All subjects underwent detailed examination by a neurologist blinded to the radiological findings, who predicted the expected site of cerebral abnormalities. MRI showed good sensitivity for the abnormalities predicted in the cortex (80-90%) and basal ganglia (100%). None of the standard MRI sequences, including DWI, DTI, and FLAIR, revealed any tissue abnormalities in cerebellum or brain stem. Apparent diffusion coefficient (ADC) values, however, were substantially and significantly elevated in several cerebellar structures, where also the volumetric (VBM) analysis revealed elevated cerebrospinal fluid volume, suggesting focal cerebellar atrophy in these CJD patients. In patients with CJD, DWI appears sensitive to the reduced diffusivity in cortex and basal ganglia but insensitive to cerebellar involvement. We propose that the radiological hallmark of cerebellar pathology in CJD is atrophy, revealed quantitatively by both VBM and elevated diffusivity, which is identifiable on ADC maps but poorly visualized in nonquantitative DWI images.

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Section: Discussionmentioning

confidence: 69%

MRI Detection of the Cerebellar Syndrome in Creutzfeldt–Jakob Disease

et al. 2009

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“…CJD occurs in new-variant and classical (sporadic and iatrogenic) forms. Iatrogenic CJD followed administration of cadaveric human pituitary hormones (192,193), dural graft transplants (194), corneal transplants (195), and the use of contaminated neurosurgical instruments (196).…”

Section: Creutzfeldt-jakob Disease (Prion Disease)mentioning

confidence: 99%

Transmission of Infection by Flexible Gastrointestinal Endoscopy and Bronchoscopy

et al. 2013

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SUMMARY Flexible endoscopy is a widely used diagnostic and therapeutic procedure. Contaminated endoscopes are the medical devices frequently associated with outbreaks of health care-associated infections. Accurate reprocessing of flexible endoscopes involves cleaning and high-level disinfection followed by rinsing and drying before storage. Most contemporary flexible endoscopes cannot be heat sterilized and are designed with multiple channels, which are difficult to clean and disinfect. The ability of bacteria to form biofilms on the inner channel surfaces can contribute to failure of the decontamination process. Implementation of microbiological surveillance of endoscope reprocessing is appropriate to detect early colonization and biofilm formation in the endoscope and to prevent contamination and infection in patients after endoscopic procedures. This review presents an overview of the infections and cross-contaminations related to flexible gastrointestinal endoscopy and bronchoscopy and illustrates the impact of biofilm on endoscope reprocessing and postendoscopic infection.

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“…Lai /Tseng Neuroepidemiology 2009;33:247-253 248 mones [6][7][8] , dural graft transplants [9] , use of dura mater in radiographic embolization procedures [10] , corneal transplants [11,12] , liver transplants [13] and the use of contaminated neurosurgical instruments or stereotactic depth electrodes [14] . An international study of the epidemiological characteristics of CJD, which included national registries in France, Germany, Italy, The Netherlands, Slovakia, UK, Australia, Austria, Canada, Spain and Switzerland, showed that the overall annual mortality rate between 1999 and 2002 was 1.67/million for all cases and 1.39/million for sporadic CJD [15] .…”

mentioning

confidence: 99%

Population-Based Epidemiological Study of Neurological Diseases in Taiwan: I. Creutzfeldt-Jakob Disease and Multiple Sclerosis

Lai

Tseng

2009

Neuroepidemiology

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Background: The purpose of the study is to investigate the epidemiology of Creutzfeldt-Jakob disease (CJD) and multiple sclerosis (MS) in Taiwan. Methods: Cases of CJD and MS were identified from the National Health Insurance Research Database from January 2000 to December 2005. Age- and sex-specific incidences of these diseases were estimated by dividing the incidence number by population data obtained from the Department of Statistics, Ministry of the Interior. Results: During the study period, 79 cases of CJD, 41 women and 38 men, were identified. The average annual incidence rate was 0.63/million. Most cases were older than 60 years. There were 1,262 cases of MS. The male-to-female ratio was 0.4. The average annual incidence rate was 0.79/100,000. The incidence reached a peak at the age group of 40–60 years for females and at the age group of over 55 years for males. The prevalence of MS was 2.96/100,000 in 2005, which is higher than those of previous studies in Taiwan. Conclusions: The annual incidence rate of CJD in Taiwan is lower than in western countries. The annual incidence rate and prevalence of MS in Taiwan are low. The prevalence of MS in Taiwan increased in recent decades.

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Human Growth Hormone–Related Iatrogenic Creutzfeldt-Jakob Disease With Abnormal Imaging

Cited by 25 publications

References 17 publications

MRI Detection of the Cerebellar Syndrome in Creutzfeldt–Jakob Disease

MRI Detection of the Cerebellar Syndrome in Creutzfeldt–Jakob Disease

Transmission of Infection by Flexible Gastrointestinal Endoscopy and Bronchoscopy

Population-Based Epidemiological Study of Neurological Diseases in Taiwan: I. Creutzfeldt-Jakob Disease and Multiple Sclerosis

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