OBJECTIVE: Iatrogenic Creutzfeldt-Jakob disease (iCJD) is mainly associated with dura mater (DM) grafts and administration of human growth hormones (hGH). Data on disease course in DM-CJD are limited. We describe the clinical and diagnostic findings in this patient group with special emphasis on MRI signal alterations. METHODS: Ten DM-CJD patients were studied for their clinical symptoms and diagnostic findings. The MRIs were evaluated for signal increase of the cortical and subcortical structures. RESULTS: DM-CJD patients had a median incubation time of 18 years and median disease duration of 7 months. The majority of patients were MM homozygous at codon 129 of the prion protein gene (PRNP) and presented with gait ataxia and psychiatric symptoms. No correlation between the graft site and the initial disease course was found. The MRI showed cortical and basal ganglia signal increase each in eight out of ten patients and thalamic hyperintensity in five out of ten cases. Of interest, patients with thalamic signal increase were homozygous for methionine. CONCLUSION: The MRI findings in DM-CJD largely resemble those seen in sporadic CJD, as the cortex and basal ganglia are mainly affected.
OBJECTIVE: Iatrogenic Creutzfeldt-Jakob disease (iCJD) is mainly associated with dura mater (DM) grafts and administration of human growth hormones (hGH). Data on disease course in DM-CJD are limited. We describe the clinical and diagnostic findings in this patient group with special emphasis on MRI signal alterations. METHODS: Ten DM-CJDpatients were studied for their clinical symptoms and diagnostic findings. The MRIs were evaluated for signal increase of the cortical and subcortical structures. RESULTS:DM-CJDpatients had a median incubation time of 18 years and median disease duration of 7 months. The majority of patients were MM homozygous at codon 129 of the prion protein gene (PRNP) and presented with gait ataxia and psychiatric symptoms. No correlation between the graft site and the initial disease course was found. The MRI showed cortical and basal ganglia signal increase each in eight out of ten patients and thalamic hyperintensity in five out of ten cases. Of interest, patients with thalamic signal increase were homozygous for methionine. CONCLUSION: The MRI findings in DM-CJD largely resemble those seen in sporadic CJD, as the cortex and basal ganglia are mainly affected.
Authors: J Huillard d'Aignaux; D Costagliola; J Maccario; T Billette de Villemeur; J P Brandel; J P Deslys; J J Hauw; J L Chaussain; Y Agid; D Dormont; A Alpérovitch Journal: Neurology Date: 1999-10-12 Impact factor: 9.910
Authors: Donald A Collie; David M Summers; Robin J Sellar; James W Ironside; Sarah Cooper; Martin Zeidler; Richard Knight; Robert G Will Journal: AJNR Am J Neuroradiol Date: 2003-09 Impact factor: 3.825
Authors: I Zerr; W J Schulz-Schaeffer; A Giese; M Bodemer; A Schröter; K Henkel; H J Tschampa; O Windl; A Pfahlberg; B J Steinhoff; O Gefeller; H A Kretzschmar; S Poser Journal: Ann Neurol Date: 2000-09 Impact factor: 10.422
Authors: P Sanchez-Juan; A Green; A Ladogana; N Cuadrado-Corrales; R Sáanchez-Valle; E Mitrováa; K Stoeck; T Sklaviadis; J Kulczycki; K Hess; M Bodemer; D Slivarichová; A Saiz; M Calero; L Ingrosso; R Knight; A C J W Janssens; C M van Duijn; I Zerr Journal: Neurology Date: 2006-08-22 Impact factor: 9.910
Authors: Hye Lim Kim; Ju Young Do; Han Jeong Cho; Yong-Chul Jeon; Seok Joo Park; Hyeo Il Ma; Jun Ho Song; Yul Lee; Hyun Choi; Kyung Chan Choi; Yong Sun Kim; Inga Zerr; Kai Kallenberg; Yun Joong Kim Journal: J Korean Med Sci Date: 2011-10-27 Impact factor: 2.153
Authors: Matt Stevenson; Lesley Uttley; Jeremy E Oakley; Christopher Carroll; Stephen E Chick; Ruth Wong Journal: Health Technol Assess Date: 2020-02 Impact factor: 4.014
Authors: Emma Jones; Holger Hummerich; Emmanuelle Viré; James Uphill; Athanasios Dimitriadis; Helen Speedy; Tracy Campbell; Penny Norsworthy; Liam Quinn; Jerome Whitfield; Jacqueline Linehan; Zane Jaunmuktane; Sebastian Brandner; Parmjit Jat; Akin Nihat; Tze How Mok; Parvin Ahmed; Steven Collins; Christiane Stehmann; Shannon Sarros; Gabor G Kovacs; Michael D Geschwind; Aili Golubjatnikov; Karl Frontzek; Herbert Budka; Adriano Aguzzi; Hata Karamujić-Čomić; Sven J van der Lee; Carla A Ibrahim-Verbaas; Cornelia M van Duijn; Beata Sikorska; Ewa Golanska; Pawel P Liberski; Miguel Calero; Olga Calero; Pascual Sanchez-Juan; Antonio Salas; Federico Martinón-Torres; Elodie Bouaziz-Amar; Stéphane Haïk; Jean-Louis Laplanche; Jean-Phillipe Brandel; Phillipe Amouyel; Jean-Charles Lambert; Piero Parchi; Anna Bartoletti-Stella; Sabina Capellari; Anna Poleggi; Anna Ladogana; Maurizio Pocchiari; Serena Aneli; Giuseppe Matullo; Richard Knight; Saima Zafar; Inga Zerr; Stephanie Booth; Michael B Coulthart; Gerard H Jansen; Katie Glisic; Janis Blevins; Pierluigi Gambetti; Jiri Safar; Brian Appleby; John Collinge; Simon Mead Journal: Lancet Neurol Date: 2020-09-16 Impact factor: 44.182
Authors: Katharina Stoeck; Pascual Sanchez-Juan; Joanna Gawinecka; Alison Green; Anna Ladogana; Maurizio Pocchiari; Raquel Sanchez-Valle; Eva Mitrova; Theodor Sklaviadis; Jerzy Kulczycki; Dana Slivarichova; Albert Saiz; Miguel Calero; Richard Knight; Adriano Aguzzi; Jean-Louis Laplanche; Katell Peoc'h; Gabi Schelzke; Andre Karch; Cornelia M van Duijn; Inga Zerr Journal: Brain Date: 2012-09-25 Impact factor: 13.501