Transmissible Spongiform Encephalopathy (Creutzfeldt-Jakob Disease)

Schoene, William C.

doi:10.1001/archneur.1981.00510080035002

Cited by 54 publications

(25 citation statements)

References 9 publications

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“…The clinical and neuropathological features of the two atypical cases of CJD are summarized in Table 1 (10,11,18). One patient (case 1) was a neurosurgeon, whereas the other (case 2) had a medical history of neurosurgery without dura mater grafting.…”

Section: Resultsmentioning

confidence: 99%

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Transmission Properties of Atypical Creutzfeldt-Jakob Disease: a Clue to Disease Etiology?

Kobayashi

Parchi

Yamada

et al. 2015

J Virol

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The genotype at polymorphic codon 129 of the PRNP gene has a profound influence on both phenotypic expression and prion strain susceptibility in humans. For example, while the most common sporadic Creutzfeldt-Jakob disease (CJD) subtype, sporadic CJD-MM1 (M1 strain), induces a single phenotype after experimental transmission regardless of the codon 129 genotype of the recipient animal, the phenotype elicited by sporadic CJD-VV2 (V2 strain), the second most common subtype, varies according to the host codon 129 genotype. In particular, the propagation of the V2 strain in codon 129 methionine homozygotes has been linked only to acquired forms of CJD such as plaque-type dura mater graft-associated CJD (dCJD), a subgroup of iatrogenic CJD with distinctive phenotypic features, but has never been observed in sporadic CJD cases. In the present report, we describe atypical CJD cases carrying codon 129 methionine homozygosity, in a neurosurgeon and in a patient with a medical history of neurosurgery without dural grafting, showing the distinctive phenotypic features and transmission properties of plaque-type dCJD. These findings raise the possibility that the two cases, previously thought to represent sporadic CJD, might actually represent acquired CJD caused by infection with the V2 strain. Thus, careful analyses of phenotypic features and transmission properties in atypical cases may be useful to distinguish acquired from sporadic cases of CJD. IMPORTANCESusceptibility to and phenotypic expression of Creutzfeldt-Jakob disease (CJD) depend on both the prion strain and genotype at polymorphic codon 129 of the PRNP gene. For example, propagation of the second most common sporadic CJD strain (V2 strain) into codon 129 methionine homozygotes has been linked to plaque-type dura mater graft-associated CJD (dCJD), a subgroup of iatrogenic CJD with distinctive phenotypic features, but has never been observed in sporadic CJD. In the present report, we describe atypical CJD cases in a neurosurgeon and in a patient with a medical history of neurosurgery without dural grafting, showing the distinctive phenotypic features and transmission properties of plaque-type dCJD. These findings raise the possibility that the two cases, previously considered to represent sporadic CJD, might actually represent acquired CJD caused by infection with the V2 strain.

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Section: Resultsmentioning

confidence: 99%

“…We found two atypical CJD cases that showed widespread kuru plaques despite their 129M/M genotype. Details of these atypical CJD cases have been reported elsewhere (10,11). In addition, clinically, genetically, and histopathologically proven sCJD and dCJD cases with the 129M/M genotype were also included in this study.…”

Section: Importancementioning

confidence: 99%

Transmission Properties of Atypical Creutzfeldt-Jakob Disease: a Clue to Disease Etiology?

Kobayashi

Parchi

Yamada

et al. 2015

J Virol

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“…Before his first hospitalization, he had peripheral neuropathy of the lower extremities, loss of vibration sense, rotatory nystagmus, and ataxic gait. 88 During the initial hospitalization, Matson carried out many of his duties from his hospital room. 18 For the centennial commemoration of Harvey Cushing, he was able to publish a selection of Cushing's works, entitled Harvey Cushing: Selected Papers on Neurosurgery.…”

Section: Illness and Deathmentioning

confidence: 99%

“…He had characteristics of Cushing syndrome due to the therapeutic course of prednisone and was moderately obtunded. 88 The faculty at PBBH put him through many clinical studies and skin biopsies in search of a diagnosis. John Shillito, Matson's colleague, noted, "The ataxia progressed.…”

Section: Illness and Deathmentioning

confidence: 99%

“…The working diagnosis, based on skin lesions and the acute and later chronic vasculitis, was Köhlmeier-Degos syndrome, a rare vasculopathy affecting small vessels in the skin, gastrointestinal tract, ocular, and central nervous systems. 36,88 At one point there was an effort to transfer him to the Veterans Hospital for further care. Matson's physicians and colleagues became increasingly upset and contacted Joseph E. Levine, the famous film producer, who was a philanthropic supporter of PBBH.…”

Section: Illness and Deathmentioning

confidence: 99%

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Unsung hero: Donald Darrow Matson’s legacy in pediatric neurosurgery

Rehder¹,

Lohani²,

Cohen³

2015

PED

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Donald Darrow Matson made seminal contributions to the field of pediatric neurosurgery. Born in 1913 in Fort Hamilton, New York, Matson was the youngest of four sons of an army colonel. He graduated from Cornell University and, years later, from Harvard Medical School. Matson selected Peter Bent Brigham Hospital for his neurosurgical training, which was interrupted during World War II. As a neurosurgeon, he worked close to the front lines under Brigadier General Elliot Cutler in Europe, earning a Bronze Star. Matson returned to Boston to become Franc Ingraham’s fellow and partner. He was a masterful surgeon and, with Ingraham, published Neurosurgery of Infancy and Childhood in 1954, the first pediatric neurosurgery textbook in the world. Upon Ingraham’s retirement, Matson became chairman of the department of neurosurgery at Boston Children’s Hospital and Peter Bent Brigham. In 1968, he became the inaugural Franc D. Ingraham Professor of Neurological Surgery at Harvard Medical School. Among his neurosurgical accomplishments, Matson served as President of the Harvey Cushing Society, later known as the American Association of Neurological Surgeons. He was unable to preside at the 1969 meeting that marked the 100th anniversary of Cushing’s birth, having contracted Creutzfeldt-Jakob disease. Matson died at the age of 55, surviving his mentor Ingraham by only 4 years.

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Scaly Papules With Atrophy

Shwayder¹

1986

Arch Dermatol

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Transmissible Spongiform Encephalopathy (Creutzfeldt-Jakob Disease)

Cited by 54 publications

References 9 publications

Transmission Properties of Atypical Creutzfeldt-Jakob Disease: a Clue to Disease Etiology?

Transmission Properties of Atypical Creutzfeldt-Jakob Disease: a Clue to Disease Etiology?

Unsung hero: Donald Darrow Matson’s legacy in pediatric neurosurgery

Scaly Papules With Atrophy

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