Prion disease: The essential facts

Clinton, J.; Roberts, Gareth W.

doi:10.1002/gps.930071203

Cited by 4 publications

(1 citation statement)

References 104 publications

(77 reference statements)

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“…Proteins that are specific for individual subunits that form the ionotropic glutamate receptors have been identified. Recently, we have shown that there are abnormalities in NMDA receptor expression as well as the expression of some of these PSD proteins in the thalamus in schizophrenia 1. The distribution and levels of expression of these molecules in the human cerebral cortex is largely unknown, however, and characterization of the cortical expression of these molecules in human brain was the goal of this study.…”

Section: Introductionmentioning

confidence: 98%

AMPA‐ and NMDA‐Associated Postsynaptic Protein Expression in the Human Dorsolateral Prefrontal Cortex

Beneyto

Meador‐Woodruff

2003

Annals of the New York Academy of Sciences

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Section: Introductionmentioning

confidence: 98%

AMPA‐ and NMDA‐Associated Postsynaptic Protein Expression in the Human Dorsolateral Prefrontal Cortex

Beneyto

Meador‐Woodruff

2003

Annals of the New York Academy of Sciences

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Frontal lobe dementia is not a variant of prion disease

Clinton

Mann

Roberts

1993

Neuroscience Letters

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Familial Creutzfeldt-Jakob Disease: A Neuropsychological Case Study

Gass

Luis²,

Meyers³

et al. 2000

Archives of Clinical Neuropsychology

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The spectrum of neuropsychological features of familial Creutzfeldt-Jakob disease (CJD) have seldom been reported, possibly because of (a) the rarity of this hereditary form of prion disease; (b) frequent delays in diagnosis, and; (c) the typically rapid demise of the patient, which affords little opportunity for comprehensive testing or serial analysis. Here we describe the neurobehavioral characteristics of a 48-year-old right-handed male (JD) who presented with complaints of poor depth perception, unsteady gait, and unusual sensory experiences in his face and neck. JD was followed serially over the final 4 months of his 5-month illness. Immediately following hospital admission, he underwent a neuropsychological evaluation that revealed moderate to severe impairment of delayed (30-minute) verbal memory, tactual performance in his right hand, and word-finding ability. In contrast, other abilities that are commonly classified within the verbal, visuospatial, and memory domains showed minimal or no compromise. Parallel studies of electroencephalographic activity revealed diffuse slowing and, later, 1-Hz rhythmical discharges over the left hemisphere, and mild prominence of the lateral ventricles and cerebral sulci on magnetic resonance imaging. Autopsy revealed spongiform changes and reactive astrocytosis, and genetic testing demonstrated a codon 200 mutation in the prion protein gene. These findings indicate that CJD can result in clinical manifestations compatible with multifocal asymmetric cerebral involvement before more diffuse neurodegeneration ensues, providing a strong impetus for the study of additional cases. This long-term understanding can help to determine whether the multiple loci of clinical involvement are specified by genetic or epigenetic factors, or both.

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Prion disease: The essential facts

Cited by 4 publications

References 104 publications

AMPA‐ and NMDA‐Associated Postsynaptic Protein Expression in the Human Dorsolateral Prefrontal Cortex

AMPA‐ and NMDA‐Associated Postsynaptic Protein Expression in the Human Dorsolateral Prefrontal Cortex

Frontal lobe dementia is not a variant of prion disease

Familial Creutzfeldt-Jakob Disease: A Neuropsychological Case Study

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