Affiliation: University of California
- Challenging the clinical utility of the 14-3-3 protein for the diagnosis of sporadic Creutzfeldt-Jakob diseaseMichael D Geschwind
Department of Neurology, University of California, San Francisco Medical Center, San Francisco, CA 94143, USA
Arch Neurol 60:813-6. 2003..Previous studies have suggested that, in the proper clinical context, the 14-3-3 protein in cerebrospinal fluid is a reliable marker for sporadic CJD...
- Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; a prospective observational studyTetsuo Ashizawa
Department of Neurology and McKnight Brain Institute, University of Florida, 1149 S, Newell Dr, L3 100, Gainesville, FL 32611, USA
Orphanet J Rare Dis 8:177. 2013..SCA1, 2, 3 and 6 are the four most common SCAs, all caused by expanded polyglutamine-coding CAG repeats. Their pathomechanisms are becoming increasingly clear and well-designed clinical trials will be needed...
- Genetic CJD with a novel E200G mutation in the prion protein gene and comparison with E200K mutation casesMee Ohk Kim
Department of Neurology, Memory and Aging Center, University of California, San Francisco UCSF, San Francisco, CA 94143, USA
Acta Neuropathol Commun 1:80. 2013....
- Rapidly progressive dementiaMichael D Geschwind
University of California San Francisco Memory and Aging Center, Department of Neurology, San Francisco, CA 94143 1207, USA
Ann Neurol 64:97-108. 2008....
- Rapidly progressive dementiaMichael D Geschwind
Department of Neurology, Memory and Aging Center, University of California, San Francisco Medical Center, San Francisco, CA 94117, USA
Neurol Clin 25:783-807, vii. 2007..This review discusses a differential diagnostic approach to RPD, emphasizing neurodegenerative, toxic and metabolic, infectious, autoimmune, neoplastic, and other conditions to consider...
- Sporadic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob diseaseJennifer Martindale
Department of Neurology, University of California San Francisco, 94143, USA
Arch Neurol 60:767-70. 2003..The determination of the form of prion disease and early diagnosis are important for prognostic, public health, and epidemiologic reasons...
- When sporadic disease is not sporadic: the potential for genetic etiologyJill S Goldman
University of California, Memory and Aging Center, San Francisco 94143 1207, USA
Arch Neurol 61:213-6. 2004..Approximately 2% of Alzheimer disease cases and 10% to 15% of prion disease cases are due to mutations in autosomal dominant genes. Mutations have been found in patients without family histories of neurological disease...
- Patterns of brain atrophy that differentiate corticobasal degeneration syndrome from progressive supranuclear palsyAdam L Boxer
Memory and Aging Center, Department of Neurology, University of California, San Francisco, CA 94143 1207, USA
Arch Neurol 63:81-6. 2006..Progressive brain atrophy is associated with the corticobasal degeneration syndrome (CBDS) and progressive supranuclear palsy (PSP). Regional differences in brain atrophy may reflect the clinical features of disease...
- Immunologically mediated dementiasMichael H Rosenbloom
UCSF Memory and Aging Center, Box 1207, San Francisco, CA 94143, USA
Curr Neurol Neurosci Rep 9:359-67. 2009..Diagnostic workup typically reveals evidence of an autoimmune process and, in some cases, cancer. In contrast to the neurodegenerative processes, many of the immunologically mediated dementias respond to immunomodulatory therapy...
- Pathologic evidence that the T188R mutation in PRNP is associated with prion diseaseMaria Carmela Tartaglia
Memory and Aging Center, University of California, San Francisco, San Francisco, California, USA
J Neuropathol Exp Neurol 69:1220-7. 2010....
- A case of enteroviral meningoencephalitis presenting as rapidly progressive dementiaVictor Valcour
Department of Neurology, Memory and Aging Center, University of California, San Francisco, CA94143, USA
Nat Clin Pract Neurol 4:399-403. 2008..He had hypophonic speech and had mildly increased axial tone. He scored 5 out of 30 on the Mini Mental State Examination and was admitted for expedited evaluation...
- Anti-GAD antibody cerebellar ataxia mimicking Creutzfeldt-Jakob diseaseChiung Chih Chang
University of California San Francisco, Department of Neurology, Memory and Aging Center, Box 1207, San Francisco, CA 94143 1207, USA
Clin Neurol Neurosurg 109:54-7. 2007..We present a case of anti-glutamic acid decarboxylase antibody (anti-GAD65) positive cerebellar ataxia associated with cognitive complaints and elevated CSF 14-3-3 protein...
- Autoimmune encephalopathiesSteven Vernino
Department of Neurology, UT Southwestern Medical Center, Dallas, Texas 75390 9036, USA
Neurologist 13:140-7. 2007..This review describes the clinical features of these potentially reversible autoimmune encephalopathies...
- The diagnostic utility of brain biopsy procedures in patients with rapidly deteriorating neurological conditions or dementiaS Andrew Josephson
Department of Neurology, University of California, San Francisco, California, USA
J Neurosurg 106:72-5. 2007....
- Diagnosis of human prion diseaseJiri G Safar
Institute for Neurodegenerative Diseases, Memory and Aging Center, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 102:3501-6. 2005..Moreover, IHC should not be used as the standard against which all other immunodiagnostic techniques are compared because an immunoassay, such as the CDI, is substantially more sensitive...
- Adult-onset drug-refractory seizure disorder associated with anti-voltage-gated potassium-channel antibodyRamon F Barajas
Department of Radiology, Neuroradiology Section, University of California, San Francisco, California 94143 1207, USA
Epilepsia 51:473-7. 2010..Once the diagnosis has been established the initiation of immunotherapy should be undertaken without delay...
- Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementiasP Vitali
Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA 94143 1207, USA
Neurology 76:1711-9. 2011..No studies, however, have demonstrated how MRI can distinguish CJD from nonprion causes of rapidly progressive dementia (npRPD). We sought to determine the diagnostic accuracy of MRI for CJD compared to a cohort of npRPD subjects...
- Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosisGeoffrey S Young
Department of Radiology, University of California, San Francisco, 74143, USA
AJNR Am J Neuroradiol 26:1551-62. 2005..To our knowledge, no large study has been conducted to determine the sensitivity and specificity of DWI and FLAIR imaging for diagnosing CJD...
- Human growth hormone-related iatrogenic Creutzfeldt-Jakob disease with abnormal imagingAaron M Lewis
Department of Neurology, University of California, San Francisco, USA
Arch Neurol 63:288-90. 2006....
- Prion proteins in subpopulations of white blood cells from patients with sporadic Creutzfeldt-Jakob diseaseEd M Choi
Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143 0518, USA
Lab Invest 89:624-35. 2009..If human WBCs harbor prion infectivity in patients with sCJD, then the levels are likely to be low...
- AMPA receptor antibodies in limbic encephalitis alter synaptic receptor locationMeizan Lai
Department of Neurology, University of Pennsylvania, Philadelphia, PA 19104, USA
Ann Neurol 65:424-34. 2009..To report the clinical and immunological features of a novel autoantigen related to limbic encephalitis (LE) and the effect of patients' antibodies on neuronal cultures...
- Correlating DWI MRI with pathologic and other features of Jakob-Creutzfeldt diseaseMichael D Geschwind
Departments of Neurology, University of California, San Francisco, UCSF Memory and Aging Center, Box 1207, SanFrancisco, CA 94143 1207, USA
Alzheimer Dis Assoc Disord 23:82-87. 2009..77; P=0.0006), followed by reactive astrocytic gliosis (r=0.63, P=0.008). This case provides further evidence that DWI abnormalities correlate well with the clinical features and with PrP(Sc) accumulation and vacuolation...
- Neuroimaging in dementiaPaolo Vitali
Department of Neurology, UCSF Memory and Aging Center, University of California San Francisco, CA 94143, USA
Semin Neurol 28:467-83. 2008..Although only clinical assessment can lead to a diagnosis of dementia, neuroimaging is clearly an invaluable tool for the clinician in the differential diagnosis...
- Human prions and plasma lipoproteinsJiri G Safar
Institute for Neurodegenerative Diseases, Department of Neurology, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 103:11312-7. 2006..Whether detection of PrP(Sc) in VLDL and LDL particles can be adapted into an antemortem diagnostic test for prions in the blood of humans, livestock, and free-ranging cervids remains to be determined...
- Psychiatric and neuroimaging findings in Creutzfeldt-Jakob diseaseJennifer L Martindale
Department of Neurology, University of California, San Francisco, Box 1207, 350 Parnassus 800, 94143, USA
Curr Psychiatry Rep 5:43-6. 2003..This article discusses how these advances may make neuroimaging the most valuable noninvasive tool for diagnosing CJD, which helps to track the progression of the disease course and provides insight into clinical-anatomic correlations...
- Voltage-gated potassium channel autoimmunity mimicking creutzfeldt-jakob diseaseMichael D Geschwind
Neuroimmunology Laboratory, Hilton 3 78F, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA
Arch Neurol 65:1341-6. 2008..Rapidly progressive dementia has a variety of causes, including Creutzfeldt-Jakob disease (CJD) and neuronal voltage-gated potassium channel (VGKC) autoantibody-associated encephalopathy...
- A 54-year-old man with slowness of movement and confusionMichael D Geschwind
Neurology 69:1881-7. 2007
- Creutzfeldt-Jakob disease in recipients of corneal transplantsRyan A Maddox
Division of Viral and Rickettsial Diseases, National Center for Zoonotic, Vector Borne, and Enteric Diseases, Centers for Disease Control and Prevention, Atlanta, GA 30333, USA
Cornea 27:851-4. 2008..We report 4 cases of CJD with a history of corneal transplantation and assess the frequency of coincidental CJD among corneal transplant recipients...