Bio

Bio


Dr. Sha received her Bachelor’s degrees in Cognitive Science and Molecular Cell Biology emphasizing in Neurobiology from UC Berkeley. She went on to obtain a Master’s degree in Physiology and MD from Georgetown University. She trained in Neurology at UCLA and Stanford University and completed a clinical and research fellowship in behavioral neurology at UCSF where she focused on identifying biomarkers for genetic forms of frontotemporal dementia and caring for patients with movement disorders and cognitive impairment.

Dr. Sha’s clinical expertise include Alzheimer’s disease, frontotemporal dementia, Lewy Body disease, corticobasal syndrome, progressive supranuclear palsy, Huntington’s disease, ataxia, multiple system atrophy, and other dementias.

Dr. Sha’s non-clinical time is spent conducting clinical trials in order to identify disease modifying treatments for dementia. She has a special interest in genetic forms of dementia and the cognitive impairment in parkinsonian-related disorders.

Clinical Focus


  • Neurology
  • Huntington Disease
  • Ataxia
  • Alzheimer Disease
  • Frontotemporal Dementia
  • Dementia

Academic Appointments


Professional Education


  • Residency:Stanford University School of Medicine (2010) CA
  • Medical Education:Georgetown University (2006) DC
  • Fellowship:University of California San Francisco (2013) CA
  • Board Certification: Neurology, American Board of Psychiatry and Neurology (2010)
  • Residency:University of California Los Angeles (2008) CA
  • Internship:California Pacific Medical Center (2007) CA

Research & Scholarship

Clinical Trials


  • Multiple Dose Study of BIIB037 (Recombinant, Fully Human Anti-Aβ IgG1 mAb) in Participants With Prodromal or Mild Alzheimer's Disease Recruiting

    The primary objective of this study is to evaluate the safety and tolerability of multiple doses of BIIB037 (recombinant, fully human anti-Aβ IgG1 mAb) in participants with prodromal or mild Alzheimer's Disease (AD). The secondary objectives of this study are to assess the effect on cerebral amyloid plaque content as measured by florbetapir-fluorine-18 (18F-AV-45F-AV-45) positron emission tomography (PET) imaging, to assess the multiple dose serum concentrations of BIIB037 and to evaluate the immunogenicity of BIIB037 after multiple dose administration in this population.

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  • Efficacy and Safety Trial of MK-8931 in Participants With Prodromal Alzheimer's Disease (MK-8931-019) Recruiting

    The purpose of this trial is to assess the efficacy and safety of MK-8931 compared with placebo in the treatment of amnestic mild cognitive impairment (aMCI) due to Alzheimer's Disease (AD), also known as prodromal AD. Participants will be randomized to receive placebo, or 12 mg or 40 mg MK-8931, once daily. The primary study hypothesis is that at least one MK-8931 dose is superior to placebo with respect to the change from baseline in the Clinical Dementia Rating scale-Sum of Boxes (CDR-SB) score at 104 weeks.

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  • The PLasma for Alzheimer SymptoM Amelioration (PLASMA) Study Recruiting

    The PLasma for Alzheimer SymptoM Amelioration (PLASMA) Study: A Randomized, Double-Blind, Placebo-Controlled, Cross-Over Trial of Intravenously-Administered Plasma From Young Donors for Treatment of Mild-To-Moderate Alzheimer's Disease

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  • A Long-Term Safety Extension Study of Studies ABE4869g And ABE4955g in Patients With Mild To Moderate Alzheimer's Disease Treated With Crenezumab Not Recruiting

    This Phase II, open-label extension (OLE), multicenter study will evaluate the long-term safety and tolerability of crenezumab in patients with mild to moderate Alzheimer's disease who have participated in and completed the treatment period of the Phase II Study ABE4869g or ABE4955g. Patients will receive crenezumab at the same dosing frequency, dose level and route of administration they were assigned to after the Week 73 visit of Study ABE4869g or ABE4955g. Patients who received placebo in Study ABE4869g or ABE4955g will receive active drug. Anticipated time on study treatment is 94 weeks.

    Stanford is currently not accepting patients for this trial.

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Publications

Journal Articles


  • Predicting amyloid status in corticobasal syndrome using modified clinical criteria, magnetic resonance imaging and fluorodeoxyglucose positron emission tomography. Alzheimer's research & therapy Sha, S. J., Ghosh, P. M., Lee, S. E., Corbetta-Rastelli, C., Jagust, W. J., Kornak, J., Rankin, K. P., Grinberg, L. T., Vinters, H. V., Mendez, M. F., Dickson, D. W., Seeley, W. W., Gorno-Tempini, M., Kramer, J., Miller, B. L., Boxer, A. L., Rabinovici, G. D. 2015; 7 (1): 8

    Abstract

    Group comparisons demonstrate greater visuospatial and memory deficits and temporoparietal-predominant degeneration on neuroimaging in patients with corticobasal syndrome (CBS) found to have Alzheimer's disease (AD) pathology versus those with underlying frontotemporal lobar degeneration (FTLD). The value of these features in predicting underlying AD pathology in individual patients is unknown. The goal of this study is to evaluate the utility of modified clinical criteria and visual interpretations of magnetic resonance imaging (MRI) and fluorodeoxyglucose positron emission tomography (FDG-PET) for predicting amyloid deposition (as a surrogate of Alzheimer's disease neuropathology) in patients presenting with CBS.In total, 25 patients meeting CBS core criteria underwent amyloid (Pittsburgh compound B; PIB) PET scans. Clinical records, MRI, and FDG scans were reviewed blinded to PIB results. Modified clinical criteria were used to classify CBS patients as temporoparietal variant CBS (tpvCBS) or frontal variant CBS (fvCBS). MRI and FDG-PET were classified based on the predominant atrophy/hypometabolism pattern (frontal or temporoparietal).A total of 9 out of 13 patients classified as tpvCBS were PIB+, compared to 2out of 12 patients classified as fvCBS (P < 0.01, sensitivity 82%, specificity 71% for PIB+ status). Visual MRI reads had 73% sensitivity and 46% specificity for PIB+ status with moderate intra-rater reliability (Cohen's kappa = 0.42). Visual FDG reads had higher sensitivity (91%) for PIB+ status with perfect intra-rater reliability (kappa = 1.00), though specificity was low (50%). PIB results were confirmed in all 8 patients with available histopathology (3 PIB+ with confirmed AD, 5 PIB- with FTLD).Splitting CBS patients into frontal or temporoparietal clinical variants can help predict the likelihood of underlying AD, but criteria require further refinement. Temporoparietal-predominant neuroimaging patterns are sensitive but not specific for AD.

    View details for DOI 10.1186/s13195-014-0093-y

    View details for PubMedID 25733984

  • Altered network connectivity in frontotemporal dementia with C9orf72 hexanucleotide repeat expansion BRAIN Lee, S. E., Khazenzon, A. M., Trujillo, A. J., Guo, C. C., Yokoyama, J. S., Sha, S. J., Takada, L. T., Karydas, A. M., Block, N. R., Coppola, G., Pribadi, M., Geschwind, D. H., Rademakers, R., Fong, J. C., Weiner, M. W., Boxer, A. L., Kramer, J. H., Rosen, H. J., Miller, B. L., Seeley, W. W. 2014; 137: 3047-3060

    Abstract

    Hexanucleotide repeat expansion in C9orf72 represents the most common genetic cause of familial and sporadic behavioural variant frontotemporal dementia. Previous studies show that some C9orf72 carriers with behavioural variant frontotemporal dementia exhibit distinctive atrophy patterns whereas others show mild or undetectable atrophy despite severe behavioural impairment. To explore this observation, we examined intrinsic connectivity network integrity in patients with or without the C9orf72 expansion. We studied 28 patients with behavioural variant frontotemporal dementia, including 14 C9orf72 mutation carriers (age 58.3 ± 7.7 years, four females) and 14 non-carriers (age 60.8 ± 6.9 years, four females), and 14 age- and sex-matched healthy controls. Both patient groups included five patients with comorbid motor neuron disease. Neuropsychological data, structural brain magnetic resonance imaging, and task-free functional magnetic resonance imaging were obtained. Voxel-based morphometry delineated atrophy patterns, and seed-based intrinsic connectivity analyses enabled group comparisons of the salience, sensorimotor, and default mode networks. Single-patient analyses were used to explore network imaging as a potential biomarker. Despite contrasting atrophy patterns in C9orf72 carriers versus non-carriers, patient groups showed topographically similar connectivity reductions in the salience and sensorimotor networks. Patients without C9orf72 expansions exhibited increases in default mode network connectivity compared to controls and mutation carriers. Across all patients, behavioural symptom severity correlated with diminished salience network connectivity and heightened default mode network connectivity. In C9orf72 carriers, salience network connectivity reduction correlated with atrophy in the left medial pulvinar thalamic nucleus, and this region further showed diminished connectivity with key salience network hubs. Single-patient analyses revealed salience network disruption and default mode network connectivity enhancement in C9orf72 carriers with early-stage or slowly progressive symptoms. The findings suggest that patients with behavioural variant frontotemporal dementia with or without the C9orf72 expansion show convergent large-scale network breakdowns despite distinctive atrophy patterns. Medial pulvinar degeneration may contribute to the behavioural variant frontotemporal dementia syndrome in C9orf72 carriers by disrupting salience network connectivity. Task-free functional magnetic resonance imaging shows promise in detecting early-stage disease in C9orf72 carriers and may provide a unifying biomarker across diverse anatomical variants.

    View details for DOI 10.1093/brain/awu248

    View details for Web of Science ID 000346760900026

    View details for PubMedID 25273996

  • Quantitative 7T Phase Imaging in Premanifest Huntington Disease AMERICAN JOURNAL OF NEURORADIOLOGY Apple, A. C., Possin, K. L., Satris, G., JOHNSON, E., Lupo, J. M., Jakary, A., Wong, K., Kelley, D. A., Kang, G. A., SHA, S. J., Kramer, J. H., Geschwind, M. D., Nelson, S. J., Hess, C. P. 2014; 35 (9): 1707-1713

    Abstract

    In vivo MR imaging and postmortem neuropathologic studies have demonstrated elevated iron concentration and atrophy within the striatum of patients with Huntington disease, implicating neuronal loss and iron accumulation in the pathogenesis of this neurodegenerative disorder. We used 7T MR imaging to determine whether quantitative phase, a measurement that reflects both iron content and tissue microstructure, is altered in subjects with premanifest Huntington disease.Local field shift, calculated from 7T MR phase images, was quantified in 13 subjects with premanifest Huntington disease and 13 age- and sex-matched controls. All participants underwent 3T and 7T MR imaging, including volumetric T1 and 7T gradient recalled-echo sequences. Local field shift maps were created from 7T phase data and registered to caudate ROIs automatically parcellated from the 3T T1 images. Huntington disease-specific disease burden and neurocognitive and motor evaluations were also performed and compared with local field shift.Subjects with premanifest Huntington disease had smaller caudate volume and higher local field shift than controls. A significant correlation between these measurements was not detected, and prediction accuracy for disease state improved with inclusion of both variables. A positive correlation between local field shift and genetic disease burden was also found, and there was a trend toward significant correlations between local field shift and neurocognitive tests of working memory and executive function.Subjects with premanifest Huntington disease exhibit differences in 7T MR imaging phase within the caudate nuclei that correlate with genetic disease burden and trend with neurocognitive assessments. Ultra-high-field MR imaging of quantitative phase may be a useful approach for monitoring neurodegeneration in premanifest Huntington disease.

    View details for DOI 10.3174/ajnr.A3932

    View details for Web of Science ID 000341639900011

    View details for PubMedID 24742810

  • Executive Functions in Premanifest Huntington's Disease MOVEMENT DISORDERS You, S. C., Geschwind, M. D., Sha, S. J., Apple, A., Satris, G., Wood, K. A., Johnson, E. T., Gooblar, J., Feuerstein, J. S., Finkbeiner, S., Kang, G. A., Miller, B. L., Hess, C. P., Kramer, J. H., Possin, K. L. 2014; 29 (3): 405-409

    Abstract

    We investigated the viability of psychometrically robust executive function measures as markers for premanifest Huntington's disease (HD).Fifteen premanifest HD subjects and 42 controls were compared on the NIH EXAMINER executive function battery. This battery yields an overall executive composite score, plus working memory, cognitive control, and fluency scores that are measured on psychometrically matched scales. The scores were correlated with two disease markers, disease burden and striatal volumes, in the premanifest HD subjects.The premanifest HD subjects scored significantly lower on the working memory score. The executive composite positively correlated with striatal volumes, and the working memory score negatively correlated with disease burden. The cognitive control and fluency scores did not differ between the groups or correlate significantly with the disease markers.The NIH EXAMINER executive composite and working memory scores are sensitive markers of cognitive dysfunction, striatal volume, and disease burden in premanifest HD.

    View details for DOI 10.1002/mds.25762

    View details for Web of Science ID 000332823000022

    View details for PubMedID 24375511

  • Interrater reliability of the new criteria for behavioral variant frontotemporal dementia NEUROLOGY LaMarre, A. K., Rascovsky, K., Bostrom, A., Toofanian, P., Wilkins, S., Sha, S. J., Perry, D. C., Miller, Z. A., Naasan, G., Laforce, R. J., Hagen, J., Takada, L. T., Tartaglia, M. C., Kang, G., Galasko, D., Salmon, D. P., Farias, S. T., Kaur, B., Olichney, J. M., Park, L. Q., Mendez, M. F., Tsai, P., Teng, E., Dickerson, B. C., Domoto-Reilly, K., McGinnis, S., Miller, B. L., Kramer, J. H. 2013; 80 (21): 1973-1977

    Abstract

    To evaluate the interrater reliability of the new International Behavioural Variant FTD Criteria Consortium (FTDC) criteria for behavioral variant frontotemporal dementia (bvFTD).Twenty standardized clinical case modules were developed for patients with a range of neurodegenerative diagnoses, including bvFTD, primary progressive aphasia (nonfluent, semantic, and logopenic variant), Alzheimer disease, and Lewy body dementia. Eighteen blinded raters reviewed the modules and 1) rated the presence or absence of core diagnostic features for the FTDC criteria, and 2) provided an overall diagnostic rating. Interrater reliability was determined by κ statistics for multiple raters with categorical ratings.The mean κ value for diagnostic agreement was 0.81 for possible bvFTD and 0.82 for probable bvFTD ("almost perfect agreement"). Interrater reliability for 4 of the 6 core features had "substantial" agreement (behavioral disinhibition, perseverative/compulsive, sympathy/empathy, hyperorality; κ = 0.61-0.80), whereas 2 had "moderate" agreement (apathy/inertia, neuropsychological; κ = 0.41-0.6). Clinician years of experience did not significantly influence rater accuracy.The FTDC criteria show promise for improving the diagnostic accuracy and reliability of clinicians and researchers. As disease-altering therapies are developed, accurate differential diagnosis between bvFTD and other neurodegenerative diseases will become increasingly important.

    View details for Web of Science ID 000319332900015

    View details for PubMedID 23635967

  • Frontotemporal dementia due to C9ORF72 mutations Clinical and imaging features NEUROLOGY Sha, S. J., Takada, L. T., Rankin, K. P., Yokoyama, J. S., Rutherford, N. J., Fong, J. C., Khan, B., Karydas, A., Baker, M. C., DeJesus-Hernandez, M., Pribadi, M., Coppola, G., Geschwind, D. H., Rademakers, R., Lee, S. E., Seeley, W., Miller, B. L., Boxer, A. L. 2012; 79 (10): 1002-1011

    Abstract

    To describe the phenotype of patients with C9FTD/ALS (C9ORF72) hexanucleotide repeat expansion.A total of 648 patients with frontotemporal dementia (FTD)-related clinical diagnoses and Alzheimer disease (AD) dementia were tested for C9ORF72 expansion and 31 carried expanded repeats (C9+). Clinical and neuroimaging data were compared between C9+ (15 behavioral variant FTD [bvFTD], 11 FTD-motor neuron disease [MND], 5 amyotrophic lateral sclerosis [ALS]) and sporadic noncarriers (48 bvFTD, 19 FTD-MND, 6 ALS).All C9+ patients displayed clinical syndromes of bvFTD, ALS, or FTD-MND. At first evaluation, C9+ bvFTD patients had more delusions and greater impairment of working memory, but milder eating dysregulation compared to bvFTD noncarriers. C9+FTD-MND patients had a trend for longer survival and had an earlier age at onset than FTD-MND noncarriers. Voxel-based morphometry demonstrated more thalamic atrophy in FTD and FTD-MND carriers than in noncarriers.Patients with the C9ORF72 hexanucleotide repeat expansion develop bvFTD, ALS, or FTD-MND with similar clinical and imaging features to sporadic cases. Other FTD spectrum diagnoses and AD dementia appear rare or absent among C9+ individuals. Longer survival in C9+ FTD-MND suggests slower disease progression and thalamic atrophy represents a novel and unexpected feature.

    View details for Web of Science ID 000308674000015

    View details for PubMedID 22875087

  • Atypical, slowly progressive behavioural variant frontotemporal dementia associated with C9ORF72 hexanucleotide expansion JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY Khan, B. K., Yokoyama, J. S., Takada, L. T., Sha, S. J., Rutherford, N. J., Fong, J. C., Karydas, A. M., Wu, T., Ketelle, R. S., Baker, M. C., Hernandez, M., Coppola, G., Geschwind, D. H., Rademakers, R., Lee, S. E., Rosen, H. J., Rabinovici, G. D., Seeley, W. W., Rankin, K. P., Boxer, A. L., Miller, B. L. 2012; 83 (4): 358-364

    Abstract

    Some patients meeting behavioural variant frontotemporal dementia (bvFTD) diagnostic criteria progress slowly and plateau at mild symptom severity. Such patients have mild neuropsychological and functional impairments, lack characteristic bvFTD brain atrophy and have thus been referred to as bvFTD 'phenocopies' or slowly progressive (bvFTD-SP). The few patients with bvFTD-SP that have been studied at autopsy have demonstrated no evidence of FTD pathology, suggesting that bvFTD-SP is neuropathologically distinct from other forms of FTD. Here, two patients with bvFTD-SP with chromosome 9 open reading frame 72 (C9ORF72) hexanucleotide expansions are described.384 patients with an FTD clinical spectrum and Alzheimer's disease diagnoses were screened for C9ORF72 expansion. Two bvFTD-SP mutation carriers were identified. Neuropsychological and functional data, as well as brain atrophy patterns, assessed using voxel based morphometry (VBM), were compared with 44 patients with sporadic bvFTD and 85 healthy controls.Both patients were aged 48 years at baseline and met possible bvFTD criteria. In the first patient, VBM revealed thalamic and posterior insula atrophy. Over 7 years, his neuropsychological performance and brain atrophy remained stable. In the second patient, VBM revealed cortical atrophy with subtle frontal and insular volume loss. Over 2 years, her neuropsychological and functional scores as well as brain atrophy remained stable.C9ORF72 mutations can present with a bvFTD-SP phenotype. Some bvFTD-SP patients may have neurodegenerative pathology, and C9ORF72 mutations should be considered in patients with bvFTD-SP and a family history of dementia or motor neuron disease.

    View details for DOI 10.1136/jnnp-2011-301883

    View details for Web of Science ID 000301296600003

    View details for PubMedID 22399793

  • Neuropsychiatric features of C9orf72-associated behavioral variant frontotemporal dementia and frontotemporal dementia with motor neuron disease ALZHEIMERS RESEARCH & THERAPY Takada, L. T., Sha, S. J. 2012; 4 (5)

    Abstract

    Earlier reports of chromosome 9p-linked frontotemporal dementia (FTD) with amyotrophic lateral sclerosis (ALS) kindreds observed psychosis as a prominent feature in some patients. Since the discovery of chromosome 9 open reading frame 72 (C9orf72) hexanucleotide expansions as a cause of FTD and ALS, research groups and consortia around the world have reported their respective observations of the clinical features associated with this mutation. We reviewed the recent literature on C9orf72-associated FTD and ALS with focus on the neuropsychiatric features associated with this mutation, as well as the experience at University of California, San Francisco. The results and methodologies varied greatly across studies, making comparison of results challenging. Four reports found that psychotic features (particularly delusions) were frequent among mutation carriers, particularly when present early during the disease course, suggesting that this symptom category may be a marker for the mutation. Disinhibition and apathy were the most commonly reported early behavioral symptoms, but these may not be helpful in distinguishing carriers and noncarriers because of the symptoms' frequency in sporadic behavioral variant FTD. Other neuropsychiatric features were reported in different frequencies across studies, suggesting either a similar behavioral phenotype in carriers and noncarriers or reflecting the heterogeneity in clinical presentation of behavioral variant FTD due to C9orf72 expansions. Further studies with larger cohorts will be necessary to determine the neuropsychiatric presentation associated with this mutation.

    View details for DOI 10.1186/alzrt141

    View details for Web of Science ID 000315195500004

    View details for PubMedID 23034079

  • Treatment implications of C9ORF72 ALZHEIMERS RESEARCH & THERAPY Sha, S. J., Boxer, A. 2012; 4 (6)

    Abstract

    Frontotemporal dementia (FTD) is a common dementia syndrome in patients under the age of 65 years with many features overlapping with amyotrophic lateral sclerosis (ALS). The link between FTD and ALS has been strengthened by the discovery that a hexanucleotide repeat expansion in a non-coding region of the C9ORF72 gene causes both familial and sporadic types of these two diseases. As we begin to understand the pathophysiological mechanisms by which this mutation leads to FTD and ALS (c9FTD/ALS), new targets for disease-modifying therapies will likely be unveiled. Putative C9ORF72 expansion pathogenic mechanisms include loss of C9ORF72 protein function, sequestration of nucleic acid binding proteins due to expanded hexanucleotide repeats, or a combination of the two. New animal models and other research tools informed by work in other repeat expansion neurodegenerative diseases such as the spinocerebellar ataxias will help to elucidate the mechanisms of C9ORF72-mediated disease. Similarly, re-examining previous studies of drugs developed to treat ALS in light of this new mutation may identify novel FTD treatments. Ultimately, research consortiums incorporating animal models and well-characterized clinical populations will be necessary to fully understand the natural history of the c9FTD/ALS clinical phenotypes and identify biomarkers and therapeutic agents that can cure the most common form of genetically determined FTD and ALS.

    View details for DOI 10.1186/alzrt149

    View details for Web of Science ID 000315195900003

    View details for PubMedID 23186535

  • Are frontotemporal lobar degeneration, progressive supranuclear palsy and corticobasal degeneration distinct diseases? NATURE CLINICAL PRACTICE NEUROLOGY Sha, r. S., Hou, C., Viskontas, I. V., Miller, B. L. 2006; 2 (12): 658-665

    Abstract

    New findings relating to the clinical, genetic and molecular bases of neurodegenerative disorders have led to a shift away from traditional nomenclatures of clinical syndromes. Historically, frontotemporal lobar degeneration (FTLD), corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) were classified on the basis of distinct clinical and pathological features. In recent years, however, advances in molecular and genetic research have led clinicians to suggest that the similar etiologies of the three disorders warrant their amalgamation into a single disorder with three subtypes. In this Review, we consider the utility and validity of combining FTLD, CBD and PSP. The earliest reports of these disorders demonstrate their distinctiveness, whereas recent findings challenge traditional nomenclatures by showing etiological overlap. For example, tau inclusions have been confirmed in patients with CBD and those with PSP, and in some patients with FTLD, implying that all three disorders are 'tauopathies'. Furthermore, most patients with progressive nonfluent aphasia, a subtype of FTLD, show PSP or CBD post-mortem. Even tau-related cases of FTLD, CBD and PSP are distinguishable on the basis of other criteria, however, and many FTLD cases do not show tau pathology. We argue, therefore, that FTLD, CBD and PSP should be considered as pathologically similar but distinct syndromes. New research criteria for CBD and PSP should note that progressive nonfluent aphasia is often a precursor of these conditions.

    View details for DOI 10.1038/ncpneuro0357

    View details for Web of Science ID 000242169100009

    View details for PubMedID 17117169

  • Distinctive neuropsychological patterns in frontotemporal dementia, semantic dementia, and Alzheimer disease. Cognitive and behavioral neurology Kramer, J. H., Jurik, J., Sha, S. J., Rankin, K. P., Rosen, H. J., Johnson, J. K., Miller, B. L. 2003; 16 (4): 211-218

    Abstract

    To assess the ability of a brief neuropsychological bedside screening battery to discriminate between Alzheimer disease, frontotemporal dementia, and semantic dementia.Subjects were 21 patients with frontotemporal dementia, 14 patients with semantic dementia, and 30 patients with Alzheimer disease comparable in terms of Mini Mental Status Examination score, age, and education. Frontotemporal dementia and semantic dementia diagnoses were made clinically using the consensus criteria of Neary et al. 1 Subjects were administered a brief neuropsychological screening assessing episodic memory, working memory, executive function, naming, spatial ability, abstract reasoning, and calculations.Both the Alzheimer disease and semantic dementia groups were significantly impaired relative to the frontotemporal dementia group on verbal memory, whereas only the Alzheimer disease group was impaired on visual memory. Frontotemporal dementia patients performed significantly worse on backward digit span and made significantly more executive errors than Alzheimer disease and semantic dementia patients. Semantic dementia patients were more impaired than Alzheimer disease and frontotemporal dementia patients on confrontation naming. Discriminant function analyses identified the 5 most discriminating variables that correctly classified 89.2% of cases.Frontotemporal dementia, semantic dementia, and Alzheimer disease are associated with distinct neuropsychological profiles that classify these dementia syndromes with considerable success. The neuropsychological profiles highlight the distinctiveness between the 3 syndromes, are consistent with the known loci of neuropathology in these conditions, and can potentially serve as an adjunct to the current clinical criteria.

    View details for PubMedID 14665820

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