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1.  Primary progressive aphasia and transient global amnesia 
Archives of neurology  2012;69(3):401-404.
Objective
To report three patients with history of transient global amnesia who developed primary progressive aphasia.
Patients
Three patients presenting to the Neurology clinic with language complaints
Setting
Tertiary care center
Results
We describe three patients with a history of transient global amnesia who were subsequently diagnosed with primary progressive aphasia. All patients had recurrent attacks of transient global amnesia. The diagnoses of primary progressive aphasia were supported by speech pathology evaluations, neuropsychometric testing and imaging findings. PET scans, for example, revealed left posterior frontal hypometabolism in one patient, predominately left temporal-parietal hypometabolism in another while single-photon emission computed tomography demonstrated decreased perfusion in the anterior left temporal and frontal lobe in the third.
Conclusions
There may be a relationship between recurrent transient global amnesia and the development of primary progressive aphasia.
doi:10.1001/archneurol.2011.1129
PMCID: PMC3904294  PMID: 22410450
2.  Clinical correlates of white matter tract degeneration in PSP 
Archives of Neurology  2011;68(6):753-760.
Objective
Progressive supranuclear palsy (PSP) is associated with degeneration of white matter tracts that can be detected using diffusion tensor imaging (DTI). However, little is known about whether tract degeneration is associated with the clinical symptoms of PSP. The aim of this study was to use DTI to assess white matter tract degeneration in PSP and to investigate correlates, between tract integrity and clinical measures.
Design
Case-control study
Setting
Tertiary care medical centre
Patients
Twenty subjects with probable PSP and 20 age and gender-matched healthy controls. All PSP subjects underwent standardized clinical testing, including the Frontal Behavioral Inventory and Frontal Assessment Battery to assess behavioral change; the PSP Rating Scale to measure disease severity, the Movement Disorder Society-sponsored revision of the Unified Parkinson’s Disease Rating Scale (parts I, II and III) to measure motor function, and the PSP Saccadic Impairment Scale to measure eye movement abnormalities.
Main outcome measures
Fractional anisotropy and mean diffusivity measured using both region-of-interest analysis and Track Based Spatial Statistics.
Results
Abnormal diffusivity was observed predominantly in superior cerebellar peduncles, body of the corpus callosum, inferior longitudinal fasciculus and superior longitudinal fasciculus in PSP compared to controls. Fractional anisotropy values in the superior cerebellar peduncles correlated with disease severity; inferior longitudinal fasciculus correlated with motor function, and superior longitudinal fasciculus correlated with severity of saccadic impairments.
Conclusions
These results demonstrate that PSP is associated with degeneration of brainstem, association and commissural fibers and that this degeneration likely plays an important role in clinical dysfunction.
doi:10.1001/archneurol.2011.107
PMCID: PMC3401587  PMID: 21670399
3.  Very Early Semantic Dementia With Progressive Left≫Right Temporal Lobe Atrophy: An Eight-Year Longitudinal Study 
Archives of neurology  2008;65(12):1659-1663.
Background
Semantic dementia (SD) is a syndrome within the spectrum of frontotemporal lobar degenerations (FTLD) characterized by fluent progressive aphasia (particularly anomia) and loss of word meaning.
Objective
To report a unique case of very early semantic dementia with slowly progressive course allowing insights into the early natural history of this disorder.
Design
Case report.
Setting
Tertiary care university hospital and academic center.
Patient
A 62-year-old female retired teacher presenting with “memory” complaints.
Main Outcome Measures
Clinical course, neuropsychological data, MRI.
Results
The patient was first evaluated when standard neuropsychological measures were normal, but subtle left anterior temporal lobe atrophy was present. Over the follow-up period of eight years, she developed profound anomia and loss of word meaning associated with progressive left anterior temporal lobe atrophy consistent with semantic dementia. In more recent years, anterograde memory impairment as well as mild prosopagnosia have evolved in association with left hippocampal atrophy and subtle atrophy in the homologous gyri of the right anterior temporal lobe. She remains functionally independent despite her current deficits.
Conclusions
Early identification of patients who will develop semantic dementia is difficult and might be missed with standard clinical, neuropsychological, and structural neuroimaging evaluations. Recognition of this relatively rare syndrome is important for early diagnosis and prognostication, and particularly for therapeutic interventions in the future.
doi:10.1001/archneurol.2008.507
PMCID: PMC2902001  PMID: 19064755
frontotemporal lobar degeneration; semantic dementia; MRI; neuropsychology
4.  Survival Profiles of Patients With Frontotemporal Dementia and Motor Neuron Disease 
Archives of neurology  2009;66(11):1359-1364.
Background
Frontotemporal dementia and amyotrophic lateral sclerosis are neurodegenerative diseases associated with TAR DNA-binding protein 43– and ubiquitin-immunoreactive pathologic lesions.
Objective
To determine whether survival is influenced by symptom of onset in patients with frontotemporal dementia and amyotrophic lateral sclerosis.
Design, Setting, and Patients
Retrospective review of patients with both cognitive impairment and motor neuron disease consecutively evaluated at 4 academic medical centers in 2 countries.
Main Outcome Measures
Clinical phenotypes and survival patterns of patients.
Results
A total of 87 patients were identified, including 60 who developed cognitive symptoms first, 19 who developed motor symptoms first, and 8 who had simultaneous onset of cognitive and motor symptoms. Among the 59 deceased patients, we identified 2 distinct subgroups of patients according to survival. Long-term survivors had cognitive onset and delayed emergence of motor symptoms after a long monosymptomatic phase and had significantly longer survival than the typical survivors (mean, 67.5 months vs 28.2 months, respectively; P<.001). Typical survivors can have simultaneous or discrete onset of cognitive and motor symptoms, and the simultaneous-onset patients had shorter survival (mean, 19.2 months) than those with distinct cognitive or motor onset (mean, 28.6 months) (P=.005).
Conclusions
Distinct patterns of survival profiles exist in patients with frontotemporal dementia and motor neuron disease, and overall survival may depend on the relative timing of the emergence of secondary symptoms.
doi:10.1001/archneurol.2009.253
PMCID: PMC2881327  PMID: 19901167
5.  Is Incidental Lewy Body Disease Related to Parkinson Disease? Comparison of Risk Factor Profiles 
Archives of neurology  2009;66(9):1114.
Objective
To explore whether associations of potential risk factors for incidental Lewy Body Disease (iLBD) may be similar to Parkinson Disease (PD).
Design, Setting, and Patients
We identified brain-autopsied residents of Olmsted County, MN and immediate vicinity(1988–2004), age>60, without evidence of neurodegenerative disease or tremor, and evaluated by at least one physician within one year of death. Analysis for “incidental” Lewy pathology was done blinded to clinical abstraction.
Main Outcome Measures
Whether risk factors previously associated with PD in Olmsted County, MN are also associated with iLBD.
Results
Of 235 subjects, 34 had iLBD(14.5%). The overall risk factor profiles for iLBD and PD were fairly similar between the two sets of OR estimates, with 11/16 ORs in the same direction. Prior Olmsted County studies documented 7 risk factors with statistically significant associations with PD; for two of these, the ORs for iLBD were in the same direction and statistically significant (physician, caffeine), whereas for three, they were in the same direction but not significant (education, head injury, number-of-children); they were in the opposite direction but not statistically significant for 2 (depression, anxiety). ILBD was not associated with various end-of-life conditions or causes-of-death, although they were slightly older and more likely cachectic.
Conclusions
Based on this exploratory study, iLBD and PD appear to have similar risk factor profiles. Thus, at least some cases of ILBD might represent preclinical PD, arrested PD or a partial syndrome due to a lesser burden of causative factors. ILBD is not explained by non-specific end-of-life brain insults.
doi:10.1001/archneurol.2009.170
PMCID: PMC2813519  PMID: 19752300
6.  Rapidly Progressive Neurodegenerative Dementias 
Archives of neurology  2009;66(2):201-207.
Background
Neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. Less common are patients with a rapidly progressive course to death.
Objective
To characterize patients with a neurodegenerative disease and a rapidly progressive course to death.
Setting
Tertiary Care Medical Center.
Design/Methods
Using a text word search for “rapid” and “dementia” in the same sentence, the Mayo Clinic Medical Records Linkage system was used to identify all patients evaluated between 1/1/00−9/30/07 with brain autopsy (N=96). Of these 96, we included only those with disease duration of <4 years to death and with histological diagnosis of a neurodegenerative disease.
Results
We identified 22 cases (10 males). Although 36% were Creutzfeldt-Jakob disease (CJD), the rest included frontotemporal lobar degenerative with motor neuron degeneration (FTLD-MND; 23%); a tauopathy (progressive supranuclear palsy or corticobasal degeneration; 18%); diffuse Lewy body disease (DLBD; 14%) or Alzheimer's disease 9%. All CJD cases died ≤12 months after onset while the others had illness duration of >12 months. Notably, all three DLBD patients, but no others, initially experienced a transient postoperative- or illness-associated encephalopathy, then relative normality for two years, before a rapidly progressive dementia and decline to death in 4−12 months.
Conclusions
Based on this cohort, although CJD is the most likely cause of a rapidly progressive neurodegenerative dementia, FTLD-MND, DLBD, tauopathies and Alzheimer's disease can also cause a rapidly progressive dementia. If illness duration is beyond 12-months, a non-CJD neurodegenerative disease may be more likely the diagnosis, than CJD.
doi:10.1001/archneurol.2008.534
PMCID: PMC2764283  PMID: 19204156
7.  Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations 
Archives of neurology  2007;64(3):371-376.
Background
Mutations in the progranulin gene (PGRN) have recently been identified as a cause of frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) in some families.
Objective
To determine whether there is a difference in the patterns of atrophy in cases with FTLD-U with and without a mutation in PGRN.
Design
Case control study
Setting
Brain bank of a tertiary care medical center
Patients
All subjects that had screened positive for mutations in PGRN and had a volumetric MRI were identified (n=8, PGRN (+)). Subjects were then matched by clinical diagnosis to a group of eight subjects with a pathological diagnosis of FTLD-U that had screened negative for mutations in PGRN (PGRN (−)). All subjects were then age and gender-matched to a control subject.
Main outcome Measures
Voxel-based morphometry was used to assess the patterns of grey matter atrophy in the PGRN (+) and (−) groups compared to controls, and compared to each other.
Results
The PGRN (+) group showed a widespread and severe pattern of grey matter loss predominantly affecting the frontal, temporal and parietal lobes. In comparison, the PGRN (−) group showed a less severe pattern of loss restricted mainly to the temporal and frontal lobes. On direct comparison the PGRN (+) group showed greater loss in the frontal and parietal lobes compared to the PGRN (−) group.
Conclusions
This study suggests that PGRN mutations may be associated with a specific and severe pattern of cerebral atrophy in subjects with FTLD-U.
doi:10.1001/archneur.64.3.371
PMCID: PMC2752412  PMID: 17353379
Frontotemporal dementia; Voxel-based morphometry; Ubiquitin; Dentate; Progranulin
8.  Visual Hallucinations in Posterior Cortical Atrophy 
Archives of neurology  2006;63(10):1427-1432.
Objective
To compare clinical and imaging features of patients with posterior cortical atrophy (PCA) with and without well-formed visual hallucinations.
Setting
Tertiary care medical center
Methods
Fifty-nine patients fulfilling criteria for PCA were retrospectively identified, and divided into two groups based on the presence (N=13) and absence (N=46) of visual hallucinations. Both groups were then compared statistically for clinical differences, as well as with voxel-based morphometry (VBM) for imaging differences.
Results
In PCA patients with hallucinations, parkinsonism and rapid eye movement sleep behavior disorder occurred more frequently (p<0.0001), as did myoclonic jerks (p=0.0002). VBM analysis showed greater atrophy in a network of structures, including the primary visual cortex, lentiform nuclei, thalamus, basal forebrain and midbrain in the patients with hallucinations.
Conclusions
Hallucinations in patients with PCA are associated with parkinsonism, rapid eye movement sleep behavior disorder, and myoclonic jerks. The results from the VBM analysis suggest that hallucinations in PCA cannot be exclusively attributed to atrophy of the posterior association cortices and may involve a circuit of thalamocortical connections.
doi:10.1001/archneur.63.10.1427
PMCID: PMC2748870  PMID: 17030659
Parkinsonism; Thalamus; Myoclonic jerks; REM sleep; Voxel based morphometry

Results 1-8 (8)