Corticobasal syndrome, Primary progressive aphasia, Dementia with Lewy bodies
Conditions
Interventions
Neuropshychological tests and Dopamine transporter scan
Sponsors
Juntendo University school of Medicine Department of Diagnosis, Prevention and Treatment of Dementia
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. corticobasal syndrome (CBS,J Neurol Neurosurg Psychiatry. 2012;83(4):405) 1. Mandatory criteria 1-1 Insidious onset and gradual progression 1-2 No sustained response to levodopa treatment 2. Major and minor criteria 2-1 Motor features Akinetic rigid syndrome Focal or segmental myoclonus Asymmetrical dystonia 2-2 Cortical motor sensory features Limb apraxia Alien limb phenomenon Cortical sensory loss or dyscalculia 2-3 Cognitive features Speech and language impairment Frontal executive dysfunction Visuospatial deficits 2. Dementia with Lewy bodies (DLB, Neurology 2005;65;1863) 1. Central feature Dementia defined as progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational function. Deficits on tests of attention, executive function, and visuospatial ability may be especially prominent. 2. Core features (two core features are sufficient for a diagnosis of probable DLB, one for possible DLB) 2-1 Fluctuating cognition with pronounced variations in attention and alertness 2-2 Recurrent visual hallucinations that are typically well formed and detailed 2-3 Spontaneous features of parkinsonism 3. Suggestive features (If one or more of these is present in the presence of one or more core features, a diagnosis of probable DLB can be made. In the absence of any core features, one or more suggestive features is sufficient for possible DLB. Probable DLB should not be diagnosed on the basis of suggestive features alone.) 3-1 REM sleep behavior disorder 3-2 Severe neuroleptic sensitivity 3-3 Low dopamine transporter uptake in basal ganglia demonstrated by SPECT or PET imaging 3. Primary progressive aphasia (PPA, Neurology 2011;76:1006) 1. Most prominent clinical feature is difficulty with language 2. These deficits are the principal cause of impaired daily living activities 3. Aphasia should be the most prominent deficit at symptom onset and for the initial phases of the disease
Exclusion criteria
Exclusion criteria: 1) Cognitive dysfunction, systemic diseases or central nervous system other than CBS, PPA, and DLB (Schizophrenia, Bipolar disorder, Parkinson's disease, Vascular dementia, Huntington disease, brain tumor, Pick disease, Creutzfeldt-Jakob disease,Normal pressure hydrocephalus, Progressive supranuclear palsy,cerebral stroke, subdural hematoma, multiple sclerosis, epilepsy, HIV infection, neurosyphilis) 2. In the presence of any other physical illness such as Vitamine deficiency 3. In case physicians judge that the participants are mismatched.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Dopamine transporter scan | — |
Secondary
| Measure | Time frame |
|---|---|
| Neurospychological battery | — |
Countries
Japan
Contacts
Public ContactYumiko Motoi
Juntendo University school of Medicine Department of Diagnosis, Prevention and Treatment of Dementia
Outcome results
None listed