Sunday, December 02, 2007

Dementia mimicking FTD with striatal hypermetabolic

state and responsive to steroids.

Leger GC,Johnson N,Horowitz SW , et al. Dememtia like presentation of striatal hypermetabolic state with antistriatal antibodies responsive to steroids. Arch Neurol 2004; 61: 757-757.

Case presentation of a 48 year old woman with a one year history of progressive changes in pesonality and attention, originally diagnosed with a frontal dementia. PET showed a hypermetabolic lesion in the left striatum and plasma antistriatal antibodies were found. Treatment with corticosteroids resulted in a return of her premorbid state. PET scan and titers of antibody resolved. the differential diagnosis in this case was Syndenham's chorea and theantiphospholipid antibody state.

the phenotype of Sydenhams is usuallykids with onset of chorea, facial grimacing, hypotonia, and loss of fine control. Elevated ASO titers are present. Mood changes and OCD behavior may occur. Epitypes of IgM ASO titers cros react with striatum. It typically is self-limited and immune modulation may be useful. SLE occassionally occurs.

Dementia in women preceded by weight loss

Knopman DS et al. Incident dementia in women is preceded by weight loss by at least a decade. Neurology 2007; 69:739-746.

Data is mixed. Weight loss isnoted at diagnosis, but obesity is a risk factor. Weight loss progresses with dementia. The Rochester Epidemiology project. Women began to lose weight 11-20 years prior to onset of dementia.

Progressive supranuclear palsy.-parkinsonism

Kaat LD, Boon AJW, Kamphorst W etal.,Frontal presentation in PSP. Neurology 2007; 69:723-729

Diagnostic accuracy is poor (70 % initial misdiagnosis, 20 % terminal misdiagnosis). International criteria include falls in first year and vertical supranuclear palsy. PSP parkinsonism is a phenotype with assymmetric onset, tremor, levodopa response and longer disease duration. (see Williams DR et al. Brain 2005). A different phenotype of PSP is behavioral changes with impaired executive functions that may overlap FTD.

Clinical signs that were significant were abnormal score on the Thurstone (word fluency) with results often less than 3 or 5; applause sign in 72 % (, and 85 % were abnormal when detailed cognitive evaluation was performed. These included especially abnormal executive functions, mental slowing, memory disturbance and change in personality.

Unreliable: dyskinesia, cortical sensory loss, dystonia and and cerebellar signs (all < 3 %). The most common misdiagnosis was FTD. Urge incontinence was both significantly higher (2x) than PD and with increased mortality, as was older age. Phenotype did not affect survival. Both had median survival around 8 years. Article suggests importance of Thurstone and personality changes (applause sign) increase the sensitivity of diagnosis.

Dementia with LB and PDD. Can MRI make difference?

editorial Seppi K, Rascol O. Dementia with lewy bodies and Parkinsondisease with dementia. Can MRI make the difference? Neurology 2007; 69: 717-718 (editorial) and aricle
Beyer MK, Larsen JP, Aarsland D. Gray matter atrophy in Parkinson disease with dementia and dementia with Lewy bodies. Neurology 2007; 69: 747-754.

Article shows DLB had more cortical atrophy than patients with PDD especially in temporal, parietal and occipital lobes. The editorial cites the clinical rule, that if dementia occurs within 12 months of the PD, its DLB, but if more than that, its PDD.

Neuropathology is not always different as both conditions share widespread ubiquitin, alpha synuclein positive neuronal inclusion LB. The editorial cautions that the issue remains unsettled whether DLB and PDD are one or two diseases.