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Measurement of volumetric changes with MR might be a useful surrogate endpoint for clinical trials in frontotemporal lobar degeneration (FTLD). Because there is only limited longitudinal imaging data currently available, we measured the rate of change over 1 year of whole brain volume (WBV) and ventricular volume (VV) in patients with FTLD.

To estimate prevalence, incidence, and rate of progression of mild cognitive impairment (MCI) to dementia and correlated vascular risk factors with incident MCI and its progression to dementia.

To examine the clinical, genetic, and neuropathologic features of posterior cortical atrophy (PCA).

To determine, in a cross-sectional evaluation of nondemented individuals, if age-related memory decline is influenced by apolipoprotein E (apoE) genotype.

Early reports suggested that corticobasal degeneration (CBD) is a distinct clinicopathologic entity. Because patients have had a fairly consistent constellation of clinical and laboratory findings, many have proposed that the pathologic diagnosis can be surmised with confidence during life.

Eight patients presented with slowly progressive focal neurologic syndromes that conformed to one of three clinically defined categories: progressive nonfluent aphasia (three patients), progressive perceptual-motor impairment (four patients), and progressive frontal lobe syndrome (one patient). Planar MRI and MRI-based surface or volume renderings demonstrated focal areas of atrophy that correlated well with clinical deficits. Single-photon emission computed tomography (SPECT) showed areas of cortical hypoperfusion that corresponded to focally atrophic regions revealed by MRI, but abnormal areas with SPECT were larger than those suggested by MRI. MRI and SPECT are useful in defining the regional structural and functional cerebral abnormalities that underlie slowly progressive focal neurologic syndromes caused by asymmetric cortical degeneration.

BACKGROUND Nonvasculitic autoimmune inflammatory meningoencephalitis and Creutzfeldt-Jakob disease can present as rapidly progressive encephalopathies with similar clinical features. Slowing of background rhythm is an electroencephalographic characteristic shown by both, but persistent periodic sharp waves are more specific for Creutzfeldt-Jakob disease and have not been reported in nonvasculitic autoimmune inflammatory meningoencephalitis or related autoimmune meningoencephalitides. OBJECTIVE To describe a patient with clinical (rapidly progressive myoclonus, dementia, and Parkinsonism) and electroencephalographic findings (persistent periodic sharp waves) that diagnostically suggest Creutzfeldt-Jakob disease. DESIGN AND SETTING A case report at the Mayo Clinic Arizona, Scottsdale. RESULTS The patient made a dramatic recovery with resolution of the periodic sharp wave complexes after treatment with high-dose corticosteroids. Our case is the first reported case of a patient with probable nonvasculitic autoimmune inflammatory meningoencephalitis and electroencephalographic periodic complexes suggestive of Creutzfeldt-Jakob disease. CONCLUSION Rapidly progressive encephalopathy with periodic sharp wave complexes can be associated with a reversible autoimmune syndrome.Arch Neurol. 2006;63:766-768 --

Five patients, age 54 to 80 years, presented between 3 weeks and 18 months after symptomatic onset of progressive cognitive decline, psychosis, and unsteady gait that proved to be due to a steroid-responsive nonvasculitic autoimmune inflammatory meningoencephalitic syndrome. CSF examination showed elevated immunoglobulin (Ig)G index and IgG synthesis rate in all three patients in whom it was checked, and brain biopsy revealed perivascular lymphocytic infiltrates without vessel wall invasion.