Multiplex families with multiple system atrophy.

Background: Multiple system atrophy (MSA) has been considered a sporadic disease, without patterns of inheritance.

Objective: To describe the clinical features of 4 multiplex families with MSA, including clinical genetic aspects.

Design: Clinical and genetic study.

Mimics of brain tumor on neuroimaging: part II.

Brain tumor is a distinct pathological entity that differs from other diseases, including cerebrovascular, demyelinating, inflammatory, infectious, and various miscellaneous diseases. Insidious onset and gradual progression of signs and symptoms are common in patients with brain tumors, whereas the onset of cerebrovascular diseases is usually acute or sudden. Patients with demyelinating, inflammatory, or infectious diseases show subacute onset.

Mimics of brain tumor on neuroimaging: part I.

Brain tumor is a distinct pathological entity that differs from other diseases, including cerebrovascular, demyelinating, inflammatory, infectious, and various miscellaneous diseases. Insidious onset and gradual progression of signs and symptoms are common in patients with brain tumors, whereas the onset of cerebrovascular diseases is usually acute or sudden. Patients with demyelinating, inflammatory, or infectious diseases show subacute onset.

MR features of diseases involving bilateral middle cerebellar peduncles.

Background And Purpose: Distribution of lesions or involvement of specific anatomic sites can suggest the diagnosis of disease. The purpose of this study was to investigate what diseases affect both middle cerebellar peduncles (MCPs) and to evaluate other MR features for differential diagnosis.

Methods: MR findings of 27 patients (14 male and 13 female; age range, 4-77 years [mean, 48.

Late-onset frontotemporal dementia with a novel exon 1 (Arg5His) tau gene mutation.

We report a case of frontotemporal dementia and parkinsonism linked to chromosome 17 of 5 years' duration in an 81-year-old man whose brother had died at age 86 years with dementia. In this patient, we found frontal and temporal neuronal loss, glial-predominant tau deposits, progressive supranuclear palsy-like straight tubules, accumulation of 4-repeat-predominant Sarkosyl-insoluble tau, and a novel exon 1 (Arg5His) tau gene mutation. This mutation decreased microtubule-promoting capacity and increased fibrillation of tau in vitro.