Amyotrophic lateral sclerosis-plus syndrome with TAR DNA-binding protein-43 pathology.

ABSTRACT:

Background

Amyotrophic lateral sclerosis (ALS)-Plus syndromes meet clinical criteria for ALS but also include 1 or more additional features such as dementia, geographic clustering, extrapyramidal signs, objective sensory loss, autonomic dysfunction, cerebellar degeneration, or ocular motility disturbance.

Methods

We performed a whole-brain and spinal cord pathologic analysis in a patient with an ALS-Plus syndrome that included repetitive behaviors along with extrapyramidal and supranuclear ocular motility disturbances resembling the clinical phenotype of progressive supranuclear palsy.

Results

There was motoneuron cell loss and degeneration of the corticospinal tracts. Bunina bodies were present. TAR DNA-binding protein-43 pathology was diffuse. Significant tau pathol

SUBMITTER: McCluskey LF 

PROVIDER: S-EPMC2702763 | biostudies-literature | 2009 Jan

REPOSITORIES: biostudies-literature