Neuroimaging and clinical features in type II (late-onset) Alexander disease.

ABSTRACT:

Objective

To describe the imaging and clinical features in type II (late-onset) Alexander disease (AxD).

Methods

We retrospectively identified all cases of type II AxD evaluated at Mayo Clinic, Rochester from January 1996 to February 2012. Clinical and neuroimaging data abstracted from the record included age at onset of symptoms, age at diagnosis, first symptom, neurologic symptoms, physical/neurologic findings on examination, genetic testing and/or biopsy (if performed), and MRI findings.

Results

Thirteen patients with type II AxD were identified. Median age at onset was 38 years (range: 12-63). Five patients were female. Eleven of 13 patients had atrophy of the medulla while all 13 had medullary T2 hyperintensity. In 7 patients, these brainstem regions showed patch

SUBMITTER: Graff-Radford J 

PROVIDER: S-EPMC3873623 | biostudies-literature | 2014 Jan

REPOSITORIES: biostudies-literature