title: Transcranial brainstem sonography as a diagnostic tool for amyotrophic lateral sclerosis.
abstract: Diagnosing amyotrophic lateral sclerosis (ALS) can be difficult, particularly in the early stage of disease; therefore, we evaluated the use of transcranial stem sonography (TCS) to improve early detection of the disease. In this cross-sectional study, 94 patients with sporadic ALS and 46 age- and gender-matched healthy controls were evaluated by TCS according to a standardized protocol used to diagnose Parkinson's disease. Approximately half (48%) of the patients with ALS showed a clear (> 0.25 cm(2)) mesencephalic hyperechogenic structure, 20% showed a possible (< 0.25 cm(2)) hyperechogenic structure and 24% patients showed no hyperechogenic structure in the brainstem. TCS findings were not correlated with gender, disease onset (spinal, bulbar), disease duration, ALSFRS-R scores, motor-evoked potentials and signal hyperintensities in conventional MRI. In 70% of the ALS patients these hyperechogenicities were found at the anatomical site of the substantia nigra. In terms of location and structure, hyperechogenicities in 30% of ALS patients were more heterogeneous than those in Parkinson's disease with pronounced extensions both rostrally and laterally. In conclusion, although the neuropathological correlation to hyperechogenicity remains unclear, TCS is an easy, feasible and reproducible technique that could serve as an additional diagnostic tool and as a surrogate biomarker in ALS.
authors.affiliation: Hans-Berger Department of Neurology, Jena University Hospital , Jena.
authors.forename: Tino
authors.initials: T
authors.lastname: Prell
authors.forename: Annekathrin
authors.initials: A
authors.lastname: Schenk
authors.forename: Otto W
authors.initials: OW
authors.lastname: Witte
authors.forename: Julian
authors.initials: J
authors.lastname: Grosskreutz
authors.forename: Albrecht
authors.initials: A
authors.lastname: Günther
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D001933:Brain Stem / Q000473:pathology / Q000648:ultrastructure*; D003430:Cross-Sectional Studies; D038524:Diffusion Magnetic Resonance Imaging; D004569:Electroencephalography; D019054:Evoked Potentials, Motor / Q000502:physiology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D012189:Retrospective Studies; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D011795:Surveys and Questionnaires; D017585:Ultrasonography, Doppler, Transcranial
keywords: Amyotrophic lateral sclerosis; corticospinal tract; substantia nigra