title: The diagnostic value of midbrain hyperechogenicity in ALS is limited for discriminating key ALS differential diagnoses.
abstract: BACKGROUND
  Hyperechogenicity of the substantia nigra was recently reported in patients with sporadic ALS with a frequency similar to PD. Data on the diagnostic utility compared to key differential diagnoses of ALS do not exist yet.
  
  
  METHODS
  We prospectively enrolled 43 patients with ALS, 29 with myasthenia gravis, 25 patients with inflammatory neuropathy, and 13 with cervical canal stenosis. All patients were examined by a blinded investigator using transcranial B-mode sonography planimetrically measuring hyperechogenic areas of the midbrain representing the substantia nigra.
  
  
  RESULTS
  Mean midbrain hyperechogenic area was increased in ALS compared to non-ALS differentials. ROC analysis revealed only small area under the curve for detecting ALS (AUC: 0.669 [95%CI: 0.56-0.78]; p = 0.006). Highest Youden index was observed for area size of <0.14 cm(2) (Youden index: 0.28). Using this cut-off score and that generated from normative data of healthy controls, area size measurements provided a sensitivity of only 46-58% and specificity of 69-83% for detecting ALS. No correlations of hyperechogenic area sizes in ALS patients were found to age, gender, ALS subtype (bulbar versus spinal form), disease duration or ALS-FRS-R score.
  
  
  CONCLUSIONS
  Midbrain hyperechogenicity is reproducibly found in ALS patients, but its diagnostic value for discriminating ALS from its key differentials is limited.
authors: Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden and German Center for NeurodegenerativeDiseases (DZNE), Research site Dresden, Fetscherstrasse 74, 01307, Dresden, Germany. andreas.hermann@uniklinikum-dresden.de.
authors: Andreas
authors: A
authors: Hermann
authors: Department of Neurology, Technische Universität Dresden, Dresden, Germany. ulrike.reuner@uniklinikum-dresden.de.
authors: Ulrike
authors: U
authors: Reuner
authors: Department of Neurology, Technische Universität Dresden, Dresden, Germany. schaefer@rcs.urz.tu-dresden.de.
authors: Jochen
authors: J
authors: Schaefer
authors: Department of Neurology, University Ulm, Ulm, Germany. panteha.fathinia@uni-ulm.de.
authors: Panteha
authors: P
authors: Fathinia
authors: Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden and German Center for NeurodegenerativeDiseases (DZNE), Research site Dresden, Fetscherstrasse 74, 01307, Dresden, Germany. tordis.leimert@uniklinikum-dresden.de.
authors: Tordis
authors: T
authors: Leimert
authors: Department of Neurology, University Ulm, Ulm, Germany. jan.kassubek@uni-ulm.de.
authors: Jan
authors: J
authors: Kassubek
authors: Department of Neurosurgery, Technische Universität Dresden, Dresden, Germany. mario.leimert@uniklinikum-dresden.de.
authors: Mario
authors: M
authors: Leimert
authors: Department of Neurology, University Ulm, Ulm, Germany. sekretariat.neurologie@rku.de.
authors: Albert C
authors: AC
authors: Ludolph
authors: Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden and German Center for NeurodegenerativeDiseases (DZNE), Research site Dresden, Fetscherstrasse 74, 01307, Dresden, Germany. alexander.storch@uniklinikum-dresden.de.
authors: Alexander
authors: A
authors: Storch
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000000981:diagnostic imaging*; D002574:Cervical Vertebrae; D003937:Diagnosis, Differential; D004453:Echoencephalography; D005260:Female; D020275:Guillain-Barre Syndrome / Q000175:diagnosis / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D008636:Mesencephalon / Q000000981:diagnostic imaging; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000175:diagnosis / Q000000981:diagnostic imaging*; D011129:Polyradiculoneuropathy / Q000175:diagnosis / Q000000981:diagnostic imaging*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis / Q000000981:diagnostic imaging; D011446:Prospective Studies; D012372:ROC Curve; D012680:Sensitivity and Specificity; D013130:Spinal Stenosis / Q000175:diagnosis / Q000000981:diagnostic imaging*; D013378:Substantia Nigra / Q000000981:diagnostic imaging*; D055815:Young Adult