title: Efferent and afferent evoked potentials in patients with adrenomyeloneuropathy.
abstract: OBJECTIVE
  This paper investigates efferent and afferent conductions of the central nervous system by various evoked potentials in patients with adrenomyeloneuropathy (AMN).
  
  
  METHODS
  Ten pure AMN patients without cerebral involvement were studied. Motor evoked potentials (MEPs), somatosensory evoked potentials (SEPs), auditory brainstem response (ABR), and pattern reversal full-field visual evoked potentials (VEPs) were recorded. For MEP recording, single-pulse or double-pulse magnetic brainstem stimulation (BST) was also performed.
  
  
  RESULTS
  Abnormal MEP was observed in all ten patients, abnormal SEP in all ten, abnormal ABR in nine, and abnormal VEP in only one. Brainstem latency was measured in three of the seven patients with central motor conduction time (CMCT) prolongation. The cortical-brainstem conduction time was severely prolonged along the normal or mildly delayed brainstem-cervical conduction time in those three patients.
  
  
  CONCLUSIONS
  The pattern of normal VEP and abnormal MEP, SEP, ABR is a clinically useful electrophysiological feature for the diagnosis. BST techniques are helpful to detect, functionally, intracranial corticospinal tract involvement, probably demyelination, in pure AMN patients.
authors: Department of Neurology, University of Tokyo, Japan. hideyukimatsumoto@mail.goo.ne.jp
authors: Hideyuki
authors: H
authors: Matsumoto
authors: Ritsuko
authors: R
authors: Hanajima
authors: Yasuo
authors: Y
authors: Terao
authors: Masashi
authors: M
authors: Hamada
authors: Akihiro
authors: A
authors: Yugeta
authors: Yuichiro
authors: Y
authors: Shirota
authors: Kaoru
authors: K
authors: Yuasa
authors: Fumio
authors: F
authors: Sato
authors: Takashi
authors: T
authors: Matsukawa
authors: Yuji
authors: Y
authors: Takahashi
authors: Jun
authors: J
authors: Goto
authors: Shoji
authors: S
authors: Tsuji
authors: Yoshikazu
authors: Y
authors: Ugawa
mesh_terms: D000074063:ATP Binding Cassette Transporter, Subfamily D, Member 1; D018528:ATP-Binding Cassette Transporters / Q000235:genetics; D000326:Adrenoleukodystrophy / Q000235:genetics / Q000503:physiopathology*; D000328:Adult; D000344:Afferent Pathways / Q000502:physiology*; D017668:Age of Onset; D001933:Brain Stem / Q000503:physiopathology; D004525:Efferent Pathways / Q000502:physiology*; D004558:Electric Stimulation; D005071:Evoked Potentials / Q000502:physiology*; D005073:Evoked Potentials, Somatosensory / Q000502:physiology; D005074:Evoked Potentials, Visual / Q000502:physiology; D007839:Functional Laterality; D006801:Humans; D008875:Middle Aged; D009154:Mutation; D046709:Pattern Recognition, Physiological; D001405:Reflex, Babinski / Q000503:physiopathology
chemical_list: C115572:ABCD1 protein, human; D000074063:ATP Binding Cassette Transporter, Subfamily D, Member 1; D018528:ATP-Binding Cassette Transporters