Efferent and afferent evoked potentials in patients with adrenomyeloneuropathy.
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.
Matsumoto
Hideyuki
H
Department of Neurology, University of Tokyo, Japan. hideyukimatsumoto@mail.goo.ne.jp
Hanajima
Ritsuko
R
Terao
Yasuo
Y
Hamada
Masashi
M
Yugeta
Akihiro
A
Shirota
Yuichiro
Y
Yuasa
Kaoru
K
Sato
Fumio
F
Matsukawa
Takashi
T
Takahashi
Yuji
Y
Goto
Jun
J
Tsuji
Shoji
S
Ugawa
Yoshikazu
Y
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
C115572:ABCD1 protein, human; D000074063:ATP Binding Cassette Transporter, Subfamily D, Member 1; D018528:ATP-Binding Cassette Transporters