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From the Department of Neurology (Drs. van Geel and Barth) and the Unit of Clinical Neurophysiology (Drs. Koelman and Ongerboer de Visser), Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.
Supported in part by grant 28-19420 from the Praeventiefonds, The Hague, the Netherlands.
Received March 22, 1995. Accepted in final form May 29, 1995.
Address correspondence and reprint requests to Dr van Geel, Department of Neurology, Academic Medical Center, Meibergdreef 9, 1105 AZ Amsterdam ZO, the Netherlands.
Article abstract-Adrenomyeloneuropathy (AMN) is one of the two most frequent phenotypes of X-linked adrenoleukodystrophy. Whether the polyneuropathy in AMN results from primary demyelination or axonal degeneration is uncertain. We examined 23 patients (18 men with AMN and five female carriers with AMN symptomatology), performed electroneurography and EMG, and compared our results with standardized electrodiagnostic criteria for primary demyelination. Both clinically and electrodiagnostically, the lower extremities were most frequently and most severely affected. A longer duration of symptoms was related to more severe pyramidal dysfunction (p < 0.004) and spasticity (p < 0.04), and to a more severe impairment of vibration sense (p < 0.05). There were no correlations between the different electrophysiologic studies and the duration of neurologic symptoms. Only two AMN patients (9%) fulfilled the electrodiagnostic criteria for primary demyelination. However, both had abnormally low compound muscle action potentials, which may have been a reflection of primary axonal degeneration. Six other patients (26%) partially fulfilled the criteria for primary demyelination, of whom five also manifested low compound muscle action potentials. In 15 patients (65%), we found polyneuropathy with predominantly axonal, sensorimotor features. We conclude that the neuropathy in AMN patients is due to primary axonal degeneration.
NEUROLOGY 1996;46: 112-118
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