Abnormal Somatosensory Evoked Potentials in Patients With Classic Galactosemia: Correlation With Neurologic Outcome
| Autor: | Peggy S. Gott, Franklin R. Manis, Francine R. Kaufman, Marvin D. Nelson, Elizabeth J. Horton, Colleen Azen, Jon A. Wolff |
|---|---|
| Rok vydání: | 1995 |
| Předmět: |
Adult
Galactosemias Male 030213 general clinical medicine Ataxia Adolescent Neural Conduction 030209 endocrinology & metabolism Nerve Fibers Myelinated 03 medical and health sciences 0302 clinical medicine Evoked Potentials Somatosensory Dysmetria Tremor Severity of illness medicine Humans Child Brain Diseases medicine.diagnostic_test Galactosemia Brain Infant Magnetic resonance imaging medicine.disease Median nerve Median Nerve Somatosensory evoked potential Child Preschool Anesthesia Pediatrics Perinatology and Child Health Female Cerebellar atrophy Neurology (clinical) Tibial Nerve medicine.symptom Psychology |
| Zdroj: | Journal of Child Neurology. 10:32-36 |
| ISSN: | 1708-8283 0883-0738 |
| DOI: | 10.1177/088307389501000109 |
| Popis: | In classic galactosemia, long-term neurologic sequelae can include low cognitive functioning and a curious neurologic syndrome with tremors, dysmetria, and ataxia. An abnormal white-matter signal on cerebral magnetic resonance imaging (MRI) is present in almost all patients; some have mild cerebral or cerebellar atrophy and focal white-matter lesions. The present study was undertaken to assess the integrity of myelinated pathways by recording somatosensory evoked potentials. Results were correlated with age at diagnosis, severity of illness, age at evoked potentials, neurologic examination, MRI studies and cognitive outcome as measured by the Woodcock-Johnson Revised Standard Cognitive Battery. Evoked potentials were abnormal in 17 (28%) of 60 patients who had median nerve, and 26 (77%) of 34 patients who had posterior tibial nerve studies. Abnormalities of the central rather than the peripheral nervous system were most common. Evoked potentials correlated with severity of presenting symptoms (P = .011), age at evoked potential testing (P = .029), and presence of focal white-matter lesions on MRI (P = .049). Results of neurophysiologic testing showed no correlation with the Woodcock-Johnson Battery. Patients with classic galactosemia may have abnormal conduction along myelinated pathways that is associated with other central deficits. Myelin, which contains galactose, may be adversely affected in this inborn error of metabolism. (J Child Neurol 1995;10:32-36). |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|