Abstract
Pelizaeus-Merzbacher disease (PMD) is classified into six types. Patients with type II PMD show congenital nystagmus, hypotonia, rigidity of the trunk and extremities, and mental and speech retardation as the main clinical symptoms. Brain MRIs show a high signal intensity in the white matter and a low signal intensity in the gray matter of the cerebrum. ABR tracings commonly show only waves I and II.
We herein report four children with Pelizaeus–Merzbacher disease whose brain MRIs demonstrated abnormalities in the cerebrum and their ABR tracings showed only waves I and II.
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References
Kaga K. Central auditory pathway disorders. Japan: Springer; 2009.
Kaga K, Tamai F, Kodama M, et al. Three young adult patients with Pelizaeus-Merzbacher disease who showed only waves I and II in auditory brainstem responses but had good auditory perception. Acta Otolaryngol. 2005;125:1018–23.
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Kaga, K. (2021). Pelizaeus–Merzbacher Disease. In: Landau-Kleffner Syndrome and Central Auditory Disorders in Children. Modern Otology and Neurotology. Springer, Singapore. https://doi.org/10.1007/978-981-10-1479-6_12
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DOI: https://doi.org/10.1007/978-981-10-1479-6_12
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Publisher Name: Springer, Singapore
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