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Neurophysiologic assessment of mucopolysaccharidosis III.

Publication ,  Journal Article
Husain, AM; Escolar, ML; Kurtzberg, J
Published in: Clin Neurophysiol
September 2006

OBJECTIVE: To describe finding of various neurophysiologic tests in patients with mucopolysaccharidosis III (MPS III) early in the disease course. METHODS: Patients were evaluated with flash visual evoked potentials (VEP), brainstem auditory evoked potentials (BAEP), electroencephalography (EEG), and nerve conduction studies (NCS) before they underwent hematopoietic stem cell transplantation (HSCT). RESULTS: Thirteen children underwent at least one neurophysiologic test before HSCT. The mean age at testing was 2.7 years. Ten of 11 (91%) patients had a normal flash VEP, and all 9 who had BAEP had normal central conduction. EEG was normal in 7/13 (54%), with the others showing diffuse slowing. NCS was normal in 10/11 (91%) patients. CONCLUSIONS: Despite extensive central nervous system involvement in MPS III, flash VEP and BAEP are almost always normal. EEG is often abnormal early in the disease. SIGNIFICANCE: This is the first report of neurophysiologic tests in a large series of MPS III patients.

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Published In

Clin Neurophysiol

DOI

ISSN

1388-2457

Publication Date

September 2006

Volume

117

Issue

9

Start / End Page

2059 / 2063

Location

Netherlands

Related Subject Headings

  • Retrospective Studies
  • Neurophysiology
  • Neurology & Neurosurgery
  • Neural Conduction
  • Mucopolysaccharidosis II
  • Male
  • Infant
  • Humans
  • Female
  • Evoked Potentials, Visual
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Husain, A. M., Escolar, M. L., & Kurtzberg, J. (2006). Neurophysiologic assessment of mucopolysaccharidosis III. Clin Neurophysiol, 117(9), 2059–2063. https://doi.org/10.1016/j.clinph.2006.05.031
Husain, Aatif M., Maria L. Escolar, and Joanne Kurtzberg. “Neurophysiologic assessment of mucopolysaccharidosis III.Clin Neurophysiol 117, no. 9 (September 2006): 2059–63. https://doi.org/10.1016/j.clinph.2006.05.031.
Husain AM, Escolar ML, Kurtzberg J. Neurophysiologic assessment of mucopolysaccharidosis III. Clin Neurophysiol. 2006 Sep;117(9):2059–63.
Husain, Aatif M., et al. “Neurophysiologic assessment of mucopolysaccharidosis III.Clin Neurophysiol, vol. 117, no. 9, Sept. 2006, pp. 2059–63. Pubmed, doi:10.1016/j.clinph.2006.05.031.
Husain AM, Escolar ML, Kurtzberg J. Neurophysiologic assessment of mucopolysaccharidosis III. Clin Neurophysiol. 2006 Sep;117(9):2059–2063.
Journal cover image

Published In

Clin Neurophysiol

DOI

ISSN

1388-2457

Publication Date

September 2006

Volume

117

Issue

9

Start / End Page

2059 / 2063

Location

Netherlands

Related Subject Headings

  • Retrospective Studies
  • Neurophysiology
  • Neurology & Neurosurgery
  • Neural Conduction
  • Mucopolysaccharidosis II
  • Male
  • Infant
  • Humans
  • Female
  • Evoked Potentials, Visual