Diffusion tensor imaging in a case of Kearns-Sayre syndrome
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Diffusion tensor imaging in a case of Kearns-Sayre syndrome : striking brainstem involvement as a possible cause of oculomotor symptoms. / Duning, Thomas; Deppe, Michael; Keller, Simon; Mohammadi, Siawoosh; Schiffbauer, Hagen; Marziniak, Martin.
In: J NEUROL SCI, Vol. 281, No. 1-2, 15.06.2009, p. 110-2.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Diffusion tensor imaging in a case of Kearns-Sayre syndrome
T2 - striking brainstem involvement as a possible cause of oculomotor symptoms
AU - Duning, Thomas
AU - Deppe, Michael
AU - Keller, Simon
AU - Mohammadi, Siawoosh
AU - Schiffbauer, Hagen
AU - Marziniak, Martin
PY - 2009/6/15
Y1 - 2009/6/15
N2 - Kearns-Sayre syndrome (KSS) is a rare autosomal dominant mitochondrial disorder affecting the central nervous system. Progressive external ophthalmoplegia is an early and characteristic clinical symptom of the disease. We describe a 22-year-old female patient with a typical KSS including early and severe external ophthalmoplegia. Conventional MRI and diffusion tensor imaging (DTI) was performed to investigate the early involvement of the central nervous system (CNS). DTI revealed substantial white matter alterations that were primarily confined to the brainstem. These early DTI changes support the hypothesis that regional affection of the brainstem may play a role in the pathogenesis of the early oculomotor symptoms. DTI might be helpful to detect an early involvement of the CNS in KSS.
AB - Kearns-Sayre syndrome (KSS) is a rare autosomal dominant mitochondrial disorder affecting the central nervous system. Progressive external ophthalmoplegia is an early and characteristic clinical symptom of the disease. We describe a 22-year-old female patient with a typical KSS including early and severe external ophthalmoplegia. Conventional MRI and diffusion tensor imaging (DTI) was performed to investigate the early involvement of the central nervous system (CNS). DTI revealed substantial white matter alterations that were primarily confined to the brainstem. These early DTI changes support the hypothesis that regional affection of the brainstem may play a role in the pathogenesis of the early oculomotor symptoms. DTI might be helpful to detect an early involvement of the CNS in KSS.
KW - Brain
KW - Brain Stem
KW - Diffusion Magnetic Resonance Imaging
KW - Female
KW - Humans
KW - Kearns-Sayre Syndrome
KW - Magnetic Resonance Imaging
KW - Nerve Fibers, Myelinated
KW - Ocular Motility Disorders
KW - Young Adult
U2 - 10.1016/j.jns.2009.03.007
DO - 10.1016/j.jns.2009.03.007
M3 - SCORING: Journal article
C2 - 19344916
VL - 281
SP - 110
EP - 112
JO - J NEUROL SCI
JF - J NEUROL SCI
SN - 0022-510X
IS - 1-2
ER -