Regional cerebral blood flow and neuropsychological performance in a Danish family with X-linked bulbo-spinal neuronopathy
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Regional cerebral blood flow and neuropsychological performance in a Danish family with X-linked bulbo-spinal neuronopathy. / Tedeschi, E; Waldemar, G; Gyring, J; Gade, A; Staehelin Jensen, T; Paulson, O B.
I: International Journal of Neuroscience, Bind 83, Nr. 1-2, 11.1995, s. 59-68.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Regional cerebral blood flow and neuropsychological performance in a Danish family with X-linked bulbo-spinal neuronopathy
AU - Tedeschi, E
AU - Waldemar, G
AU - Gyring, J
AU - Gade, A
AU - Staehelin Jensen, T
AU - Paulson, O B
PY - 1995/11
Y1 - 1995/11
N2 - Regional Cerebral Blood Flow (rCBF) measurements and neuropsychological evaluation were performed in 6 patients from a Danish family affected with X-linked Bulbo-Spinal Neuronopathy (XBSN). This inherited form of motor neuron disease (MND) has been shown to affect various functions within the nervous and the endocrine systems. We investigated the possibility that focal or diffuse cortical deficits, already demonstrated in MND, were present in XBSN. The global CBF values of the patients, when compared to age- and sex-matched healthy controls, were found reduced, but no focal rCBF alterations, nor increased regional side-to-side asymmetry were observed. The neuropsychological evaluation showed no cognitive impairments. We conclude that cortical perfusion and cognitive functions are not significantly altered in XBSN.
AB - Regional Cerebral Blood Flow (rCBF) measurements and neuropsychological evaluation were performed in 6 patients from a Danish family affected with X-linked Bulbo-Spinal Neuronopathy (XBSN). This inherited form of motor neuron disease (MND) has been shown to affect various functions within the nervous and the endocrine systems. We investigated the possibility that focal or diffuse cortical deficits, already demonstrated in MND, were present in XBSN. The global CBF values of the patients, when compared to age- and sex-matched healthy controls, were found reduced, but no focal rCBF alterations, nor increased regional side-to-side asymmetry were observed. The neuropsychological evaluation showed no cognitive impairments. We conclude that cortical perfusion and cognitive functions are not significantly altered in XBSN.
KW - Adult
KW - Brain/blood supply
KW - Denmark
KW - Female
KW - Humans
KW - Male
KW - Middle Aged
KW - Motor Neuron Disease/diagnosis
KW - Neuropsychological Tests
KW - Pedigree
KW - Regional Blood Flow
KW - Tomography, Emission-Computed, Single-Photon
KW - Tomography, X-Ray Computed
KW - X Chromosome
U2 - 10.3109/00207459508986325
DO - 10.3109/00207459508986325
M3 - Journal article
C2 - 8746749
VL - 83
SP - 59
EP - 68
JO - International Journal of Neuroscience
JF - International Journal of Neuroscience
SN - 0020-7454
IS - 1-2
ER -
ID: 275018132