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Magnetic Resonance Imaging and Magnetic Resonance Spectroscopy in a Patient with Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

Helena Šarac
Marija Žagar
Davorka Vranješ
Neven Henigsberg
Ervina Bilić
Goran Pavliša

Puni tekst: engleski pdf 272 Kb

str. 205-210

preuzimanja: 713



Magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) have been investigated in a single
neurodegenerative disease manifesting as either amyotrophic lateral sclerosis (ALS) or frontotemporal dementia (FTD)
alone, but have not been examined in combined disorders such as ALS with FTD (ALS-FTD). To our knowledge, this
study is the first attempt to demonstrate relationship between MRI abnormalities and MR spectroscopic metabolite changes
of the motor cortex, frontal white matter and corticospinal tract in a patient with the diagnosis of ALS with probable
upper motor neuron signs (ALS-PUMNS) and FTD. Patient presented underwent MRI of the brain and MRS. The ratio
of N-acetylaspartate (NAA) to creatine (Cr), choline to Cr, myo-inositol (mI) to Cr and glutamate-glutamine (Glx) to Cr
were derived from peak area measurement. Spectra from the right motor cortex, frontal white matter and corticospinal
tract were obtained. MR images were evaluated for sulcus centralis enlargement, corticospinal tract hyperintensity and
frontal lobes atrophy. Spectra showed reduced NAA/Cr and Glx/Cr ratio, yet the ratio of Cho/Cr exhibited significant elevation.
MR images revealed sulcus centralis enlargement, high signal intensity of corticospinal tract and atrophy of both
frontal lobes. Proton spectroscopic metabolic changes in a current patient fully correlate with previously reported MRS
metabolic changes in ALS alone. Surprisingly, normal mI (glial marker) values have been found in almost all measured
voxels of interest except in the frontal white matter. These findings differ from the previous findings in ALS or FTD
alone. In conclusion, these findings support the concept that ALS, FTD and ALS-FTD may represent different manifestations
of a single pathological continuum.

Ključne riječi

magnetic resonance imaging; magnetic resonance spectroscopy; amyotrophic lateral sclerosis; frontotemporal dementia

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