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|IMAGES IN CLINICAL MEDICINE
|Year : 2015 | Volume
| Issue : 3 | Page : 206-208
Symptoms of degeneration of the pyramidal tracts in conventional magnetic resonance imaging and diffusion tensor imaging in a young woman with primary lateral sclerosis
S Budrewicz1, P Szewczyk2, K Slotwinski1, M Koszewicz1
1 Department of Neurology, Interventional Radiology and Neuroradiology, Wroclaw Medical University, Wroclaw, Poland
2 Department of Neurology; Department of General Radiology, Interventional Radiology and Neuroradiology, Wroclaw Medical University, Wroclaw, Poland
|Date of Submission||12-Jul-2014|
|Date of Decision||29-Sep-2014|
|Date of Acceptance||16-Oct-2014|
|Date of Web Publication||26-Jun-2015|
Department of Neurology, Interventional Radiology and Neuroradiology, Wroclaw Medical University, Wroclaw
Source of Support: None, Conflict of Interest: None
Primary lateral sclerosis (PLS) is one of the forms of motor neuron disease (MND), affecting only upper motor neurons. The diagnosis of PLS should be made on different diagnostic criteria, for example, Pringle or Gordon, but it is usually a diagnosis of exclusion. There are no characteristic findings in standard laboratory and electrophysiological assessment. We present details of a 31-year-old woman who had suffered from progressive paraparesis with right-side predominance. Conventional MRI of brain and spinal cord and diffusion tensor imaging (DTI) studies showed Hyperintense lesions in the upper part of the cervical spinal cord at the level C1 in lateral funicules, in the medulla oblongata at the pyramidal decussation and in the midbrain. Brain DTI revealed changes along the corticospinal tracts on fractional anisotropy (FA) maps. MRI of the thoracic spinal cord showed in T2-weighted images hyperintensive regions in the course of the lateral corticospinal tracts. This aided in PLS recognition.
Diffusion tensor imaging, MRI, primary lateral sclerosis
|How to cite this article:|
Budrewicz S, Szewczyk P, Slotwinski K, Koszewicz M. Symptoms of degeneration of the pyramidal tracts in conventional magnetic resonance imaging and diffusion tensor imaging in a young woman with primary lateral sclerosis. J Postgrad Med 2015;61:206-8
|How to cite this URL:|
Budrewicz S, Szewczyk P, Slotwinski K, Koszewicz M. Symptoms of degeneration of the pyramidal tracts in conventional magnetic resonance imaging and diffusion tensor imaging in a young woman with primary lateral sclerosis. J Postgrad Med [serial online] 2015 [cited 2020 Jul 11];61:206-8. Available from: http://www.jpgmonline.com/text.asp?2015/61/3/206/150901
Primary lateral sclerosis (PLS) is one of the forms of motor neuron disease (MND), affecting only upper motor neurons. PLS diagnosis should be based on different diagnostic criteria, for example, Pringle or Gordon, but it is usually a diagnosis of exclusion. There are no characteristic findings in standard laboratory and electrophysiological assessment. 
A 31-year-old woman, after bone-marrow transplantation from an unrelated donor because of the paroxysmal nocturnal hemoglobinuria, was admitted thrice to our Neurological Department. She had suffered from progressive paraparesis with right-side predominance since August 2009 and her family history was negative.
Her first hospitalization revealed enhanced straight reflexes in the upper limbs with right-side predominance, paraparesis of the lower limbs was equal to 3 points on the Lovett scale on the right side and 4 points on the left, deep reflexes were enhanced bilaterally. Babinski sign was positive on both sides; without sensory and cerebellar disturbances, bulbar and pseudobulbar symptoms, or bladder impairment. We noted an increase in the paraparesis between November 2009 and June 2010.
Blood biochemistry, morphology, B12, level, cerebrospinal fluid with peptide immunoelectrophoresis analysis and IgG index were normal. Lues and intoxication of heavy metals were excluded. Borrelia burgdorferi, Herpes, ECHO, Coxackie, HTLV1 viruses antigens were negative.
The latencies of the visual evoked potentials were slightly prolonged, auditory brain stem evoked potentials, somatosensory evoked potentials, EEG, electroneurography, electromyography, pain, temperature and vibration sensation thresholds were normal. EMG was normal during the whole time of the observation.
Conventional brain MRI and spinal cord as well as diffusion tensor imaging (DTI) studies were performed with a 1.5T Signa Hdx MR scanner; we used a high resolution 16-channel HNS coil, brain DTI was performed with single-shot spin-echo-type echo-planar imaging. Brain MRI revealed the presence of areas of increased signal in T2-weighted images [Figure 1] and FLAIR sequence along the pyramidal tracts. Hyperintense lesions were visible in the upper part of the cervical spinal cord at the level C1 in lateral funicules [Figure 1]a, in the medulla oblongata at the pyramidal decussation [Figure 1]b and in the midbrain - in the ventral part of the left cerebral peduncle [Figure 1]c and d. Hyperintense lesions in the left cerebral peduncle were also clearly visible in diffusion-weighted images (DWI) [Figure 1]e and f. Brain DTI also revealed changes along the corticospinal tracts on fractional anisotropy (FA) maps. Focal decrease of the FA parameter was visible at the C1 spinal cord level in the lateral funicule region [Figure 2]a, at the pyramidal decussation [Figure 2]b, with changes more strongly marked on the left side, and in the ventral left cerebral peduncle [Figure 2]c and d. MRI of the thoracic spinal cord showed in T2-weigted images hyperintensive regions in the course of the lateral corticospinal tracts [Figure 3]a and b. The signal of the lateral pyramidal tracts was also increased, but to a lesser extent in the T1-weighted images [Figure 3]c and d. There were no atrophies of the spinal cord.
|Figure 1: Brain MRI, axial and coronal T2-weighted images showing increased signal intensity (arrows) along the corticospinal (pyramidal) tracts in lateral funicules at the C1 level of the spinal cord (a) at the level of medullary pyramids (b) and in the left cerebral peduncle (c and d). On diffusion-weighted images (DWI) (e and f) hyperintense lesions are clearly visible in the left cerebral peduncle (arrows)|
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|Figure 2: Diffusion tensor imaging (DTI), fractional anisotropy (FA) maps reveal focal decrease in the FA values in the course of pyramidal tracts (arrows). As in structural MRI changes are visible in lateral funicules of the spinal cord at the level of C1 (a), in the pyramidal decussation (b) and in the left cerebral peduncle (c and d)|
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|Figure 3: MRI, axial T2-weighted images show hyperintensities along the lateral pyramidal tracts (lateral funicules) at the level of the upper (a) and lower (b) thoracic spinal cord. Increased signal intensity of the lateral funicules is also visible on T1-weighted images without contrast medium (c and d)|
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PLS recognition is based on clinical and EMG examination after the exclusion of other diseases, and according to either Pringle's or Gordon's criteria.  In our patient conventional brain MRI revealed hyperintense lesions in T2-weighted images along the pyramidal tracts. Similar changes were found in earlier neuroimaging and neuropathological studies in patients with MND. , Hyperintensity probably reflects the level of gliosis and demyelination.  We did not observe any changes in the cortex of the precentral gyrus and other cortical regions as other authors. ,, Focal decrease in the FA value on brain DTI maps found in the course of corticospinal tracts, hyperintensity in T2-weighted images in the lateral corticospinal tracts of the thoracic spinal cord indicated axonal degeneration of the pyramidal tracts in our patient. ,, Wang et al.  indicated the meaning of changes in posterior limb internal capsule (PLIC) in the diagnosis of PLS. In our case, the DTI showed areas of decreased FA value in PLIC, cerebral peduncles as well as in the spinal cord, mostly the C1 segment. To the best of our knowledge there have been few studies on MRI changes in the spinal cord in patients with different types of MND. The asymmetry of pyramidal tract lesions closely corresponded to the clinical findings (asymmetry of paresis). According to Agosta et al.  the DTI could be useful in differential diagnosis between PLS and ALS patients with the highest accuracy of corpus callosum FA values, but they did not perform MRI of the spinal cord.
The authors emphasize the importance of neuroimaging in PLS recognition. A good quality, high-resolution MRI is able to visualize changes in pyramidal tracts at the level of the spinal cord and DTI study at the level of the brain in PLS patients. This suggests the utility of spinal cord abnormality disclosure as an analytical tool for PLIC changes in MND patients.
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[Figure 1], [Figure 2], [Figure 3]