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Multiparametric 3T MRI evaluation of hereditary spastic paraplegia: A case report

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ABSTRACT

Hereditary spastic paraplegia (HSP) is a rare heterogeneous group of familial neurodegenerative disorders characterized by degeneration of the corticospinal tracts and posterior column of the spinal cord. Previously described radiological findings included nonspecific brain abnormalities such as brain atrophy and white matter lesions, as well as atrophy of the corpus callosum and spinal cord. Magnetic resonance spectroscopy may reveal reduced concentrations of normal brain metabolites and elevated levels of myoinositol. Diffusion tensor imaging shows increased mean diffusivity and reduced fractional anisotropy in the periventricular white matter, which is compatible with damaged myelinated axons. We present here two cases of HSP in a single family with typical imaging findings.

No MeSH data available.


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(A) MRI brain sagittal T2-weighted image showing thin anterior corpus callosum (white arrow) with mild atrophy. (B) MRI brainaxial T2 FLAIR showing periventricular frontal (ears of lynx appearance) white matter hyperintensity (white open arrow) and thin genu of corpus callosum (white solid arrow). (C) MRS brain showing no abnormal metabolite peaks. (D) DTI showing significantly reduced fractional anisotropy in anterior part of corpus callosum. (E) DTI showing decreased oriental coherence of fiber tracts. (F) MRI dorsal spine axial T2 Weighted image showing spinal cord atrophy and hyperintensity in left posterolateral aspect of the cord (open white arrow)
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Figure 1: (A) MRI brain sagittal T2-weighted image showing thin anterior corpus callosum (white arrow) with mild atrophy. (B) MRI brainaxial T2 FLAIR showing periventricular frontal (ears of lynx appearance) white matter hyperintensity (white open arrow) and thin genu of corpus callosum (white solid arrow). (C) MRS brain showing no abnormal metabolite peaks. (D) DTI showing significantly reduced fractional anisotropy in anterior part of corpus callosum. (E) DTI showing decreased oriental coherence of fiber tracts. (F) MRI dorsal spine axial T2 Weighted image showing spinal cord atrophy and hyperintensity in left posterolateral aspect of the cord (open white arrow)

Mentions: Hematological investigations were normal. Cerebrospinal fluid analysis was mildly positive for oligoclonal band. Electromyography and nerve conduction studies were normal. MRI brain and spinal cord was done using a 3 T machine, which revealed a thin anterior part of body and genu of corpus callosum (CC) with mild brain atrophy [Figure 1A]. Abnormal high signal intensities were noted in periventricular white matter and centrum semiovale on fluid-attenuated inversion recovery (FLAIR) images [Figure 1B]. MRspectroscopy (MRS) did not show significant alterations in normal metabolites [Figure 1C]. Diffusion tensor imaging (DTI) with voxel-based analysis of fractional anisotrophy (FA) and mean diffusivity (MD) revealed reduced FA and increased MD predominantly in the anterior CC [Figures 1D and E]. FA values in the genu and splenium of CC were 0.41 and 0.78, respectively; MD values were 1.42 and 0.83 × 10-6 mm2/s. Mild atrophy was noted in the dorsal cord. Abnormal high signal intensity was seen in the dorsal column of thoracic cord at the level of D3-D4 [Figure 1F].


Multiparametric 3T MRI evaluation of hereditary spastic paraplegia: A case report
(A) MRI brain sagittal T2-weighted image showing thin anterior corpus callosum (white arrow) with mild atrophy. (B) MRI brainaxial T2 FLAIR showing periventricular frontal (ears of lynx appearance) white matter hyperintensity (white open arrow) and thin genu of corpus callosum (white solid arrow). (C) MRS brain showing no abnormal metabolite peaks. (D) DTI showing significantly reduced fractional anisotropy in anterior part of corpus callosum. (E) DTI showing decreased oriental coherence of fiber tracts. (F) MRI dorsal spine axial T2 Weighted image showing spinal cord atrophy and hyperintensity in left posterolateral aspect of the cord (open white arrow)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5036329&req=5

Figure 1: (A) MRI brain sagittal T2-weighted image showing thin anterior corpus callosum (white arrow) with mild atrophy. (B) MRI brainaxial T2 FLAIR showing periventricular frontal (ears of lynx appearance) white matter hyperintensity (white open arrow) and thin genu of corpus callosum (white solid arrow). (C) MRS brain showing no abnormal metabolite peaks. (D) DTI showing significantly reduced fractional anisotropy in anterior part of corpus callosum. (E) DTI showing decreased oriental coherence of fiber tracts. (F) MRI dorsal spine axial T2 Weighted image showing spinal cord atrophy and hyperintensity in left posterolateral aspect of the cord (open white arrow)
Mentions: Hematological investigations were normal. Cerebrospinal fluid analysis was mildly positive for oligoclonal band. Electromyography and nerve conduction studies were normal. MRI brain and spinal cord was done using a 3 T machine, which revealed a thin anterior part of body and genu of corpus callosum (CC) with mild brain atrophy [Figure 1A]. Abnormal high signal intensities were noted in periventricular white matter and centrum semiovale on fluid-attenuated inversion recovery (FLAIR) images [Figure 1B]. MRspectroscopy (MRS) did not show significant alterations in normal metabolites [Figure 1C]. Diffusion tensor imaging (DTI) with voxel-based analysis of fractional anisotrophy (FA) and mean diffusivity (MD) revealed reduced FA and increased MD predominantly in the anterior CC [Figures 1D and E]. FA values in the genu and splenium of CC were 0.41 and 0.78, respectively; MD values were 1.42 and 0.83 × 10-6 mm2/s. Mild atrophy was noted in the dorsal cord. Abnormal high signal intensity was seen in the dorsal column of thoracic cord at the level of D3-D4 [Figure 1F].

View Article: PubMed Central - PubMed

ABSTRACT

Hereditary spastic paraplegia (HSP) is a rare heterogeneous group of familial neurodegenerative disorders characterized by degeneration of the corticospinal tracts and posterior column of the spinal cord. Previously described radiological findings included nonspecific brain abnormalities such as brain atrophy and white matter lesions, as well as atrophy of the corpus callosum and spinal cord. Magnetic resonance spectroscopy may reveal reduced concentrations of normal brain metabolites and elevated levels of myoinositol. Diffusion tensor imaging shows increased mean diffusivity and reduced fractional anisotropy in the periventricular white matter, which is compatible with damaged myelinated axons. We present here two cases of HSP in a single family with typical imaging findings.

No MeSH data available.


Related in: MedlinePlus