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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.


Related in: MedlinePlus

(A) MRI brainaxial T2 FLAIR showing white matter hyperintensities in bilateral centrum semiovale region (white open arrow). (B) MRI cervical spine axial T2-weighted image showing spinal cord atrophy and hyperintensity in the centrolateral aspect of the cord (open white arrow)
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Figure 2: (A) MRI brainaxial T2 FLAIR showing white matter hyperintensities in bilateral centrum semiovale region (white open arrow). (B) MRI cervical spine axial T2-weighted image showing spinal cord atrophy and hyperintensity in the centrolateral aspect of the cord (open white arrow)

Mentions: Physical examination was unremarkable except for bilateral pes cavus. Neurological examination showed dysarthria. There was no evidence of muscle wasting. Power in both the lower limbs was grade III in all groups of muscles. Deep tendon reflexes in both upper and lower limbs were symmetrically brisk and plantar response was bilaterally extensor. MRI brain revealed diffuse atrophy and long repetition time (TR) hyperintensities in periventricular white matter and centrum semiovale [Figure 2A]. CC appeared unremarkable morphologically. Significant changeswith increased MD and FA reduction were found in the visible lesions and CC (FA in genu and splenium were 0.61 and 0.72, respectively, and MD was 1.01 and 0.87 × 10-6 mm2/s in the genu and corpus callosum, respectively). MRS, however, showed normal metabolites. There was evidence of mild atrophy of cord in cervicothoracic region with hyperintensity in the centrolateral aspect which possibly signifies degeneration in the lateral corticospinal tract [Figure 2B].


Multiparametric 3T MRI evaluation of hereditary spastic paraplegia: A case report
(A) MRI brainaxial T2 FLAIR showing white matter hyperintensities in bilateral centrum semiovale region (white open arrow). (B) MRI cervical spine axial T2-weighted image showing spinal cord atrophy and hyperintensity in the centrolateral 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 2: (A) MRI brainaxial T2 FLAIR showing white matter hyperintensities in bilateral centrum semiovale region (white open arrow). (B) MRI cervical spine axial T2-weighted image showing spinal cord atrophy and hyperintensity in the centrolateral aspect of the cord (open white arrow)
Mentions: Physical examination was unremarkable except for bilateral pes cavus. Neurological examination showed dysarthria. There was no evidence of muscle wasting. Power in both the lower limbs was grade III in all groups of muscles. Deep tendon reflexes in both upper and lower limbs were symmetrically brisk and plantar response was bilaterally extensor. MRI brain revealed diffuse atrophy and long repetition time (TR) hyperintensities in periventricular white matter and centrum semiovale [Figure 2A]. CC appeared unremarkable morphologically. Significant changeswith increased MD and FA reduction were found in the visible lesions and CC (FA in genu and splenium were 0.61 and 0.72, respectively, and MD was 1.01 and 0.87 × 10-6 mm2/s in the genu and corpus callosum, respectively). MRS, however, showed normal metabolites. There was evidence of mild atrophy of cord in cervicothoracic region with hyperintensity in the centrolateral aspect which possibly signifies degeneration in the lateral corticospinal tract [Figure 2B].

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