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Holoprosencephaly in children: diffusion tensor MR imaging of white matter tracts of the brainstem--initial
Sait Albayram1, Elias R Melhem, Susumu Mori
1Department of Radiology and Radiological Sciences, Johns Hopkins Medical Institutions, Baltimore, MD, USA.
Insights
Diffusion tensor MRI can identify brainstem white matter tract abnormalities in children with holoprosencephaly. These abnormalities correlate with holoprosencephaly type and neurodevelopmental scores, aiding in diagnosis and understanding disease severity.
Area of Science:
- Neuroimaging
- Developmental Neuroscience
- Neurology
Background:
- Holoprosencephaly is a congenital disorder characterized by incomplete separation of the forebrain.
- Brainstem white matter tracts are crucial for neurological function and are thought to be relatively spared in holoprosencephaly.
- Diffusion tensor magnetic resonance (MR) imaging offers advanced visualization of white matter microstructure.
Purpose of the Study:
- To evaluate the dimensions of specific brainstem white matter tracts in children with holoprosencephaly using diffusion tensor MR imaging.
- To correlate these white matter tract abnormalities with the severity of forebrain malformation and neurologic deficits.
- To assess the utility of diffusion tensor MR imaging in identifying in vivo abnormalities.
Main Methods:
- Thirteen patients with holoprosencephaly underwent diffusion tensor MR imaging.
- White matter color maps were generated to visualize specific tracts.
- Spearman rank correlation analysis was used to correlate tract dimensions with holoprosencephaly type and neurodevelopmental scores.
Main Results:
- Diffusion tensor MR imaging successfully identified brainstem white matter tracts in most patients.
- Cortico-ponto-spinal tracts (CPSTs) were absent bilaterally in alobar holoprosencephaly.
- Holoprosencephaly type and neurodevelopmental scores showed a strong correlation with CPST and middle cerebellar peduncle (MCP) dimensions.
Conclusions:
- Diffusion tensor MR imaging is effective for in vivo identification of brainstem white matter tract abnormalities in holoprosencephaly.
- Abnormalities in these tracts are linked to the severity of holoprosencephaly and associated neurodevelopmental outcomes.
- This imaging technique aids in understanding the neurological impact of holoprosencephaly.
Purpose:
To evaluate the dimensions of specific white matter tracts in the brainstems (region of brain thought to be least affected) of children with holoprosencephaly by using diffusion tensor magnetic resonance (MR) imaging and to correlate these abnormalities with forebrain malformation severity and neurologic deficit severity.
Materials And Methods:
Thirteen patients with holoprosencephaly underwent diffusion tensor MR imaging, with which white matter color maps were generated. Type of holoprosencephaly was correlated with presence or absence of specific brainstem white matter tracts. Furthermore, patient rank based on cortico-ponto-spinal tract (CPST) and middle cerebellar peduncle (MCP) dimensions was correlated with holoprosencephaly type and neurodevelopmental score by using Spearman rank correlation analysis.
Results:
Two patients had alobar holoprosencephaly, five had the semilobar type, one had the lobar type, and one had the middle-hemisphere-variant type. Four patients were excluded from analysis. In the two patients with alobar holoprosencephaly, the CPSTs were absent bilaterally. In all of the remaining patients except one, who had semilobar holoprosencephaly in which the CPSTs could not be identified at the level of the medulla oblongata, all tracts were present bilaterally. Holoprosencephaly type and neurodevelopmental score correlated strongly with CPST and MCP dimensions (P <.01) over and above the effect of age.
Conclusion:
In vivo identification of brainstem white matter tract abnormalities in patients with holoprosencephaly can be achieved by performing diffusion tensor MR imaging.
