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Brainstem dysfunction in patients with late-onset Lennox-Gastaut syndrome: Voxel-based morphometry and tract-based
Kang Min Park1, Yun Jung Hur2, Sung Eun Kim1
1Department of Neurology, Haeundae Paik Hospital, Inje University College of Medicine, Haeundae-gu, Busan 612-896, India.
Annals of Indian Academy of Neurology
|December 21, 2016
Summary
Late-onset Lennox-Gastaut syndrome (LGS) presents differently from early-onset LGS, with distinct electroencephalography (EEG) findings. Brainstem white matter abnormalities may play a role in the development of this late-onset neurological disorder.
Area of Science:
- Neurology
- Neuroscience
- Medical Research
Background:
- Lennox-Gastaut syndrome (LGS) is typically diagnosed in early childhood.
- Rare cases of LGS developing in the second decade of life have been reported.
- Understanding late-onset LGS is crucial for identifying potential pathogenic pathways.
Purpose of the Study:
- To investigate the electroclinical presentation of patients with late-onset LGS.
- To evaluate structural brain abnormalities in late-onset LGS.
- To explore potential common pathogenic mechanisms in LGS.
Main Methods:
- Recruitment of patients diagnosed with late-onset LGS.
- Collection of electroclinical characteristics.
- Structural brain abnormalities assessed using voxel-based morphometry (VBM) and tract-based spatial statistics (TBSS).
Main Results:
- Three subjects diagnosed with late-onset LGS were studied.
- Patients exhibited normal cognitive function and background EEG activity, with generalized paroxysmal fast activities during sleep.
- TBSS revealed reduced fractional anisotropy in brainstem white matter compared to controls; VBM showed no significant differences.
Conclusions:
- Late-onset LGS exhibits distinct clinical and EEG characteristics compared to early-onset LGS.
- Brainstem dysfunction is implicated in the pathogenesis of late-onset LGS.
- Further research into brainstem involvement in LGS is warranted.

