Brain activity levels potential diagnostic for SPG4 HSP

Differences with disease severity identified


Prof. Lu Shen

Certain changes measured in brain activity in people with SPG4 type HSP correlate with disease severity and therefore represent potential diagnostic markers.


Differences in both resting state activity levels in many regions of the brain, and the connectivity between regions, were identified in this study.



The study aimed to investigate the functional alterations of spontaneous brain activity in patients with spastic paraplegia type 4 (SPG4), and the relationship with the severity of spasticity.



Twelve patients with SPG4 and ten healthy controls underwent resting-state functional magnetic resonance imaging (rs-fMRI). Amplitude of low-frequency fluctuation (ALFF) and regional homogeneity (ReHo) were used to characterize regional neural function, and functional connectivity (FC) was used to evaluate the functional integration of the brain network.



Compared to healthy controls, patients with SPG4 exhibited significantly decreased ReHo values in the medial superior frontal gyrus. ALFF values were lower in left insula and higher in right precentral and superior frontal gyrus of the patient group. Increased ALFF values in the right precentral gyrus negatively correlated with Spastic Paraplegia Rating Scale (SPRS) scores in the patients. The connectivity study showed that the SPG4 patients had one increased FC between the left middle frontal gyrus to the left middle orbitofrontal gyrus, and pairs of decreased FC.



Our findings confirm that the baseline regional neural activity and interregional connectivity are altered in many brain regions in patients with SPG4, and certain changes are correlated with disease severity, providing potential diagnostic markers for SPG4.


SOURCE: Journal of the Neurological Sciences, Volume 384, 15 January 2018, Pages 1-6,


Resting state fMRI studies in SPG4-linked hereditary spastic paraplegia


Xinxin Liao a, Mufang Huang a, Wu Xing b, Xinwei Wu a, Weihua Liao b, Xiaoyi Wang b, Beisha Tang a,c,d,e,f,g, Lu Shen a,c,d


a Department of Neurology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China

b Department of Radiology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China

c Key Laboratory of Hunan Province in Neurodegenerative Disorders, Central South University, Changsha, Hunan 410008, China

d State Key Laboratory of Medical Genetics, Changsha, Hunan 410078, China

e Parkinson’s Disease Center of Beijing Institute for Brain Disorders, Beijing 100069, China

f Collaborative Innovation Center for Brain Science, Shanghai 200032, China

g Collaborative Innovation Center for Genetics and Development, Shanghai 200433, China

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