Investig Magn Reson Imaging.  2019 Jun;23(2):148-156. 10.13104/imri.2019.23.2.148.

Multi-Parametric Quantitative MRI for Measuring Myelin Loss in Hyperglycemia-Induced Hemichorea

• Youn SW
• Kwon OD
• Hwang MJ

• KMID: 2452529
• DOI: http://doi.org/10.13104/imri.2019.23.2.148

Abstract

Hyperglycemia-induced hemichorea (HGHC) is a rare but characteristic hyperkinetic movement disorder involving limbs on one side of the body. In a 75-year-old woman with a left-sided HGHC, conventional brain MR imaging showed very subtle T1-hyperintensity and unique gadolinium enhancement in the basal ganglia contralateral to movements. Multi-parametric MRI was acquired using pulse sequence with quantification of relaxation times and proton density by multi-echo acquisition. Myelin map was reconstructed based on new tissue classification modeling. In this case report of multi-parametric MRI, quantitative measurement of myelin change related to HGHC in brain structures and its possible explanations are presented. This is the first study to demonstrate myelin loss related to hyperglycemic insult in multi-parametric quantitative MR imaging.

Keyword

Hyperglycemia; Hemichorea; Movement disorder; Multi-parametric MRI, Quantitative, Myelin

MeSH Terms

Aged
Basal Ganglia
Brain
Classification
Extremities
Female
Gadolinium
Humans
Hyperglycemia
Hyperkinesis
Magnetic Resonance Imaging*
Movement Disorders
Myelin Sheath*
Protons
Relaxation
Gadolinium
Protons

Figure

• 

  Fig. 1 A 75-year-old female presented with hyperglycemia-induced hemichorea. (a) Body 18F-fludeoxyglucose positron emission tomography (FDG PET) at one year prior demonstrated multifocal nodal masses distributed in the neck, chest, and abdomino-pelvic area, suggesting large B cell lymphoma. She has achieved complete resolution by six sessions of chemotherapy. (b) Non-enhanced CT at time of hyperglycemia (one month before hemichorea) did not show significant abnormality. Conventional MRI at three weeks after hemichorea included T1-weighted image (T1WI) and gadolinium-enhanced T1WI. (c) T1WI of the basal ganglia level demonstrated an area of very subtle hyperintensity involving the right head of putamen with no surrounding edema. (d) Gadolinium-enhanced T1WI revealing enhancement in the right putamen.

• 

  Fig. 2 Time table of symptoms and imaging studies. At a month before hemichorea occurrence, non-ketotic hyperglycemia developed. It was controlled by insulin within 10 days. Non-contrast brain CT was obtained at that time. At 10 days after hemichorea occurrence, first MRI was performed including multi-parametric image acquisition. At three weeks, second MRI including gadolinium-enhanced T1WI enabled suspicion of non-ketotic hyperglycemia-induced hemichorea syndrome in correlation with clinical findings. Third MRI including multi-parametric image was performed at one month after PET acquisition. Multi-parametric quantitative MRIs (first and third) were retrospectively reconstructed and analyzed. Hemichorea persisted after PET acquisition at one month after symptom onset.

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  Fig. 3 Quantitative T1, T2, and proton density (PD) maps synthesized from multi-parametric MRI in correlation with arterial spin labeling cerebral blood flow (ASL CBF) map and FDG PET. (a) T1 map of the brain showing very subtle low signal intensity in the right putamen as compared to the left side. (b) T2 map and (c) PD map showing no signal difference between right and left brain structures. (d) ASL CBF revealing no cerebral blood flow abnormality. (e) On FDG PET, local cerebral glucose metabolism was decreased in the contralateral putamen to chorea, supporting the notion of cerebral glucose metabolic failure in lesions.

• 

  Fig. 4 Multi-parametric quantitative MRI measuring myelin loss in hyperglycemia-induced hemichorea. (a) Myelin-water-fraction (MWF) map was reconstructed from multi-parametric MRI with pulse sequence with quantification of relaxation times and proton density (PD) by multi-echo acquisition of a saturation-recovery using turbo spin-echo readout. (b) Synthetic T1, T2, PD, R1, R2, and MWF map of hyperglycemia-induced hemichorea showing no major difference between right and left brain structures. To obtain average values of normal controls, six age- and sex-matched normal volunteers without any neurologic deficit were registered into Montreal Neurological Institute template and values of predetermined area in putamen (put), pallidum (pall), caudate nucleus (caud), thalamus (thal), red nucleus (RN), and substantia nigra (SN) were averaged. MWFs were markedly reduced in the caudate nucleus, thalamus, substantia nigra, and red nucleus compared to average values of normal controls. On follow-up imaging, loss of MWF was slightly restored in substantia nigra and red nucleus in accordance with mild symptomatic improvement. On the other hand, it seemed that MWFs were further decreased in thalamus. avg-hc = averaged healthy control group; fu = follow up

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