Accelerated myelination in early Sturge-Weber syndrome: MRI-SPECT correlations

Adamsbaum, C.; Pinton, Florence; Rolland, Y.; Chiron, Catherine; Dulac, Olivier; Kalifa, G.

doi:10.1007/bf01396195

Cited by 65 publications

(28 citation statements)

References 10 publications

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“…Developmental myelination is highly synchronized with CNS plasticity and neuronal network organization and thus, it is conceivable that neurological dysfunction could also result from precocious increased myelin formation. While accelerated myelination in the developing human brain has been poorly investigated, such a situation is known to occur in children with hemimegalencephaly [56,57]; following increased neuronal activity associated to epilepsy [57,58]; and in Sturge-Weber syndrome, a congenital condition linked to a variety of symptoms including among others seizures, visual deficits, and cognitive impairment [59]. In this regard, a recent study demonstrated that maturation of the rat striatum is accompanied by a well-defined developmental switch in the activity of complex gene networks which culminate with the expression of myelin-related genes [60].…”

Section: Discussionmentioning

confidence: 99%

The Opioid System and Brain Development: Effects of Methadone on the Oligodendrocyte Lineage and the Early Stages of Myelination

Vestal-Laborde

Eschenroeder²,

Bigbee³

et al. 2014

Dev Neurosci

120

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Oligodendrocytes express opioid receptors throughout development, but the role of the opioid system in myelination remains poorly understood. This is a significant problem as opioid use and abuse continue to increase in two particular populations: pregnant addicts (in whom drug effects could target early myelination in the fetus and newborn) and adolescents and young adults (in whom late myelination of ‘higher-order' regions takes place). Maintenance treatments for opioid addicts include the long-lasting opioids methadone and buprenorphine. Similar to our previous findings on the effects of buprenorphine, we have now found that early myelination in the developing rat brain is also altered by perinatal exposure to therapeutic doses of methadone. Pups exposed to this drug exhibited elevated brain levels of the 4 major splicing variants of myelin basic protein, myelin proteolipid protein, and myelin-oligodendrocyte glycoprotein. Consistent with the enrichment and function of these proteins in mature myelin, analysis of the corpus callosum in these young animals also indicated an elevated number of axons with already highly compacted myelin sheaths. Moreover, studies in cultured cells showed that methadone exerts direct effects at specific stages of the oligodendrocyte lineage, stimulating the proliferation of progenitor cells while on the other hand accelerating the maturation of the more differentiated but still immature preoligodendrocytes. While the long-term effects of these observations remain unknown, accelerated or increased oligodendrocyte maturation and myelination could both disrupt the complex sequence of synchronized events leading to normal connectivity in the developing brain. Together with our previous observations on the effects of buprenorphine, the present findings further underscore a crucial function of the endogenous opioid system in the control of oligodendrocyte development and the timing of myelination. Interference with these regulatory systems by opioid use or maintenance treatments could disrupt the normal process of brain maturation at critical stages of myelin formation.

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Section: Discussionmentioning

confidence: 99%

The Opioid System and Brain Development: Effects of Methadone on the Oligodendrocyte Lineage and the Early Stages of Myelination

Vestal-Laborde

Eschenroeder²,

Bigbee³

et al. 2014

Dev Neurosci

120

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“…Although MRI signs of ‘accelerated myelination’ have been described in infants with SWS [23], only a few recent MRI studies focused on the significance of white matter pathology using quantitative volumetry [3] or water diffusion measurements [4–6]. MRI studies of cerebral perfusion performed in small groups of SWS patients demonstrated mostly decreased perfusion in regions with meningeal enhancement [11, 12]; additional perfusion defects were seen in areas of deep venous abnormalities.…”

Section: Discussionmentioning

confidence: 99%

Clinical Correlates of White Matter Blood Flow Perfusion Changes in Sturge-Weber Syndrome: A Dynamic MR Perfusion-Weighted Imaging Study

Miao¹,

Juhász²,

Wu³

et al. 2011

AJNR Am J Neuroradiol

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Background and Purpose Low brain tissue perfusion due to abnormal venous drainage is thought to be a central mechanism of brain damage in Sturge-Weber syndrome (SWS). In the present study, high-resolution perfusion-weighted imaging (HR-PWI) was used to quantify white matter perfusion abnormalities and correlate these with brain atrophy and clinical variables. Materials and Methods Fourteen children (age: 0.8–10.0 years) with unilateral SWS underwent MRI examinations, including HR-PWI. Relative cerebral blood volume (rCBV), cerebral blood flow (rCBF) and mean transit time (MTT) in the affected white matter (WM) and in contralateral homotopic WM were measured. Asymmetry index (AI) for each perfusion parameter was correlated with age, brain atrophy, motor and seizure variables as well as IQ. Results Increased perfusion was seen in the affected hemisphere in 5 children and decreased perfusion in 9. Brain atrophy was more severe in the low-perfusion group (p=0.01) and was related to both CBF-AI and CBV-AI (r = −0.69, p = 0.007; r = −0.64, p = 0.014, respectively). Older children had lower CBV values on the affected side (r = −0.62, p = 0.02). Longer duration of epilepsy was related to lower CBF (more negative CBF-AI, r=−0.58, p=0.03) and low CBV (r=−0.55, p=0.04) on the affected side. Lower perfusion was associated with more frequent seizures (rCBF-AI: r=−0.56, p=0.04; rCBV-AI: r=−0.63, p=0.02). Conclusion Increased perfusion in the affected cerebral WM may indicate an early stage of SWS without severe brain atrophy. Decreased perfusion is associated with frequent seizures, long duration of epilepsy and brain atrophy.

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“…Accelerated myelination in the involved hemisphere may be an early diagnostic feature before 6 months of age. Adamsbaum et al [4] measured the regional blood flow using xenon-133 and correlated these findings with MRI T1 and T2 sequences. Their data suggested that the accelerated myelination is not related to ischemia, but to transient hyperperfusion.…”

Section: Discussionmentioning

confidence: 99%

Accelerated myelination associated with venous congestion

et al. 2005

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Magnetic resonance imaging is currently the gold standard in the assessment of brain myelination. The normal pattern of brain myelination conforms to a fixed chronological sequence. Focal accelerated myelination is a usual pathological state and previously has only been associated with Sturge-Weber syndrome. The purpose of our study is to describe alternate causes for accelerated myelination. We retrospectively reviewed serial MR scans, MR angiography, conventional angiography and the clinical progress of three children with accelerated myelination. Two patients with accelerated myelination had an underlying cerebral sinovenous thrombosis. The third patient had Sturge-Weber syndrome. Our study strongly suggests that cerebral venous thrombosis with the consequent restriction of venous outflow could be a key factor in the induction of accelerated myelination. We recommend that in patients with accelerated myelination, the search for an underlying etiology should include careful evaluation of the intracranial vascular pathology, especially cerebral venous thrombosis.

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Accelerated myelination in early Sturge-Weber syndrome: MRI-SPECT correlations

Cited by 65 publications

References 10 publications

The Opioid System and Brain Development: Effects of Methadone on the Oligodendrocyte Lineage and the Early Stages of Myelination

The Opioid System and Brain Development: Effects of Methadone on the Oligodendrocyte Lineage and the Early Stages of Myelination

Clinical Correlates of White Matter Blood Flow Perfusion Changes in Sturge-Weber Syndrome: A Dynamic MR Perfusion-Weighted Imaging Study

Accelerated myelination associated with venous congestion

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