Teaching NeuroImage: Superior Cerebellar Peduncle Tract Sign in SCA27B.

Many studies have reported motor impairments in autistic spectrum disorders (ASD). However, the brain mechanism underlying motor impairment in ASD remains unclear. Recent neuroimaging studies have suggested that underconnectivity between the cerebellum and other brain regions contributes to the features of ASD. In this study, we investigated the microstructural integrity of the cerebellar pathways, including the superior, middle, and inferior cerebellar peduncles, of children with and without ASD by using diffusion tensor imaging (DTI) tractography to determine whether the microstructural integrity of the cerebellar pathways is related to motor function in children with ASD. Thirteen children with ASD and 11 age-, gender-, handedness-, and IQ-matched typically developing (TD) controls were enrolled in this study. DTI outcome measurements, such as fractional anisotropy (FA), axial diffusivity (AD), and radial diffusivity (RD), for the cerebellar pathways were calculated. The Movement Assessment Battery for Children 2 (M-ABC 2) was used for assessing motor functions. There were no significant differences between the two groups in RD. However, compared to the TD subjects, patients with ASD had a significantly lower FA in the right superior cerebellar peduncle and lower AD in the left superior cerebellar peduncle, in addition to a significantly lower score in ball skills and the total test score of M-ABC 2. There was a significant positive correlation between the total test score of M-ABC 2 and FA in the right superior cerebellar peduncle in the ASD group. These findings suggest that the altered microstructural integrity of the superior cerebellar peduncle may be related to motor impairment in ASD.

Mobility impairment is associated with reduced microstructural integrity of the inferior and superior cerebellar peduncles in elderly with no clinical signs of cerebellar dysfunction

A better understanding of the surgical anatomy of the cerebellar peduncles in different surgical approaches and their relationship with other neural structures are delineated through cadaveric dissections. We aimed to revisit the surgical anatomy of the cerebellar peduncles to describe their courses along the brain stem and the cerebellum and revise their segmental classification in surgical areas exposed through different approaches. Stepwise fiber microdissection was performed along the cerebellar tentorial and suboccipital surfaces. Multiple surgical approaches in each of the cerebellar peduncles were compared in eight silicone-injected cadaveric whole heads to evaluate the peduncular exposure areas. From a neurosurgical point of view, the middle cerebellar peduncle (MCP) was divided into a proximal cisternal and a distal intracerebellar segments; the inferior cerebellar peduncle (ICP) into a ventricular segment followed by a posterior curve and a subsequent intracerebellar segment; the superior cerebellar peduncle (SCP) into an initial congregated, an intermediate intraventricular, and a distal intramesencephalic segment. Retrosigmoid and anterior petrosectomy approaches exposed the junction of the MCP segments; telovelar, supratonsillar, and lateral ICP approaches each reached different segments of ICP; paramedian supracerebellar infratentorial, suboccipital transtentorial, and combined posterior transpetrosal approaches displayed the predecussation SCP within the cerbellomesencephalic fissure, whereas the telovelar approach revealed the intraventricular SCP within the superolateral recess of the fourth ventricle. Better understanding of the microsurgical anatomy of the cerebellar peduncles in various surgical approaches and their exposure limits constitute the most critical aspect for the prevention of surgical morbidity during surgery in and around the pons and the upper medulla. Our findings help in evaluating radiological data and planning an operative procedure for cerebellar peduncles.

The infratentorial paramedian supracerebellar approach is useful for lesions involving the upper brain stem or the cerebellar pedunculi. We provide a precise description of this approach and its indications. Four patients were operated on using this approach. The lesions included a squamous cell carcinoma, a glioblastoma multiforme, a hematoma, and a cavernoma. The lesions involved the quadrigeminal plate, the superior cerebellar peduncle, the middle cerebellar peduncle, and the quadrangular lobule of the cerebellum. Postoperatively, there was no apparent aggravation of neurological symptoms. The main advantage of the approach was that the operation can be performed with a wide horizontal view without sacrificing the deep veins. It is concluded that lesions in the superior and inferior colliculus, superior and middle cerebellar peduncles, and quadrangular lobules of the cerebellum can be safely operated on with this approach.

To develop a new tract imaging technique for visualising small fibre tracts of the brainstem and for detecting the abnormalities in multiple system atrophy of the cerebellar type (MSA-C) using a phase difference enhanced (PADRE) imaging technique, in which the phase difference between the target and surrounding tissue is selectively enhanced. Two neuroradiologists compared the high-spatial-resolution PADRE imaging, which was acquired from six healthy volunteers, three patients with MSA-C, and 7 patients with other types of neurodegenerative diseases involving the brainstem or cerebellum. Various fine fibre tracts in the brainstem, the superior and inferior cerebellar peduncles, medial lemniscus, spinothalamic tract, medial longitudinal fasciculus, central tegmental tract, corticospinal tract and transverse pontine fibres, were identified on PADRE imaging. PADRE imaging from MSA-C demonstrated the disappearance of transverse pontine fibres and significant atrophy of the inferior cerebellar peduncles, while the superior cerebellar peduncles were intact. PADRE imaging also demonstrated that the transverse pontine fibres and inferior cerebellar peduncle were not involved in the other neurodegenerative diseases. PADRE imaging can offer a new form of tract imaging of the brainstem and may have the potential to reinforce the clinical utility of MRI in differentiating MSA from other conditions.

To confirm the hypothesis that brain white matter damage is involved in the pathogenesis and disease progression of Langerhans cell histiocytosis (LCH)-associated neurodegenerative disease (ND), we aimed to analyze pediatric patients with LCH using diffusion tensor imaging (DTI). We enrolled 33 patients with LCH and obtained 33 DTI datasets. Using DTI-based tractography, fractional anisotropy (FA), apparent diffusion coefficient (ADC), axial diffusivity (AD), and radial diffusivity (RD) were measured in the cerebral and cerebellar white matter tracts. The participants were divided into three groups-non-ND, ND without clinical symptoms (r-ND), and ND with clinical symptoms (c-ND)-according to their clinical status during the examination with DTI. We compared the DTI parameters in white matter tracts were compared among the three groups. In the order of non-ND, r-ND, and c-ND groups, the FA in superior cerebellar peduncle (SCP) and middle cerebellar peduncle (MCP) significantly decreased, the ADC, AD, and RD of MCP, and the RD of SCP were significantly elevated (FA-SCP; p < 0.001, FA-MCP; p = 0.026, ADC-MCP; p < 0.001, AD-MCP; p = 0.002, RD-MCP; p = 0.003, and RD-SCP; p = 0.018). Furthermore, in the simple linear regression analysis, the FA, ADC, AD, and RD values in the MCP and the FA value in the SCP were significantly influenced by the presence of neurological symptoms and ND findings on MRI (all p < 0.001). In LCH-ND, we identified microstructural damage in the SCP and MCP. DTI parameters in these tracts may help monitor LCH-ND; therefore, future studies are required to validate these results in a large cohort.

Cerebellar mutism syndrome (CMS) is a morbid complication of posterior fossa surgery in children. This review focuses on the current understanding of pathophysiology in the white matter tracts (WMT) using diffusion tensor imaging (DTI). A series of 38 patients operated on for posterior fossa tumors in our institute between December 2019 till May 2021 were evaluated neurologically along with characteristics of mutism and DTI imaging (fractional anisotropy) in preoperative and postoperative periods. We also noted tumor size, location, volume, brainstem compression, cerebellar peduncle involvement, extent of resection, surgical approach, and histopathology. Cerebellar mutism developed in five patients. The mean age, male sex, tumor size >5cm, superior cerebellar peduncle involvement, and vermian incision were found to have positive correlation with the development of CMS. They showed reduction in fractional anisotropy in superior cerebellar peduncle (SCP) following resection; however, others' WMT did not show any significant change in fractional anisotropy values pre- or post-surgery. Our study suggests that functional disruption of WMT, i.e., superior cerebellar peduncle and dentato-thalamo-cortical tract (DTC) is the underlying pathophysiological component of CMS. Taking this into consideration, we need to deploy techniques to limit the damage to the superior cerebellar peduncle and DTC using neurophysiological monitoring.

The cerebellum communicates with the cerebral cortex via the superior, middle, and inferior cerebellar peduncles (CPs). To preserve the structure and function of the brainstem and cerebellum, which is compressed in various pathological conditions, it is important to delineate the spatial interrelationship of the CPs for presurgical planning and intraoperative guidance. Diffusion tensor tractography (DTT) is a technique capable of depicting the major fiber bundles in CPs. However, routine use of this technology for brainstem visualization remains challenging due to the anatomical smallness and complexity of the brainstem and susceptibility-induced image distortions. Here, we attempt to visualize CPs using high-resolution DTT in a commercial equipment for the application of this technique in normal clinical settings. DTT and fast imaging employing steady-state acquisition-cycled phases (FIESTA) of the whole brainstem were performed. We rendered the DTT fiber bundle using a region-of-interest-based fiber tracking method onto the structural image generated in FIESTA by automatic image coregistration. Fibers of the CPs were clearly visualized by DTT. The DTT-FIESTA overlaid image revealed the cross-sectional and three-dimensional anatomy of the pyramidal tract and the ascending sensory fibers, in addition to the CPs. This could indicate a geometrical relationship of these fibers in the brainstem. The CPs could be visualized clearly using DTT within clinically acceptable scanning times. This method of visualizing the exact pathway of fiber bundles and cranial nerves in the skull base helps in the planning of surgical approaches.

Over the last decades, the importance of cerebellar processing for cortical functions has been acknowledged and consensus was reached on the strict functional and structural cortico-cerebellar interrelations. From an anatomical point of view strictly contralateral interconnections link the cerebellum to the cerebral cortex mainly through the middle and superior cerebellar peduncle. Diffusion MRI (dMRI) based tractography has already been applied to address cortico-cerebellar-cortical loops in healthy subjects and to detect diffusivity alteration patterns in patients with neurodegenerative pathologies of the cerebellum. In the present study we used dMRI-based tractography to determine the degree and pattern of pathological changes of cerebellar white matter microstructure in patients with focal cerebellar lesions. Diffusion imaging and high-resolution volumes were obtained in patients with left cerebellar lesions and in normal controls. Middle cerebellar peduncles and superior cerebellar peduncles were reconstructed by multi fiber diffusion tractography. From each tract, measures of microscopic damage were assessed, and despite the presence of unilateral lesions, bilateral diffusivity differences in white matter tracts were found comparing patients with normal controls. Consistently, bilateral alterations were also evidenced in specific brain regions linked to the cerebellum and involved in higher-level functions. This could be in line with the evidence that in the presence of unilateral cerebellar lesions, different cognitive functions can be affected and they are not strictly linked to the side of the cerebellar lesion.

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In this study, we aimed to evaluate the importance of cerebellum in freezing of gait (FOG) pathophysiology. Due to the fundamental role of the cerebellum in posture and gait control, we examined cerebellar structural and functional connectivity (FC) in patients with PD and FOG. We recruited 15 PD with FOG (PD-FOG), 16 PD without FOG (PD-nFOG) patients, and 16 healthy subjects (HS). The FOG Questionnaire (FOG-Q) assessed FOG severity. Three tesla-MRI study included resting-state functional MRI, diffusion tensor imaging (DTI), and 3D T1-w images. We located seed regions in the cerebellar locomotor region, fastigial, and dentate nucleus to evaluate their FC. DTI parameters were obtained on the superior, middle, and inferior cerebellar peduncles. Global and lobular cerebellum volumes were also calculated. Cerebellar locomotor and fastigial FC was higher in cerebellar and posterior cortical areas in PD-FOG than in HS. FC of the cerebellar locomotor region with cerebellar areas positively correlated with FOG-Q. Dentate FC was lower in the prefrontal and parieto-occipital cortices in PD-FOG than in HS and in the brainstem, right basal ganglia, and frontal and parieto-occipital cortices than in PD-nFOG. DTI parameters in superior and middle cerebellar peduncles were altered in PD-FOG compared with PD-nFOG and significantly correlated with FOG-Q. There were no differences in cerebellar volumes between PD-FOG and either PD-nFOG or HS. Our results suggest that altered connectivity of the cerebellum contributes to the pathophysiology of FOG. FC of the cerebellar locomotor region and white matter (WM) properties of cerebellar peduncles correlate with FOG severity, supporting the hypothesis that abnormal cerebellar function underlies FOG in PD.

The connections between the cerebellum and the hypothalamus have been well documented. However, the specific cerebellar peduncle through which the hypothalamo-cerebellar and cerebello-hypothalamic connections pass has not been demonstrated. The present study aims to define the specific cerebellar peduncle through which connects the cerebellum to specific hypothalamic nuclei. Seventeen male albino rats received 20-50-nl pressure injections of either Fluoro-Gold (FG) or biotinylated dextran amine (BDA) tracer into the superior (SCP), middle (MCP), and inferior (ICP) cerebellar peduncle. Following 7-10days of survival period, the animals were processed according to the appropriate protocol for the two tracers used. Labeled cells and axons were documented using light or fluorescence microscopy. The present study showed connections between the hypothalamus and the cerebellum via both the SCP and the MCP but not the ICP. The hypothalamo-cerebellar connections via the SCP were from the lateral, dorsomedial, paraventricular, and posterior hypothalamic nuclei, and cerebello-hypothalamic connections were to the preoptic and lateral hypothalamic nuclei. The hypothalamo-cerebellar connections via the MCP were from the lateral, dorsomedial, ventromedial, and mammillary hypothalamic nuclei; and cerebello-hypothalamic connections were to the posterior, arcuate, and ventromedial hypothalamic nuclei. The hypothlamo-cerebellar connections were denser compared to the cerebello-hypothlamic connections via both the SCP and the MCP. The connection between the cerebellum and the hypothalamus was more prominent via the SCP than MCP. Both the hypothlamo-cerebellar and cerebello-hypothalamic connections were bilateral, with ipsilateral preponderance. Reciprocal connections were with the lateral hypothalamic nucleus via the SCP and the ventromedial nucleus via the MCP were observed. Cerebellum takes part in the higher order brain functions via its extensive connections. The knowledge of hypothalamo-cerebellar and cerebello-hypothalamic connections conveyed within the SCP and MCP can be important for the lesions involving the MCP and SCP. These connections can also change the conceptual architecture of the cerebellar circuitry and deepen current understanding.

This study used diffusion tensor imaging tractography at 3T MRI to relate cognitive function to white matter tracts in the brain. Brain T2 fluid attenuated inversion recovery-weighted and diffusion tensor 3T MRI scans were acquired in thirty-three healthy participants without mild cognitive impairment or dementia. They completed a battery of neuropsychological tests including the Montreal Cognitive Assessment, Stroop test, Trail Making Test B, Wechsler Memory Scale-III Longest span forward, Wechsler Memory Scale-III Longest span backward, Mattis Dementia Rating Scale, California Verbal Learning Test Version II Long Delay Free Recall,and Letter Number Sequencing. Tractography was generated by the Fiber Assignment by Continuous Tracking method. The corpus callosum, cingulum, long association fibers, corticospinal/bulbar tracts, thalamic projection fibers, superior cerebellar peduncle, middle cerebellar peduncle and inferior cerebellar peduncle were manually segmented. The fractional anisotropy (FA) and mean diffusivity (MD) of these tracts were quantified. We studied the association between cognitive test scores and the MD and FA of tracts while controlling for age and total white matter hyperintensities volume. Worse scores on the Stroop test was associated with decreased FA of the corpus callosum, corticospinal/bulbar tract, and thalamic projection tracts. Scores on the other cognitive tests were not associated with either the FA or MD of measured tracts. In healthy persons the Stroop test appears to be a better predictor of the microstructural integrity of white matter tracts measured by DTI tractography than other cognitive tests.

Cortical lesions of the supplementary motor area (SMA) are important in balance control and postural recovery in stroke patients, while the role of subcortical lesions of the SMA has not been studied. This study aimed to investigate the subcortical projections of the SMA and its relationship with ataxia in supratentorial stroke patients. Thirty-three patients with hemiparesis were divided into 3 groups (severe ataxia, n = 9; mild to moderate ataxia, n = 13; no ataxia, n = 11). Ataxia severity was assessed using the Scale for Ataxia Rating Assessment. Diffusion tensor imaging analysis used the fractional anisotropy (FA) values and tract volume as parameters of white matter tract degeneration. The FA values of regions related to ataxia were analyzed, that is the SMA, posterior limb of the internal capsule, basal ganglia, superior cerebellar peduncle, middle cerebellar peduncle, inferior cerebellar peduncle, and cerebellum. Tract volumes of the corticostriatal tract and cortico-ponto-cerebellar (CPC) tract originating from the SMA were evaluated. There were significant differences among the 3 groups in FA values of the subcortical regions of the CPC tract. Furthermore, the volume of the CPC tract originating from the SMA showed significant negative correlation with ataxia severity. There was no correlation between ataxia and corticostriatal tract volume. Therefore, we found that subcortical lesions of the CPC tract originating from the SMA could contribute to ataxia severity in stroke patients with ataxic hemiparesis.

The sequence of myelination in the brainstem of the rat monitored by myelin basic protein immunohistochemistry