Criteria-unfulfilled multiple system atrophy at an initial stage exhibits laterality of middle cerebellar peduncles.

To elucidate clinically useful imaging characteristics of multiple system atrophy with predominant cerebellar ataxia (MSA-C) at the initial stage showing pure cerebellar ataxia but unfilling consensus criteria (MSA-pc), clinical and neuroradiological analyses on cerebral MRI and single-photon emission computed tomography (SPECT) for measuring regional cerebral blood flow (rCBF) were performed. Seven MSA-pc patients meeting the above condition at an initial evaluation were identified, and all the MSA-pc patients later developed autonomic dysfunction and finally fulfilled the criteria for probable or possible category of MSA-C. For comparison, two patients with spinocerebellar ataxia type 6 and three patients with idiopathic cerebellar ataxia who did not exhibit autonomic dysfunction for more than three years were enrolled in this study (non-MSA-pc). As non-ataxic controls without cerebellar involvement, seven patients with Parkinson's disease were also enrolled. As a result, MRI analysis clarified a smaller pontine area and significant laterality of middle cerebellar peduncle (MCP) width in MSA-pc in comparison to non-MSA-pc and controls. SPECT analysis revealed that pontine rCBF was reduced even at the initial stage of MSA-pc. Moreover, the laterality of cerebellar rCBF values and the laterality of MCP width in MSA-pc patients exhibited a significant positive correlation, indicating anatomical and functional laterality of afferent projections to cerebellum is a characteristic finding for MSA-pc. These neuroimaging characteristics could be clinically useful to consider the possibility of the criteria-unfulfilled MSA and promote an earlier intervention after obtaining a diagnosis of probable MSA-C.

Diagnostic efficacy of the magnetic resonance T1w/T2w ratio for the middle cerebellar peduncle in multiple system atrophy and spinocerebellar ataxia: A preliminary study.

BACKGROUND: The standardized T1-weighted/T2-weighted (sT1w/T2w) ratio for the middle cerebellar peduncle (MCP) has been reported to be sensitive for detecting degenerative changes in the cerebellar subtype of multiple system atrophy (MSA-C), even in the early stages. We aimed to investigate the diagnostic value of the MCP sT1w/T2w ratio for differentiating between MSA-C and spinocerebellar ataxia (SCA). METHODS: We included 32 MSA-C, 8 SCA type 3 (SCA3), 16 SCA type 6 (SCA6) patients, and 17 controls, and the MCP sT1w/T2w ratio was analyzed using a region-of-interest approach. The diagnostic performance of the MCP sT1w/T2w ratio in discriminating among MSA-C, SCA3, and SCA6 was assessed and compared with diagnosis based on visual interpretation of MCP hyperintensities and the "hot cross bun" (HCB) sign. RESULTS: MCP sT1w/T2w ratio values were markedly lower in patients with MSA-C than in those with SCA3, those with SCA6, and controls (p < 0.001). The MCP sT1w/T2w ratio showed high diagnostic accuracy for distinguishing MSA-C from SCA3 (area under curve = 0.934), SCA6 (area under curve = 0.965), and controls (area under curve = 0.980). The diagnostic accuracy of the MCP sT1w/T2w ratio for differentiating MSA-C from SCA3 or SCA6 (90.0% for MSA-C vs. SCA3, and 91.7% for MSA-C vs. SCA6) was comparable to or superior than that of visual interpretation of MCP hyperintensities (80.0-87.5% in MSA-C vs. SCA3 and 87.6-97.9% in MSA-C vs. SCA6) or the HCB sign (72.5-80.0% in MSA-C vs. SCA3 and 77.1-93.8% in MSA-C vs. SCA6). CONCLUSIONS: The MCP sT1w/T2w ratio might be a sensitive imaging-based marker for detecting MSA-C-related changes and differentiating MSA-C from SCA3 or SCA6.

How I do it: horizontal fissure approach to the middle cerebellar peduncle.

BACKGROUND: The horizontal fissure approach is a workhorse for brainstem lesions in the central and dorsolateral pons and middle cerebellar peduncle (MCP). The cerebellopontine fissure is a V-shaped fissure with a superior and inferior limb between the cerebellum, pons, and MCP. The horizontal or petrosal fissure is at the apex of the cerebellopontine fissure and extends laterally to divide the petrosal surface of the cerebellum into superior and inferior parts. Splitting this fissure exposes the posterolateral aspect of the MCP without excessive retraction or transgression of the cerebellum. METHOD: We demonstrate and describe the horizontal fissure operative approach to the middle cerebellar peduncle for resection of a pontine cavernoma with illustrative figures and operative video. CONCLUSION: Splitting the horizontal (petrosal) fissure of the cerebellum brings the middle cerebellar peduncle into view behind the root entry zone of the trigeminal nerve, providing an expanded, safe corridor to the central and dorsolateral pons.

Primary intracranial germ cell tumour originating from right brachium Pontis with hypertrophic Olivary degeneration: a case report.

BACKGROUND: Primary right brachium pontis germinoma with hypertrophic olivary degeneration (HOD) is extremely rare. A preoperative diagnosis is challenging due to the absence of characterized clinical and neuroimaging features, and biopsy should be considered. CASE PRESENTATION: A 20-year-old male patient presented with a case of primary intracranial germinoma originating from right brachium pontis with HOD manifesting as ocular myoclonus, nystagmus in both eyes, ataxic gait and incoordination of the limbs. Magnetic resonance imaging (MRI) revealed an irregular patchy lesion with hyperintensity on T2-weighted images (T2WI) and T2 fluid-attenuated inversion recovery (FLAIR) without enhancement by gadolinium (Gd). Furthermore, a focal hyperintense nodule on T2WI in the left inferior olive nucleus (ION) of the medulla oblongata was considered hypertrophic olivary degeneration (HOD) based on the patient's symptoms and neuroimaging findings. Due to suspected demyelinating disease and low-grade glioma (LGG), a biopsy was planned. The pathological diagnosis was germinoma. Subsequently, he received chemoradiation therapy, resulting in the improvement of neurological deficits and the disappearance of the lesion on MRI. CONCLUSION: A case of "Primary right brachium pontis germinoma with HOD" is reported for the first time. A preoperative diagnosis is challenging due to the fact of absence of clinical signs and symptoms and neuroimaging characteristics. However, patients can have favourable prognoses with appropriate evaluation and treatment.

Clinical Significance of Middle Cerebellar Peduncle Ischemia After Translabyrinthine Vestibular Schwannoma Resection.

OBJECTIVE: To assess clinical symptoms, signs, and radiographic evolution of middle cerebellar peduncle (MCP) diffusion restriction (DR) abnormalities following vestibular schwannoma (VS) resection. STUDY DESIGN: Retrospective chart and imaging review. SETTING: Tertiary-referral neurotology and neurosurgery practice. PATIENTS: All consecutive patients who underwent translabyrinthine VS resection over a 2-year period (August 2017-May 2019). INTERVENTION: Translabyrinthine craniotomy for VS resection. MAIN OUTCOME MEASURES: Magnetic resonance imaging (MRI) obtained on postoperative day 1 were reviewed for DR within the pons and cerebellum, with 3 months follow-up MRI to assess for evolution of these vascular changes. RESULTS: Of the 31 patients who met inclusion criteria, MRI demonstrated MCP DR consistent with acute ischemia in 29% (9/31). Of those, two showed corresponding T2 signal abnormalities on follow up MRI consistent with cerebrovascular accident (CVA) within the MCP. Both had severe gait ataxia and dysmetria requiring acute rehabilitation admission and significantly larger tumors (p = 0.02). The remaining seven were asymptomatic, and DR abnormality resolved without lasting radiographic changes. Brainstem compression was present in 100% of patients with postoperative MCP DR (mean MCP ipsilateral:contralateral ratio 0.59 ±â€Š0.19), and 68.1% of those without (mean MCP ratio 0.71 ±â€Š0.25), a difference that was not statistically significant (p = 0.14). In the two patients with CVA, MCP asymmetry persisted, whereas the asymmetry resolved in all others. CONCLUSIONS: Asymptomatic acute MCP ischemia discovered incidentally does not require intervention. However, when the ischemic area is large and patients are symptomatic, especially if an acute rehabilitation admission is required, surgeons should suspect true CVA.

Characterization and diagnostic potential of diffusion tractography in multiple system atrophy.

INTRODUCTION: Microstructural integrity of the middle cerebellar peduncle (MCP) and the putamen captured by diffusion-tensor imaging (DTI) is differentially affected in the parkinsonian and cerebellar variants of multiple system atrophy (MSA-P, MSA-C) compared to Parkinson's disease (PD). The current study applied DTI and tractography in order to 1) characterize the distribution of DTI metrics along the tracts of the MCP and from the putamen in MSA variants, and 2) evaluate the usefulness of combining these measures for the differential diagnosis of MSA-P against PD in the clinical setting. METHODS: Twenty-nine MSA patients (MSA-C, n = 10; MSA-P, n = 19), with a mean disease duration of 2.8 ± 1.7 years, 19 PD patients, and 27 healthy controls (HC) were included in the study. Automatized tractography with a masking procedure was employed to isolate the MCP tracts. DTI measures along the tracts of the MCP and within the putamen were acquired and jointly used to classify MSA vs. PD, and MSA-P vs. PD. Putamen volume was additionally tested as classification feature in post hoc analyses. RESULTS: DTI measures within the MCP and putamen showed significant alterations in MSA variants compared to HC and PD. Classification accuracy for MSA vs. PD and MSA-P vs PD using diffusion measures was 91.7% and 89.5%, respectively. When replacing the putaminal DTI measure by a normalized measure of putamen volume classification accuracy improved to 95.8% and 94.7%, respectively. CONCLUSION: Multimodal information from MCP tractography and putamen volume yields excellent diagnostic accuracy to discriminate between early-to-moderately advanced patients with MSA and PD.

Neuroanatomical MRI study: reference values for the measurements of brainstem, cerebellar vermis, and peduncles.

OBJECTIVES: To set age-specific normal reference values for brainstem, cerebellar vermis, and peduncles measurements and characterize values' variations according to gender, age, and age by gender interaction. METHODS: 565 normal brain magnetic resonance examinations with normal anatomy and signal intensity of the supra- and infratentorial structures were categorized into six age groups (infant, child, adolescent, young adult, middle-age adult, and old aged adults). Patients with congenital malformations, gross pathology of the supra- or infratentorial brain, brain volume loss, developmental delay, metabolic disorders, and neuropsychological disorders (n = 2.839) were excluded. On midsagittal T1 weighted and axial T2 weighted images specific linear diameters and ratios of the brainstem, cerebellar vermis, and peduncles were attained. Two observers assessed a random sample of 100 subjects to evaluate the inter- and intraobserver reproducibility. Intraclass correlation coefficients, means ± standard deviation, one and two-way analysis of variance tests were used in the statistical analysis. RESULTS: Good to excellent inter- and intraobserver measurements' reproducibility were observed, except for the transverse diameter of the midbrain, the anteroposterior diameter of the medulla oblongata at the pontomedullary and cervicomedullary junctions, cerebellar vermis anteroposterior diameter, and thickness of the superior cerebellar peduncle. Age-specific mean values of the investigated measurements were established. A significant gender-related variation was recorded in the anteroposterior diameter of the basis pontis (p = 0.044), the anteroposterior diameter of the medulla oblongata at the cervicomedullary junction (p = 0.044), and cerebellar vermis height (p = 0.018). A significant age-related change was detected in all measurements except the tectal ratio. Age by gender interaction had a statistically significant effect on the tectal ratio, inferior, and middle cerebellar peduncles' thickness (p = 0.001, 0.022, and 0.028, respectively). CONCLUSION: This study provides age-specific normal mean values for various linear dimensions and ratios of the posterior fossa structures with documentation of measurements' variability according to gender, age, and their interaction. ADVANCES IN KNOWLEDGE: It provides a valuable reference in the clinical practice for easier differentiation between physiological and pathological conditions of the posterior fossa structures especially various neurodegenerative diseases and congenital anomalies.

Delayed Maturation of the Middle Cerebellar Peduncles at Near-Term Age Predicts Abnormal Neurodevelopment in Preterm Infants.

BACKGROUND: The infant brain grows quickly with elaborate microstructural development during the neonatal period. The white matter, during critical periods of development, is selectively vulnerable to altered maturation and impaired growth in very-low-birth-weight (VLBW) infants. OBJECTIVE: To evaluate whether abnormal white matter maturation in VLBW infants is associated with poor neurodevelopmental outcomes at 18 months of corrected age. METHODS: Between 2015 and 2017, we recruited 60 VLBW infants at 24-32 weeks of gestational age and 15 full-term controls. All participants underwent magnetic resonance imaging at near-term age and were assessed at 18 months of corrected age with the Bayley Scales of Infant and Toddler Development, Third Edition. The associations between regional white matter fractional anisotropy (FA) and mean diffusivity on diffusion tensor imaging (DTI) and developmental outcomes were explored using multivariable linear regression after correcting for gestational age, postmenstrual age at DTI scan, and maternal education level. RESULTS: The FA values of the splenium of the corpus callosum (p = 0.032), corticospinal tract (p = 0.025), middle cerebellar peduncle (MCP) (p < 0.001), and cingulum (p = 0.043) were significantly related to cognitive scores; however, only the association corresponding to the MCP remained significant after correcting for multiple comparisons. The MCP FA (p = 0.008) was associated with motor scores after correction for multiple comparisons (p = 0.008). Cognitive impairment (area under the curve [AUC] = 0.823, 95% confidence interval [CI] = 0.722-0.911) and motor impairment (AUC = 0.776, 95% CI = 0.656-0.899) were predicted by MCP FA. CONCLUSIONS: The FA of MCP at near-term age may predict developmental outcomes of VLBW infants at 18 months of corrected age.

Individual voxel-based morphometry adjusting covariates in multiple system atrophy.

INTRODUCTION: This study aimed to evaluate whether novel individual voxel-based morphometry adjusting covariates (iVAC), such as age, sex, and total intracranial volume, could increase the accuracy of a diagnosis of multiple system atrophy (MSA) and enable the differentiation of MSA from Parkinson's disease (PD). METHODS: We included 53 MSA patients (MSA-C: 33, MSA-P: 20), 53 PD patients, and 189 healthy controls in this study. All participants underwent high-resolution T1-weighted imaging (WI) and T2-WI with a 3.0-T MRI scanner. We evaluated the occurrence of significant atrophic findings in the pons/middle cerebellar peduncle (MCP) and putamen on iVAC and compared these findings with characteristic changes on T2-WI. RESULTS: On iVAC, abnormal findings were observed in the pons/MCP of 96.2% of MSA patients and in the putamen of 80% of MSA patients; however, on T2-WI, they were both observed at a frequency of 60.4% in MSA patients. On iVAC, all but one MSA-P patient (98.1%) showed significant atrophic changes in the pons/MCP or putamen. By contrast, 69.8% of patients with MSA showed abnormal signal changes in the pons/MCP or putamen on T2-WI. iVAC yielded 95.0% sensitivity and 96.2% specificity for differentiating MSA-P from PD. CONCLUSION: iVAC enabled us to recognize the morphological characteristics of MSA visually and with high accuracy compared to T2-WI, indicating that iVAC is a potential diagnostic screening tool for MSA.

Primary Middle Cerebellar Peduncle Lymphoma with Primary Cervical Lymphoma: Repeated Misdiagnosis.

The coexistence of primary intracranial and extracranial lymphomas is relatively rare. In this study, we report a misdiagnosed case of primary middle cerebellar peduncle lymphoma (PMCPL) with primary cervical lymphoma. This case illustrates that hearing loss may be the only manifestation of PMCPL, which can be easily misdiagnosed as sudden deafness or acoustic neuroma. Patients with PMCPL may also have primary extracranial lymphoma, which should not be misdiagnosed as metastatic tumor.

Magnetic resonance T1w/T2w ratio in the middle cerebellar peduncle might be a sensitive biomarker for multiple system atrophy.

OBJECTIVE: We aimed to investigate the use of a myelin-sensitive MRI contrast, the standardized T1-weighted/T2-weighted (sT1w/T2w) ratio, for detecting early changes in the middle cerebellar peduncle (MCP) in cerebellar subtype multiple system atrophy (MSA-C) patients. METHODS: We included 28 MSA-C patients, including a subset of 17 MSA-C patients within 2 years of disease onset (early MSA-C), and 28 matched healthy controls. T1w and T2w scans were acquired using a 3-T MR system. The sT1w/T2w ratio in MCP was analyzed using SPM12 by utilizing a region-of-interest approach in normalized space. The diagnostic performance of the MCP sT1w/T2w ratio in discriminating MSA-C and the subgroup of early MSA-C from the matched controls was assessed. Correlation analyses were performed to evaluate the relationship between the MCP sT1w/T2w ratio and other clinical parameters including the International Cooperative Ataxia Scale (ICARS) score for quantifying cerebellar ataxia. RESULTS: Compared to controls, the sT1w/T2w ratio in the MCP was markedly lower in all (p < 0.001) MSA-C patients and 17 early (p < 0.001) MSA-C patients. The MCP sT1w/T2w ratio had high sensitivity (96%) and specificity (100%) to distinguish MSA-C from controls (area under the curve = 0.99), even for the early MSA-C group (area under the curve = 0.99; sensitivity = 94%, specificity = 100%). The MCP sT1w/T2w ratio correlated with the ICARS score in early MSA-C. CONCLUSIONS: The sT1w/T2w ratio can detect MSA-C-related changes in the MCP, even in the early stages of the disorder, and could be a sensitive biomarker for MSA-C. KEY POINTS: • The sT1w/T2w ratio can detect MSA-C-related changes in the middle cerebellar peduncle, even in the early stages of the disorder. • The middle cerebellar peduncle sT1w/T2w ratio correlated with a cerebellar ataxia score in early MSA-C patients.

Outcomes at 6 months are related to brain structural and white matter microstructural reorganization in idiopathic tinnitus patients treated with sound therapy.

This study aimed to explore brain structural and white matter microstructural reorganization in the early stage of tinnitus and identify brain alterations that contribute to its relief after 6 months of sound therapy. We studied 64 patients with idiopathic tinnitus, including 29 patients who were categorized into an effective group (EG) and 35 who were categorized into an ineffective group (IG) according to the 6-month follow-up improvement of the Tinnitus Handicap Inventory score, along with 63 healthy controls (HCs). All participants underwent structural and diffusion tensor imaging scanning on a 3-T magnetic resonance system. Differences in brain gray/white matter volume and white matter microstructure were evaluated using voxel-based morphometry analysis and tract-based spatial statistics among the three groups. Associations between brain reorganization and the improvement of tinnitus symptoms were also investigated. Compared with EG patients, IG patients experienced a significant gray matter volume decrease in the right middle frontal gyrus (MFG)/right precentral gyrus (PreCG). Meanwhile, both EG and IG patients showed significant changes (decrease or increase) in brain white matter integrity in the auditory-related or nonauditory-related white matter fiber tracts compared with HCs, while EG patients showed decreased axial diffusivity in the bilateral middle cerebellar peduncle (MCP) compared with IG patients. We combined the gray matter change of the MFG/PreCG and the white matter integrity of the bilateral MCP as an imaging indicator to evaluate the patient's prognosis and screen patients before treatment; this approach reached a sensitivity of 77.1% and a specificity of 82.8%. Our study suggests that there was a close relationship between brain reorganization and tinnitus improvement. The right MFG/PreCG and bilateral MCP may be indicators that can be used to predict prognoses in patients with idiopathic tinnitus and may be used to screen patients before sound therapy. These findings may provide new useful information that can lead to a better understanding of the tinnitus mechanism.

The switch from Gd-DTPA to Gd-DOTA is not associated with decrease of the T1 signal intensity of the pallidus and dentate in a pediatric population.

BACKGROUND: The switch from the linear gadolinium-based contrast agent (GBCA) gadopentate dimeglumine (Gd_DTPA) to the macrocyclic GBCA gadobutrol is associated with a decrease of the T1 signal intensity (SI) in brain gray matter nuclei. The effects of the switch to other macrocyclic GBCAs are not yet established. PURPOSE: To explore the effects of switching from Gd-DTPA to the macrocyclic GBCA gadoterate meglumine (Gd-DOTA) in pediatric patients. MATERIAL AND METHODS: We measured the pallidus/middle cerebellar peduncle (MCP) SI ratio and the dentate/MCP SI ratio in pre-contrast sagittal T1-weighted spin-echo images in nine patients who had received ≥6 administrations of Gd-DTPA and then of Gd-DOTA, in 18 patients who had received ≥6 administrations of Gd-DOTA alone, and in nine age-matched controls without prior GBCA administrations. Serial assessment was performed in patients who switched from Gd-DTPA to Gd-DOTA. Finally, the rate of change of pallidal/MCP and dentate/MCP SI ratios between the first and last Gd-DOTA administrations was compared. RESULTS: The pallidal/MCP and dentate/MCP SI ratios were (P < 0.05) higher in patients with prior Gd-DTPA and Gd-DOTA administrations compared to the controls. After the switch, the pallidal/MCP SI ratio increased in nine patients and the dentate/MCP ratio in seven patients. The rate of change of pallidal/MCP SI ratio after Gd-DOTA was higher (P < 0.01) in patients who had previously received Gd-DTPA (mean 2.89 ± 2.6%) than in patients who had received Gd-DOTA alone (mean 0.53 ± 0.89%). CONCLUSION: T1 SI in gray matter nuclei does not decrease after switching from Gd-DTPA to Gd-DOTA. The switch effects from Gd-DTPA to each macrocyclic GBCA should be individually evaluated.

Bilateral MCP Infarct Due to Vertebral Giant Cell Arteritis.

We present the case of an 83-year-old woman with an isolated bilateral middle cerebellar peduncles stroke caused by complete occlusion of the right vertebral artery and focal occlusion of the left vertebral artery due to giant cell arteritis. The diagnosis was achieved by integrating MRI, ultrasound study, laboratory data and subsequent pathology analysis after biopsy of the temporal artery.