Motor Organization in Schizencephaly: Outcomes of Transcranial Magnetic Stimulation and Diffusion Tensor Imaging of Motor Tract Projections Correlate with the Different Domains of Hand Function.

OBJECTIVE: Schizencephaly is a rare congenital malformation that causes motor impairment. To determine the treatment strategy, each domain of the motor functions should be appropriately evaluated. We correlated a color map of diffusion tensor imaging (DTI) and transcranial magnetic stimulation (TMS) with the hand function test (HFT) to identify the type of hand function that each test (DTI and TMS) reflects. Further, we attempted to demonstrate the motor neuron organization in schizencephaly. METHOD: This retrospective study was conducted on 12 patients with schizencephaly. TMS was conducted in the first dorsal interosseous (FDI), biceps (BB), and deltoid muscles of the upper extremity, and contralateral MEP (cMEP) and ipsilateral MEP (iMEP) were recorded. The HFT included the grip strength, box and block (B&B), and 9-hole peg test. The schizencephalic cleft was confirmed using magnetic resonance imaging, and the corticospinal tract (CST) was identified using the color map of DTI. The symmetry indices for the peduncle and CST at pons level were calculated as the ratios of the cross-sectional area of the less-affected side and that of the more-affected side. RESULT: In the more-affected hemisphere TMS, no iMEP was obtained. In the less-affected hemisphere TMS, the iMEP response was detected in 9 patients and cMEP in all patients, which was similar to the pattern observed in unilateral lesion. Paretic hand grip strength was strongly correlated with the presence of iMEP (p = 0.044). The symmetry index of the color map of DTI was significantly correlated with the B&B (p = 0.008, R 2 = 0.416), whereas the symmetry index of the peduncle was not correlated with all HFTs. CONCLUSION: In patients with schizencephaly, the iMEP response rate is correlated with the hand function related to strength, while the symmetricity of the CST by the color map of DTI is correlated with the hand function associated with dexterity. Additionally, we suggest the possible motor organization pattern of schizencephaly following interhemispheric competition.

The lateral cerebral peduncle approach to ventrally placed intra-axial midbrain tumors: A technical note.

We describe the anatomical landmarks and surgical feasibility of a novel 'safe' brainstem entry zone to approach ventrally placed intra-axial midbrain tumors. The anatomy of the brainstem was specifically studied to evaluate safe surgical entry zone in the midbrain on two formalin fixed silicon injected cadaver head specimens. A novel entry point through the lateral one - fifth of the cerebral peduncle was identified to be 'safe' to approach lesions of the ventral midbrain. Three patients, having oculomotor schwannoma, peduncular glioma and a peduncular cavernoma were operated using this safe entry zone. To approach the midbrain, retrosigmoid lateral supracerebellar route was used in two patients and a basal subtemporal avenue was deployed in one patient. On the basis of fine microanatomical dissection on cadavers, a novel entry point through the lateral one-fifth of the cerebral peduncle, 5 mm anterior to the lateral mesencephalic sulcus and approximately 5 mm superior to the fourth nerve was identified. The proposed brainstem entry point traverses the parieto-temporo-occipital pontine fibers and the trajectory is between the corticospinal tracts ventrally and the substantia nigra dorsally. Three patients were operated successfully using the approach. There were no post-operative motor, sensory or extra-pyramidal deficits. The corridor through the lateral one-fifth of the cerebral peduncle presents a safe and relative 'easy' surgical route to approach ventrally placed intra-axial midbrain tumors.

An Eyebrow, Supracarotid Triangle Approach for Lesions at the Ventral Thalamopeduncular Junction: A Technical Report.

BACKGROUND: Lesions arising at the ventral thalamopeduncular junction are difficult to resect. In addition to being relatively inaccessible, these lesions are located in one of the most sensitive areas of the brain. A critical question is whether new approaches could be developed to allow surgeons to adequately resect these lesions with reasonable outcomes. In the present report, we describe our approach to resect lesions in this region of the brain using an eyebrow craniotomy approach with a trajectory through the supracarotid triangle. METHODS: Through retrospective data collection, we present a small series of patients who had undergone an eyebrow, supracarotid triangle approach to resect lesions located at the thalamopeduncular junction. We describe our surgical technique and report patient outcomes using this approach. RESULTS: Three patients had undergone an eyebrow, supracarotid approach for resection of a lesion arising at the ventral thalamopeduncular junction. Two patients had presented with a cavernoma and one with a pilocytic astrocytoma. Complete resection of all 3 lesions was achieved during surgery without any intraoperative complications. No patient developed permanent contralateral weakness despite entering the peduncle during surgery. One patient developed permanent paresthesia in his left hand. CONCLUSIONS: Lesions arising at the ventral thalamopeduncular junction can be adequately resected with reasonable outcomes using an eyebrow, supracarotid triangle approach. This operative technique establishes another potential operative corridor by which neurosurgeons can resect lesions arising within this relatively inaccessible part of the brain.

Kernohan-Woltman Notch Phenomenon in Two Patients with Subdural Hematoma and Ipsilateral Hemiparesis.

CASE 1: A 9-yr-old boy presents with right subdural hematoma and ipsilateral hemiparesis. Clinical presentation and imaging are consistent with Kernohan-Woltman notch phenomenon. CASE 2: A 19-yr-old man presents with right subdural hematoma and ipsilateral hemiparesis. Clinical presentation is consistent with Kernohan-Woltman notch phenomenon. CLINICAL PEARL: Kernohan-Woltman notch phenomenon is a false-localizing neurologic sign that presents with hemiparesis ipsilateral to the primary lesion. It occurs in the setting of transtentorial herniation, during which the contralateral cerebral peduncle is compressed against the free edge of the tentorium, causing compression of descending corticospinal tract fibers.

Holmes Tremor due to Artery of Percheron Infarct: Clinical Case and Treatment Using Deep Brain Stimulation of the Vim and ZI Targets.

Background: Holmes tremor (HT) arises from disruption of the cerebellothalamocortical pathways. A lesion can interrupt the projection at any point, resulting in this tremor. We describe a case of HT due to the rare artery of Percheron infarct and its successful treatment using deep brain stimulation. Case report: A 62-year-old woman with a right medial cerebral peduncle and bilateral thalamic stroke developed HT. Ventral intermediate nucleus (Vim) zona incerta (ZI) deep brain stimulation (DBS) surgery was performed, with improvement in her tremor. Discussion: Our case supports the theory that the more caudal ZI target in combination with Vim is beneficial in treating poorly DBS-responsive tremors such as HT.

Test-retest reliability and minimal detectable change of corticospinal tract integrity in chronic stroke.

Diffusion tensor imaging (DTI) can be used to index white matter integrity of the corticospinal tract (CST) after stroke; however, the psychometric properties of DTI-based measures of white matter integrity are unknown. The purpose of this study was to examine test-retest reliability as determined by intraclass correlation coefficients (ICC) and calculate minimal detectable change (MDC) of DTI-based measures of CST integrity using three different approaches: a Cerebral Peduncle approach, a Probabilistic Tract approach, and a Tract Template approach. Eighteen participants with chronic stroke underwent DTI on the same magnetic resonance imaging scanner 4 days apart. For the Cerebral Peduncle approach, a researcher hand drew masks at the cerebral peduncle. For the Probabilistic Tract approach, tractography was seeded in motor areas of the cortex to the cerebral peduncle. For the Tract Template approach, a standard CST template was transformed into native space. For all approaches, the researcher performing analyses was blind to participant number and day of data collection. All three approaches had good to excellent test-retest reliability for fractional anisotropy (FA; ICCs >0.786). Mean diffusivity, axial diffusivity, and radial diffusivity were less reliable than FA. The ICC values were highest and MDC values were the smallest for the most automated approach (Tract Template), followed by the combined manual/automated approach (Probabilistic Tract) then the manual approach (Cerebral Peduncle). The results of this study may have implications for how DTI-based measures of CST integrity are used to define impairment, predict outcomes, and interpret change after stroke.

A fixel-based analysis of micro- and macro-structural changes to white matter following adult traumatic brain injury.

Diffusion tensor imaging is often used to assess white matter (WM) changes following traumatic brain injury (TBI), but is limited in voxels that contain multiple fibre tracts. Fixel-based analysis (FBA) addresses this limitation by using a novel method of analysing high angular resolution diffusion-weighted imaging (HARDI) data. FBA examines three aspects of each fibre tract within a voxel: tissue micro-structure (fibre density [FD]), tissue macro-structure (fibre-bundle cross section [FC]) and a combined measure of both (FD and fibre-bundle cross section [FDC]). This study used FBA to identify the location and extent of micro- and macro-structural changes in WM following TBI. A large TBI sample (Nmild = 133, Nmoderate-severe = 29) and control group (healthy and orthopaedic; N = 107) underwent magnetic resonance imaging with HARDI and completed reaction time tasks approximately 7 months after their injury (range: 98-338 days). The TBI group showed micro-structural differences (lower FD) in the corpus callosum and forceps minor, compared to controls. Subgroup analyses revealed that the mild TBI group did not differ from controls on any fixel metric, but the moderate to severe TBI group had significantly lower FD, FC and FDC in multiple WM tracts, including the corpus callosum, cerebral peduncle, internal and external capsule. The moderate to severe TBI group also had significantly slower reaction times than controls, but the mild TBI group did not. Reaction time was not related to fixel findings. Thus, the WM damage caused by moderate to severe TBI manifested as fewer axons and a reduction in the cross-sectional area of key WM tracts.

Wallerian Degeneration of the Cerebral Peduncle and Association with Motor Outcome in Childhood Stroke.

BACKGROUND: To evaluate the presence of Wallerian degeneration and its relationship with sensorimotor deficits following childhood-onset arterial ischemic stroke (AIS). METHODS: Children surviving unilateral AIS older than one month of age were assessed for severity of sensorimotor neurological deficit with the Pediatric Stroke Outcome Measure at least one year post stroke (mean follow-up = 2.9 years, S.D. = ±1.6). The area (mm3) of each cerebral peduncle was measured on T2-weighted magnetic resonance images to calculate an Asymmetry Index (AI). The AI between patients with childhood stroke (cases) and controls (children with normal MRI) was compared. In the stroke group, the AI between patients with good and poor motor outcome, and the correlation between the AI and motor outcome was calculated. RESULTS: Asymmetry was compared in 52 children with stroke (cases) and 20 controls (normal brain MRIs). The AI was greater in patients with stroke (mean = 6.8%, S.D. = ±5.9) compared with controls (mean = 3.4%, S.D. = ±3.5, P < 0.02). Patients with poor outcome had an AI of 10% or greater compared with patients with good outcome (mean 10.4 versus 4, P < 0.001), and the AI was moderately correlated with motor deficit severity (r = 0.582, P = 0.001). CONCLUSIONS: Asymmetry of the cerebral peduncle is a feasible method of assessing Wallerian degeneration in children with unilateral AIS. The degree of asymmetry in the cerebral peduncles was moderately correlated with neurological outcome severity and reflects the degree of motor deficit in children following stroke.

Assessment of the Corticospinal Tract Profile in Pure Lower Motor Neuron Disease: A Diffusion Tensor Imaging Study.

AIM: The aim of this study was to evaluate the corticospinal tract (CST) diffusion profile in pure lower motor neuron disease (pLMND) patients who at baseline did not show any clinical or electrophysiological involvement of upper motor neurons (UMN), and in amyotrophic lateral sclerosis (ALS) patients. MATERIALS AND METHODS: Fifteen ALS patients with delayed central motor conduction time (CMCT) and 14 pLMND patients with normal CMCT were enrolled together with 15 healthy controls. Fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) maps were obtained. The tract profile of CST was reconstructed with the automated fiber quantification tool and its diffusion properties were quantified voxel-by-voxel and then compared pairwise between groups. Moreover, a random forest (RF) classifier was trained to evaluate the ability of CST diffusion metrics in distinguishing pairwise the groups from the controls. RESULTS: ALS patients presented wide microstructural abnormalities in the entire CST as assessed by FA decrease and RD increase while pLMND patients showed focal FA decrease and a larger AD increase in the cerebral peduncle and posterior limb of the internal capsule in comparison with controls. RF revealed that diffusion tensor imaging (DTI) metrics accurately distinguished ALS patients and pLMND patients from controls (96.67 and 95.71% accuracy, respectively). CONCLUSIONS: Our study demonstrates that the CST was impaired in both ALS and pLMND patients, thus suggesting that DTI metrics are a reliable tool in detecting subtle changes of UMN in pLMND patients, also in the absence of clinical and CMCT abnormalities.

Rare presentation of an isolated bilateral cerebral peduncular infarction: A case report and review of the literature.

RATIONALE: Due to the rarity of bilateral cerebral peduncular infarction (BCPI), its symptoms and prognosis are not clear. It is necessary to collect cases of pure cerebral peduncular infarction, explore the etiology and anatomy of midbrain infarction in depth, and develop meaningful tools for explaining clinical symptoms and predicting prognosis of patients. PATIENT CONCERNS: We here provide a case of isolated BCPI with uncommon symptoms of ataxia, dysarthria, sensory disturbance, normal muscular strength, and full eye movements. DIAGNOSES: Diffusion weighted images and apparent diffusion coefficient map of our patient revealed acute and isolated bilateral peduncle cerebrum infarction. INTERVENTIONS: Drugs that could improve circulation and antiplatelet were used in therapy. OUTCOMES: The infarct size was enlarged and new infarction was identified in the splenium of the corpus callosum and pons. The patient developed progressed disorder of consciousness and died at the eleventh day. LESSONS: We inferred that the symptoms of ataxia, dysarthria, sensory disturbance, and mild paresis of the extremities could be prominent features of patients with pure cerebral peduncular infarction. We hypothesize that pure BCPI is also related to severe basilar artery stenosis or occlusion and there is no collateral circulation from PCA. This may explain the corresponding distribution of cerebral peduncular infarction and its poor prognosis. For these reasons, exploring etiology and anatomy of midbrain infarction in depth would have clinical value for predicting symptoms and prognosis.

The Interpeduncular Angle: A Practical and Objective Marker for the Detection and Diagnosis of Intracranial Hypotension on Brain MRI.

BACKGROUND AND PURPOSE: Classic findings of intracranial hypotension on MR imaging, such as brain stem slumping, can be variably present and, at times, subjective, potentially making the diagnosis difficult. We hypothesize that the angle between the cerebral peduncles correlates with the volume of interpeduncular cistern fluid and is decreased in cases of intracranial hypotension. We aimed to investigate its use as an objective assessment for intracranial hypotension. MATERIALS AND METHODS: Brain MRIs of 30 patients with intracranial hypotension and 30 age-matched controls were evaluated by 2 fellowship-trained neuroradiologists for classic findings of intracranial hypotension and the interpeduncular angle. Group analysis was performed with a Student t test, and receiver operating characteristic analysis was used to identify an ideal angle threshold to maximize sensitivity and specificity. Interobserver reliability was assessed for classic findings of intracranial hypotension using the Cohen κ value, and the interpeduncular angle, using the intraclass correlation. RESULTS: The interpeduncular angle had excellent interobserver reliability (intraclass correlation coefficient value = 0.833) and was significantly lower in the intracranial hypotension group compared with the control group (25.3° versus 56.3°; P < .001). There was significant correlation between the interpeduncular angle and the presence of brain stem slumping (P < .001) and in cases with ≥3 classic features of intracranial hypotension (P = .01). With a threshold of 40.5°, sensitivity and specificity were 80% and 96.7%, respectively. CONCLUSIONS: The interpeduncular angle is a sensitive and specific measure of intracranial hypotension and is a reliably reproducible parameter on routine clinical MR imaging.

White Matter Correlates of Cognitive Performance on the UCSF Brain Health Assessment.

OBJECTIVE: White matter (WM) microstructural changes are increasingly recognized as a mechanism of age-related cognitive differences. This study examined the associations between patterns of WM microstructure and cognitive performance on the University of California, San Francisco (UCSF) Brain Health Assessment (BHA) subtests of memory (Favorites), executive functions and speed (Match), and visuospatial skills (Line Orientation) within a sample of older adults. METHOD: Fractional anisotropy (FA) in WM tracts and BHA performance were examined in 84 older adults diagnosed as neurologically healthy (47), with mild cognitive impairment (19), or with dementia (18). The relationships between FA and subtest performances were evaluated using regression analyses. We then explored whether regional WM predicted performance after accounting for variance explained by global FA. RESULTS: Memory performance was associated with FA of the fornix and the superior cerebellar peduncle; and executive functions and speed, with the body of the corpus callosum. The fornix-memory association and the corpus callosum-executive association remained significant after accounting for global FA. Neither tract-based nor global FA was associated with visuospatial performance. CONCLUSIONS: Memory and executive functions are associated with different patterns of WM diffusivity. Findings add insight into WM alterations underlying age- and disease-related cognitive decline.

Cerebral Peduncle Volume and Motor Function Following Adult Hemispherectomy.

BACKGROUND: Hemispherectomy is a successful and well-described treatment option for pediatric patients with hemispheric ictal onset, but adult outcomes have been far less studied. We describe the outcomes in adult patients with medically refractory epilepsy and hemispheric disease and the relationship to cerebral peduncle volume. CASE DESCRIPTIONS: We retrospectively reviewed adult hemispherectomy patients at our institution from 2015 to 2018. Patient data including demographic information, pathologic changes, seizure-free outcomes, and ipsilateral (i.e., surgical side) and contralateral (i.e., functional side) cerebral peduncle volume data were collected. We identified 4 adult patients who underwent hemispherectomy. The mean age at surgery was roughly 25 years. All patients were categorized as Engel I or II, and motor scores at last follow-up were unchanged. The mean volume for contralateral and ipsilateral cerebral peduncle means were 1.42 and 0.78 cm3, respectively (P = 0.01). CONCLUSIONS: These findings suggest that smaller ipsilateral cerebral peduncle size could potentially be associated with unchanged postoperative hemiparesis. We hypothesize that smaller ipsilateral peduncle size could have represented corticospinal tract reorganization in childhood, implying that the removed brain matter was mostly noncontributory to contralateral motor function.

What is the role of diffusion tensor imaging (DTI) in detecting subclinical pyramidal tract dysfunction in Behçet's and neuro-Behçet's cases?

The aim of this study is to investigate the pyramidal tract integrity with DTI in Behçet's and neuro-Behçet's cases. We performed this technique in two subgroups of neuro-Behçet's patients (parenchymal and vascular), and Behçet's cases without neurological involvement and control group. Totally, 28 patients were investigated. The control group was composed of 14 healthy people. Cranial MR and DTI were performed in three patient groups and the control group. At DTI, circular regions of interest (ROI) were symmetrically drawn on axial slices on the left and right sides along the pyramidal tract pathway at two levels: middle one third of the cerebral peduncle and posterior limb of the internal capsule. Fractional anisotropy (FA) values for each ROI were obtained by averaging all voxels within the ROI. Calculated FA values on both sides (left and right) of the posterior limb of the internal capsule and cerebral peduncle are significantly lower in all three patient groups when compared to the control group. But there is no any difference of FA values in the selected brain regions of three patient groups. FA values on the posterior limb of the internal capsule and cerebral peduncle do not show a statistically significant difference in parenchymal neuro-Behçet's cases. Our study demonstrates that DTI can detect subclinical pyramidal tract dysfunction in neuro-Behçet's and Behçet's patients. Detection of subclinical nervous system involvement is crucial for morbidity in Behçet's disease. For this reason, studies based on DTI, which include a large number of patients and explore different brain regions, are needed to guide clinicians.

Neuroimaging Identifies Patients Most Likely to Respond to a Restorative Stroke Therapy.

BACKGROUND AND PURPOSE: Patient heterogeneity reduces statistical power in clinical trials of restorative therapies. Valid predictors of treatment responsiveness are needed, and several have been studied with a focus on corticospinal tract (CST) injury. We studied performance of 4 such measures for predicting behavioral gains in response to motor training therapy. METHODS: Patients with subacute-chronic hemiparetic stroke (n=47) received standardized arm motor therapy, and change in arm Fugl-Meyer score was calculated from baseline to 1 month post-therapy. Injury measures calculated from baseline magnetic resonance imaging included (1) percent CST overlap with stroke, (2) CST-related atrophy (cerebral peduncle area), (3) CST integrity (fractional anisotropy) in the cerebral peduncle, and (4) CST integrity in the posterior limb of internal capsule. RESULTS: Percent CST overlap with stroke, CST-related atrophy, and CST integrity did not correlate with one another, indicating that these 3 measures captured independent features of CST injury. Percent injury to CST significantly predicted treatment-related behavioral gains (r=-0.41; P=0.004). The other CST injury measures did not, neither did total infarct volume nor baseline behavioral deficits. When directly comparing patients with mild versus severe injury using the percent CST injury measure, the odds ratio was 15.0 (95% confidence interval, 1.54-147; P<0.005) for deriving clinically important treatment-related gains. CONCLUSIONS: Percent CST injury is useful for predicting motor gains in response to therapy in the setting of subacute-chronic stroke. This measure can be used as an entry criterion or a stratifying variable in restorative stroke trials to increase statistical power, reduce sample size, and reduce the cost of such trials.

Differences in neural pathways are related to the short- or long-term benefits of constraint-induced movement therapy in patients with chronic stroke and hemiparesis: a pilot cohort study.

Background No previous studies have determined how the post-stroke integrity of non-corticospinal neural pathways relates to the efficacy of constraint-induced movement therapy (CIMT). Objectives We aimed to clarify the relationship between several non-corticospinal neural pathway integrities and the short- and long-term benefits of CIMT. Methods This was a pilot cohort study (UMIN registration number: R00027136UMIN000023566), for which we enrolled 13 patients with chronic stroke and hemiparesis who had undergone CIMT. We assessed patients' motor function improvement by comparing the Fugl-Meyer Assessment (FMA) scores, as well as the Amount of Use (AOU) and Quality of Movement (QOM) scales of the Motor Activity Log before, immediately after (short-term), and 6 months after (long-term) CIMT. We assessed neural pathway integrity by calculating fractional anisotropy (FA) in diffusion tensor images acquired before CIMT. We then assessed correlations between FA and short- and long-term post-CIMT motor function improvements. Results The patients showed significant improvements in all functional assessments at both short- and long-term follow-ups. Immediate FMA score improvements were significantly correlated with FA of the affected anterior limb of the internal capsule (ALIC), body of the corpus callosum, column and body of the fornix (CBF), cingulate cortex (CgC), cerebral peduncle (CP), and posterior limb of the internal capsule. Six-month FMA score improvements were significantly correlated with FA of the affected ALIC, CgC, CBF, CP, and superior frontooccipital fasciculus. Conclusions The integrity of the affected corticospinal and non-corticospinal motor pathways was associated with CIMT-induced motor learning at least 6 months after CIMT.

Utility of Fractional Anisotropy in Cerebral Peduncle for Stroke Outcome Prediction: Comparison of Hemorrhagic and Ischemic Strokes.

BACKGROUND: Diffusion-tensor fractional anisotropy (FA) has been used for predicting stroke outcome. However, most previous studies focused on patients with either hemorrhagic or ischemic stroke. The aim of this study was to assess the correlation between FA and outcome for patients with hemorrhagic stroke and those with ischemic stroke, and then compare their correlation patterns. METHODS: This study sampled 40 hemorrhagic and 40 ischemic stroke patients from our previously published reports. Diffusion-tensor images were obtained on days 14-21, and FA images were generated, after which the ratio of FA within the cerebral peduncles of the affected and unaffected hemispheres (rFA) was calculated. Outcome was assessed using Brunnstrom stage (BRS), motor component of the functional independence measure (FIM-motor), and total length of hospital stay (LOS) at discharge from our affiliated rehabilitation hospital. The data were then compared between the hemorrhage and the infarct groups. Correlation analyses between rFA and outcome assessments were performed separately for both groups and then were compared between the groups. RESULTS: The hemorrhage group exhibited significantly more severe BRS, longer LOS, and lower rFA than the infarct group. The correlations between rFA and outcome measures were all statistically significant for both the hemorrhage and the infarct groups. The correlation patterns for BRS and LOS were very similar between the hemorrhage and the infarct groups. However, such similarity was not evident for FIM-motor. CONCLUSIONS: FA in the cerebral peduncles may be used to predict extremity functions and LOS for both types of stroke.

[Symmetrical lesions of the cerebral peduncles and internal capsules on MRI in a patient with extranodal NK/T cell lymphoma].

A 72-year-old man was admitted to our hospital because of subacute progressive consciousness disturbance. FLAIR images showed diffuse high intensity areas in the bilateral thalamus, basal ganglia, and white matter. Chest CT revealed a mass in the right lung. The patient was diagnosed with extranodal natural killer/T cell lymphoma (ENKL) according to the results of a biopsy of the right pulmonary mass. The FLAIR images showed diffuse white matter lesions, without a mass such as lymphomatosis cerebri. On diffusion-weighted images, symmetrical high-intensity lesions of the cerebral peduncle and the internal capsule suggested high-density lymphoma cells around neurons. ENKL of the central nervous system and lung is a very rare neoplasm, and this case showed characteristic images.

MR Imaging Features of the Cerebellum in Adult-Onset Neuronal Intranuclear Inclusion Disease: 8 Cases.

Neuronal intranuclear inclusion disease is a neurodegenerative disorder pathologically characterized by eosinophilic hyaline intranuclear inclusions. A high-intensity signal along the corticomedullary junction on DWI has been described as a specific MR imaging finding of the cerebrum in neuronal intranuclear inclusion disease. However, MR imaging findings of the cerebellum in neuronal intranuclear inclusion disease have not been fully evaluated. Here, we review MR imaging findings of the cerebellum in a series of 8 patients with pathologically confirmed neuronal intranuclear inclusion disease. The MR imaging results showed cerebellar atrophy (8/8 patients) and high-intensity signal on FLAIR images in the medial part of the cerebellar hemisphere right beside the vermis (the "paravermal area") (6/8) and in the middle cerebellar peduncle (4/8). The paravermal abnormal signals had a characteristic distribution, and they could be an indicator of the diagnosis of neuronal intranuclear inclusion disease even when using the results of past MR imaging examinations in which DWI findings were not examined.