Culprit Plaques of Large Parent Arteries, Rather than Cerebral Small Vessel Disease, Contribute to Early Neurological Deterioration in Stroke Patients with Intracranial Branch Atheromatous Disease.

INTRODUCTION: Intracranial branch atheromatous disease (BAD) has been applied to occlusions that occur at the origin of large caliber penetrating arteries due to the microatheromas or large parent artery plaques. This study aimed to explore the association between culprit plaques of large parent arteries, neuroimaging markers of cerebral small vessel disease (CSVD), and the risk of early neurological deterioration (END) in stroke patients with BAD. METHODS: A total of 97 stroke patients with BAD in the vascular territories of the lenticulostriate arteries or paramedian pontine arteries, diagnosed using high-resolution magnetic resonance imaging, were prospectively recruited in this observational study. A culprit plaque in the middle cerebral artery was defined as the only arterial plaque on the ipsilateral side of an infarction visible on diffusion-weighted imaging. A culprit plaque in the basilar artery (BA) was identified when it was observed within the same axial slices of an infarction or on the adjacent upper or lower slice, whereas a plaque within the BA located in the ventral region was considered non-culprit. If more than one plaque was present in the same vascular territory, the most stenotic plaque was chosen for the analysis. Four CSVD neuroimaging markers, including white matter hyperintensity, lacunes, microbleeds, and enlarged perivascular spaces, were evaluated in accordance with the total CSVD score. The associations between neuroimaging features of lesions within large parent arteries, neuroimaging markers of CSVD, and the risk of END in stroke patients with BAD were investigated using logistic regression analysis. RESULTS: END occurred in 41 stroke patients (42.27%) with BAD. The degree of large parent artery stenosis (p < 0.001), culprit plaques of large parent arteries (p < 0.001), and plaque burden (p < 0.001) were significantly different between the END and non-END groups in stroke patients with BAD. In logistic regression analysis, culprit plaques of large parent arteries (odds ratio, 32.258; 95% confidence interval, 4.140-251.346) were independently associated with the risk of END in stroke patients with BAD. CONCLUSIONS: Culprit plaques of large parent arteries could predict the risk of END in stroke patients with BAD. These results suggest that lesions in the large parent arteries, rather than damage to the cerebral small vessels, contribute to END in stroke patients with BAD.

Enhancement pattern of demyelination-mimicking cerebral B-cell lymphoma.

BACKGROUND AND PURPOSE: Central nervous system (CNS) B-cell lymphoma-mimicking demyelinating diseases creates a diagnostic dilemma. This study aimed to determine the specific magnetic resonance imaging (MRI) features of CNS B-cell lymphoma to facilitate the early identification of the disease. METHODS: We retrospectively reviewed the brain MRI of biopsy-confirmed CNS B-cell lymphoma patients. They were initially diagnosed with CNS demyelination, and these images were compared with those of actual patients with demyelinating diseases. RESULTS: A total of 20 patients with CNS B-cell lymphoma and 12 patients with demyelination were included in this study. Cohesive enhancement with satellite enhancing foci surrounded by prominent non-enhancing areas of oedema is the major contrast-enhancing pattern of lymphoma patients, accounting for 81% (13) of patients with primary diffuse large B-cell lymphoma (DLBCL). This imaging pattern revealed a sensitivity of 81% and a specificity of 75% for lymphoma in the differential diagnosis between primary DLBCL and demyelinating disease in our cohort. Among these lesions, most of the nodules were located deeply, which yielded a specificity of 100% and a sensitivity of 69% for primary DLBCL. Enhancement in a single pattern (mainly ring-like, patchy or punctate; 57%) and no enhancement (30%) were commonly observed in demyelinating lesions, distinct from primary DLBCL (p < 0.05). CONCLUSIONS: Lesions with cohesive enhancement and satellite foci on T1 contrast-enhanced imaging could be a specific hallmark of CNS B-cell lymphoma, suggesting the need to withdraw steroidal therapy and biopsy confirmation.

Myelitis: A Common Complication of Tuberculous Meningitis Predicting Poor Outcome.

Background: Myelitis is an important complication in patients with tuberculous meningitis (TBM). However, a paucity of publications exists on the spectrum of neurological and MRI findings of TBM-related myelitis. The risk factors and prognosis of myelitis in patients with TBM are not fully understood. Therefore, this study aims to identify the risk factors, clinicoradiological features, and prognostic impact of myelitis for patients with TBM. Methods: We conducted a retrospective study in our institution. Patients with TBM who were consecutively admitted during the period of August 2015 to December 2019 were included. We reviewed the demographic characteristics, clinical, laboratory and MRI findings, and clinical outcomes of all of the included patients. The diagnosis of myelitis was identified by a hyperintensity on T2-weighted images that were associated with cord edema, enlargement, and marginal or no enhancement on contrast-enhanced images. Results: A total of 114 patients were included. Myelitis occurred in 19 (16.7%) patients, five of whom paradoxically developed myelitis. The common clinical signs of myelitis were paraparesis (738.9%), quadriparesis (844.4%), urinary retention or constipation (1,477.8%), and paresthesias in the lower limbs (1,052.6%). In the MRI findings, the hyperintensities on T2-weighted images involved more than 3 spinal cord segments. Myelitis was often combined with other forms of spinal cord injury, including 10 patients (52.6%) with spinal meningeal enhancement, 7 patients (36.8%) with enlargement of the central canal of the spinal cord, 6 patients (31.6%) with tuberculoma, and 4 patients (21.1%) with arachnoiditis and 1 patient (5.3%) with cerebrospinal fluid (CSF) loculations. None of the 5 patients with paradoxical myelitis were complicated with spinal meningeal enhancement and arachnoiditis, while 4 patients were complicated with enlargements of the central canal of the spinal cord. In multivariable analysis, a grade III disease severity on admission [p = 0.003, odds ratio (OR) = 8.131, 95% CI: 2.080-31.779] and high CSF protein (p = 0.033, OR = 1.698, 95% CI: 1.043-2.763) were independent risk factors for myelitis. After the 6 months follow-up, myelitis (p = 0.030, OR = 13.297, 95% CI: 1.283-137.812) and disturbance of consciousness (p = 0.042, OR = 12.625, 95% CI: 1.092-145.903) were independent risk factors for poor outcomes. Conclusion: Myelitis was a common complication of TBM and independently predicted a poor outcome. A grade III disease severity and high CSF protein on admission were independent risk factors for myelitis. Paradoxical myelitis was rarely complicated with spinal meningeal enhancements and arachnoiditis, indicating that the immune reaction may play a dominant role.

The Distributional Characteristics of Multiple Sclerosis Lesions on Quantitative Susceptibility Mapping and Their Correlation With Clinical Severity.

Background: Multiple sclerosis (MS) patients have a wide spectrum of severity and responses to therapy; the personalization of treatment relies on sensitive and specific biomarkers. Previous studies have suggested that susceptibility contrast in demyelinated plaques is associated with iron-related pathology in multiple sclerosis which may indicate clinical severity. The aims of this study were to characterize the spatial distribution of MS lesions with different iron patterns by using quantitative susceptibility mapping and to explore neuroradiological findings that correlate with poor clinical outcome. Methods: Twenty-six patients with relapsing-remitting MS [14 men, 12 women; mean age, 29 ± 8 (standard deviation) years; age range, 21-52 years] were included in this study. Differences in lesion number, T2 volume, and susceptibility were compared among lesions subcategorized by location and by the presence or absence of a hyperintense rim on quantitative susceptibility mapping. Associations between these imaging features and clinical outcomes including Expanded Disability Status Scale scores and annual relapse rates were investigated. Results: A total of 811 unifocal MS lesions were included, and their QSM patterns were nodular hyperintensity with no rim (rim-, 540, 67%) or with a hyperintense rim on the edge (rim+, 172, 21%) and nodular isointensity (99, 12%). Rim+ lesions had significantly larger volume (115 ± 142 vs. 166 ± 185 mm3, p < 0.001) and lower susceptibility (4 ± 15 vs. 8 ± 16 ppb, p < 0.05) than rim- lesions. More rim+ lesions were found in periventricular areas [median, 45%; interquartile range (IQR), 36%], whereas a larger proportion of rim- lesions were distributed in juxtacortical (median, 32%; IQR, 21%) and deep white matter (median, 38%; IQR, 22%) areas. The annual relapse rate was positively correlated with the proportion of periventricular rim+ lesions (p < 0.001, r = 0.65) and the proportion of subtentorial rim+ lesions (p < 0.05, r = 0.40). Additionally, a significant association was found between the burden of periventricular rim+ lesions (ß = 0.64, p < 0.001) and the burden of subtentorial rim- lesions (ß = 0.36, p < 0.05). Conclusions: A high number or lesion burden of periventricular rim+ lesions or subtentorial lesions is associated with frequent clinical relapses.

CLCN2-related leukoencephalopathy: a case report and review of the literature.

BACKGROUND: Loss-of-function mutations in the CLCN2 gene were recently discovered to be a cause of a type of leukodystrophy named CLCN2-related leukoencephalopathy (CC2L), which is characterized by intramyelinic edema. Herein, we report a novel mutation in CLCN2 in an individual with leukodystrophy. CASE PRESENTATION: A 38-year-old woman presented with mild hand tremor, scanning speech, nystagmus, cerebellar ataxia in the upper limbs, memory decline, tinnitus, and dizziness. An ophthalmologic examination indicated macular atrophy, pigment epithelium atrophy and choroidal capillary atrophy. Brain magnetic resonance imaging (MRI) showed the diffuse white matter involvement of specific white matter tracts. Decreased diffusion anisotropy was detected in various brain regions of the patient. Diffusion tensor tractography (DTT) showed obviously thinner tracts of interest than in the controls, with a decreased fiber number (FN), increased radial diffusivity (RD) and unchanged axial diffusivity (AD). A novel homozygous c.2257C > T (p.Arg753Ter) mutation in exon 20 of the CLCN2 gene was identified. CONCLUSION: CC2L is a rare condition characterized by diffuse edema involving specific fiber tracts that pass through the brainstem. The distinct MRI patterns could be a strong indication for CLCN2 gene analysis. The findings of our study may facilitate the understanding of the pathophysiology and clinical symptoms of this disease.

Susceptibility-weighted imaging in the differential diagnosis of autoimmune central nervous system vasculitis and multiple sclerosis.

OBJECTIVES: To determine the differentiating features of autoimmune central nervous system (CNS) vasculitis and multiple sclerosis (MS) on susceptibility-weighted imaging (SWI). METHODS: Seventy-three patients (27 with autoimmune CNS vasculitis and 46 with MS) who underwent magnetic resonance imaging with SWI sequence were included. The features of lesions and distinct SWI findings were investigated in both diseases. RESULTS: On SWI, autoimmune CNS vasculitis presented with a higher prevalence of multiple microbleeds (48.1%), cortical superficial siderosis (70.4%), and tortuosity of the vascular route (59.3%) than were found in MS (p < 0.001, p < 0.001, and p = 0.001, respectively). Multivariable logistic analysis showed that multiple microbleeds and cortical superficial siderosis were associated with a much higher probability of a diagnosis of autoimmune CNS vasculitis than of MS (OR 19.09, 95% CI 1.13-321.18, p = 0.041; and OR 13.20, 95% CI 2.22-78.30, p = 0.005, respectively). The presence of more than eleven lesions was associated with a lower probability of a diagnosis of autoimmune CNS vasculitis than of MS (OR 0.14, 95% CI 0.03-0.73, p = 0.020). CONCLUSIONS: SWI may be a useful adjunct in distinguishing autoimmune CNS vasculitis from MS. The identification of multiple microbleeds and cortical superficial siderosis can point to a diagnosis of autoimmune CNS vasculitis.