Balo's concentric sclerosis involving bilateral thalami

Balo's concentric sclerosis (BCS) is a rare inflammatory demyelinating disease of central nervous system, pathologically characterized by alternate bands of demyelination and preserved myelin tissue. Before the era of magnetic resonance imaging (MRI), most cases of BCS were diagnosed on postmortem examination. MRI allows for noninvasive diagnosis by demonstrating characteristic changes which closely parallels the histopathological features of BCS. We report a case of 26-year-old female with BCS involving bilateral thalami, with typical MRI appearance.     

Antemortem diagnosis of Balo's concentric sclerosis based on MRI. Case report

Balo's concentric sclerosis is a rare variant of multiple sclerosis. It is pathologically characterized by alternating rings of demyelination and spared myelin. Recently, MRI was applied to demonstrate characteristic patterns in this disease. We report a case of Balo's concentric sclerosis diagnosed by the typical MRI findings of concentric rings of demyelination.     

Aquaporin-4 astrocytopathy in Baló’s disease

Baló’s concentric sclerosis (BCS) is considered to be a rare variant of multiple sclerosis and characterized by alternating rings of demyelinated and preserved myelin layers. The mechanism underlying BCS remains to be elucidated. Recently, occurrence of concentric rings of Baló was described in the brainstem of a patient with neuromyelitis optica (NMO). Because selective loss of aquaporin-4 (AQP4) and vasculocentric deposition of complement and immunoglobulins are characteristic in NMO, we aimed to assess AQP4 expression in the concentric demyelinating lesions of BCS patients. We evaluated AQP4 expression relative to expression of another astrocytic marker (glial fibrillary acidic protein), the extent of demyelination, lesion staging and perivascular deposition of complement and immunoglobulin in four cases with BCS, and 30 individuals with other neurological diseases. All cases with BCS demonstrated extensive AQP4 loss in both demyelinated and myelinated layers of all actively demyelinating lesions, with perivascular lymphocytic cuffing of T cells, but no deposition of immunoglobulins or complement around vessels. These findings suggest that AQP4 loss occurs in heterogeneous demyelinating conditions, namely NMO and BCS. Furthermore, acute BCS lesions are characterized by extensive AQP4 loss without vasculocentric deposition of complement or immunoglobulin.     

Balò's concentric sclerosis in a North-African patient

INTRODUCTION: Balo's concentric sclerosis is a rare variant of multiple sclerosis described by Balo in 1928. It is characterized by alternating rings of demyelination and spared myelin. CASE REPORT: We report a case of Balo's concentric sclerosis diagnosed by the typical MRI findings of concentric rings of demyelination. Medullar and brain localisation were found and clinical course was good under intravenous corticosteroids. CONCLUSION: MRI provides the best diagnostic information for Balo's concentric sclerosis, allowing early diagnosis and treatment.     

Technology insight: can neuroimaging provide insights into the role of ischemia in Baló's concentric sclerosis?

Baló's concentric sclerosis (BCS) has long been considered to be a variant of multiple sclerosis. Although BCS was initially described over 100 years ago, relatively few antemortem cases have been identified, and the exact pathogenesis remains unknown. Inflammatory protective ischemic preconditioning has recently been suggested as a mechanism by which the typical concentric rings of the BCS lesion are formed. Advanced neuroimaging can provide important in vivo markers of disease progression that can assist in the diagnosis and management of patients with BCS. In this Review, we discuss evidence from longitudinal neuroimaging studies that supports the role of ischemic preconditioning in BCS.     

Balo's concentric sclerosis. Evolution of active demyelination demonstrated by serial contrast-enhanced MRI

We report a case of Balo's concentric sclerosis with peculiar MRI findings. Unlike previously published cases all concentric rings in our case showed marked enhancement, supporting the view of synchronous active demyelination in the lesion. Follow-up MRI disclosed a change of the lesions into a confluent pattern more resembling a typical large MS plaque. There were no oligoclonal bands or intrathecal Ig-G synthesis. The hypothesis concerning the pathophysiology of the lesions' typical and peculiar morphological appearance and its relationship to multiple sclerosis are briefly discussed. Received: 18 August 2001, Received in revised form: 9 November 2001, Accepted: 21 November 2001     

Histopathology and serial, multimodal magnetic resonance imaging in a multiple sclerosis variant

Defining tools in magnetic resonance imaging (MRI) representing specific pathological processes is needed to understand the complex relationship between inflammation, myelin breakdown, axonal injury and clinical symptoms in multiple sclerosis (MS) and its variants. Here, we describe a case of histologically-defined MS, in which the radiological appearance of the lesion and clinical course support the diagnosis of Balo's concentric sclerosis. Serial magnetization transfer, diffusion tensor imaging and 1H-magnetic resonance spectroscopy, from 14 days to 13 months after biopsy, allow the contextual interpretation of specific pathological changes. In our case, acute inflammation was sensitively traced by fractional anisotropy and increased lactate in spectroscopy. In contrast, magnetization transfer ratio and the apparent diffusion coefficient monitor the sequential loss of tissue in selected rings of the lesion. The delay from the peak of symptoms in a dramatic clinical course to the maximum tissue destruction indicated through MRI suggests that compromise of axonal function may be decisive for the acute clinical situation. This is the first report comparing 1H-magnetic resonance spectroscopy, magnetization transfer and diffusion tensor imaging with histopathology in a patient with Balo's concentric sclerosis.     

Balo's disease showing benign clinical course and co-existence with multiple sclerosis-like lesions in Chinese

Baló's concentric sclerosis (BCS) is a rare demyelinating disorder usually considered a variant of multiple sclerosis (MS). However, its pathogenesis and its correlation with MS remains unclear and controversial. This report presents seven Hans Chinese subjects diagnosed as BCS on the basis of the pathognomonic MR (magnetic resonance) findings. Upon diagnosis, all the cases displayed good responses to corticosteroids and showed an overall benign prognosis during a follow-up period of 4-13.5 years, although three relapsed later. MR findings suggest that the characteristic concentric lesions of BCS frequently (5/7) coexist with multiple sclerosis-like lesions. During follow-up, the Baló-like lesions may either dissolve over time or transform into an MS-like lesion. Moreover, the Balóand MS-like lesions occurred one after another at the onset and relapse phases of the same patient in two cases. These clinical features suggest that Baló's disease showing benign clinical course and co-existence of multiple sclerosis (MS)-like lesion is not rare among the Chinese, and strengthens the notion that BCS correlates intrinsically with MS.     

Contribution of proton magnetic resonance spectroscopy to the diagnosis of Balo's concentric sclerosis

INTRODUCTION: Balo's concentric sclerosis is a neuropathological type of multiple sclerosis characterized by alternating rings of spared myelin and demyelination. Diagnosis is based on MRI, but very few data are available concerning the lesion features using serial proton magnetic resonance spectroscopy (1H-MRS). METHODS: We report 1H-MRS initial findings and disease course in one case of Balo's concentric sclerosis. RESULTS: The first 1H-MRS study of 2 concentric ring-enhanced lesions showed a decreased N-acetyl-aspartate (NAA) peak, an increased choline peak, 2 broad lactate peaks and the presence of a lipid peak at 0.9 ppm. Six months later, 1H-MRS showed a decrease of choline peak, whereas the lactate peak had disappeared. The NAA peak was still at a low level. CONCLUSION: These findings are similar to those reported in demyelinating disorders, such as multiple sclerosis. Thus, in Balo's concentric sclerosis, 1H-MRS may provide neurochemical arguments for inflammation and demyelination, and indicate the severity of axonal damage and recovery.     

Baló's concentric sclerosis associated with primary human herpesvirus 6 infection

BACKGROUND: Baló's concentric sclerosis (BCS) is a demyelinating disorder believed to be a rare variant of multiple sclerosis (MS). Human herpesvirus 6 (HHV-6) is a highly neurotropic virus causing severe central nervous system (CNS) infections predominantly following reactivation of latent HHV-6 in immunocompromised individuals. Primary infection with HHV-6 usually occurs in early childhood manifesting as exanthema subitum. The clinical spectrum of primary infection in adolescents or adults has not yet been evaluated. Case report: A previously healthy 13 year old girl developed acute hemianopsia and anomia 5 days after an episode of fever and malaise of unknown origin. Cerebral MRI revealed three white matter lesions, one with ring-like contrast enhancement. Lumbar puncture showed mononuclear pleocytosis of 30 cells/microl, oligoclonal IgG, and a normal protein level. Follow up cerebral MRI scans revealed lamellar concentric hemispheric lesions characteristic of BCS. The first neurological symptoms of the patient coincided with primary HHV-6 CNS infection, diagnosed by a positive PCR test of the CSF together with seroconversion. Response to antiviral and corticosteroid treatment was only temporary, but immunoglobulin treatment has so far been followed by clinical stability for 30 months. CONCLUSIONS: To our knowledge, this is the first report both of an association between HHV-6 and BCS and of immunoglobulin treatment of BCS. A late primary infection with HHV-6 might be associated with BCS. Further studies in patients with this rare disease are needed to confirm this association and to evaluate the efficacy of antiviral and immunoglobulin treatment.     

Balo''s concentric sclerosis: a clinical case study of brain MRI, biopsy, and proton magnetic resonance spectroscopic findings.

The antemortem diagnosis of Balo's concentric sclerosis was made in a 52 year old woman with subacute right hemiparesis on the basis of brain MRI and stereotactic brain biopsy, which showed multiple ring-like lesions of lamellated demyelination alternating with spared white matter. Proton magnetic resonance spectroscopy (1H-MRS) was carried out one and nine months after the onset of illness. The first 1H-MRS showed a decreased N-acetyl aspartate peak, an increased choline peak, presence of large lipid peaks, and high resonance at 1.4 ppm. The second 1H-MRS disclosed changes such as a decrease of lipid signal, a decrease of resonance at 1.4 ppm, and an increase in the myoinositol peak. These findings are similar to those reported for multiple sclerosis. It seems that this is the first report of 1H-MRS findings in Balo's concentric sclerosis.     

Balo同心圆性硬化磁共振成像与弥散加权成像特点及其机制

目的 总结同心圆硬化的MRl及弥散加权成像(DWI)特点,并探讨其影像学变化与可能发生机制的相关性.方法 总结临床确诊的同心圆硬化患者3例,均行头颅MRI及DWI检查,其中1例患者行氢质子波谱成像(1H-MRS).结果 3例患者均为多发病灶,病灶多位于中央白质区,均可见特征性的"同心圆样"或"洋葱皮样"改变,增强扫描旱环形或半环形强化.DWI见病灶呈高、低交替环形信号,表观扩散系数(ADC)值随同心圆病理的脱髓鞘区与髓鞘保留区相问存在而出现高低相间变化.结论 Balo同心圆硬化MRI和DWI表现具有特征性,可与病理改变对应,为加深该病发生机制认识提供影像学证据.     

多发性硬化脊髓型合并周围神经病变

目的：评价多发性硬化（MS）脊髓型的临床特征,脊髓磁共振成像（MRI）,神经电生理检查和腓肠神经活检的诊断价值。方法：分析14例MS脊髓型临床表现,脊髓MRI,神经电生理和腓神经活组织病理检查。结果：其临床表现为两侧肢体不对称性运动和感觉障碍的脊髓内部损害,可伴有发作性症状,有缓解和复发,脊髓MRI可以明确脱髓鞘部位,表现髓内纵行条带状或斑片状长T1长T2信号,脊髓略增粗,诱发电位有极高的敏感性,肌电图和腓肠神经病理提示周围神经脱髓鞘改变。结论：MRI、神经电生理检查及周围神经活检对MS脊髓型的早期诊断和治疗起重要作用。     

同心圆硬化12例临床及影像学分析

目的 探讨同心圆硬化的临床表现、影像学特征及其诊断价值.方法 回顾12例同心圆硬化患者(门诊2例,住院10例)的临床表现、实验室检查结果、影像学特点及病理结果,并对患者进行随访.结果 12例患者(男4例,女8例)发病年龄8～54岁(平均36.8岁),多以淡漠少语、反应迟钝起病(7例),病程中其他临床表现以智能减退(10例)、肢体活动障碍(8例)、大小便失禁(7例)、肌力减退(7例)、锥体束征阳性(6例)较多见.8例患者行脑脊液髓鞘碱性蛋白检查,其中7例增高.6例行脑活体组织检查,均可见分层脱髓鞘改变,有炎性细胞浸润.病灶数量1例为单发,11例为多发.4例患者行头颅CT检查,病灶均呈低密度影,增强后无强化.MRI检查均可见圆或类圆形影像,增强扫描8例可见环状或半环状强化,易被误诊为脑肿瘤;3例呈同心圆样强化.结论 同心圆硬化常亚急性或慢性起病,首发症状多为认知功能障碍,易被误诊,头颅MRI有特征表现,有诊断价值,激素治疗有效,预后良好,呈良性临床过程.

Abstract：

Objective To explore the clinical features, imaging and their diagnosis vaule of Baló's concentric sclerosis (BCS). Methods The clinical manifestation, laboratory examination, neuroimaging characteristics, pathology and follow-up of 12 (2 outpatients and 10 inpatients) cases of BCS were analyzed retrospectively. Results The onset age of 12 patients (male 4, female 8) ranged from 8 to 54 years old (mean 36.8 years). Unconcerned and slowness response were common in the disease onset (7 cases).Other symptoms and signs such as hypophrenia ( 10 cases), limitation of limb or hand movement (8 cases),urinary and fecal incontinence (7 cases), hypo-myodynamia (7 cases), positive pyramidal sign (6 cases)were not rare in course of disease. Eight cases underwent the examination of cerebrospinal fluid myelin basic protein, which increased in 7 cases. Demyelinating change and perivascular inflammatory infiltration were shown in 6 brain biopsies. The involvements of multi-lesions were found in 11 cases. Four patients underwent CT scanning and all the cases showed with hypodense lesions and couldn' t be enhanced. On MRI, all cases appeared round or round-like in shape. Enhanced lesions showed ring-like or half-ting-like shape (8 cases) and concentric ring (3 cases). It's easy to be misdiagnosed as brain tumor (8 cases).Conclusions BCS is a kind of demyelinating disease of subacute or chronic onset accompanied with cognitive disorder being the most common initiating symptoms. It's prone to be misdiagnosed. Diagnosis depends on MRI and corticosteroid treatment is effective. BCS has a good prognosis and presents with benign clinical course.     

MRI study of Baló's concentric sclerosis before and after immunosuppressive therapy

Summary An intra vitam diagnosis of Baló's concentric sclerosis in a 39-year-old male is described. To our knowledge this is the first reported case of ante-mortem diagnosis based solely on magnetic resonance imaging (MRI) findings. MRI showed features unique to this process. After high-dose intravenous therapy with prednisone, the clinical symptoms improved, and MRI showed marked changes of the concentric lesions.     

颅脑多发性硬化的临床及MRI诊断

目的 探讨多发性硬化(MS)的临床及MRJ特征,提高对多发性硬化的认识及诊断水平.方法 对20例颅脑MS患者临床资料、病灶部位、形态、MR信号及强化特点、胼胝体改变进行回顾性分析评价.结果 MS以青、中年女性稍多见,急性、亚急性起病,多以视觉障碍或肢体感觉、运动障碍为首发症状.视觉诱发电位大多数异常.MRJ检查18例发现病灶,敏感性90%(18/20).病灶以双侧侧脑室旁、额叶皮层及皮层下、半卵圆中心多发.病灶大、小不等,多数为圆形、卵圆形."直角脱髓鞘征"及"白质变脏征"是两个较为典型的征象.T1WI上表现为等、低信号,T2WI及Flair序列上表现为高信号,Flair序列显示病灶更清晰.增强扫描病灶可呈结节状强化、环状强化、弧形强化或无强化.结论 MS的临床及MRI表现具有一定特征.MRI有助于脑部MS的诊断及鉴别诊断,是诊断MS最敏感的成像方法.     

MRI Lesion and Epileptogenic Focus in Temporal Lobe Epilepsy

Abstract: The spatial relationship between a circumscribed lesion in the temporal lobe detected by MRI and an epibptogenic focus identified by ictal depth EEG along with a correlation, of the MRI lesion with neuropath logical findings were investigated in patients with medically intractable temporal lobe epilepsy but without any focal lesion on CT. Four parameters (an areal ratio of the temporal lobe against the hemisphere, area and calculated T1, T2 values of the hippocampus) were used to determine the abnormal MRI side. An agreement was reached in 67–72%, of 18 patients between the abnormal values of the hippocampus area and of calculated T1, T2 and the side of the epileptogenic focus. In 14 of 17 patients, typical hippocampus sclerosis was demonstrated in respected tissue in accordance with the MRI lesions (atrophy and/or prolonged T2 of hippocampus). These results imply: 1) MRI abnormality thus defined may, if not all, indicate the side of the epileptogenic focus, and 2) also the presence of hippocampal sclerosis. It was emphasized that the MRI lesion would be a usable instrument to explore the causal relationship of hippocampus sclerosis to a generation of epileptogenic lesions as well as for presurgical evaluation.     

MRI lesion and epileptogenic focus in temporal lobe epilepsy

The spatial relationship between a circumscribed lesion in the temporal lobe detected by MRI and an epileptogenic focus identified by ictal depth EEG along with a correlation of the MRI lesion with neuropathological findings were investigated in patients with medically intractable temporal lobe epilepsy but without any focal lesion on CT. Four parameters (an areal ratio of the temporal lobe against the hemisphere, area and calculated T1, T2 values of the hippocampus) were used to determine the abnormal MRI side. An agreement was reached in 67-72% of 18 patients between the abnormal values of the hippocampal area and of calculated T1, T2 and the side of the epileptogenic focus. In 14 of 17 patients, typical hippocampal sclerosis was demonstrated in resected tissue in accordance with the MRI lesions (atrophy and/or prolonged T2 of hippocampus). These results imply: 1) MRI abnormality thus defined may, if not all, indicate the side of the epileptogenic focus, and 2) also the presence of hippocampal sclerosis. It was emphasized that the MRI lesion would be a usable instrument to explore the causal relationship of hippocampal sclerosis to a generation of epileptogenic lesions as well as for presurgical evaluation.     

Acute astrocyte activation in brain detected by MRI: new insights into T(1) hypointensity.

Increases in the T(1) of brain tissue, which give rise to dark or hypointense areas on T(1)-weighted images using magnetic resonance imaging (MRI), are common to a number of neuropathologies including multiple sclerosis (MS) and ischaemia. However, the biologic significance of T(1) increases remains unclear. Using a multiparametric MRI approach and well-defined experimental models, we have experimentally induced increases in tissue T(1) to determine the underlying cellular basis of such changes. We have shown that a rapid acute increase in T(1) relaxation in the brain occurs in experimental models of both low-flow ischaemia induced by intrastriatal injection of endothelin-1 (ET-1), and excitotoxicity induced by intrastriatal injection of N-methyl-D-aspartate (NMDA). However, there appears to be no consistent correlation between increases in T(1) relaxation and changes in other MRI parameters (apparent diffusion coefficient, T(2) relaxation, or magnetisation transfer ratio of tissue water). Immunohistochemically, one common morphologic feature shared by the ET-1 and NMDA models is acute astrocyte activation, which was detectable within 2 h of intracerebral ET-1 injection. Pretreatment with an inhibitor of astrocyte activation, arundic acid, significantly reduced the spatial extent of the T(1) signal change induced by intrastriatal ET-1 injection. These findings suggest that an increase in T(1) relaxation may identify the acute development of reactive astrocytes within a central nervous system lesion. Early changes in T(1) may, therefore, provide insight into acute and reversible injury processes in neurologic patients, such as those observed before contrast enhancement in MS.     

A case of demyelinating disease showing a peculiar honeycomb-like and lamellar structure on magnetic resonance imaging

A case of subacute demyelinating disease showing large honeycomb-like and lamellar lesions in bilateral cerebral white matter on MRI was reported. A 22 year-old woman felt general fatigue on sept. 30, 1988, and developed complete quadriplegia and mutism in about 10 days. She recovered shortly after high-dose corticosteroid therapy. She had neither fever nor meningeal irritation. There were no inflammatory changes in examinations of blood and CSF. On MRI, high and low signal areas of irregular shape in the cerebral white matter and brainstem, were observed on T2 and T1 weighted images, respectively, suggesting that the lesions are of demyelinating nature. These lesions appeared lamellar or honeycomb-like structures on T1 and T2 weighted image. This patient was diagnosed as having a subacute type of demyelinating disease similar to Balo's disease (concentric sclerosis).