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.     

Balo-s Concentric Sclerosis Report of Two Patients with Magnetic Resonance Imaging Follow-up

Balo's concentric sclerosis was diagnosed antemortem in 2 patients, by magnetic resonance (MR) imaging showing striking concentric alternating rings in 1 patient and by characteristic histopathological features in the other. The course of the lesions and the concentric pattern were followed by MR imaging for 3 years and 18 months, respectively. One patient demonstrated spontaneous remission that has not been reported in Balo's disease . Balo's disease may not have a fulminant course as described in the past and the MR appearance of the chronic lesion may resemble that of a chronic multiple sclerosis plaque.     

Hemidystonia secondary to cervical demyelinating lesions

Hemidystonia is usually associated with a structural lesion in the contralateral basal ganglia. We report a patient with definite multiple sclerosis, according to Poser's criteria, presenting with an acute-onset sustained left hemidystonia. Cranial T2-weighted magnetic resonance imaging (MRI) showed several hyperintense lesions in the centri semiovali and in the periventricular area without basal ganglia involvement. Moreover cervical spinal cord T2-weighted MRI showed two hyperintense lesions in the left posterolateral spine at C2 and C3, and one lesion in the right posterolateral spine at C4 levels. The hemidystonia improved completely after daily treatment with 1000 mg of methylprednisolone, and cervical MRI was performed after the improvement which showed that the lesions had become smaller and less intense. Finally we consider that the hemidystonia may be caused by the cervical spinal cord lesions of multiple sclerosis.     

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.     

Electroconvulsive therapy in patient with psychotic depression and multiple sclerosis

Safety of electroconvulsive therapy (ECT) in depressive patients with multiple sclerosis (MS) is still discussed and based solely on case reports. This kind of therapy was used in both unipolar depression and depression in bipolar disorder. It was suggested that ECT might cause the deterioration of neurological state (new MS lesions in magnetic resonance imaging). Moreover, there were also data indicating some anesthesiological complications and difficulties in patients with MS. We have presented a case of a patient who was treated with ECT and developed grand mal seizure after 14th electroconvulsive treatment.     

Korsakoff's syndrome as the initial presentation of multiple sclerosis

Summary A 37-year-old man presented with an acute amnestic syndrome of Korsakoff's type and an upper brain-stem oculomotor syndrome. After a moderate improvement with steroid therapy, he developed progressive behavioural changes due to a frontal lobe syndrome, in addition to motor and visual impairment. Memory performance was investigated on several occasions during an 11-year follow-up. Diagnosis of laboratory-supported definite multiple sclerosis was established and magnetic resonance imaging showed disseminated white matter lesions, especially in both medial temporal lobes. No other cause than multiple sclerosis was found for the amnestic syndrome. Among the rare cases of Korsakoff's syndrome in the course of multiple sclerosis, this is to our knowledge the first case in which a memory deficit was the initial manifestation of the disease.     

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.     

Azathioprine therapy in a case of pediatric multiple sclerosis that was seropositive for MOG-IgG

There is a lack of evidence for treatment of pediatric multiple sclerosis (PedMS). Treatment using azathioprine for PedMS has not been reported. A 10-year-old boy with multiple sclerosis who was seropositive for antibodies against myelin oligodendrocyte glycoprotein (MOG)-IgG was treated with azathioprine plus oral methylprednisolone. The patient showed clinical and magnetic resonance imaging stability, with MOG-IgG seroconversion. There were no major side effects over a 5-year period. Azathioprine may be a treatment option, particularly in poor medical resource areas, for pediatric patients with multiple sclerosis who are seropositive for MOG-IgG.     

Multiple sclerosis and mitochondrial myopathy: An unusual combination of diseases

A woman with definite multiple sclerosis (MS) and mitochondrial myopathy is described. There were widespread white matter lesions on magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) abnormalities and evoked response changes. Muscle biopsy showed ragged red fibres (RRFs) and cytochrome c oxidase (CoX) deficiency. Southern blot analysis revealed a large deletion of mitochondrial DNA (mtDNA). The patient may be affected by two unrelated diseases, MS and mitochondrial myopathy, but this combination has never previously been reported.     

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'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     

Balo''s concentric sclerosis diagnosed intravitam on brain biopsy

A 28-year-old woman presented with acute right hemiparesis evolving to bilateral hemiplegia and a mute state within 1 week on a background of paranoid psychosis of 8 years duration. CT scan revealed multiple bilateral hypodense enhancing lesions in the central white matter. Biopsy of the lesion showed lamellar alternating zones of demyelination and remyelination, typical of Balo's concentric sclerosis, an acute variant of multiple sclerosis. Immunohistochemical staining for neurofilament showed greater damage to the axis cylinders by the disease than generally perceived by silver stains. Steroid therapy produced a gratifying and sustained improvement. Follow up CT scans and psychometry revealed regression of the demyelinating lesions and improvement of the cognitive state.     

Enhancing magnetic resonance imaging lesions and cerebral atrophy in patients with relapsing multiple sclerosis

OBJECTIVE: To examine the relation between the frequency of enhancing magnetic resonance imaging lesions and their characteristics of enhancement and atrophy in patients with early relapsing multiple sclerosis. DESIGN: Analysis of number of enhancing lesions, ventricular volumes and diameters, and lesion characteristics on monthly magnetic resonance imaging scans during natural history follow-up. SETTING: A clinical research institution. PATIENTS: Sixteen patients with confirmed early relapsing multiple sclerosis. MAIN OUTCOME MEASURE: Cerebral atrophy as measured by ventricular enlargement. RESULTS: Numbers of enhancing lesions correlated well with an increase of ventricular size. This correlation was strongest for patients with a high proportion of concentric ring-enhancing lesions with central contrast pallor. CONCLUSIONS: Inflammatory events, especially those within lesions with associated blood-brain barrier breakdown, affect the ensuing loss of brain parenchyma. Patients with a high proportion of lesions with central contrast pallor, which is likely associated with more extensive tissue damage, have a higher rate of atrophic changes.     

Disappearance of hypointense multiple sclerotic lesions on FLAIR MRI.

A child is presented who displayed hypointense multiple sclerotic lesions on fluid-attenuated inversion recovery sequences by magnetic resonance imaging, with the possible pathologic tissue changes of these hypointense lesions evaluated. The magnetic resonance imaging results in this patient demonstrated the disappearance of low-signal lesions on fluid-attenuated inversion recovery in multiple sclerosis, and the improvement of this patient's condition was likely compatible with sequential magnetic resonance imaging findings. Some hypointense lesions in the supratentorial white matter that appear on fluid-attenuated inversion recovery images in multiple sclerosis patients may include reversible brain lesions, suggesting extracellular fluid collection not accompanied by axonal loss or gliosis.     

Serial magnetic resonance imaging in patients with Balò's concentric sclerosis: natural history of lesion development.

We reviewed the magnetic resonance imaging scans from 22 serial studies of 5 patients with Balò's concentric sclerosis collected during the past 3 years. The data showed the concentric lesions did not occur simultaneously but developed step by step in a centrifugal direction. The development of lesions was preceded by an enhancing ring relatively devoid of demyelination and was followed by progressive demyelination occurring mainly at the inner aspect of the enhancement. The same process recurred on the edge of the previous enhanced zone. Thus, an appearance of concentric rings with alternating demyelinated and relatively myelin-preserved bands was formed.     

Gd-DTP A-enhanced lesions in the brain of patients with acute optic neuritis

The cerebral hemispheres of 19 patients with acute monosymptomatic optic neuritis (AMON) were investigated using magnetic resonance imaging (MRI) and Gadolinium-DTPA (Gd-DTPA). Using T1-weighted and T2-weighted imaging sequences it was disclosed that 14 of 19 patients had lesions and that enhancement was seen in seven patients. It is known that patients with AMON and silent lesions in the CNS have a highly increased risk of developing multiple sclerosis (MS) later on. If it is accepted that a silent lesion in patients with AMON does represent a multiple sclerosis (MS) plaque, and that an enhancing silent lesion represents an acute MS lesion, we may hypothesize that the disease process ultimately leading to MS starts long before the first symptom or sign ever appears. It would seem that at least half of the silent lesions in the cerebral hemispheres of patients with AMON had existed before the onset of the AMON, and that the disease actually started before the onset of the AMON.     

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.     

Cerebrospinal fluid and MRI findings in three patients with multiple sclerosis and systemic lupus erythematosus

Three patients fulfilling the diagnostic criteria of both multiple sclerosis (MS) and systemic lupus erythematosus (SLE) were examined clinically, immunologically and by magnetic resonance imaging (MRI). In all three patients MRI showed several high-signal lesions compatible with MS and, additionally, non-specific small white matter lesions suggesting small vessel occlusion were seen. In CSF the cytoimmunological abnormalities were variable and showed only slight to moderate immunoactivation within the CNS at the time of sampling.     

Infantile multiple sclerosis with extensive white matter lesions

A boy developed a right hemiparesis at 13 months of age which disappeared spontaneously at 19 months. Computed tomography and magnetic resonance imaging revealed extensive low-density areas with left-sided predominance in the white matter at age 17 months. The flash visual evoked potential revealed abnormal findings of wave V. At 20 months of age, paraplegia developed; as it receded 3 months later, a left hemiparesis developed. At that time, computed tomography demonstrated a new low-density area in the right centrum semiovale which disappeared at 28 months of age concomitant with the recovery of the left hemiparesis. Overall, he had experienced 3 independent episodes, 2 of which corresponded to each of the lesions depicted by computed tomography and magnetic resonance imaging. Multiple sclerosis was diagnosed at 23 months of age which is earlier than in any previously reported patient. Unlike most typical findings of multiple sclerosis on computed tomography and magnetic resonance imaging studies, our patient demonstrated extensive white matter lesions.     

Magnetic resonance imaging and cognitive functioning in multiple sclerosis

Summary The relationship between cognitive impairment in multiple sclerosis and brain lesions seen on magnetic resonance imaging (MRI) was studied. Three groups of 11 patients with multiple sclerosis, matched for the variables of disability, duration of illness, age and sex, were included. On the basis of neuropsychological testing, the groups were seen to differ in their level of cognitive impairment. The first group showed no cognitive impairment, the second group a moderate, and the third group a serious cognitive impairment. These differences between the groups were reflected by MRI, which revealed more abnormalities in the groups with cognitive impairment compared with the group with normal cognitive function. However, by MRI it was not possible to distinguish between the groups with moderate and that with serious cognitive impairment.