Progressive Multifocal Leukoencephalopathy

Progressive multifocal leukoencephalopathy is a subacute demyelinating disease that occurs in patients with defects in cell-mediated immunity, including those with AIDS and lymphoproliferative disorders. It is caused by reactivation of JC virus (JCV), which infects 70% to 90% of the population by adulthood, but remains latent in normal hosts. Once reactivated, JCV infects oligodendrocytes and astrocytes, with resultant cell lysis, leading to focal areas of demyelination and necrosis in cerebral white matter causing focal neurologic deficits and characteristic findings on MRI. Polymerase chain reaction for the detection of JCV is a sensitive and specific test, replacing brain biopsy as the initial diagnostic test in the appropriate clinical setting. Historically, the prognosis of progressive multifocal leukoencephalopathy is poor, with most patients dying within 6 months of diagnosis. Antiviral medications targeted against JCV have shown little success. However, with the use of highly active antiretroviral therapy, survival of AIDS patients with progressive multifocal leukoencephalopathy has improved.     

Are behaviour and motor performances of rheumatoid arthritis patients influenced by subclinical cognitive impairments? A clinical and neuroimaging study

OBJECTIVE: To determine whether some behavioural manifestations and poor motor performances in patients affected by rheumatoid arthritis (RA) are due to subclinical cognitive defects. METHODS: We performed a psychometric assessment of 30 patients affected by RA exploring several cognitive domains such as memory, visual-spatial integration, motor planning, mental flexibility, relating performances with morphological and functional neuroimaging (MRI and SPECT). We also related the cognitive data with the Ritchie and Lee indexes and other clinical parameters. RESULTS: We found an impairment in visual-spatial tasks in 71% of patients with a high correlation to activity and disease severity as expressed by the Ritchie and Lee indexes (p < 0.005; p < 0.01). Furthermore, we detected in 38% of patients some difficulties in mental flexibility related to the Lee Index (p < 0.05). These poor performances are related to hypoperfusion of the frontal and parietal lobes as detected by brain SPECT; this finding is more evident in patients with brain white matter alterations on MRI. CONCLUSIONS: Our data allow us to hypothesize that manual dexterity could be due to a disconnection between subcortical white matter and parietal-frontal lobes because of microangiopathy; furthermore, a chronic reduction in sensorial stimuli by impaired joints could lead to produce an alteration in motor planning cognitive processes.     

Transient tetraparesis after intrathecal and high-dose systemic methotrexate

 Aggressive polychemotherapy, intrathecal cytostatic prophylaxis and cranial irradiation have contributed to the remarkable improvement in the prognosis of acute lymphoblastic leukemia (ALL) and subtypes of high-grade non-Hodgkin's lymphoma (NHL) and the reduction of central nervous system (CNS) relapses. Early and late neurologic changes have been observed after different CNS-directed therapies. We report on the rare event of an acute tetraparesis after methotrexate (MTX) without other CNS-directed therapy. A young female with a diffuse large B-cell lymphoma developed signs of meningeal irritation a few hours after intrathecal prophylaxis with MTX, cytosine-arabinoside and dexamethasone. She recovered quickly. Ten days after her last course of systemic chemotherapy including high dose MTX she was admitted with a tetraparesis and motoric aphasia. A computer assisted tomography (CT) scan was normal. On magnetic resonance imaging (MRI) hyperintense white matter lesions were visible in the periventricular white matter. Initially, the radiologic signs were progressive while the patient's clinical condition improved. MRI controls after complete neurologic normalization revealed delayed partial regression of the white matter abnormalities. The patient has now been free of neurologic symptoms for 16 months. This case report demonstrates acute and subacute neurotoxic effects of MTX in the same patient and illustrates that radiologic CNS changes can persist irrespective of the disappearance of clinical symptoms. Received: March 13, 1998 / Accepted: July 29, 1998     

儿童亚急型与慢型克山病临床对比观察

对133例儿童亚急型和60例慢型克山病进行了临床对比观察,结果表明:1、亚急型患者年龄较慢型小:2亚急型出现奔马律较慢型多,而慢型出现心律失常及并发症者明显多于亚急型:3、心电图改变亚急型窦性心动过速,低电压较慢型多,而慢型房室扩大、过早搏动明显多于亚急型:4、慢型心脏扩大程度明显重于亚急型:5、慢型克山病预后较差。     

Susac syndrome

Susac syndrome is a mysterious vasculopathy affecting brain, retina and inner ear in young women. Main features of the disease are increasingly recognized: subacute encephalopathy often mimicking psychosis and frequently heralded with unusual ophthalmic migraine; frequent subclinical meningitis; brain MRI with multiple and bilateral white and gray matter nuclei lesions, with prominent involvement of corpus callosum; bilateral involvement of central retina artery branches, not only with occlusions but also with peculiar leakage of fluorescein through arteriolar walls on late stages of angiography; non-specific bilateral cochleovestibular symptoms with audiogram showing perception hypoacousia that predominates on low frequencies. Outcome, prognosis, pathogenesis and a rational basis for treatment are discussed in this review. A key message for the clinician should be to perform brain MRI, audiogram and retinal angiography whatever the mode of entry, in order not to miss one (or two) features of this syndrome triad.     

Autopsy case of primary central nervous system lymphoma with subacute mental deterioration and progressive white matter lesions in an elderly man

An autopsy case is reported here of a 70-year-old Japanese man with primary central nervous system lymphoma (PCNSL) manifesting subacute mental deterioration and progressive white matter lesions. Brain magnetic resonance imaging (MRI) revealed irregular hyperintense lesions on T₂-weighted images of the white matter of the bilateral frontal lobes. The hyperintense lesions then spread to the corpus callosum, internal capsule, globus pallidus and along the pyramidal tract in the brainstem without a mass effect. The U-fibers were also involved in some regions. Neither brain swelling nor a significant atrophy was observed. The clinical manifestations and imaging findings of PCNSL are diverse. Recently, some reports have revealed atypical MR imaging of PCNSL exhibiting diffuse infiltration in the brain parenchyma without mass formation and enhancement of the lesions after the administration of a contrast medium. The authors also encountered an interesting case of PCNSL presenting as progressive white matter lesions without brain swelling. It is now possible to finely observe white matter lesions by brain MRI, particularly in T₂-weighted images. Recently the number of immunocompetent elderly patients with PCNSL appears to be increasing in Japan. Autopsy of the present case revealed an intermediate lymphocytic malignant lymphoma. Because imaging and laboratory findings are not completely specific for the diagnosis of PCNSL at present, brain biopsy is more strongly recommended for the final diagnosis of PCNSL even in elderly patients, based on which appropriate treatment is administered.     

Chronic alcoholism with diffuse brain atrophy

An analysis of clinical manifestations and CT scan of 9 cases of chronic alcoholism was reported. It is shown that there are diffuse brain atrophy in chronic alcoholism patients with neuro-psychiatric symptoms. There were significant relations among clinical history, amount of alcohol consumed and brain atrophy. It should also be mentioned that the number of young alcoholics is more than that of elderly. This problem must be paid attention to.     

An autopsy case of chronic empyema-associated angiosarcoma that presented with a rapidly growing chest wall mass]

A 76-year-old woman with chronic tuberculous empyema presented with bloody sputa and anorexia in April 2005. Chest X-ray and CT scan showed no obvious change compared with previous films. She complained of a left chest pain in June 2005, and chest CT scan showed a tumor shadow enhanced heterogeneously on the left chest wall and chronic empyema. After admission, the chest wall mass grew rapidly. Neither CT- nor sonographically guided biopsy yielded a definitive diagnosis. Dynamic MRI showed a high signal intensity unlike hematoma. She died of respiratory failure 2 months after the onset of her chest pain. Autopsy revealed angiosarcoma. We should always keep in mind the early diagnosis of malignant tumor and tuberculosis in patients presenting with a chest wall mass and constitutional symptoms during follow-up of chronic tuberculous empyema.     

In vivo detection and functional correlates of white matter microstructural disruption in chronic alcoholism

BACKGROUND: Postmortem studies report degradation of brain white matter microstructure in chronic alcoholism, but until recently, in vivo neuroimaging could provide measurement only at a macrostructural level. The development of magnetic resonance diffusion tensor imaging (DTI) for clinical use offers a method for depicting and quantifying the diffusion properties of white matter expressed as intravoxel and intervoxel coherence of tracts and fibers. METHODS: This study used DTI to examine the intravoxel coherence measured as fractional anisotropy (FA) and intervoxel coherence (C) of white matter tracts of the genu and splenium of the corpus callosum and of the centrum semiovale in 15 detoxified alcoholic men and 31 nonalcoholic control subjects. Exploratory correlational analyses examined the relationships between regional DTI measures and tests of attention and working memory in the alcoholic patients. RESULTS: The alcoholic group had lower regional FA than the control group. C was lower in the alcoholics than controls in the splenium only. Working memory correlated positively with splenium FA, whereas attention correlated positively with genu C. CONCLUSIONS: These results provide in vivo evidence for disruption of white matter microstructure in alcoholism and suggest that interruption of white matter fiber coherence contributes to disturbance in attention and working memory in chronic alcoholism.     

胰腺囊实性乳头状上皮性肿瘤临床病理特点

目的 评价胰腺囊实性乳头状上皮性肿瘤的临床病理特点及其治疗。方法 分析我院2003年收治的经手术病理证实的2例胰腺囊实性乳头状上皮性肿瘤的临床病理资料，并复习国内外相关文献。结果 该病好发于青年女性，临床表现主要为上腹部肿物，腹痛，上腹不适等，无特异表现，超声、CT、MRI等影像学检查有诊断价值，最终诊断依靠病理学检查。本组2例均行手术切除，疗效满意。结论 胰腺囊实性乳头状上皮性肿瘤为低度恶性肿瘤，预后较好，治疗首选手术切除。     

Acute liver failure as the initial manifestation of acute leukaemia.

BACKGROUND/AIMS: Haematological malignancies seldom cause clinically significant liver disease. Acute liver failure as the initial manifestation of acute leukaemia is very rare and carries a very poor prognosis. METHODS/RESULTS: Three cases of acute liver failure secondary to acute leukaemia are described. Each case presented initially as acute liver failure of uncertain cause. Specific treatment for the leukaemia was instituted; however, all three patients died as a consequence of the liver failure. We describe the clinical course and relevant investigations of these patients and discuss possible mechanisms of acute liver failure in this setting. CONCLUSION: Acute leukaemia presenting as acute liver failure has a very poor prognosis. Although a rare cause of acute liver failure, it should be considered in any patient presenting with acute liver failure with prodromal symptoms and a raised peripheral white cell count, lactate dehydrogenase and uric acid.     

Current role of conventional and digital aortography in the diagnosis of aortic disease

The availability of the less invasive techniques such as CT, MRI, and digital angiography require reexamination of the indications for conventional screen-film aortography. Because of poor image quality, IV digital subtraction angiography should be limited to congenital aortic disease and follow-up cases. Intraarterial digital angiography can be used as a supplement to or as a replacement for conventional aortography in most cases. Care should be used in substituting intraarterial digital angiography for conventional studies in aortic dissection and aortic rupture; continuing problems with digital subtraction artifacts may introduce error in cases with subtle abnormalities. In aortic aneurysm, CT is usually sufficient for diagnosis and surgical planning with angiography used for inconclusive examinations or more accurate determination of branch involvement. CT is the primary diagnostic examination in suspected chronic or subacute dissection and is the method of choice in sequential studies of patients following medical or surgical therapy for dissection. In acute aortic dissection, either angiography or CT may be used and are equally diagnostic. Angiography is most helpful in aortic dissection with suspected brachiocephalic vessel involvement, coexistant coronary artery disease, or in cases of indeterminate CT. In blunt chest trauma, aortography remains the examination of choice in the diagnosis of aortic transection. CT may play a role in excluding aortic damage in stable patients with a low clinical suspicion of aortic transection.     

肝脏影像学检查在土三七引起的肝小静脉闭塞性疾病中的诊断价值

目的探讨肝脏影像学改变在肝小静脉闭塞性疾病(HVOD)患者诊断中的价值。方法对45例HVOD患者临床表现、肝脏影像学检查及肝穿刺组织学变化进行分析。结果 45例患者肝脏超声检查有28例患者可见"斑片状"或"豹纹状"低回声区;CT检查均有"地图状"密度改变,肝脏MRI T1WI均呈大片状低信号,T2WI信号呈略高信号,增强后呈"地图状"不均匀强化。肝脏超声诊断HVOD符合率62.2%(28/45);肝脏CT、MRI诊断HVOD符合率100%,二者差异有统计学意义(χ2=15.06,P<0.05)。21例作肝组织穿刺确诊的HVOD患者,检查均显示肝脏有"地图状"的高、低密度改变。结论肝脏CT、MRI诊断HVOD较肝脏超声检查有更高敏感性和特异性。肝脏CT、MRI可替代肝穿刺组织学检查诊断HVOD。     

我国慢性胰腺炎的临床特点——附132例分析

本文分析了132例慢性胰腺炎的临床资料,描述了我国慢性胰腺炎的临床特点。与西方国家不同,我国慢性胰腺炎最常见的病因是胆道疾病而不是酒精中毒。脂肪痢和胰腺钙化在我国慢性胰腺炎病人中不很多见。本文还讨论了ERCP,B型超声,CT和PABA试验等项检查在诊断慢性胰腺炎中的价值。     

Multiple cerebral lesions in a patient with refractory celiac disease: A case report

BACKGROUNDEnteropathy-associated T cell lymphoma (EATL) is an aggressive intestinal T cell lymphoma derived from intraepithelial lymphocytes, which occurs in individuals with celiac disease (CD). Cerebral involvement is an extremely rare condition and as described so far, lymphoma lesions may present as parenchymal predo-minantly supratentorial or leptomeningeal involvement. We describe a case of EATL with multifocal supra- and infratentorial brain involvement in a patient with refractory celiac disease (RCD).CASE SUMMARYA 58-years old man with known CD developed ulcerative jejunitis and was diagnosed with RCD type II. Six months later he presented with subacute cerebellar symptoms (gait ataxia, double vision, dizziness). Cranial magnetic resonance imaging (MRI) revealed multifocal T2 hyperintense supra- and infratentorial lesions. Laboratory studies of blood and cerebrospinal fluid were inconspicuous for infectious, inflammatory or autoimmune diseases. ¹⁸F-fluorodeoxyglucose-positron emission tomography/computed tomography (¹⁸FDG-PET/CT) scan showed a suspect hypermetabolic lesion in the left upper abdomen and consequent surgical jejunal resection revealed the diagnosis of EATL. During the diagnostic work-up, neurological symptoms aggravated and evolved refractory to high-dosage cortisone. Recurrent MRI scans showed progressive cerebral lesions, highly suspicious for lymphoma and methotrexate chemotherapy was initiated. Unfortunately, clinically the patient responded only transiently. Finally, cerebral biopsy confirmed the diagnosis of cerebral involvement of EATL. Considering the poor prognosis and deterioration of the performance status, best supportive care was started. The patient passed away three weeks after diagnosis.CONCLUSIONEATL with cerebral involvement must be considered as a possible differential diagnosis in patients with known RCD presenting with neurological symptoms.     

Extramedullary Soft Tissue Involvement and Discrepant Osseous Uptake on Tc-99m MDP and Ga-67 Citrate Scintigraphy in a Patient With Multiple Myeloma: A Case Report and Literature Review

Multiple myeloma (MM) is a plasma cell neoplasm with skeletal destruction which could also spread to extramedullary regions. Common diagnostic imaging modalities include skeletal radiography, computed tomography (CT), magnetic resonance imaging (MRI). Recently, PET/CT is proposed as an ideal tomographic tool for diagnosis and follow-up, but impending factors includes high cost, limited availability of cameras and radiotracers. Bone scan and gallium scan are usually considered of limited clinical value.Herein, we present a 66-year-old Taiwanese man with MM, who was hospitalized to our hospital for bone pain control. Bone and gallium scintigraphies were obtained for bone pain and infection workup. However, unexpected features of discordant osseous uptake with high gallium-to-bone uptake ratio and extramedullary gallium uptake were noted which both indicated poor prognosis of MM. The patient then passed away due to rapid disease progression.In conclusion, although gallium and bone scintigraphies are considered less sensitive for MM, combined use may be a good alternative for ¹⁸F-FDG PET/CT in evaluation of disease extent and prognosis, especially in high-risk patients or with suspicion of disease progression.     

结节性硬化症39例CT及MRI影像学特点分析

目的探讨结节性硬化症(TS)的CT、MRI影像学特征表现及诊断价值。方法回顾性分析39例经临床和手术确诊为TS患者的影像学资料,根据脑内病灶所处的位置以及合并脑内或其他脏器病变进行影像学分型。结果Ⅰ型10例分布在室管膜下,Ⅱ型21例,除分布在室管膜下外,还分布在皮层、白质等部位,Ⅲ型8例合并其它脏器肿瘤。结论 CT、MRI表现是脑内TS确诊的主要依据,二者相互结合对明确诊断有重要意义;对TS进行影像学分型发现结节位置及合并症是TS预后的决定因素。     

Lysability of arterial thrombi assessed by magnetic resonance imaging

BACKGROUND: Intravascular thrombi change in time due to retraction and organization, which is reflected in the appearance of magnetic resonance images of clots. We have hypothesized that MRI has the potential to improve patient selection for thrombolytic treatment. The aim of our study was to analyze occlusive arterial thrombi with MRI, and to correlate the MRI parameters with the therapeutic outcome in patients with occlusive atherothrombotic disease of the superficial femoral artery who were treated with catheter-directed thrombolysis by streptokinase. PATIENTS AND METHODS: We included 13 patients with subacute (2 weeks to 3 months old) occlusive arterial thrombi and 4 patients with chronic (more than 6 months old) arterial occlusions. We measured the MRI signal intensity on gradient echo images of 98 axial slices of the subacute occlusive thrombi and in 45 slices of 4 chronic thrombi. Following MRI, the patients with subacute history were treated with catheter-directed thrombolysis. RESULTS: Thrombolysis was successful in 11/13 patients. The normalized MRI signal intensity was significantly higher in the unsuccessfully treated thrombi than in the successfully treated thrombi (1.10 +/- 0.08 vs. 0.72 +/- 0.17, p < 0.003), but the subacute and chronic thrombi did not differ in signal intensity. CONCLUSIONS: High signal intensity of arterial thrombi on gradient echo MRI might predict resistance to thrombolytic therapy.     

Acute haemorrhagic leukoencephalopathy: two case reports and review of the literature

Acute haemorrhagic leukoencephalopathy is a rare, rapidly progressive disease of cerebral white matter associated with high fatality. The few known patients surviving have all been treated with early instigation of immuno-modulatory therapy. Here we describe two cases of this disease and underscore the importance of considering this diagnosis in the differential for any individual presenting with progressive neurological signs, an antecedent flu-like illness, and early changes on CT showing widespread disease of white matter.     

Disorders in neuropsychological development and their relation to computed tomography brain scan in children with acute lymphoblastic leukemia in long-term remission

In a group of 71 children with acute lymphoblastic leukemia in long-term remission lasting 4 to 15 years different disorders in their neuropsychological development were found. The disturbances observed were as follows: Emotional disorders in 88%, personality development disorders in 65%, mental retardation in 58%, signs of CNS dysfunction in 54%, and neurological disorder in 31% of children. Abnormal computed tomography brain scans were present in 49% of patients. They consisted mainly of two types: Hypodense areas of the white matter were observed in 23% and widening of the ventricular system and subarachnoidal spaces was present in 26% of children. Statistical evaluation showed significant relations between CT brain scan abnormalities (mainly hypodense changes) and neurological disorders as well as the presence of signs of CNS dysfunction and disorders of personality development. Emotional and mental changes did not correlate with abnormalities of CT brain scan findings.