Whipple's disease confined to the central nervous system

We report a 49-year-old woman with a leaft parietal lesion, shown on CT and MRI as an isolated ring-enhancing mass. The diagnosis of cerebral Whipple's disease was made by brain biopsy; there were no gastrointestinal symptoms nor periodic-acid Schiff-positive inclusions in the jejunal mucosa. This case illustrates atypical Whipple's disease, confined exclusively to the central nervous system.     

Second brain tumors following central nervous system radiotherapy in childhood

Objective:

The second tumour (ST) occurrence is a relatively uncommon late complication of radiotherapy but represents one of the most significant issues, especially in childhood oncology. We describe our experience with patients who developed second brain neoplasm following cranial irradiation in childhood.

Methods:

We identified nine patients who received radiotherapy owing to central nervous system tumour in childhood and subsequently developed the second brain tumour. The full clinical and radiological documentation and histopathological reports were reviewed. Risk factors such as age at irradiation, latency period to ST diagnosis, radiotherapy doses and volumes and other therapy methods were evaluated. We correlated the ST location with the three levels of irradiation dose (high, >40 Gy; medium, 25–40 Gy; and low <25 Gy).

Results:

Five meningiomas and four gliomas occurred as the ST after the mean time of 11.7 years after radiotherapy. The average age of children during irradiation was 4.6 years. The shorter latency time to the ST induction was found in children treated with chemotherapy (9 years vs 17.2 years). Seven STs developed in the area of high and moderate dose (>25 Gy), only two low-grade gliomas appeared in the low-dose region.

Conclusion:

Our data suggest that the STs usually develop in the brain tissues that received doses >25 Gy in patients irradiated at a young age.

Advances in knowledge:

The low-dose volume seems not to be so significant for second brain neoplasm induction. Therefore, the modern intensity-modulated radiotherapy technique could be safely applied in paediatric patients.The second tumour (ST) occurrence is a multifactorial event, depending on the factors associated with therapy and with the clinical characteristics of the patient, such as age or genetic predisposition.^1–5 The cranial irradiation, especially the radiation dose and the field size, is known as the significant risk factor for developing second brain neoplasms.^1,6–11 However, details of this relationship are still not clear. The mechanisms of the tumour induction are complex.² The radio-induced tumour is defined by many authors as a new mass, histologically different from the original tumour, occurring after delay in irradiated areas, and not related to phacomatosis.^6,9,10 This late complication of radiotherapy is not common but is especially important in young patients with a long life expectancy. Among factors that limit better understanding of ST''s aetiology is the lack of long-term follow-up that is often interrupted when irradiated children become adults. In this study, a single institution''s experience was presented with nine second brain tumours that appeared following central nervous system (CNS) radiotherapy in childhood.     

Neuroblastoma presenting as central nervous system disease

Neuroblastoma may be extremely difficult to recognize, particularly when the tumor presents as a primary central nervous system disease. Central nervous system involvement may be considered as primary intracerebral neuroblastoma, metastases to the cranium from an occult primary, primary intraorbital neuroblastoma originating in the ciliary ganglion, metastatic intraorbital neuroblastoma from an occult primary, primary intraspinal neuroblastoma originating in dorsal root ganglia, intraspinal metastatic disease, and distant effects such as myoclonic encephalopathy. Primary neuroblastoma within the ciliary ganglion and primary intraspinal neuroblastoma are extremely rare entities. Illustrative cases the demonstrate the broad spectrum of neurologic presentations are offered. The second known report of neuroblastoma in association with primary pulmonary hypoventilation (Ondine curse) is included.     

Imaging characteristics of Rosai-Dorfman disease in the central nervous system

Background and purpose

Rosai-Dorfman disease (RDD) is a rare, lymphoproliferative disorder of uncertain etiology. The Central Nervous System (CNS) is a very rare site for RDD and only a few imaging appearances have been described. The purpose of this study is to present the largest series of cases in the CNS imaging literature to increase familiarity with this entity and further identify features that may distinguish RDD from meningioma.

Materials and methods

Findings from imaging examinations in 10 patients with pathologically confirmed RDD were retrospectively reviewed. Two radiologists evaluated the lesion location, shape, size, number, edge, cerebral edema, homogeneous or heterogeneous appearance, attenuation and signal intensity, degree of enhancement, and the relation between lesions and meninges.

Results

RDD in CNS showed similar features in imaging: an extra-axial, well-circumscribed, dura-based mass, isodense or hyperdense on CT, isointensity on T1-weighted imaging and isointensity with hypointensity on T2-weighted imaging. The mass enhanced markedly and homogeneously after the administration of contrast agent and demonstrated dural tail sign in all cases. Significant perifocal edema was associated with the masses. Remarkably, seven patients (77.8%) showed strong hypointensity within isointensity on T2-weighted or FLAIR images and no calcification was observed in CT images or pathologic specimens.

Conclusions

Although RDD in the CNS is a rare process, it should be considered in the differential diagnoses for meningioma. We believe that a typical representation of hypointensity irrelevant to calcification on T2-weighted or FLAIR images can suggest the diagnosis of RDD.     

Sarcoidosis of the central nervous system.

A case of central nervous system sarcoidosis verified by direct biopsy and skin tests and without evidence of any other system involvement is reported. The computed tomography findings are described in detail.     

Schwannomas of the central nervous system.

Isolated schwannomas may occur in many different situations within the central nervous system. Most neurilemmomas are benign, and enucleation after longitudinal dissection of the nerve will usually affect a cure. It is for this reason that the roentgenological appearances should be appreciated and this diagnosis considered so as to attempt total surgical removal.     

Nuclear magnetic resonance imaging in central nervous system disease

From the preliminary work of many investigators, it appears that proton nuclear magnetic resonance (NMR) imaging will have wide application in the diagnostic assessment (and potential management) of patients with vascular, neoplastic, and demyelinating diseases of the central nervous system (CNS). Findings in isolated cases and small series suggest that NMR imaging may play a role in the evaluation of patients with other CNS conditions including hydrocephalus, malformations, infections, developmental and metabolic disorders, and degenerative processes. Because of the dynamic nature of disease processes involving the CNS, the precise meaning of NMR image parameters (rho, T1, and T2) remains unclear. A comprehensive study correlating NMR images in neurologic disease with precise neuropathologic examination is required. In the future, with accurate quantitative measurements of these NMR parameters, in vivo imaging may provide insight into the dynamic nature of neurologic disease.     

Gadolinium-enhanced MRI in central nervous system Behçet's disease

Summary Two cases of central nervous system Behçet's disease, studied by gadolinium-enhanced MRI, are presented. In one patient, whose clinical picture was dominated by a brain stem syndrome, the gadolinium enhancement resolved with clinical improvement, although the hyperintense areas in the mesencephalon on T2-weighted images persisted. In the second, who had a pseudobulbar palsy and a mild right hemiparesis, there were many abnormal areas, but an enhancing focus in the posterior limb of the left internal capsule was probably the lesion responsible for the hemiparesis.     

Hemorrhagic vasculopathy after treatment of central nervous system neoplasia in childhood: diagnosis and follow-up.

PURPOSETo review the clinical data, imaging findings, and intermediate outcomes of a series of children with hemorrhagic vasculopathy after treatment for intracranial neoplasia.METHODSWe retrospectively analyzed the medical records and imaging examinations of 20 pediatric patients (ages 1 to 15 years) with intracranial neoplasia in whom delayed intracranial hemorrhage developed after cranial irradiation or radiation combined with systemic or intrathecal chemotherapy. Patients with intracranial hemorrhage from other identifiable causes were excluded. Histopathologic analysis was available in four patients.RESULTSTwenty patients with delayed intracranial hemorrhage received cranial irradiation alone (n = 9) or combined radiation and chemotherapy (n = 11) for primary brain tumors (n = 13), leukemia (n = 6), or lymphoma (n = 1). Imaging findings were consistent with hemorrhages of varying ages. The hemorrhages were not associated with tumor recurrence nor second tumors. Except for location of the hemorrhage, no significant relationship was established between outcome and original diagnosis, radiation dose (range, 1800 to 6000 centigray), chemotherapeutic agent or dosage, age at treatment, or interval between therapy and hemorrhage (mean, 8.1 years). Only brain stem hemorrhage was associated with a poor outcome.CONCLUSIONIn children with central nervous system neoplasia who have undergone cranial irradiation, or radiation combined with chemotherapy, delayed intracranial hemorrhage may develop.     

MR imaging of the central nervous system in divers.

A group of 70 professional divers and 47 healthy control subjects who had never dived were examined with magnetic resonance (MR) imaging to determine the prevalence of focal white matter changes in the brain. Spots of high signal intensity in white matter on proton density- and/or T2-weighted spin-echo images were detected in 42% of the control subjects and in 34% of the divers. In the control subjects, the prevalence of more than three changes was related to smoking, use of alcohol, head trauma, age of more than 35 years, and a combination of several cerebrovascular risk factors. This relationship was not present in the divers. The prevalence of changes in divers was inversely related to diving depth, amount of diving, participation in "unsafe diving," and decompression sickness. The reasons for these results could not be ascertained. The results are compared with those of MR imaging studies of white matter changes recently presented by other research groups.     

Superficial siderosis in the central nervous system

We describe a rare entity, superficial siderosis of the central nervous system, due to multiple small episodes of subarachnoid haemorrhage from any source. Non-specific neurological findings are associated with deposition of ironcontaining pigments in the leptomeninges and superficial layers of the cortex. T2-weighted magnetic resonance imaging demonstrates characteristic low signal in the meninges.     

中枢神经系统Rosai-Dorfman病的CT和MRI表现

目的 总结中枢神经系统Rosai-Dorfman病(RDD)的CT和MRI影像特点,增加对该病的认识,减少临床误诊率.方法 结合文献回顾性分析4例病理确诊的中枢神经系统RDD患者的临床表现和影像特点.结果 2例患者行头颅CT平扫,4例患者行头颅MRI平扫加增强扫描;1例患者头颅CT显示脑水肿,1例头颅CT平扫未见异常;3例患者头颅MRI显示鞍区T1WI等或低信号,T2WI等或低信号,其中2例呈环形强化,1例呈均匀强化;1例患者右上颌窦、右鼻腔、右眶内及右颅前窝多发病灶,T1WI呈等和低混杂信号,T2WI呈等信号,呈团块样强化.4例患者病灶均基于脑膜生长.结论 临床发热、头痛、血沉增快,血免疫球蛋白升高的患者,如影像检查发现颅内基于脑膜或者脊膜生长的独立或者多发病灶,特别是鞍区的病灶,应考虑到中枢神经系统RDD的可能.