Aspergillosis of the central nervous system

Aspergillosis of the central nervous system is rare. The spread to the CNS is usually from the lungs and uncommonly from paranasal sinuses. Four cases of CNS aspergillosis with spread of infection from the paranasal sinuses are described. Two patients had meningitis, one intracranial granuloma and one presented with subarachnoid haemorrhage resulting from a fungal mycotic aneurysm. Difficulties in establishing the diagnosis are discussed.     

Aspergillosis with involvement of the central nervous system

Clinical and pathological findings in a patient with central nervous system lesions due to a disseminated aspergillosis are reported. Symptoms and signs were suggestive of a meningoencephalitis with onset two years after nephrectomy for cancer and four months after a corticotherapy was started for post-radiation digestive disorders. A review of the literature shows that C.N.S. is rarely involved in this disease, that the diagnosis is difficult in immunodepressed patients and that the prognosis is poor in the absence of a specific treatment.     

原发性中枢神经系统血管炎1例及文献复习

1 临床资料患者,男,26岁,工程师.因言语不利伴短暂性意识模糊1周于2010年7月10日收治于上海中医药大学附属曙光医院神经内科.患者于1周前剧烈运动后,突感双下肢乏力,并伴有言语不利、短暂性意识模糊及咽痒感,无恶心、呕吐、眩晕等不适症状,即至我院急诊就诊,查血常规示白细胞9.73×109/L,中性粒细胞51.8%,红细胞5.15×1012/L,血红蛋白159.5 g/L,血小板284×109/L.头颅CT平扫未见明显异常.考虑短暂性缺血发作(transient ischemic attack, TIA)合并感染可能,给予抗生素、中药活血化瘀通络等治疗.治疗后,咽痒感症状明显改善,但仍有言语不利及偶发的短暂性意识模糊.入院时,患者诉肢体偶有麻木感,发作性言语不利,舌体僵硬感,无恶心、呕吐、眩晕等不适症状.患者近4月来常有短暂性眩晕感并伴有剧烈头痛发作,可自愈且无后遗症,未进行正规治疗.     

Aspergillosis of the central nervous system: clinicopathological analysis of 17 patients

The clinical, laboratory, and pathological features of aspergillosis of the central nervous system (CNS) were studied in a series of 17 autopsied patients. Two groups were defined. Group A consisted of 8 patients with diseases commonly associated with CNS aspergillosis: leukemia, lymphoma, aplastic anemia, and renal transplantation. Group B contained 9 patients with various illnesses not generally known to be associated with CNS aspergillosis. CNS aspergillosis was diagnosed and treated before death in only 1 patient. Patients in Group A received cytotoxic drugs, often had granulocytopenia, less commonly had focal neurological deficits, and seldom had seizures. Group B patients were not granulocytopenic, received no cytotoxic agents, underwent nontransplant surgery, and more frequently had focal neurological deficits. Eleven of the 17 patients (65%) had focal deficits, most of them hemiparesis. Meningeal signs were rare, but the cerebrospinal fluid was usually abnormal. The principal neuropathological process was Aspergillus invasion of blood vessels causing hemorrhagic infarction. Focal clinical deficits correlated neuroanatomically with Aspergillus lesions. In 2 patients, such lesions were detected by 99mTc-DTPA or cerebral angiography before computed tomographic scanning. The lungs were the usual portal of entry, but isolated CNS lesions occurred in 2 patients. CNS aspergillosis should be considered as a cause of new onset of focal neurological deficits in patients with illnesses that are more diverse than has generally been appreciated.     

Primary central nervous system angiosarcoma: A case report and literature review

Angiosarcoma is a rare vascular malignant neoplasm that mainly occurs in skin and soft tissues. Intracranial localization is very rare and only a few cases have been reported. This report intends to present the clinical, radiological and pathological pictures of a primary central nervous system angiosarcoma along with a review of the literature. A 35‐year‐old woman presented at our institution with weakness and sensory disturbances of her right hand. Neuroimaging revealed a roughly round, hemorrhagic and moderately enhancing lesion in the left frontal posterior region. The tumor was totally removed under awake anesthesia and continuous monitoring of motor and language functions. Histopathology revealed an epithelioid angiosarcoma. Radical removal, followed by adjuvant radiotherapy and chemotherapy, is able to completely control the disease for a relatively long period.     

Primary central nervous system lymphoma report of 32 cases and review of the literature

We retrospectively analyzed 32 cases of primary central nervous system lymphoma (PCNSL). Five cases were diagnosed in the period 1987-1994, for 27 cases in the period 1995-2002. There were 17 men and 15 women whose median age was 69 years. Three patients were immunodeficient. The commonest symptoms were focal deficit (16 patients) and cognitive/behaviour disturbances (14 patients). Radiologically, a total of 47 contrast-enhancing lesions were observed in 32 patients; 18 patients had deep-seated lesions. All but two patients underwent histological diagnosis following craniotomy (11 patients) and/or stereotaxic biopsy (22 patients); diagnosis was obtained on CSF cytology in one patient with a third ventricle tumour. In the last patient, the diagnosis was based on the finding of marked tumour shrinkage under corticotherapy, despite two negative histological examinations. Treatment included surgical resection (10 patients), chemotherapy (25 patients) and/or radiotherapy (12 patients). According to the therapeutic recommendations of the GELA (Groupe d'Etude des Lymphomes de l'Adulte), 19 patients received at least two courses of high-dose methotrexate; intrathecal chemotherapy was used in 20 patients with methotrexate and/or cytosine arabinoside. Radiation therapy consisted of whole brain irradiation followed by a boost on tumour site. Nine patients received a combined treatment of chemotherapy and radiotherapy. Twelve patients showed rapid progression to death. At the time of last contact, 28/32 patients (88%) had died, all from PCNSL disease or from complications due to its treatment. The median survival time was 13.9 months. We conclude that PCNSL is an increasingly frequent tumour. The diagnosis is obtained by stereotactic biopsy in the majority of cases. The prognosis appears dismal despite an intensive multidisciplinary therapeutic approach.     

神经纤维瘤病在中枢神经系统的临床表现和治疗(附14例报告及文献复习)

目的回顾分析侵犯中枢神经系统的神经纤维瘤病之临床特点和治疗经验。方法14例诊断明确的神经纤维瘤病患者，Ⅰ型者（2例）主要以神经胶质细胞或神经细胞发育不良（巨脑回）、成瘤性（视神经胶质瘤）以及颅骨缺损为特征；Ⅱ型者（12例）病变主要侵犯脑的被覆组织，如脑膜和神经鞘膜，表现为许旺细胞瘤和脑膜瘤病。针对不同肿瘤类型和主要病征采取个体化治疗方案．其中Ⅰ型蝶骨发育缺损导致进展性突眼者采取眶后壁钛板塑形修补术，脑发育异常引起癫痫发作者予以药物治疗：Ⅱ型双侧听神经瘤者，根据肿瘤大小、听力损伤程度选择手术切除；有占位效应的并发性肿瘤（多发脑膜瘤、神经鞘瘤）患者也分别采取手术切除；对侧未手术的听神经瘤或多发脑膜瘤、手术后残留听神经瘤复发者施以γ-刀治疗。结果14例患者平均随访27个月，12例Ⅱ型患者中11例经综合治疗后所有肿瘤控制良好，1例死于颅内多发肿瘤侵袭生长。10例13耳行听神经瘤切除术，3例复发．1例死亡：仅3耳保存听力。结论神经纤维瘤病在中枢神经系统中表现多样，多部位、多种类型肿瘤和畸形并存．使治疗十分困难，应当根据不同病变类型和主要的临床症状采取个体化的治疗方案。     

Vascular amyloid in the aging central nervous system. Clinico-pathological study and literature review

The clinico-pathological features of five patients with vascular amyloid restricted to the central nervous system are presented. In three normotensive patients, intracerebral hemorrhage was the dramatic manifestation of amyloid angiopathy. In two other cases, one of amyloid in an arteriovenous malformation, the other of amyloid following therapeutic radiation, amyloid deposition was asymptomatic. Clinically, amyloid angiopathy must be considered in the different diagnosis of intracerebral hemorrhage, independent of the presence of dementia. Pathologically, a factor common to the syndrome of cerebrovascular amyloid appears to be locally increased vascular permeability resulting from a variety of previous tissue injuries.     

Whipple''s disease and the central nervous system a case report and a review of the literature

A 65-year-old man was suffering from recurrent manic psychosis accompanied by weight loss. He also had a history of pleural effusion, aspecific migratory non-deforming seronegative polyarthritis, sensorineural hearing loss and semicircular canal paresis. Whipple's disease (WD) had been diagnosed at the age of 63 years. On admission to hospital)he had weight loss, diarrhoea in combination with an organic, brain syndrome, hemiparesis and ophthalmoplegia, including internuclear ophthalmoplegia (INO). A clinical diagnosis of central nervous system (CNS) WD was made. MRI revealed a thalamus lesion that halved in size during sulfamethoxazole-trimethop:rim treatment. The organic brain syndrome and ophthalmoplegia diminished also, as did the cerebrospinal fluid (CSF) IgG level. A review of CNS WD is presented and implications for treatment are discussed.     

Primary central nervous system Hodgkin lymphoma: A case report and review of the literature

Central nervous system (CNS) involvement in the context of relapsed/refractory Hodgkin lymphoma (HL) is a quite rare, but well-known complication. Nevertheless, primary CNS–HL is an exceedingly rare condition, which diagnosis is based on well-defined morphological and immunohistochemical features, in addition to isolated involvement of the CNS.In spite of limited casuistry (just over twenty cases reported in the literature), available data agree that primary and isolated CNS–HL, when treated with a combination of surgery followed by some form of adjuvant therapy (radiotherapy ± chemotherapy), carries a better prognosis than those cases with CNS involvement in the context of relapsed/refractory HL or those with CNS non-Hodgkin lymphoma.We herein report a case of a 55-year-old female patient who was diagnosed with primary CNS–HL. The patient was treated with complete surgical resection followed by intrathecal chemotherapy and whole brain radiotherapy (WBRT), showing fourteen months of disease-free survival at the time of this case report. A review of the available literature is also presented.     

不典型性中枢神经系统Whipple病:一例报告并文献复习

目的结合文献探讨中枢神经系统Whipple病的诊断与治疗特点,以提高对该病的认识。方法回顾分析1例以头痛、左侧肢休无力,伴记忆力减退为首发症状的不典型性中枢神经系统Whipple病的临床诊断与治疗经过,并进行文献复习。结果女性患者,35岁。首发症状表现为头痛、肢体无力及记忆力减退,但不伴发热、癫癎发作。病程进展过程中相继出现阵发性四肢抽动、右侧下肢无力、小便失禁、多食、体质量增加、停经、体温波动,大剂量糖皮质激素及青露素、复方磺胺甲噁唑等抗炎药物治疗无效,随着颅内压逐渐升高,脑疝形成。腰椎穿刺脑脊液检测仅蛋广白定量显著升高。脑电图提示右侧前额颞区慢波。MRI呈以右侧大脑半球、额顶颢叶、半卵圆中心及基底节为主的大片长T₁、长T₂信号,并不均匀疏松团状强化,病灶周围水肿,占位效应明显,并累及左侧大脑半球。病理学检查呈现大片状坏死,脑组织及血管周围大量淋巴细胞和浆细胞浸润,伴大量格子细胞渗出,胞质丰富,内含大量六胺银和PAS染色阳性的细小颗粒状物质。排除中枢神经系统肿瘤、脱髓鞘病变及炎性假瘤等疾病。结论中枢神经系统Whipple病极为罕见,临床及影像学表现复杂多样,病理学检查仅能提示特殊感染,治疗困难,误诊率及病死率高。早期进行组织活检,结合临床表现及病理学特征可以明确诊断,经规范的抗生素治疗,患者可获得良好预后。     

原发性中枢神经系统血管炎一例报道及文献复习

患者女性,20岁,间歇性头痛3个月.于当地医院未经任何辅助检查予对症药物治疗,症状缓解.2007年12月6日再次头痛发作,伴喷射状呕吐.     

Isolated lymphomatoid granulomatosis of the central nervous system: A case report and literature review

Lymphomatoid granulomatosis (LYG) is an angiocentric and angiodestructive lymphoproliferative disease which can involve multiple organs of the body and is most common in the lungs. Its pathological features are proliferation of large atypical B‐cells related to Epstein–Barr virus, T‐cell infiltration and tissue necrosis. This disease is rare, and LYG which uniquely involves the central nervous system (CNS) is extremely rare. In this paper, we report a case of isolated lymphomatoid granulomatosis of the CNS (iCNS‐LYG) diagnosed by histological biopsy and we review the clinical features of all similar cases reported in the past 46 years. A total of 49 cases of iCNS‐LYG have been reported to date. The clinical, imaging and pathological features of iCNS‐LYG are discussed in combination with a literature review.     

原发性中枢神经系统非霍奇金淋巴瘤1例报道并文献复习

目的探讨原发性中枢神经系统非霍奇金淋巴瘤(PCNSL)的病因,诊断方法及治疗措施。方法结合文献回顾性分析我院收治的1例PCNSL患者的临床及影像学资料,进行诊断及鉴别诊断。结果病理活检明确诊断为PCNSL,患者依方案行化疗治疗,随访1年未见病变复发。结论 PCNSL是一种罕见的颅内肿瘤,发病隐匿,对放化疗敏感,临床需与多种颅内肿瘤相鉴别。     

中枢神经系统囊性海绵状血管瘤2例报告及文献复习

中枢神经系统海绵状血管瘤的人群发生率约为 0 .4 %～0 .8% [1] ,近年来随着MRI的广泛应用 ,本病检出率日渐增多。绝大多数海绵状血管瘤表现为实质性病变 ,极少见到囊性海绵状血管瘤 ,临床上罕有报道。我科在 1993～ 2 0 0 1年期间共收治颅内海绵状血管瘤患者 16 0例 ,其中 2例在影像学上表现为实质性肿块周围出现囊性病变 ,现报告如下。病例 1:男性 ,5 4岁 ,因头痛伴呕吐 3个月收住入院。体检未见明显阳性体征。MRI示 :第四脑室底部囊性占位 (图 1)。术中见一囊性病灶 ,囊液呈淡黄色 ,囊肿壁上可见一暗红色实质性肿块 ,呈圆型 ,大小约 2…     

瘤样原发性中枢神经系统血管炎的诊治分析（附6例报道并文献复习）

目的 探讨瘤样原发性中枢神经系统血管炎（TLP-PACNS）的诊断、治疗及预后,以提高对该病的认识。方法 回顾性分析2011年3月～2021年2月手术治疗的6例TLP-PACNS的临床资料,并对相关文献进行复习。结果 术前头颅MRI显示单发局限性病灶3例,考虑胶质瘤;多发病灶3例,考虑转移瘤。6例均行开颅病灶切除术,术后病理证实为PACNS。2例术后给予激素治疗后明显好转;1例多发病灶术后激素治疗后无变化,加用环磷酰胺后好转;3例单发病灶术后自然缓解;随访3个月～2年无复发。结论 TLP-PACNS是一种非常少见的疾病,临床表现和影像学表现缺乏特异性,术前易误诊,病理检查是诊断金标准,激素治疗是一种有效的方法。