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1.
Paracoccidioidomycosis is a systemic mycosis, endemic in South and Central America, that affects the central nervous system (CNS) in almost 10% of patients. Neurological involvement includes two different clinical forms: meningeal and granulomatous, also known as the pseudotumor form. Five patients with biopsy-proved systemic paracoccidioidomycosis and neurological complaints were studied by magnetic resonance imaging. CNS involvement was detected in all patients in the form of multiple round or lobulated lesions, predominantly hypointense on T2-weighted images and ring or nodular enhancement on post-gadolinium T1-weighted images. The lesions were distributed diffusely, with a slight predominance in the supratentorial compartment, although infratentorial lesions were also observed, mainly in the cerebellum. Hypointense lesions on T2-weighted images persisted in all 3 patients reexamined after treatment, whereas enhancing lesions on post-gadolinium T1-weighted images turned isointense in 2 patients. Magnetic resonance imaging is a sensitive method in documenting CNS paracoccidioidomycosis, most frequently as supratentorial and infratentorial multiple, round or lobulated hypointense lesions on T2-weighted images.  相似文献   

2.
Neurological manifestations of the oculodentodigital dysplasia syndrome   总被引:4,自引:0,他引:4  
Oculodentodigital dysplasia (ODDD) (MIM 164200) is a rare autosomal dominant inherited disorder affecting the development of the face, eyes, limbs and dentition. Neurological complications are thought to be occasional manifestations of the disorder. This report illustrates the neurological manifestations by a pedigree of two ODDD patients with spastic paraparesis, cerebral white matter hyperintensity and basal ganglia hypointensity. A systematic review of the English, French, German and Italian literature on ODDD is also provided to summarize the neurological manifestations of the disorder. 243 previously described ODDD cases presented a spectrum of neurological manifestation including spasticity (25), subcortical white matter lesions (9) and basal ganglia changes (6) on MRI. Additional findings consisted of gaze palsy and squinting (28), bladder and bowel disturbances (21), visual loss (20) and blindness (4), hearing loss (15), ataxia (11), nystagmus (9), muscle weakness (5) and paresthesias (3). Neurological manifestations, including spasticity associated with MRI changes, are an underrecognized feature in the ODDD phenotype. A clinical guide to the neurological manifestations of ODDD may assist in the assessment of patients with this condition. Received: 17 May 2001, Received in revised form: 26 September 2001, Accepted: 8 October 2001  相似文献   

3.
急性播散性脑脊髓炎12例临床分析   总被引:1,自引:0,他引:1  
目的探讨急性播散性脑脊髓炎临床特点及治疗原则。方法回顾1990年5月-2010年12月住院治疗且诊断明确的急性播散性脑脊髓炎患者临床资料,结合文献分析其临床表现、实验室检查及影像学特点。结果共12例患者符合入组条件,男性10例,女性2例,年龄6~69岁(中位年龄34.50岁)。其中9例发病前出现前驱感染症状如上呼吸道感染(6例)或无诱因发热(3例),以及诱发因素如麻疹疫苗接种史(1例)或麻疹病史(1例)。临床主要表现为发热,恶心、呕吐,头痛,神经系统症状与体征以脑神经受累(展神经常见),瘫痪(偏瘫、截瘫、四肢瘫),感觉异常,膀胱功能障碍[尿失禁和(或)尿潴留],脑膜刺激征阳性,以及不同程度意识障碍为主。大剂量糖皮质激素冲击疗法或人血丙种球蛋白静脉注射治疗有效。结论尽管急性播散性脑脊髓炎发病凶险,但早期明确诊断及鉴别诊断,及时治疗,可明显改善患者预后。  相似文献   

4.
Sixty patients (51 women/9 men) with diagnosis of SLE were studied for finding out the frequency of nervous system involvement in SLE, the time of of appearance of neurological involvement after diagnosis establishing, the coexistence of the antiphospholipid syndrome, and the character of changes in MR, CT and CSF. Nervous system involvement was found in 40 cases (67%), with 34 cases (56%) had involvement of the CNS, 6 patients (10%) had symptoms of peripheral nervous system dysfunction, and 3 (5%) had involvement of both systems. In 4 cases polineuropathy and transverse spinal cord lesion, and in 3 cases psychiatric symptoms were the first manifestations of SLE. Changes due to involvement of cerebral vessels (TIA, stroke) were observed in 20 patients (33%), and psychiatric symptoms in 16 cases (26.6%). No difference was found in the occurrence of stroke or TIA between SLE patients with and without antiphospholipid syndrome, and no correlations were noted between the presence of neurological or psychiatric symptoms and other SLE symptoms. CT scans demonstrated corticosubcortical atrophy in 28.3% of cases, while in MRI in T2 images small hyperintense lesions were situated mainly in the white matter (33.9%). In 5 cases oligoclonal band was found in the CSF, but without any correlation with specific neurological symptoms.  相似文献   

5.
Beh?et's disease (BD), a systemic vasculitis of unknown cause, affects many organs and systems. Neurological involvement is seen in 5-15% of the patients, and the two major forms of neurological disease seen in BD are central nervous system (CNS) parenchymal involvement and cerebral venous sinus thrombosis. We report a 14-year-old boy with BD who had neuro-parenchymal involvement. The diagnosis of the systemic disease was not made until the onset of the neurological manifestations, which led to an MRI study that revealed findings suggestive of CNS involvement of BD. We therefore emphasize the importance of the localization and appearance of other characteristics of the lesions on MRI in the differential diagnosis of parenchymal neuro-Beh?et syndrome.  相似文献   

6.
Erdheim–Chester disease (ECD) is a rare disorder characterized by accumulation of non‐Langerhans cell histiocytes in multiple organs. The clinical manifestations are protean and vary from asymptomatic focal disease to potentially fatal multisystem disorder. The commonest presentation is symmetric osterosclerotic lesions of lower extremity long bones; other organs, including cardiovascular, nervous, and endocrine system may be affected. Central nervous system involvement can occur in up to 50% cases and is associated with poor prognosis. The disease pathogenesis involves organ involvement secondary to histiocytic infiltration and systemic inflammation driven by Th1 cytokine activation. The recent discovery of activating mutations in proto‐oncogene B‐rapidly accelerated fibrosarcoma (BRAF) V600E and other genes involved in mitogen‐activated protein kinase (MAPK) pathways has led to redefinition of ECD as a myeloid neoplastic disorder. The diagnosis requires histochemical and molecular analysis of histiocytes in tissue biopsies in patients with compatible clinical and imaging features. The treatment options include interferon‐alpha, anakinra, and immunosuppressive therapies. Better understanding of disease pathogenesis has led to development of novel targeted and effective therapies including BRAF and MEK inhibitors. The rarity of the disease and variable clinical features and course often results in diagnostic errors and delays. Rare primary neurological presentation can occur mimicking CNS inflammatory, neoplastic, or demyelinating disorders. We report an unusual case of ECD presenting with progressive encephalopathy and ataxia along with multifocal brainstem and cerebellar lesions. A comprehensive review of clinical and neuroimaging features and immunohistochemical and molecular characteristic of ECD are presented along with review of neuroimaging findings in two previously reported cases.  相似文献   

7.
OBJECTIVES: Accurate prediction of outcome in acute stroke would help in identifying subgroups of patients for therapeutic trials and intravenous thrombolysis. The purpose of this study was to prospectively test the hypothesis that brain SPECT, with (99m)Tc-L, L-ethylcysteinate dimer (ECD), a tracer sensitive to cell function, performed in the first hours after stroke onset, adds predictive power to concomitant neurological evaluation. METHODS: Twenty-four patients with a first-ever middle cerebral artery stroke were prospectively studied with ECD-SPECT within 12 h after stroke onset. Neurological evaluation was performed using Orgogozo's scale at admission and 3 months later in order to calculate the percent Martinez-Vila evolution indices (EI%). Semiquantitative visual analysis of SPECT images was performed in 6 cortical regions relevant for carotid artery territory. Both the extent and the intensity of cortical reduced ECD uptake were calculated, leading to an 'ischemia' score, corresponding to the sum of regions of interest (ROI) where ECD uptake was between 40 and 80% of the contralateral healthy hemisphere, and an 'irreversibly damaged tissue' (IDT) score, corresponding to an uptake below 40%, and a total score (ischemia + IDT). Each patient was assigned to one of three patterns: (1) pattern I with severe ECD cortical uptake reduction defined by at least one ROI with uptake under 40%, (2) pattern II with moderate ECD cortical uptake reduction (40-80%) only and (3) pattern III with normal ECD uptake. RESULTS: There were 11 patients (46%) with pattern I ECD-SPECT. This group had almost invariably (10/11 patients) a poor outcome. The 12 patients (50%) classified in pattern II had a variable clinical outcome, ranging from improvement to deterioration. The single patient with a normal SPECT (pattern III) had a full clinical recovery. Both total score and IDT score were strongly significantly correlated with neurological recovery EI% (respectively p = 0.006 and 0.004). Their predictive value was significantly higher than, and independent of, day 0 neurological evaluation. No patient had an increased ECD uptake. CONCLUSION: Our results show that the degree of ECD cortical uptake reduction, measured on early brain SPECT, is a strong predictor of neurological recovery. ECD-SPECT data have a higher predictive value than day 0 neurological evaluation. The apparently better predictive value of ECD over hexamethylpropyleneamine oxime may reflect this tracer's brain retention mechanisms which are weighted more towards cell function than towards perfusion. ECD-SPECT is easily obtainable and may help in selecting out from therapy those patients who are likely to have either very good or very poor spontaneous outcome, and thus improve the assessment of acute stroke and the choice of therapeutic strategy.  相似文献   

8.
Abstract Adamantiades-Beh?et disease (ABD) is characterised by oral and genital ulcerations, skin lesions and ocular manifestations and, rarely, by central nervous system (CNS) involvement. Neuro-Beh?et disease (NBD) is categorised to parenchymal or non-parenchymal, while combined CNS disease is rarely reported in the literature. A case of NBD, with severe relapsing ocular and neurological disease of combined pattern is presented. Neurological complications included brainstem manifestations, as well as neurovascular involvement, while ocular involvement consisted of bilateral uveitis and branch retinal vein occlusion. Manifestations responded to corticosteroid plus cyclophosphamide pulse therapy. Maintenance therapy included cyclosporine A, azathioprine and corticosteroids. Case individualities are discussed, focusing on scepticism concerning treatment of NBD relapses in the long term.  相似文献   

9.
目的探讨韦格纳肉芽肿病的神经系统损害特点。方法回顾分析33例韦格纳肉芽肿病患者的临床特点。结果33例患者中16例累及神经系统,主要临床表现为:痫性发作2例;轻偏瘫1例;脑神经损害7例,其中多脑神经损害3例;脊神经损害4例,其中多发性单神经病3例,远端对称性感觉神经病1例;风湿性多肌痛2例。1例头MRI显示眶内浸润性病变,2例头MRI和DSA显示病变浸润颅底组织,均累及颈内动脉,DSA证实颈内动脉颅内段狭窄,其中1例累及垂体,并伴有脑梗死,另外1例证实伴有静脉窦血栓形成。9例鼻腔黏膜或皮肤、肾活检病理显示坏死性肉芽肿性小血管炎,11例血清胞质型抗中性粒细胞抗体(C-ANCA)阳性。激素及免疫抑制剂治疗后病情可缓解。结论韦格纳肉芽肿病可累及中枢神经系统、周围神经系统和肌肉,神经系统损害可为疾病的首发表现,血清C-ANCA检测和组织学检查有助于诊断。  相似文献   

10.
IntroductionNeurological manifestations of Behçet disease (BD) are polymorphic and serious. The purpose of this study was to analyze the clinical patterns and outcome of neurological involvement in BD.MethodsThe medical records of patients with neurological manifestations of BD were reviewed retrospectively. All patients fulfilled the International Study Group Criteria for the Diagnosis of BD. Patients with headache and normal findings (neurological examination, cerebrospinal fluid, computed tomography scan, magnetic resonance imaging) were excluded.ResultsSixty-seven patients had clinical evidence of neurological involvement. There were 53 men and 14 women. A male/female sex ratio was 3.78. The average age of onset of neurological involvement was 31.5. The meningoparenchymal (MP) central nervous system involvement was found in 83.58%. The most common findings were pyramidal signs. Eleven patients (16.41%) without parenchymal central nerouvous system involvement were noted. In this group, there were six cases of intracranial thrombosis, one case of intracranial aneurysm and four cases of idiopathic intracranial hypertension. Other clinical features were reported: extrapyramidal signs and isolated spinal cord involvement. The course of disease was favorable in 70% of cases, and unfavorable in 30%.ConclusionClinical patterns of neurological involvement in BD are various and particularly serious in the MP group.  相似文献   

11.
Beh?et's disease (BD) is a chronic relapsing multisystem disorder. While most frequently occurring around the Mediterranean and in Japan, isolated cases of BD have been reported in Africa south of the Sahara and in the Caribbean. The aim of this study was to describe our experience of BD in Guadeloupe (French West Indies) where the presence of the disease has not been reported previously. We analysed retrospectively the charts, and clinical and imaging features of patients native to Guadeloupe who were diagnosed with neurological manifestations of BD between 1989 and 1999. In our series of 13 cases, seven had neurological involvement. Neurological manifestations included meningoencephalitis or meningoencephalomyelitis in four cases, cerebral venous thrombosis in one case and peripheral neuropathy in two cases associated with myositis in one. Patients received treatment with colchicine (n=7), corticosteroids (n=6), immunosuppressive therapy (azathioprine and/or cyclophosphamide; n=4), acetylsalicylic acid (n=2) and oral anticoagulation for venous thrombosis (n=1). Long-term sequelae occurred only in patients with recurrent neurological disease. This study suggests that the frequency of BD in this Afro-Caribbean population is higher than this reported in Caucasian populations. Meningoencephalitis is associated with a poor prognosis while other patients achieved recovery.  相似文献   

12.
Comparison of MRI, EEG, EPs and ECD-SPECT in Wilson's disease   总被引:3,自引:0,他引:3  
OBJECTIVES: The purpose of this study is to evaluate the efficiency of a few methodologies in detecting anatomo-functional brain abnormalities in patients with Wilson's disease. MATERIALS AND METHODS: Twenty-three patients with Wilson's disease underwent almost simultaneously brain magnetic resonance imaging (MRI), computerized electroencephalography (EEG), multimodal evoked potentials (EPs) and ECD single photon computerized tomography (SPECT) evaluation. The clinical picture was of the neurologic type in 8 patients and of the hepatic type in 15. RESULTS: MRI was abnormal in 7 patients with neurological manifestations. The EPs proved pathologic in 7 neurologically symptomatic patients and in 4 cases with hepatic form. These results agree with those reported in other case studies. The EEG records were abnormal only in 3 cases. Nevertheless, the most interesting finding of this study is the particular frequency (86%) of diffuse or focal decrease of ECD uptake shown by brain SPECT. CONCLUSION: We highlight the use of this interesting procedure in the therapeutic monitoring of this disease.  相似文献   

13.
目的探讨儿童急性播散性脑脊髓炎(ADEM)临床特点和预后。方法回顾性分析66例ADEM患儿的临床资料。应用Kurtzke扩展残疾状况量表(EDSS)评估入院时神经功能损伤情况,应用mRS评估最后一次随访时神经功能恢复情况,并分组比较其临床资料。结果66例ADEM患儿中42例(63.6%)有前驱感染史,5例(7.6%)发病于疫苗接种后。所有患儿均有脑病表现,其中有意识障碍50例(75.8%)、认知障碍9例(13.6%)、精神症状6例(9.1%)、认知障碍合并精神症状1例(1.5%)。其他临床症状包括发热46例(69.7%),锥体束征33例(50.0%),惊厥32例(48.5%),头痛27例(40.1%),共济失调26例(39.4%),言语障碍14例(21.2%),颅神经受累11例(16.7%)(面神经受累7例,展神经受累1例,视神经受累2例,面神经合并视神经1例),锥体外系症状9例(13.6%),感觉异常7例(10.6%)。MRI病灶以皮质下白质(84.8%)、深部核团(36.4%)、脑干(16.7%)及小脑(15.2%)多见,脊髓受累(7.6%)较少。不同神经功能损伤组临床资料差异无统计学意义(均P>0.05)。56例给予大剂量甲泼尼龙治疗,10例给予地塞米松治疗;两种糖皮质激素治疗方案患者最后一次随访mRS评分差异无统计学意义(t=1.47,P=0.15)。康复组、好转组及恢复不佳组年龄(F=4.47,P=0.02)、入院EDSS评分(F=38.62,P<0.01)及惊厥(χ^2=9.64,P=0.01)、言语障碍(χ^2=17.64,P<0.01)及认知障碍(χ^2=24.15,P<0.01)比率差异有统计学意义。结论儿童ADEM多有前驱感染史或疫苗接种史。临床表现多样,以脑病最为常见。MRI对诊断具有重要意义。多数患儿经糖皮质激素治疗后预后良好,预后可能与发病年龄、入院时EDSS、发病时有无皮质受累有关。  相似文献   

14.
Summary Neurological manifestations were analysed in 13 patients with left atrial myxoma confirmed by histological study. In this group, 6 patients had neurological complaints at initial presentation, 5 had cardiac symptoms, and 4 patients presented with signs of systemic disease. CT was performed in 10 and MRI in 7 patients. There were signs of ischaemic or haemorrhagic infarction (6 of 12) and one lesion suggestive of myxoma metastasis; other findings mainly comprised non-specific white matter lesions revealed by MRI. Neurological follow-up 0.5–8 years after tumour resection revealed no delayed neurological complications, follow-up CT and MRI findings were not suggestive of lesions acquired after operation. It is suggested that this rare condition of cardiac myxoma should be suspected in young patients with CNS symptoms or signs lacking cerebrovascular or cardiovascular risk factors and should be included in the differential diagnosis of vasculitis. With timely diagnosis and resection of the cardiac tumour the long-term prognosis can be considered improved.  相似文献   

15.
The aim of this study was to assess the underlying causes of isolated cranial nerve (CN) manifestations in cancer patients. In a prospective study over a 16 month period, 20 of 242 patients presented with isolated CN manifestations, and were assessed for the following: primary site; CN symptoms and signs; length of time between primary diagnosis and neurological involvement; and survival following the neurological diagnosis. The most frequent primary site was determined as hematological malignancy (9/20). Thirteen of the 20 patients presented with single CN manifestations. Underlying causes of CN manifestations in 16 of the 20 patients were related to metastases. They included meningeal carcinomatosis (10/16), brain stem metastases (3/16), primary brain astrocytomas (1/16), and metastases out of the central nervous system (2/16). The remaining four patients' CN manifestations related to non-metastatic conditions. Although most of the isolated CN manifestations were due to systemic metastasis, in particular to the meninges, up to 20% were related to benign conditions.  相似文献   

16.
Primary HIV-1 infection is a relevant period for its virological and epidemiological consequences. Most patients present a symptomatic disease that can be potentially serious, but neurological involvement during primary HIV-1 infection has been poorly studied. The aim of this study was to describe the characteristics and outcomes of primary HIV-1 infection patients presenting neurological symptoms and to compare them with primary HIV-1 infection patients without neurological involvement. Retrospective case-control study (1:3) comparing primary HIV-1 infection patients with and without neurological involvement enrolled in the Acute/Recent Hospital Clinic PHI Cohort between 1997 and 2016. Matching criteria included age (±10 years), gender, year of diagnosis (±4 years), and Fiebig stage. The conditional logit model was used for comparisons. Fourteen out of 463 patients (3.02%) enrolled in the Acute/Recent Hospital Clinic PHI Cohort between 1997 and 2016 presented neurological symptoms. 28.5% of cases presented as meningitis and 71.5% as meningoencephalitis. Cerebrospinal fluid showed non-specific findings, including pleocytosis with lymphocyte predominance and increased protein levels. All cases required hospitalisation, whereas only 19% of the controls did. No other pathogen was identified in any case, but five patients initiated empirically antimicrobial treatment for other aetiologies until diagnosis was confirmed. CD4/CD8 ratio was significantly lower (p = 0.039) and plasmatic viral load significantly higher in the case group, compared to controls (p = 0.028). Risk factors, HIV-1 tropism, subtype distribution, and prescribed ART regimens were comparable between cases and controls. After 6 months on ART, 92% of cases had undetectable viral load, similar to controls, and CD4/CD8 ratio became also comparable between groups. All cases recovered rapidly with ART and were discharged without sequels. Neurological involvement during primary HIV-1 infection is unusual but serious, always requiring hospitalisation. Diagnosis is difficult because of the wide range of symptoms and similarities with other viral aetiologies. Neurological manifestations during primary HIV-1 infection are associated with a lower CD4/CD8 ratio and with a higher viral load than controls. Immediate ART initiation and rapid viral load decrease are required, allowing complete clinical recovery.  相似文献   

17.
INTRODUCTION: Identifying tumor infiltration or compression in patients with non-Hodgkin's malignant lymphoma presenting peripheral neuropathy can be a difficult task. METHODS: We collected a series of patients with peripheral neuropathy with demonstrated lymphomatous infiltration or compression managed between October 1977 and October 2001 to search for clinico-pathological correlations. RESULTS: Ten cases were reviewed. Neurological manifestations were the inaugural symptom of the disease in 7 patients. Clinical presentations included 5 focal (3 cranial nerve palsies, 2 brachial radiculopathies) and 5 diffuse neuropathies (3 polyradiculoneuropathies, 1 polyneuropathy and 1 mononeuritis multiplex). The mechanisms of peripheral nerve involvement were classified into lymphomatous meningoradiculitis (5 cases), involvement of cranial nerves or spinal roots in their extraneuraxial course (3 cases) and infiltration of distal peripheral nerves (2 cases). Four long lasting survivals after treatment were observed. CONCLUSIONS: Prognosis depends much more on the haematological disease than on the neurological symptoms or tumor location.  相似文献   

18.
OBJECTIVES: To determine the prevalence of neurological involvement in Behcet's disease in a prospective study, and to describe the clinical patterns of neurological presentation in this disease in patients attending a multidisciplinary clinic in Baghdad. METHODS: All patients attending the clinic who fulfilled the international study group criteria for the diagnosis of Behcet's disease were studied during a two year period starting in April 1999. Patients were assessed neurologically by a neuro-Behcetologist. All those with clinical neurological manifestations were sent for CSF examination, cranial magnetic resonance imaging, and magnetic resonance venography and were followed up to explore the patterns of neurological relapse. RESULTS: 140 patients with Behcet's disease were studied. Their mean age was 34.2 years (range 16 to 66); 105 (75%) were men and 35 (25%) were women. The mean duration of the disease was 4.2 years (range 0.4 to 26). Twenty patients (14%) had neurological involvement (neuro-Behcet's disease); 14 of these (70%) were men and six (30%) women. The mean age at the first neurological presentation was 34.1 years. The mean duration of follow up of patients with neuro-Behcet's disease was 20.7 months. Ten patients with neuro-Behcet's disease (50%) presented with parenchymal CNS involvement, six (30%) with intracranial hypertension, and four (20%) with a mixed pattern of both parenchymal CNS involvement and intracranial hypertension. CONCLUSIONS: Careful neurological assessment of patients with Behcet's disease may show a relatively high prevalence of neuro-Behcet features, and though the clinical patterns of presentation are characteristic a mixed pattern may occur.  相似文献   

19.
Cranial neuropathies were present in 5 patients with positive serology for the human immunodeficiency virus. Two patients presented with abnormalities of ocular movements (3rd, 4th), two with an isolated unilateral facial nerve palsy and one with a lesion of the accessory nerve. Neurological symptoms and signs are present in approximately 60 to 80 p. 100 of cases of the acquired immunodeficiency syndrome, but cranial neuropathies affect only 2 to 3 p. 100 of the patients. The isolation of the human immunodeficiency virus from the nerve suggest a direct role, but an indirect immune mechanism may also be present. Some of the patients with aseptic meningitis or subacute encephalopathy have demonstrated involvement of cranial nerves, mainly 2nd, 7th and 8th. Systemic tumors (lymphoma) may involve the central nervous system by diffuse meningeal invasion with lesions of 3rd, 4th and 6th nerves. Opportunist infections like Herpes zoster or Cytomegalovirus may produce cranial neuropathies (2nd, 5th). Isolated mononeuropathies or cranial nerves palsies have also been reported in patients treated with chemotherapeutic agents like vinca alkaloids.  相似文献   

20.
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