麻痹性痴呆(附2例报告)

目的 探讨麻痹性痴呆（GPI）的临床表现、实验室检查、影像学改变和治疗。方法 回顾性分析2例GPI患者的临床资料结果2例患者均为男性,以记忆力下降和精神障碍为主要临床表现,1例出现内脏危象,血清及脑脊液快速血浆反应素环状卡片试验（RPR）、梅毒螺旋体明胶凝集试验（TPPA）均（＋）,MRI检查均有脑室扩大经驱梅治疗后,2例患者病情均明显好转。结论 GPI多见于中年男性,误诊率高,对不明原因的年轻痴呆患者,应想到GPI的可能,及时诊断、积极治疗对其预后有重要意义。     

麻痹性痴呆患者临床资料分析

目的：探讨麻痹性痴呆患者的临床表现、实验室检查、影像学特征，为早期诊断提供依据。方法对21例麻痹性痴呆患者的临床资料进行回顾性分析。结果本组患者均无自知力，并伴有精神病性症状、记忆力下降，部分患者伴有共济失调等症状；简易智力状态检查量表评分为（10＆#177;3）分，实验室检查梅毒螺旋体血凝试验及快速血浆反应素环状卡片试验均为阳性；其中13例患者行头颅C T检查，提示脑萎缩3例。结论麻痹性痴呆早期临床表现复杂多样，容易导致误诊误治，应详细询问病史，重视梅毒血清和脑脊液抗体检查，并结合影像学提高确诊率。     

脑脊液非螺旋体抗原试验对神经梅毒诊断效率系统评价

目的：评价脑脊液非螺旋体抗原试验对神经梅毒的诊断效率。方法采用 Cochrame 系统评价方法检索电子数据库中公开发表的脑脊液非螺旋体抗原试验（VDRL、RPR、TRUST）的研究文献及参考文献，制订纳入和排除标准，提取被纳入研究文献的数据，采用 MetaDisc1．4分析软件进行统计分析。结果6个研究符合纳入标准被纳入本次系统评价，含神经梅毒 CSF 样品225例，非梅毒患者 CSF 样品1224例。Spearman 相关系数为-0．03（P ＝0．957），各研究间不存在阈值效应。加权合并功效指标及95％置信区间（CI）分别为：敏感度0．773（0．713～0．826）、特异性0．988（0．980～0．993）、阳性似然比53．5（24．3～118．0）、阴性似然比0．233（0．180～0．296）、诊断优势比229．9（117．3～450．9）、综合受试者工作特征曲线下面积0．981及 Q 指数0．938。按方法分组进行 Meta 回归分析：RDOR＝1．07[95％CI ：0．19～5．96]。结论脑脊液非螺旋体抗原试验对神经梅毒有较高的诊断功效，RPR 和 TRUST 可替代 VDRL 用于脑脊液样品。     

A Case of Cerebral Gumma Presenting as Brain Tumor in a Human Immunodeficiency Virus (HIV)-Negative Patient

Syphilis, along with the recent increase of human immunodeficiency virus (HIV) patients, has also been on the rise. It has a broad spectrum of clinical manifestations, among which cerebral gumma is, a kind of neurosyphilis, however, it is rare and can be cured by penicillin. Thus, cerebral gumma needs to be differentially diagnosed from other brain masses that may be present in syphilis patients. We have experienced a case where the patient was first suspected of brain tumor, but confirmed by surgery to be cerebral gumma due to neurosyphilis. This is the first such case encountered in Korea, therefore, we report it here in. A 40-year old woman complaining of headaches was found to have a brain mass on her CT scans and MRI. Suspecting a brain Tumor, a resection was performed on the patient, and histological results revealed that the central portion of the mass contained necrotic material and the peripheral region was infiltrated with plasma cells. Warthin-Starry staining of the region revealed spirochetes, and the patient was thus diagnosed as brain gumma. Venereal Disease Research Laboratory (VDRL) of cerebrospinal fluid (CSF) was reactive. After an operation, penicillin-G at a daily dose of 24×10⁶ U was given for 10 days from post-operative day 10, and thereafter, the mass disappeared.     

Syphilis and HIV: a dangerous duo

HIV and syphilis affect similar patient groups and coinfection is common. All patients presenting with syphilis should be offered HIV testing and vice versa. Syphilis can enhance the transmission of HIV. Detection and treatment of syphilis can probably help to reduce HIV transmission. Syphilis may present with atypical features in the HIV-positive patient, for example, there is a higher rate of asymptomatic primary syphilis, and proportionately more HIV-positive patients present with secondary disease. Secondary infection may be more aggressive and there is an increased rate of early neurologic and ophthalmic involvement. Diagnosis is generally made with serology, but the clinician should be aware of the potential for false-negative serology in both primary and, less commonly, in secondary syphilis. All HIV-positive patients should be treated with a penicillin-based regimen, and alternative therapies should be used with caution. All HIV-positive patients should be considered for the evaluation of neurosyphilis. Relapse is a real concern and careful follow up is required. This review will explore the differences in clinical manifestations in HIV-coinfected individuals, and will discuss data to warrant different management in HIV-coinfected individuals.     

Downbeat nystagmus

Localizing significance: Downbeat nystagmus (DBN) was found in lesions in the posterior fossa close to the medullocervical (craniocervical) junction, of the floor of the 4th ventricle and of the cerebellum, especially of the cerebellar flocculi. The occurrence of DBN in a patient demands special differential-diagnostic considerations in Neuro-Ophthalmology. Both congenital and acquired diseases were found in patients with this special kind of nystagmus.

Clinical evaluation has proved a multifactorial cause of DBN. About one-third of the patients with this sign show an Arnold-Chiari malformation. In a cerebellar degeneration this vertical nystagmus type is not rare. It is essential to investigate all the family members to make diagnosis sure if a hereditary disease is suspected. Familial DBN due to spinocerebellar degeneration is one of the commonest causes of DBN. Hereditary downbeat nystagmus was described without additional neurological findings. Magnetic resonance imaging is the method of choice for investigating patients with DBN.

Clonazepam, prism therapy, or a Kestenbaum-type operation, or artificial retinal image stabilization can be helpful in patients who suffer from nystagmus-induced oscillopsia.

The mechanism of DBN remains controversial. The mechanism postulated for DBN supposes that it reflects imbalance of tonic vestibular control in the vertical canal system or, alternatively, that it results from defective smooth pursuit mechanisms in the vertical plane.

A large series of 62 patients with DBN showed that in about 40% the cause remained undiagnosed despite detailed neurological and neuroradiological investigations.     

Intravenous Ceftriaxone for Syphilitic Uveitis

Purpose: Report of clinical/multimodal imaging outcomes of patients with syphilitic uveitis alternatively treated with intravenous(IV) ceftriaxone, due to unavailability of penicillin G.

Methods: Chart review of all cases of syphilitic uveitis presenting to Hospital São Geraldo/HC-UFMG and treated with intravenous ceftriaxone, between January and August 2014. Clinical, serological and ophthalmological data were collected.

Results: Twelve consecutive patients with syphilitic uveitis receiving IV ceftriaxone were identified. All 24 eyes had active intraocular inflammation on clinical examination. All patients received IV ceftriaxone (2–4 g daily) for 14–21 days, supplemented with oral corticosteroid as needed in 9 patients (75%), after documented clinical response. Improvement in intraocular inflammation was seen in all 24 eyes, with median best-corrected visual acuity (BCVA) increasing from 20/50 to 20/20, after a mean follow-up of 5.3 months.

Conclusion: IV ceftriaxone may be an effective alternative for treatment of syphilitic uveitis, in the setting of unavailability of penicillin G.