Magnetic resonance imaging of the spinal cord in a man with tabes dorsalis

Abstract

Background

Tabes dorsalis is a late manifestation of untreated syphilis that is characterized by ataxia, lancinating pains, and urinary incontinence. A form of tertiary syphilis or neurosyphilis, it is the result of slow, progressive degeneration of the nerve cells in the spinal cord.

Method

Case report.

Findings

A 39-year-old man presented with tingling paresthesia in the lower limbs, difficulty in walking, and loss of vision. Magnetic resonance imaging (MRI) of the dorsal spine showed intramedullary hyperintensity and cord atrophy, similar to changes seen in subacute combined degeneration. MRI features of tabes dorsalis have not been described previously to the best of our knowledge.

Conclusion

MRI findings in this patient with tabes dorsalis were similar to those seen in subacute combined degeneration, which is characterized predominantly by cord atrophy and intramedullary hyperintensities.     

Voiding dysfunction due to neurosyphilis

Three patients with neurosyphilis presenting with urinary frequency, incontinence and voiding dysfunction were investigated. Unlike the previously reported finding of areflexia in tabes dorsalis, all 3 had hypocompliant detrusor hyper-reflexia with detrusor-sphincter dyssynergia and post-micturition residual urine. One patient also had bladder neck dyssynergia treated by bladder neck incision. The other 2 patients were initially managed by intermittent catheterisation but 1 ultimately underwent urinary diversion. The clinical relevance of these findings and the treatment of this condition are discussed.     

Magnetic resonance imaging of the spinal cord in a man with tabes dorsalis

Background

Tabes dorsalis is a late manifestation of untreated syphilis that is characterized by ataxia, lancinating pains, and urinary incontinence. A form of tertiary syphilis or neurosyphilis, it is the result of slow, progressive degeneration of the nerve cells in the spinal cord.

Method

Case report.

Findings

A 39-year-old man presented with tingling paresthesia in the lower limbs, difficulty in walking, and loss of vision. Magnetic resonance imaging (MRI) of the dorsal spine showed intramedullary hyperintensity and cord atrophy, similar to changes seen in subacute combined degeneration. MRI features of tabes dorsalis have not been described previously to the best of our knowledge.

Conclusion

MRI findings in this patient with tabes dorsalis were similar to those seen in subacute combined degeneration, which is characterized predominantly by cord atrophy and intramedullary hyperintensities.     

脑脊液寡克隆区带在神经梅毒诊断中的临床意义

目的探讨脑脊液寡克隆区带（OB）检测在神经梅毒患者诊断中的临床意义。 方法收集2018年6月至2018年12月于首都医科大学附属北京地坛医院初诊、未经治疗的HIV阴性梅毒患者共163例。入组患者跟根据诊断结果分为隐性梅毒组（39例）、无症状神经梅毒组（43例）和有症状神经梅毒组（81例）。应用等电聚焦电泳联合免疫固定法检测入组患者脑脊液及血清OB，并判定脑脊液OB阳性结果。比较隐性梅毒和神经梅毒患者OB阳性率的差异，同时分析OB阳性和阴性神经梅毒患者的脑脊液生化、常规指标的差异，并通过ROC曲线评估脑脊液OB诊断神经梅毒的效能。 结果入组163例梅毒患者中97例脑脊液OB阳性。隐性梅毒组、无症状神经梅毒组和有症状神经梅毒组患者脑脊液OB阳性率分别为12.8%（5/39）、65.1%（28/43）和79.0%（64/81）。有症状[64（79.0%）]及无症状神经梅毒[28（65.1%）]患者脑脊液OB阳性率显著高于隐性梅毒患者[5（12.8%）]，差异有统计学意义（Bonferroni校正，P < 0.0167）。有症状神经梅毒亚型的OB阳性率分别为脊髓痨93.3%（14/15），麻痹性痴呆89.5%（34/38），脑膜血管型81.8%（9/11）和眼梅毒41.2%（7/17）。以眼梅毒OB阳性率最低，且与麻痹性痴呆、脊髓痨间差异有统计学意义（Bonferroni校正，P < 0.008）。晚期神经梅毒患者OB阳性率（90.6%、48/53）显著高于早期神经梅毒（68.5%、37/54）患者，差异有统计学意义（χ² = 7.96、P = 0.005）。OB阳性神经梅毒患者IgG指数及脑脊液白细胞、IgG、IgG合成率、总蛋白较OB阴性患者显著升高。脑脊液OB阳性诊断神经梅毒的特异性和灵敏度分别为87.2%和74.2%，与本研究采用的神经梅毒诊断"金标准"一致率为78.6%，ROC曲线下面积为0.81。 结论与隐性梅毒相比，神经梅毒患者脑脊液OB阳性率显著升高，提示脑脊液OB可能对神经梅毒有一定的辅助诊断价值。脑脊液OB能够反映中枢神经系统体液免疫，为脑脊液OB检测在神经梅毒诊断中的应用提供一定的客观依据。     

Case Report: Neuropathic Arthropathy of the Hip as a Sequela of Undiagnosed Tertiary Syphilis

Background  

Neuropathic arthropathy is characterized by rapidly progressive bone destruction in the setting of impaired nociceptive and proprioceptive innervation to the involved joint. It is seen most commonly in the foot and ankle, secondary to peripheral neuropathy in patients with diabetes mellitus. Other less common sites of involvement may include the knee, hip, shoulder, and spine, depending on the underlying etiology. Neuropathic arthropathy can be associated with tabes dorsalis, a unique manifestation of late, tertiary neurosyphilis that may arise in individuals with untreated syphilis many years after initial infection, and usually involves the knee, or less commonly, the hip.     

Treatment of neurosyphilis

Opinion statement In 2001, the incidence of primary and secondary syphilis increased in the United States for the first time in a decade. Increasing rates of early syphilis among men who have sex with men have been reported in many American cities, with similar outbreaks noted in Canada and Europe. In San Francisco, the increase has been particularly sharp and accompanied by an increase in the incidence of neurosyphilis. Early neurosyphilis develops within weeks to years of primary infection and primarily involves the meninges. Syndromes include syphilitic meningitis (often accompanied by cranial neuropathies), meningovascular syphilis (with associated ischemic stroke), or asymptomatic neurosyphilis. Late neurosyphilis occurs years to decades after exposure as cerebral or spinal gummatous disease or the classic parenchymal forms affecting the brain (general paresis or syphilitic encephalitis) or spinal cord and nerve roots (tabes dorsalis). Treponema pallidum, the causative agent, cannot be cultured in vitro, and microscopic techniques are laborious. Thus, diagnosis depends on serologic tests and cerebrospinal fluid (CSF) examination. The suboptimal sensitivity and specificity of these tests complicate diagnosis, particularly among patients coinfected with HIV. CSF examination should be performed to evaluate for neurosyphilis in all patients with positive serum syphilis serology and neurologic, ophthalmic, or tertiary disease, or in those who have failed therapy, and in HIVinfected patients with late latent syphilis or syphilis of unknown duration. Intravenous penicillin G is the recommended treatment for all forms of neurosyphilis and for syphilitic eye disease. An outpatient alternative, if adherence can be assured, is intramuscular benzathine penicillin with oral probenecid. Newer drugs that penetrate CSF, such as ceftriaxone or azithromycin, have not yet been adequately tested for neurosyphilis. Syphilis facilitates transmission of HIV (and vice versa), and thus all patients diagnosed with syphilis should be offered HIV testing.     

抗-HIV阴性神经梅毒患者的临床特点与实验室检测分析

目的分析抗-HIV阴性神经梅毒患者的临床表现、影像学检查和实验室指标检测结果,以提高对神经梅毒患者的认知和诊疗水平。 方法回顾性分析2014年1月至2015年10月本院收治的20例抗-HIV阴性神经梅毒患者的临床症状、体征,影像学（MRI、CT）检查等临床资料和实验室检查结果。数据资料统计学分析采用SPSS 20.0软件。 结果20例神经梅毒患者中男女比例4︰1,年龄29～78岁,平均年龄（52.6±12.4）岁。其中梅毒性脑膜炎型患者1例（5%）,脑膜血管型患者8例（40%）,麻痹性痴呆型患者8例（40%）,脊髓痨型患者2例（10%）,麻痹性痴呆合并脊髓痨型患者1例（5%）;有明确梅毒史患者6例（30%）,首诊误诊率高达70%。所有患者梅毒血清学检测梅毒螺旋体明胶颗粒凝集试验（TPPA）和快速血浆反应素（RPR）均为阳性（1:2～1:128）,脑脊液TPPA均阳性,RPR阳性17例（85%）（1:2～1:16）。脑脊液白细胞> 10 × 10⁶/L患者18例（90%）,均为单核细胞升高;蛋白升高患者17例（85%）,葡萄糖异常患者15例（75%）,氯化物水平和脑脊液压力均正常;17例行脑脊液IgG检测16例患者升高（94.12%）;15例患者脑脊液IgG寡克隆带检测13例阳性（86.67%）;13例患者中CSF-24 h IgG合成率升高者12例（92.31%）。各临床型患者脑脊液实验室检测结果差异无统计学意义（χ²= 1.63、P = 0.95）。 结论神经梅毒以壮年男性多见,临床表现多样,是易误诊的可治性疾病,血清及脑脊液梅毒检查对指导早期诊疗和预后具有重要价值。     

Neurogenic bladder and the lumbar spine

From a large number of patients with urologic symptoms, 8 were selected in six years as having “true neurogenic bladder” with chronic and mild symptoms in the lumbar spine. Symptoms and neurologic and radiologic findings in the preoperative period are described. The type of urologic evaluation is briefly presented with characteristic examples. The postoperative results of these and other series are presented and questioned because of the lack of improvement on urodynamic studies in patients deemed better clinically. The need for a complete evaluation with more sophisticated urologic tests before and after surgery is stressed before a final decision regarding the relation between neurogenic bladder and “asymptomatic” cauda equina compression can be reached.     

Disorders of micturition in tabes dorsalis

The aim of this study was to determine whether the disorders of micturition that occur in patients with tabes dorsalis can be attributed exclusively to disturbed bladder sensation, as has been previously suggested. Urodynamic studies were performed in 8 patients with tabes dorsalis who had no obvious organic obstructive urological disease. The results indicated that not only disturbed bladder sensation but also suprasacral and/or sacral lesions innervating the detrusor could be responsible for voiding dysfunction in such patients.     

Spinal Charcot arthropathy

Charcot joints of the spine are well-documented clinical entities most commonly associated with tabes dorsalis. Spinal neuropathic joints, however, may be produced by other disease processes including syringomyelia. In this review, the authors discuss the cause and treatment of spinal Charcot arthropathy with emphasis on surgical therapy and results.     

Shoulder arthropathy (Charcot joint) and syringomyelia: report of two patients

Neuropathic arthropathy of the shoulder is a relatively rare disorder and is mainly caused by tabes dorsalis and syringomyelia. Sensory deficit has been implicated as its causative factor. It is frequently misdiagnosed because its early symptoms may suggest a mild infection, minor fracture, tendon rupture, or a similar benign problem. The authors describe two patients with this disorder affecting the shoulder that initially presented a diagnostic dilemma. The two patients were initially misdiagnosed until X-Rays revealed destruction of the shoulder joint with marked resorption of the humeral head, and magnetic resonance imaging revealed a syrinx in the central cord associated in one patient with Arnold-Chiari malformation.     

The effect of rectosigmoidectomy and Duhamel-type pull-through procedure on lower urinary tract function in children with Hirschsprung's disease

BACKGROUND/PURPOSE: The aim of this study was to investigate the effect of rectosigmoidectomy and Duhamel-type pull-through procedure on lower urinary tract function in children with Hirschsprungs disease. METHODS: During a 3-year period the authors assessed 11 consecutive children with Hirschsprung's disease prospectively by standard urodynamic investigations, before and after surgery. Urodynamics included simultaneous measurement of abdominal pressure, bladder pressure, detrusor pressure, and pelvic floor electromyography during filling and voiding. All children were submitted to laparoscopic resection of the aganglionic bowel segment below the cul de sac and a Duhamel-type pull-through procedure. Postoperatively, the children were assessed urodynamically and evaluated every 3 months for urologic problems. RESULTS: Mean age at first urodynamic study was 5 months (range, 2 to 10). Postoperative urodynamics were performed at a mean age of 10 months (range, 5 to 159). The mean interval between operation and postoperative urodynamic study was 6 months (range, 2 to 10). No child had structural urologic anomalies or urologic problems before surgery, and all had normal preoperative urodynamic findings. After surgery, urodynamics were considered normal in 3 children. In 7 children cystometric bladder capacity (CBC) was abnormally large, and 6 of these children had significant residuals. However, all had detrusor contractility and were able to void spontaneously. One child had low bladder compliance postoperatively. Despite the urodynamic changes, no child had clinical urologic problems at further follow-up. Mean follow-up after surgery was 24 months. CONCLUSIONS: This study found that after rectosigmoidectomy below the cul de sac alterations of bladder function can be observed. In 7 of the 11 patients studied, mean cystometric bladder capacity was 87% higher than capacity estimated for age. Moreover, postoperative residuals were 156% higher than the preoperative values. These findings suggest that partial detrusor denervation is likely in these patients. However, because detrusor contractility was present, and none of the children had retention or any urologic problems, the findings must be interpreted carefully. Because children with Hirschsprung's disease generally do not have preexisting urologic problems, routine preoperative urodynamic screening is not necessary. However, children with voiding problems after operation should be investigated urodynamically. For legal reasons parents should be informed of possible urologic problems, especially if subtotal resection of the aganglionic bowel segment is planned.     

Charcot-Marie-Tooth disease presenting as a nonhealing ulcer in a 26-year-old man

A 26-year-old man presented with a nonhealing ulcer on the plantar aspect of the left foot of five years duration. Initial investigations were unremarkable. It was only after careful neurological examination that an inherited neuropathy was suspected. This was confirmed by nerve conduction studies and serum electrophoresis. He subsequently underwent partial great toe amputation for the ulcer and underlying first phalangeal osteomyelitis with uneventful healing. Neuropathic ulcers are usually associated with several well-known disorders including diabetes mellitus, tabes dorsalis, pernicious anemia, and sickle cell disease. A rarer cause is Charcot-Marie-Tooth Disease (CMTD). The report gives a review of CMTD and emphasizes that when faced with a nonhealing ulcer in the younger age group, such an underlying hereditary neuropathic cause must be considered.     

Severe lightning pain during spinal anesthesia

A 74-yr-old man was scheduled for the biopsy of prostatic tumor under spinal anesthesia. Preoperative serologic test for syphilis was highly positive. Spinal anesthesia was performed in the sitting position, and 2 ml of hyperbaric Neo-percamine S (a mixture of 0.24% dibucaine and 0.12% T-caine) was administered uneventfully with the onset of warm sensation on the perineal region. Ten minutes later, however, he began to complain of severe lightning sensation on the feet. After giving pentazocine intravenously, the short cystoscopic procedure was completed. As a cause of severe pain, an erroneous anesthetic solution or direct neuronal injury had been excluded because of rapid and complete recovery after anesthesia. Two weeks later, he was scheduled for TUR-P. He again complained of severe lightning pain after the successful spinal anesthesia with the same anesthetic solution. Because the pain was not relieved by analgesics, he was then anesthetized with enflurane and N2O in oxygen, and there were no neurological complications after anesthesia. Several cases of severe pain during spinal anesthesia have been reported in patients with tabes dorsalis. Although the patient lacks clear symptoms of neurosyphilis, positive serologic examination for syphilis without any other possible causes suggests altered sensitivity of the spinal cord to anesthetic solutions.     

Abdominal wall electromyography: a noninvasive technique to improve pediatric urodynamic accuracy

Abdominal wall electromyography, using sticky surface patch electrodes placed on the rectus abdominus muscles, was used during noninvasive urodynamic studies in 60 children to detect straining. Straining, which usually was imperceptible to the examiner, occurred in 64 per cent of neurologically normal children. Urodynamics identified detrusor-sphincter dyssynergia during straining to void but reverted to normal when the same children voided without straining. Because the urodynamic findings that characterize detrusor-sphincter dyssynergia are mimicked by straining and other common urologic entities, the potential for misdiagnosis is great unless some assessment of intra-abdominal pressure is made during neurourologic testing.     

Lancinating pain in post-laminectomy chronic sciatica

Summary Lancinating pain, as described in tabes dorsalis, was noted in four patients with chronic sciatica after several months of laminectomy. The pain responded well to carbamezapine therapy. Abnormal or ephaptic neural transmission of impulses in the roots was considered to be the cause of such pain.     

Infravesical obstruction caused by a retrovesical malignant schwannoma

A 77-year-old male presented himself with acute urinary retention, and a history of infravesical obstruction. Cystourethroscopy failed to show the obstruction. A malignant schwannoma (MS) causing impression in the bladder was removed through a laparotomy. Postoperatively there were no urologic symptoms, and the urodynamic investigations had changed to normal.     

Patient costs for urodynamic testing

Urodynamic investigations are not completely standardized. However, the number of exhibits of urodynamic equipment at urologic meetings by commercial firms suggests that these studies have considerable applicability and relevance to everyday practice. Investment by a urologist in equipment for urodynamic testing should be cautious in the light of the costs for these investigations reported from centers in Canada, the United States, and Western Europe. The figures suggest that sophisticated urodynamic testing is not a screening test -- it is too expensive, but large urodynamic laboratories appear to charge only for a portion of the actual cost of such testing. Since urodynamic testing is much more expensive than actual patient charges, it is probably not yet cost effective in its present state of development.     

The Incidence and Etiology of Overactive Bladder in Patients After Cerebrovascular Accident

Cerebrovascular accident or stroke is a devastating neurologic event that can have both short and long term urologic complications. The purpose of this article is to provide an up-to-date review of the incidence and causes of voiding dysfunction after stroke, the evaluation of voiding dysfunction in patients after stroke, and the recommendations on the management of voiding dysfunction following stroke. The reported incidence of urinary incontinence varies from 28-79?% and the causes of urinary incontinence following stroke are multifactorial. Detrusor overactivity is predominant and detrusor underactivity is somewhat less prevalent. Urodynamic findings in patients with stroke vary depending upon timing of the study and associated comorbidities. Currently there are no large longitudinal studies linking urodynamic findings with location or degree of infarct. Based on current studies, we conclude that patients with detrusor underactivity should be managed with clean intermittent catheterization or indwelling Foley catheter, while timed voiding with or without anticholinergic therapy may be an effective treatment for patients with detrusor overactivity after stroke.     

Screening urography in asymptomatic cryptorchid patients.

Excretory urograms in 140 cryptorchid patients free of associated urologic symptoms are reviewed. Our findings are presented and compared to those of previously reported series. Our results do not indicate a specific indication for the routine use of excretory urography in asymptomatic male subjects with cryptorchidism.