Late neurological dysfunction in adult lumbosacral lipoma

The case of a 53-year-old woman with adult-onset neurological dysfunction secondary to spinal dysraphia and lumbosacral lipoma is presented. This condition not only affects children, but may produce neurological troubles also in adult age. This observation confirms that lumbosacral lipomas do not represent a static condition but carry the potential for late neurological dysfunction and therefore they should be treated in early life to prevent late complications. Besides the adult-onset urinary dysfunction and lumbosacral and sciatic pain should suggest the possibility of spinal dysraphia and lumbosacral lipoma.     

Evaluation and therapeutic approaches of voiding and erectile dysfunction in neurological Beh?et's syndrome.

PURPOSE: Beh?et's syndrome is a progressive inflammatory disease which involves multiple systems. It is characterized by 3 main symptoms of iridocyclitis, and oral and genital ulcerations. Nervous system involvement is seen rarely in this clinical entity and is known as neurological Beh?et's syndrome. Inflammation usually occurs in the brain stem, cerebellum and medulla spinalis. Voiding and erectile dysfunction can be due to progressive inflammatory reactions in the nervous and vascular systems. We prospectively evaluated the dysfunctional bladder and penis, and therapeutic options were evaluated prospectively. MATERIALS AND METHODS: A total of 24 consecutive patients diagnosed with neurological Beh?et's syndrome after neurological evaluation were enrolled in this study. Neurological involvement and localization of the nervous system were proved on evaluation. Voiding and erectile dysfunction was evaluated regardless of the presence of related symptoms, and the results were compared with those of controls. Patients with voiding dysfunction on urodynamic study were treated and reevaluated symptomatically after 3 and urodynamically after 6 months. RESULTS: The rate of erectile dysfunction in neurological Beh?et's syndrome was 63%. Mixed type vasculogenic impotence, arterial insufficiency, veno-occlusive dysfunction and neurogenic impotence were identified in 7, 2, 2 and 1 patient, respectively. Detrusor instability was demonstrated in 12 patients with urgency incontinence, including 3 with detrusor-sphincter dyssynergia. Brain stem localization was determined in these patients on neurogenic evaluation. Significant improvement was observed with anticholinergic treatment and clean intermittent catheterization in 3 patients with detrusor-sphincter dyssynergia. Hypersensitive and hypocompliant detrusor was noted in patients with neurological Beh?et's syndrome who had normal voiding habits. CONCLUSIONS: Incontinence or irritable bladder symptoms should not be considered innocuous clinical findings in neurological Beh?et's syndrome. Lower urinary tract function should be evaluated in all patients with this neurological syndrome. The incidence of erectile dysfunction is approximately 65% and the therapeutic approach should be determined according to lower urinary tract function.     

不同脑保护方法对深低温停循环主动脉手术后短暂性神经功能障碍的影响

目的评价不同脑保护方法对深低温停循环（DHCA）主动脉手术后短暂性神经功能障碍（TND）的影响。方法对78例行DHCA主动脉手术患者的临床资料进行回顾性分析,比较逆行性脑灌注（RCP）和选择性顺行脑灌注（SCP）两种不同脑保护方法术后TND的发生情况,同时考察DHCA时程对TND发生率的影响。结果RCP组TND的发生率为34。9％（15／43）,SCP组则为11．4％（4／35）,两组间比较差异有统计学意义（P〈0．05）。同时长DHCA时程（〉50min）的TND的发生率亦明显高于短DHCA时程（〈50min）的TND发生率（P〈0．05）。结论采用SCP作为脑保护方法和缩短DHCA时程可以降低TND的发生率,能够更好的保护脑功能。     

Reversible arteriovenous malformation-induced venous hypertension as a cause of neurological deficits.

A case of a dural arteriovenous malformation with prominent localizing neurological deficits is reported. The venous drainage of the lesion and the lack of a significant pial supply implicate venous hypertension as the mechanism of neurological dysfunction. This mechanism is supported further by the angiographic changes and the prompt resolution of the deficits after endovascular treatment. This case illustrates the potential for this frequently postulated but rarely confirmed pathophysiological mechanism to cause reversible neurological dysfunction.     

Tumor-associated neurological dysfunction prevented by lazaroids in rats

The efficacy of U-74006F and U-78517F in the treatment of blood-tumor barrier permeability and tumor-associated neurological dysfunction was evaluated in a brain-tumor model in rats. U-74006F is a 21-aminosteroid and U-78517F is a 2-methylamino chroman. Rats with stereotactically implanted Walker 256 tumors were treated with methylprednisolone, U-74006F, U-78517F, or vehicle (0.05 N HCl) on Days 6 through 10 following implantation. Neurological function and vascular permeability were assessed on Day 10. Methylprednisolone and U-74006F were equally effective at preventing neurological dysfunction compared to the control group (p less than 0.01); U-78517F was slightly less effective than U-74006F and methylprednisolone but was significantly better than vehicle in preventing neurological dysfunction. Delivery of methylprednisolone resulted in a significant decrease in tumor vascular permeability (p less than 0.006) while U-74006F and U-78517F had no effect on permeability. This suggests that U-74006F and U-78517F prevented tumor-associated neurological dysfunction by a mechanism other than decreasing permeability in tumor capillaries, and that U-74006F or U-78517F could prove useful in the treatment of brain tumors.     

Results of aortic arch repair with hypothermic circulatory arrest and retrograde cerebral perfusion

BACKGROUND: Repair of aortic arch pathology is reliably performed with hypothermic circulatory arrest, but the best method of brain protection is controversial. METHODS: We reviewed a consecutive series of 67 patients who had aortic arch repair with hypothermic circulatory arrest. Retrograde perfusion of arterial blood into the superior vena cava (SVC) during systemic arrest was used in 87%. Average age was 65 years. Acute dissection was present in 25%. Average circulatory arrest time was 37 minutes, and average temperature 17.7 degrees C. RESULTS: Hospital mortality was 1.5%. Strokes occurred in 4.5%. Temporary neurological dysfunction occurred in 16%. Multivariate logistic regression analysis showed that acute dissection was the only independent predictor of the combined risk of stroke and temporary neurological dysfunction (odds ratio 8.5). Duration of circulatory arrest and patient age were not risk factors for adverse neurological outcome. CONCLUSION: Continuous arterial perfusion of the SVC during hypothermic circulatory arrest provides excellent cerebral protection for aortic arch repair. Acute dissection is an independent risk factor for adverse neurological outcome. Arrest time is not a predictor of neurological dysfunction.     

Management of severe cerebral edema in the metabolic encephalopathy of Reye-Johnson syndrome.

Fifteen critically ill children with the diagnosis of Reye-Johnson syndrome were treated with techniques developed to maintain adequate cerebral perfusion pressure and levels of circulating blood glucose. One child died, three sustained neurological deficit, and nine children (70%) recovered without significant neurological dysfunction. The technique developed during the period these children were treated, the indications for their use, and factors that can interfere with maintaining adequate cerebral perfusion in patients with increased intracranial pressure from metabolic encephalopathy are described. The results suggest that neurological damage in this syndrome results from neuronal injury secondary to inadequate cerebral perfusion and/or hypoglycemia, and that neurological dysfunction like hepatic dysfunction should produce minimal mortality and morbidity if cerebral perfusion and adequate levels of circulating blood glucose are sustained during the period of increased intracranial pressure and liver failure.     

Update on the neurology of Parkinson's disease

The differential diagnosis of a patient with apparent Parkinson's Disease (PD) and bladder symptoms is considered and the bladder dysfunction of Multiple System Atrophy (MSA) is reviewed. Recent insights into the progression of the neuropathology of PD have enabled thinking about the stage of the disease at which bladder dysfunction is likely to occur and the expected clinical context of the problem. Bladder symptoms of neurological origin are likely in a patient who has had treated motor symptoms for some years and in whom the ongoing neuropathology has progressed beyond involvement of the basal ganglia, so that symptoms due to cortical dysfunction as well as the adverse effects of dopaminergic medication are also confounding factors. Bladder symptoms in a man with lesser neurological disability should be investigated to exclude underlying outflow obstruction. Possible management options are considered.     

Normal pressure hydrocephalus in children and young adults

Normal pressure hydrocephalus (NPH) is generally considered to be a disorder of adult and geriatric patients. We report four patients who are children or young adults with chronic neurological disorders, recent deterioration of their levels of function, normal cerebrospinal fluid (CSF) pressures, and ventricular enlargement. All four patients improved after the placement or revision of a ventriculoperitoneal shunt. Frequent symptoms and signs included irritability (three patients), vomiting (three patients), and abnormal limb posturing (two patients). Correct diagnosis was hampered by two factors: (a) Multiple or prolonged recordings of CSF pressures were invariably well within the normal ranges with respect to age, and (b) the patients had chronic neurological deficits. After ventriculoperitoneal shunting, subjective and objective improvement was seen in all cases. Young patients with large ventricles may benefit from shunting procedures despite low CSF pressures. These patients may be clinically identified by symptoms of new neurological dysfunction in cases of previously static neurological disease or acceleration of slowly progressive neurological dysfunction. Some of these underlying neurological disorders may predispose children and young adults to NPH.     

Voiding dysfunction in young,nulliparous women: symptoms and urodynamic findings

The objective was to determine urodynamic findings in young, premenopausal, nulliparous women with bothersome lower urinary tract symptoms and assess whether or not symptoms are predictive of specific urodynamic abnormalities. The records of 57 women were reviewed. Those with neurological disease or a primary complaint of stress incontinence were excluded. All completed the American Urological Association Symptom Index (AUASI) and underwent videourodynamics. Symptoms were compared in patients with and without bladder dysfunction and/or voiding phase dysfunction. Bladder dysfunction was diagnosed in 86% of patients with urge incontinence vs. 17% of those without (p<0.0001). Patients with voiding phase dysfunction had higher total and voiding AUASI scores. Occult neurological disease was later diagnosed in 4 women (24%) with urge incontinence and bladder dysfunction. Urge incontinence and voiding symptoms are frequently associated with urodynamically demonstrable abnormalities. Urge incontinence and bladder dysfunction may be a sign of occult neurological disease in this population. The presenting symptoms are useful in determining the utility of urodynamics in this population.Editorial Comment: Voiding dysfunction in young women tends to be an incompletely described phenomenon. This patient cadre is often treated presumptively, and as we see from this study, incorrectly. The clinical tendency for minimalist evaluation represents a disservice to these individuals as accruing data supports the use of urodynamics as an intrinsic component in the evaluation of these women. Obviously, data on pressure / flow dynamics would provide further insight into subtle pelvic floor and sphincteric dysfunctions manifested in this group. The authors are to be commended for this opus     

脊柱转移瘤的外科治疗

目的：脊柱转移瘤可引起顽固性疼痛及脊髓压迫，其外科治疗仍存在许多争议，探讨脊柱转移瘤外科治疗的临床效果。方法：自1998年7月－2001年7月，北京大学人民医院骨肿瘤科手术治疗脊柱转移瘤患者62例，包括颈椎转移6例，胸椎转移37例，腰椎转移19例。无明显神经系统受累者19例；出现神经系统受损者43例，其中完全瘫痪者19例，不完全瘫痪者24例。病变累及一个脊柱节段者43例，二个脊柱节段者14例，三具脊柱节段者5例。结果：62例患者中，58例（94％）术后疼痛得到缓解。43例有神经功能损害的患者中，33例术后麻痹症状改善。25例术前膀胱及直肠功能受损的患者中，术后12例膀胱及直肠功能明显改善。术前Frankel A、B级的患者，5例术后恢复到E级或D级，9例改善为C或D级。结论：从肢体功能减退到完全瘫痪所经历的时间是最重要的预后因素，在48h内完全瘫痪为预后不良的重要因素；膀胱和直肠功能的完全丧失也提示预后不良。甲状腺癌和乳腺癌的脊柱转移一般预后较好，肺癌和肝癌的脊柱转移通常存活期较短。     

Normothermic cardiopulmonary bypass: effect on the incidence of persistent postoperative neurological dysfunction following coronary artery bypass graft surgery

We retrospectively reviewed the records of 250 consecutive patients undergoing coronary artery bypass graft surgery (CABG) from January 1994 through January 1996 to determine the incidence of persistent postoperative neurological dysfunction after CABG and to compare normothermic and moderate hypothermic cardiopulmonary bypass (CPB). Normothermic CPB was used in 128 patients (36°–37°C) and hypothermic CPB (27°–28°C) in 122 patients. Postoperative neurological dysfunction included focal motor deficits, delayed recovery of consciousness (>24h) after surgery, and seizures within 1 week postoperatively. Persistent neurological dysfunction was diagnosed if complete resolution had not occurred within 10 days of surgery. The incidence of persistent postoperative neurological dysfunction was 4.1% in the hypothermic CPB group and 2.3% in the normothermic CPB group. There were no statistically significant differences between the two groups (P=NS). These results suggest that normothermic CPB did not increase the incidence of persistent postoperative neurological dysfunction compared to hypothermic CPB.     

Case report: Acute postoperative neurological impairment from fat embolism syndrome

PURPOSE: To describe a case of fat embolism syndrome (FES) following elective tendon contracture release in a patient with myotonic dystrophy, to highlight the importance of considering this entity in the differential diagnosis of acute postoperative neurocognitive dysfunction. CLINICAL FEATURES: A 34-yr-old man with myotonic dystrophy underwent uneventful tendon contracture release under regional anesthesia. In the immediate postoperative period, neurological and respiratory complications developed, requiring intensive care support. The patient showed the classical clinical triad of hypoxemia, neurological impairment and a petechial rash associated with the FES. A diagnosis of FES was made and, despite therapy including fluid and inotropic support, the patient succumbed to the condition. There was no demonstrated intracardiac shunt, suggesting a physiological intrapulmonary shunt was responsible for the development of systemic manifestations of FES. CONCLUSIONS: Postoperative neurological dysfunction is a difficult condition with numerous possible causes. All possible etiologies, including FES, need to be considered in the differential diagnosis and postoperative management of patients developing acute postoperative neurological impairment and hypoxemia.     

The role of sacroiliac joint dysfunction in the genesis of low back pain: the obvious is not always right

Background context It is a common practice to the link low back pain with protruding disc even when neurological signs are absent. Because pain caused by sacroiliac joint dysfunction can mimic discogenic or radicular low back pain, we assumed that the diagnosis of sacroiliac joint dysfunction is frequently overlooked. Purpose To assess the incidence of sacroiliac joint dysfunction in patients with low back pain and positive disc findings on CT scan or MRI, but without claudication or objective neurological deficits. Methods Fifty patients with low back pain and disc herniation, without claudication or neurological abnormalities such as decreased motor strength, sensory alterations or sphincter incontinence and with positive pain provocation tests for sacroiliac joint dysfunction were submitted to fluoroscopic diagnostic sacroiliac joint infiltration. Results The mean baseline VAS pain score was 7.8 ± 1.77 (range 5–10). Thirty minutes after infiltration, the mean VAS score was 1.3 ± 1.76 (median 0.000E+00 with an average deviation from median = 1.30) (P = 0.0002). Forty-six patients had a VAS score ranging from 0 to 3, 8 weeks after the fluoroscopic guided infiltration. There were no serious complications after treatment. An unanticipated motor block that required hospitalization was seen in four patients, lasting from 12 to 36 h. Conclusions Sacroiliac joint dysfunction should be considered strongly in the differential diagnosis of low back pain in this group of patients.     

Bladder dysfunction in the elderly

Bladder dysfunction in the elderly is an enormous public health problem. Laboratory research using animal models has to date not yielded clinically useful new information. Demographic and urodynamic studies in selected elderly patients indicate that neurological disease and bladder outlet obstruction are the primary causes for voiding dysfunction. The effect of aging per se on the urinary bladder is not known. Elderly patients who have bladder dysfunction should be evaluated with a careful history and physical examination combined with a practical urodynamic evaluation rather than simply being labeled as having "bladder dysfunction in the elderly."     

Conus medullaris syndrome without lower-extremity involvement in L-1 burst fractures: report of four cases

Conus medullaris injury from burst fractures is known to occur in conjunction with other neurological deficits, including lower-extremity motor weakness or sensory changes. Rarely does an isolated conus medullaris injury occur from an extradural cause without other neurological deficits. The authors report four cases of L-1 burst fractures in which conus medullaris dysfunction was the sole neurological injury in the absence of lower-extremity involvement.     

Post-traumatic syringomyelia

Progressive post-traumatic cystic syringomyelia is an uncommon and increasingly recognized cause of morbidity following spinal cord injury. We hereby report a 35-year-old gentleman who sustained wedge compression fracture of L-1 vertebral body 15 years back and had complete paraplegia with bowel/bladder involvement. The neurological deficit recovered with minimal residual motor deficits and erectile dysfunction. He presented now with increasing neurological deficits associated with pain and paresthesia. The MRI spine showed a syrinx extending from the site of injury up to the medulla. He underwent a syringo-peritoneal shunt and at followup his pain and motor functions had improved but erectile dysfunction was persisting.     

Hormonal etiology in erectile dysfunction

The proper function of erection mechanisms depend on correct interrelationship between psychological, vascular, neurological and hormonal factors. Endocrine diseases affect sexual function, and sexual dysfunction may be one of the symptoms of some hormonal anomalies. Diabetes mellitus is the endocrine disease most frequently causing erectile dysfunction due to the frequent vascular and neurological complications associated. It is important to determine blood glucose in the initial evaluation of a male with erectile dysfunction, as well as to try an adequate control of blood glucose levels to avoid worsening. Diabetic male erectile dysfunction is multifactorial, more severe and has worse response to oral treatment. Hyperprolactinemia causes disorders of the sexual sphere because it produces a descent of testosterone. In these cases, sexual symptoms are treated by correcting the levels of prolactin. Routine determination of prolactin is not clear and it seems it should be determined when testosterone levels are diminished. Thyroid hormone disorders (both hyper and hypotyroidism) are associated with erectile dysfunction, which will subside in half the patients with thyroid hormone normalization. The role of adrenal hormones in erectile function is not clear and their routine determination is not considered in the diagnostic evaluation of erectile dysfunction. The role of estradiol in the regulation of the erection mechanism is not well known either, although it is known that high levels may cause erectile dysfunction. Among endocrine-metabolic disorders we point out dyslipemias, with hypercholesterolemia as an important risk factor for erectile dysfunction and, though its correction may prevent vascular system deterioration, the role of statins in erectile dysfunction is not clear.     

Urinary dysfunction in transverse myelitis

Six men and 2 women with a history of transverse myelitis and persistent lower urinary tract symptoms underwent neurourological evaluation. Of the patients, 4 were neurologically intact, while the remainder had residual neurological deficits. Urodynamic studies revealed detrusor-external sphincter dyssynergia in 6 patients. Two patients had detrusor hyperreflexia, of whom 1 also had an incompetent sphincter. Erectile or ejaculatory dysfunction was reported by 3 men. We conclude that prolonged bladder and sexual dysfunction, caused by spinal cord inflammatory insult, may persist despite a systemic neurological recovery. Therefore, bladder management guided by initial and followup urodynamics is recommended.     

Bulbocavernosus reflex: its validity as a diagnostic test of neurogenic impotence

Measurement of the bulbocavernosus reflex is used widely to diagnose underlying neurogenic disorders in erectile dysfunction. A prolonged bulbocavernosus reflex latency (that is more than 45 msec.) or the absence of a reflex response of the bulbocavernosus muscles during electrical stimulation of the glans penis is considered a sign of neurological disease. Since only a few experimental studies have been performed in man related to the neurophysiological mechanism of erection, and since the results of these studies were contradictory the diagnostic validity of bulbocavernosus reflex measurement was reassessed. We determine whether men with abnormal bulbocavernosus reflex latencies have concomitant organic erectile dysfunction as confirmed by nocturnal plethysmographic and rigidity recordings. The bulbocavernosus reflex was recorded in 90 subjects and 19 had abnormal bulbocavernosus reflex latencies. Of these 19 subjects 8 had normal nocturnal erections, thus, confirming a diagnosis of psychogenic impotence. These results cast some doubts on the validity of bulbocavernosus reflex measurement for the diagnosis of organic erectile dysfunction due to a neurological disease.