Bruns Garland Syndrome as the first presentation of type 2 diabetes: two case reports and a practical approach to diagnosis.

BACKGROUND: Diabetes is a global health problem causing a significant burden on the healthcare systems both due to the disease itself and associated complications. Diabetic radiculoplexus neuropathies or Bruns-Garland syndrome constitutes a rare form of microvascular complications, more commonly affecting the lumbosacral plexus and, very rarely, the cervical plexus. We describe two Sri Lankan males who presented with diabetic lumbosacral radiculoplexus neuropathy and diabetic cervical radiculoplexus neuropathy as the initial manifestation of diabetes. CASE DESCRIPTION: Case 1: a 49-year-old Sri Lankan hotel chef presented with subacute painful weakness and wasting of the left upper arm for 3 months and weight loss. Left upper limb proximal muscles were wasted with diminished power and reflexes. A nerve conduction study showed comparative amplitude reduction. An electromyogram revealed positive sharp waves, frequent fibrillations, and high amplitude polyphasic motor unit potentials with reduced recruitment in proximal muscles of left upper limb. Case-2: a 47-year-old Sri Lankan carpenter presented with subacute progressive asymmetrical painful weakness and wasting of bilateral thighs for 5 months and weight loss. Lower limb proximal muscles were wasted with reduced power and knee jerks. The nerve conduction study was normal. The electromyogram was similar to case 1 involving both quadratus femoris muscles, which was more prominent on the left side. The work up for an underlying etiology revealed only elevated fasting blood glucose and HbA1c, suggesting a new diagnosis of diabetes associated with neurological symptoms. Patient 1 was diagnosed with diabetic cervical radiculoplexus neuropathy and patient 2 with diabetic lumbosacral radiculoplexus neuropathy. Both showed significant improvement following optimization of glycemic control together with symptomatic treatment and physiotherapy. CONCLUSION: Diagnosis of diabetic radiculoplexus neuropathy requires a comprehensive workup to rule out other sinister pathologies. This case report has a dual importance; it describes diabetic radiculoplexus neuropathy as the very first manifestation of two previously healthy people, giving rise to a new diagnosis of diabetes and, at the same time, reporting on diabetic cervical radiculoplexus neuropathy, which is extremely rare and has never been previously reported in Sri Lanka.

Variations in the course of the superior gluteal artery in relation to the lumbosacral plexus.

The course of the superior gluteal artery (SGA) as it passes through the lumbosacral plexus is variable. The variations of the arterial course in relation to the lumbosacral plexus have focused on statistical analysis, and it is limited arterial diversity. In this study, we investigated the positional relation between the SGA and the furcal nerve (FN): guide to segmentation of the lumbosacral plexus, arising from the L4, ie, the contribution to the femoral nerve, obturator nerve, and lumbosacral trunk. We could classify the pathway of the SGA into three types based on its positional relation to the FN. The SGA courses under the ramus from which the FN originates (Type A), under the ramus one segment below the origin of the FN (Type B), or between the obturator nerve and the lumbosacral trunk (Type C). The SGA pathway in Types A and B showed a cranial or caudal shift along with cranial or caudal deviation of the FN. In summary, the variation in the SGA pathway was correlated with cranial or caudal shift of the FN. Our findings indicate that variations of the SGA pathway are associated not only with arterial transformation, but also with diversity of the lumbosacral plexus.

Complicated abdominal pregnancy with placenta feeding off sacral plexus and subsequent multiple ectopic pregnancies during a 4-year follow-up: a case report.

BACKGROUND: Abdominal pregnancy (pregnancy in the peritoneal cavity) is a very rare and serious type of extrauterine gestation that accounts for approximately 1.4% of all ectopic pregnancies. It also represents one of the few times an ectopic pregnancy can be carried to term. Early strategic diagnosis and management decisions can make a critical difference with regards to severity of morbidity and mortality risk. After an extensive search of the English language medical literature, we are unaware of any case of abdominal pregnancy in which the placenta was receiving its vascular supply from the sacral plexus. CASE PRESENTATION: A 26-year-old African-American woman, primigravida, at 16 weeks 4 days' gestation, presented to our Emergency Department with abdominal pain. She did not complain of any vaginal bleeding. A physical examination revealed mild abdominal tenderness and no blood in the vaginal vault. Laboratory findings corresponded to an increased level of beta human chorionic gonadotropin; magnetic resonance imaging confirmed an abdominal pregnancy. She underwent feticide, administration of methotrexate and a laparotomy was done which was immediately deferred due to perceived increased bleeding risk. She was found to have an intra-abdominal ectopic pregnancy with the placenta attached to her omentum, cul-de-sac and rectosigmoid, with unusual and extensive vascularity from the sacral plexus. A repeat laparotomy was performed 11 weeks later, aimed at removal of the gestational sac and placenta that were left in situ on the first laparotomy. This time, we achieved successful removal of the peritoneal gestation, lysis of adhesions, ligation of vascular supply and cautery of the diminished vasculature. Subsequently, she had two ectopic pregnancies, which were managed with both medical and surgical interventions. CONCLUSIONS: Ectopic pregnancies should be identified early and evaluated for the etiology of the presentation. Rarely, an ectopic pregnancy implants at an extratubal location. Today, early intervention saves lives and reduces morbidity, but ectopic pregnancy still accounts for 4 to 10% of pregnancy-related deaths and leads to a high incidence of ectopic site gestations in future pregnancies. Medical management has emerged as a safe alternative to surgery and holds promise for preservation of future fertility; however, surgery remains an acceptable modality. We found that careful and strategic choice of management pathway can make all the difference to a favorable outcome. As emergency physicians, we need to be aware of the possibility of abdominal ectopic pregnancy in such presentations and its severe consequences if it remains undiagnosed.

Surgical management of presacral bleeding.

INTRODUCTION: Presacral venous bleeding is an uncommon but potentially life threatening complication of rectal surgery. During the posterior rectal dissection, it is recommended to proceed into the plane between the fascia propria of the rectum and the presacral fascia. Incorrect mobilisation of the rectum outside the Waldeyer's fascia can tear out the lower presacral venous plexus or the sacral basivertebral veins, causing what may prove to be uncontrollable bleeding. METHODS: A systematic search of the MEDLINE(®) and Embase™ databases was performed to obtain primary data published in the period between 1 January 1960 and 31 July 2013. Each article describing variables such as incidence of presacral venous bleeding, surgical approach, number of cases treated and success rate was included in the analysis. RESULTS: A number of creative solutions have been described that attempt to provide good tamponade of the presacral haemorrhage, eliminating the need for second operation. However, few cases are reported in the literature. CONCLUSIONS: As conventional haemostatic measures often fail to control this type of haemorrhage, several alternative methods to control bleeding definitively have been described. We propose a practical comprehensive classification of the available techniques for the management of presacral bleeding.

Aspectos anatómicos y topográficos del nervio pudendo en la región glútea / Anatomical and topographical aspects of the pudendal nerve in gluteal region

El nervio pudendo distribuye ramos motores y sensitivos para la región perineal y órganos genitales externos. Tiene importancia funcional en la micción, defecación, erección y parto. Desde el punto de vista clínico, se realiza bloqueo anestésico del mismo en la práctica obstétrica, se electroestimula en casos de incontinencia fecal o urinaria, entre otros procedimientos. Investigaciones anatómicas han señalado que puede presentar variaciones en su conformación y topografía. Con el propósito de complementar el conocimiento sobre este nervio en su trayecto por la región glútea, se estudió su conformación, biometría y relaciones con los vasos pudendos internos y ligamentos adyacentes. Se disecaron 30 regiones glúteas de 15 cadáveres formolizados de individuos brasileños, adultos, de ambos sexos, observando la conformación del nervio, número de ramos, disposición respecto a los vasos pudendos internos y ligamentos sacrotuberoso y sacroespinoso, registrando también su ancho en el trayecto entre los forámenes isquiático mayor y menor. El nervio pudendo se presentó como tronco único en 53,3 por ciento de los casos y dividido en ramos en 46,7 por ciento (dos ramos en 36,7 por ciento, tres en 6,7 por ciento y cuatro en 3,3 por ciento). Cuando estaba dividido, en 36,7 por ciento los ramos permanecieron separados y en 10 por ciento se unieron antes de ingresar en el foramen isquiático menor. El nervio (único o dividido) fue medial a los vasos pudendo internos en 70 por ciento y lateral a ellos en 3,3 por ciento. En el 26,7 por ciento restante, estaba dividido en dos o tres ramos, que se situaban medial y lateralmente a los vasos o los cruzaban posteriormente. Su posición fue anterior al ligamento sacrotuberoso en 93,3 por ciento. El nervio pudendo presenta interesantes variaciones en su conformación y topografía, que deben ser consideradas durante los procedimientos clínicos y quirúrgicos que lo involucren.

The pudendal nerve distributes motor and sensory branches to the perineum and genital external organs. It has functional importance in the micturition, defecation, erection and labor. From the clinical point of view, anaesthetic blockade of the same one is realized in the obstetric practice, electroestimulation in cases of fecal or urinary incontinence, among other procedures. Anatomical investigations have indicated that it can present variations in its conformation and topography. The objective of this study was complete knowledge about this nerve in its course through the gluteal region, its conformation, biometry and its relationship with the internal pudendal vessels and adjacent ligaments were studied. We dissected 30 gluteal regions of 15 corpses fixed in formaldehyde 10 percent of Brazilian individuals, adult, of both sexes, observing the conformation of the nerve, number of branches, disposition with regard to the internal pudendal vessels and sacrotuberous and sacrospinous ligaments, also recording its external diameter in the distance between greater sciatic foramen and lesser sciatic foramen. The pudendal nerve appeared as a single trunk in 53.3 percent of the cases and divided in branches in 46.7 percent (two branches in 36.7 percent, three in 6.7 percent and four in 3.3 percent). When it was divided, in 36,7 percent the branches remained separated and in 10 percent they joined before the lesser sciatic foramen. The nerve (single or divided) was medial to the internal pudendal vessels in 70 percent and lateral to them in 3.3 percent. In 26.7 percent, it was divided in two or three branches, which were located medially and laterally to these vessels or crossing posterior to them. Its position was anterior to the sacrotuberous ligament in 93.3 percent. The pudendal nerve presents interesting variations in its conformation and topography which must be considered during the clinical and surgical procedures.

Laparoscopic therapy for endometriosis and vascular entrapment of sacral plexus.

OBJECTIVE: To report our experience with endopelvic causes for sacral radiculopathies and sciatica. DESIGN: Prospective cohort study. SETTING: Tertiary referral advanced laparoscopic gynecology and neuropelveologic unit. PATIENT(S): Two hundred thirteen women who underwent laparoscopic management of sacral radiculopathy (sciatica, pudendal, gluteal pain) of unknown genesis in the period between November 2004 and February 2010. INTERVENTION(S): Selective, clinically oriented, laparoscopic exploration of the sacral plexus with nerve decompression. MAIN OUTCOME MEASURE(S): Complication rates and the short-term cure at 6-month follow-up with use of the Visual Analogue Scale. RESULT(S): Laparoscopic exploration showed isolated endometriosis of the sciatic nerve in 27 patients, deeply infiltrating parametric endometriosis with sacral plexus infiltration in 148 patients, sacral plexus vascular entrapment in 37 patients, and pyriformis syndrome in one patient. A reduction in mean ± SEM) Visual Analogue Scale score of patient pain from 7.7 (± 1.16; range 6-10) before surgery to 2.6 (± 1.77; range 0-6) at 6-month follow-up was obtained for sacral plexus endometriosis and from 6.6 (± 1.43; range 5-9) to 1.5 (± 1.27; range 0-4) for vascular entrapment. CONCLUSION(S): In patients with chronic pelvic pain, preoperative anamnesis and examination should include evaluation of symptoms of sacral radiculopathies (pudendal, gluteal pain) and sciatic neuralgia. In patients with sacral radiculopathy or sciatica of unknown genesis, suspicion of endopelvic pathology such as endometriosis or vascular entrapment must be raised, and laparoscopic exploration of the sacral plexus and/or sciatic nerve is then advisable.

Efectividad de la simpatectomía lumbar en el tratamiento de la arteriopatía periférica oclusiva de miembros inferiores: revisión sistemática / Effectiveness of lumbar sympathectomy in the treatment of occlusive peripheral vascular disease in lower limbs: Systematic review

Fundamento y Objetivos: Este trabajo revisa la efectividad de la simpatectomía lumbar en el tratamiento de la enfermedad arterial periférica de miembros inferiores mediante una revisión sistemática. Material y Método: Revisión sistemática de la bibliografía. Fuentes de datos utilizadas: Medline, Embase, Cochrane Library, INHATA, CRD, BioMed Central y el registro de ensayos clínicos ClinicalTrials.gov. Los términos MeSH usados fueron “sympathectomy”, “peripheral vascular disease”, “lower extremity”, “vascular therapy”, “iliac artery”, “femoral artery” y “popliteal artery”. Se incluyeron pacientes adultos con arteriopatía periférica oclusiva de miembros inferiores tratados con simpatectomía lumbar. La calidad de los estudios se evaluó mediante los criterios del listado de comprobación CASP (Critical Appraisal Skills Programme) y un cuestionario realizado ad hoc. Resultados: Se incluyeron 4 ensayos clínicos y 4 estudios observacionales. La simpatectomía no aportó diferencias significativas en cuanto a mortalidad, amputaciones y gravedad de la claudicación intermitente. Cuando se valoró frente a anestésicos locales o prostaglandina E1 tampoco hubo diferencias. La simpatectomía química registró mejores resultados en relación con la estancia hospitalaria que la simpatectomía quirúrgica.Conclusiones: La evidencia sobre la eficacia de la simpatectomía no ofrece diferencias en relación con los tratamientos farmacológicos convencionales. Ambas técnicas de simpatectomía (quirúrgica y química) no presentan diferencias estadísticamente significativas en términos de mortalidad ni amputaciones (AU)

Background and Objectives: The aim of this work has been to check the scientific evidence on the efficacy of lumbar sympathectomy in the treatment of peripheral arterial disease of lower limbs. Material and Methods: Systematic review of the literature. Data Source: MedLine, Embase, Cochrane Library, INHATA, CRD, BioMed Central, and ClinicalTrials.gov register. Mesh terms: sympathectomy, peripheral vascular disease, lower extremity, vascular therapy, iliac artery, femoral artery, popliteal artery. Inclusion criteria: adult patients with occlusive peripheral arterial disease of lower limbs treated with sympathectomy. The quality was evaluated by means of the criteria of the CASP checklist and “ad hoc” questionnaire. Results: Four clinical trials and four observational studies were included. Sympathectomy did not show significant differences for mortality, amputations and grade of intermittent claudication. When it was assessed regarding local anaesthetics or prostaglandin-E1, differences were not detected. Chemical sympathectomy showed better results than surgical sympathectomy in hospital stance.Conclusions: Evidence on the efficacy of sympathectomy does not show differences related to conventional treatments. Both sympathectomy techniques (surgical and chemical) do not report statistical differences regarding mortality or amputations (AU)

Nicolau livedoid dermatitis following intramuscular benzathine penicillin injection.

We report the case of a 64-year-old male presenting with a rapidly enlarging painful violaceous plaque in the left buttock and posterior thigh, following a gluteal intramuscular injection of benzathine penicillin. Associated urinary incontinence and lower left limb paresis were consistent with sciatic and lower sacral nerve damage, as confirmed by electromyography. Additional underlying muscular damage was observed in ultrasound and computer tomodensitometry scans and supported by high serum levels of creatine kinase and lactate dehydrogenase. Aggressive treatment was performed with fluid expansion, intravenous steroid bolus, vasodilators and anticoagulation, resulting in slow improvement of cutaneous and muscular lesions. However, no significant effect was observed on neurologic dysfunction after 6 months of regular neuromuscular rehabilitation. Nicolau Livedoid Dermatitis is a rare and potentially fatal condition showing variable levels of tissue impairment and unpredictable course and prognosis. Specific treatment is not consensual and the efficacy of any particular treatment remains to be established.

Intravenous immunoglobulin for the treatment of diabetic lumbosacral radiculoplexus neuropathy.

OBJECTIVE: The objective of this study was to evaluate the effect of intravenous immunoglobulin (IVIg) therapy in diabetic lumbosacral radiculoplexus neuropathy (DLRPN) patients who did not respond to analgesic drug therapy and corticosteroids. Background. DLRPN is a rare painful condition that may occur in diabetes mellitus (DM). At the moment, there are limited therapeutic options for DLRPN. METHODS: We recruited five patients affected by type 2 DM and DLRPN. They were selected from a cohort of 13 consecutive DLRPN patients. Inclusion criteria were severe pain (visual analog scale [VAS] > 4/10) and no response to pain symptomatic therapy and corticosteroids. Patients were treated with IVIg (0.4 g/kg/day for 5 days). Outcome measures were VAS, time of onset and duration of pain relief, the Medical Research Council (MRC) scale for lower limb muscle strength, and walking distance. Electrophysiology and needle electromyography (EMG) were retested after IVIg. RESULTS: Four of the patients had positive pain response after IVIg. VAS reduction started 5-10 days after IVIg infusion. Two patients underwent additional IVIg infusions due to pain reappearance after 7-18 months, again with positive response. VAS, MRC scale, and walking distance significantly improved at 1 month (Wilcoxon nonparametric test, two-tailed, P < 0.05). Electrodiagnostic testing was unchanged, but needle EMG showed reduction of denervation signs after IVIg. CONCLUSIONS: IVIg may rapidly reduce pain and improve motor function in DLRPN despite previous negative response to corticosteroids. IVIg may be repeated in those patients who experience disease relapse. Future double-blind trials are needed to evaluate the role of IVIg in DLRPN.

Microvasculitis in diabetic lumbosacral radiculoplexus neuropathy.

We present a case of a 60-year-old man with mild type 2 diabetes mellitus and step-wise progression of bilateral lower limb weakness, numbness, and pain over a 1-year period. At the time of evaluation, he used a walker. He had elevated cerebrospinal fluid protein, abnormal cooling and heat-pain thresholds on quantitative sensory testing, and nerve conduction studies/electromyography consistent with bilateral lumbosacral radiculoplexus neuropathies. Because it was not clear whether the disease was still active, a right superficial peroneal nerve biopsy was performed and showed evidence of active axonal degeneration, ischemic injury, and microvasculitis. On the basis of these results, the patient was diagnosed with diabetic lumbosacral radiculoplexus neuropathy and was treated with weekly intravenous methylprednisolone with marked improvement of neurologic symptoms and signs. This case illustrates the typical clinical, electrophysiologic, and pathologic features of diabetic lumbosacral radiculoplexus neuropathy and the utility of nerve biopsy to judge ongoing disease activity.

[Ischemic bilateral lumbosacral plexopathy and walking disability following surgical treatment of secondary aortoenteric fistulas: a case report and review of literature]. / Ischémie bilatérale des plexus lombosacrés avec atteinte de la marche suite à une réparation de fistule aortodigestive secondaire: à propos d'un cas Revue de la littérature.

OBJECTIVE: Aortoenteric fistula is a rare and serious pathology with a high mortality rate (around 50%). The surgery's neurological complications are also rare (around 0.25% of reconstructive interventions); two-thirds are spinal cord infarcts, with the remaining one-third corresponding to cases of ischemic polyradiculopathy. The latter condition is rarely described and appears to have a better functional prognosis. METHODOLOGY: We report a patient case with aortoduodenal fistula complicated, first by acute haemorrhage and then, by polyradicular damage and a sensorimotor impairment in both legs. RESULTS: EMG revealed bilateral damage to the lumbosacral plexus but NMR did not detect any abnormalities. The motor impairment in the legs was linked to sensory damage. We monitored the patient for two years. The initial functional disability score was 68 but it worsened steadily to a maximum value of 126 two years later. CONCLUSION: The paraclinical data (EMG, NMR) and the functional worsening strengthened our diagnosis of bilateral lumbosacral plexus ischemia, which is rarely described. However, the prognosis appears to be better than for infarction of the conus medullaris, the principal differential diagnosis.

Lumbalgia secundaria a dilatación del plexo venoso epidural lumbar por compresión de la vena cava inferior / Low back pain secondary to lumbar epidural venous plexus dilatation due to compression of the inferior vena cava

Se presenta el caso de un paciente que acudió al Servicio de Urgencias, con una lumbalgia subaguda radiada a ambos miembros inferiores, en el que se hizo el diagnóstico por ecografía y tomografía computarizada abdominal de una masa adenopática retroperitoneal que comprimía la vena cava inferior. Una resonancia magnética de la columna lumbar, además de la masa retroperitoneal, mostró una dilatación y tortuosidad de los vasos del plexo venoso epidural lumbar a la que se atribuyó la lumbalgia y la radiculalgia que presentaba el paciente. El diagnóstico anatomopatológico de la masa retroperitoneal fue de linfoma no Hodgkin folicular. La dilatación del plexo venoso epidural lumbar puede ser causa de dolor lumbar y radicular

We present the case of a patient presenting at the emergency department with subacute low back pain radiating to both lower limbs in whom ultrasonography and abdominal computed tomography diagnosed a retroperitoneal adenopathic mass compressing the inferior vena cava. Magnetid resonance imagin of the lumbar spine showed the retroperitoneal mass and also showed dilatation and tortuosity of the vessels of the lumbar epidural venous plexus, which was considered responsible for the radiating low back pain. Histological study defined the retroperitoneal mass as follicular non-Hodgkin's lymphoma. The dilatation of the lumbar epidural venous plexus can cause lumbar and radicular pain

Lower limb paralysis from ischaemic neuropathy of the lumbosacral plexus following aorto-iliac procedures.

OBJECTIVES: Neurological injuries following aorto-iliac procedures are rare, unpredictable and cause significant morbidity. We report four cases of lower limb paralysis following aorto-iliac procedures, in which two patients suffered internal iliac occlusion and discuss potential aetiological factors. METHODS: Four male patients, age ranging between 56 and 77 years, underwent aorto-iliac procedures. Three patients underwent repair of infra-renal abdominal aortic aneurysm (2 open and 1 endovascular repair) and one patient had percutaneous angioplasty of the internal iliac artery. RESULTS: All patients developed a unilateral lower limb paralysis early post procedure. Neurophysiological studies were performed in three patients and confirmed the injury to the lumbosacral plexus in two cases. MRI scan performed in two patients did not show any abnormality. In two of the cases, occlusion of one internal iliac artery was implicated as the cause of lumbo-sacral plexopathy: one with the coverage of the internal artery origin with the stent, the other due to thrombotic occlusion of common and internal iliac in arteries after an elective open repair of abdominal aortic aneurysm with a bifurcated graft. Follow up ranged between 2 and 4 months. Only one patient recovered completely; the other three were left with permanent disability. CONCLUSIONS: Ischaemic neuropathy following aorto-iliac intervention, whether open or endovascular, remains a rare, unpredictable and devastating complication. When it occurs it is likely to result in permanent neurological disability. It is important to note that it may be related to internal iliac artery thrombosis.

Ischaemic lumbosacral plexopathy following aorto-iliac bypass graft: case report and review of literature.

A 77-year-old man had aorto-iliac bypass for an abdominal aortic aneurysm (AAA). This was complicated by occlusion that needed extension of the graft to the right femoral artery. He was unable to move his right leg with numbness after surgery. This was caused by extensive lumbosacral plexopathy on the right side. Lumbosacral plexopathy is uncommon because the plexus has a rich blood supply. The incidence of ischaemic lumbosacral plexopathy is higher with re-operative and emergency AAA reconstruction. This may predispose the lumbosacral plexus to ischaemic injury. Consideration should be given to maintaining retrograde perfusion of the internal iliac artery.