MULTIPLE PERIPHERAL NERVE ENTRAPMENT IN FORESTIER'S DISEASE (DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS)

A patient with typical diffuse idiopathic skeletal hyperostosisand characteristic new bone formation around the elbows exhibitedbilateral ulnar nerve entrapment at these sites as well as mediannerve compression at one wrist. KEY WORDS: Ulnar nerve, Median nerve, Heterotopic bone, Surgical decompression     

Ulnar entrapment neuropathy in patients with type 2 diabetes mellitus: An electrodiagnostic study

Aims

This study aimed to assess the prevalence and electrophysiological features of ulnar entrapment neuropathy in patients with type 2 diabetes mellitus (DM).

Methods

Nerve conduction studies (NCS) were performed in a sample of consecutive diabetic patients aged 25–75 years, referred by the Diabetology Unit. NCS of the median, ulnar, radial, peroneal and sural nerves were performed on the non-dominant side. Median entrapment neuropathy at the wrist (MNW) and ulnar neuropathy at the elbow (UNE) and wrist (UNW) were diagnosed according to standard electrodiagnostic criteria.

Results

Sixty-four patients were enrolled, 28 male (44%), average age 61, average DM duration 14.5 years. Polyneuropathy was diagnosed in 45 subjects (70%). UNE was detected in 22 patients (34%) (4 did not have polyneuropathy), in the abductor digiti minimi in 16, the first interosseus in 14 and in both in 8. UNW was detected in 7 (11%) subjects and MNW in 40 (63%). NCS alterations consistent with ulnar neuropathy were detected in a high proportion of patients (45%), suggesting that the ulnar nerve is very susceptible to focal entrapment in DM.

Conclusions

Upper limb sensory and motor NCS, including motor conduction velocity across the elbow, should be considered in the staging of DM patients.     

Marinacci anastomosis (reverse Martin-Gruber anastomosis): A case report

Rationale:The incidence of Martin-Gruber anastomosis ranges from 5% to 34%, which is characterized by crossing over from the median to the ulnar nerve and innervating the first dorsal interosseous, thenar or hypothenar muscles. However, the reverse Martin-Gruber anastomosis, or Marinacci anastomosis, is far less discussed and appears in recent literature.Patient concerns:A 56-year-old man presented to the clinic of a university hospital because of left neck soreness with numbness radiating to the left lateral shoulder. The neck discomfort was aggravated while the neck rotated or tilted to the right.Diagnosis:Higher compound muscle action potential over the abductor pollicis brevis on elbow stimulation than on the wrist was found during upper limb nerve conduction velocity study. Ulnar to median anastomosis was identified.Intervention:We performed cervical spine X-ray and electrophysiological examinations and monitored the patient.Outcomes:We identified that this patient had left C5 and C6 subacute radiculopathy with active denervation and left subclinical ulnar sensory neuropathy, and verified the existence of ulnar-to-median anastomosis.Lessons:We demonstrated a pure motor ulnar-to-median anastomosis without sensory correspondence and higher CMAP over the abductor pollicis brevis on elbow stimulation of the ulnar nerve than on the wrist. The prevalence might be underestimated in a Chinese population-based published study.     

Acute ulnar neuropathy at the wrist: a case report and review of the literature

Acute ulnar neuropathy at the wrist is an extremely uncommon condition, at times requiring a high index of suspicion for the diagnosis. Clinical presentations of ulnar nerve lesions at the wrist and hand show variations due to the complex anatomic course of the nerve in distal sites. We report a case of acute ulnar neuropathy at the wrist caused by a ganglion in Guyon’s canal, being initially misinterpreted as flexor tenosynovitis. The accurate diagnosis of selective distal motor neuropathy of ulnar nerve was made electrophysiologically. Magnetic resonance imaging revealed a well defined soft tissue mass consistent with a ganglion, compressing the ulnar nerve in Guyon’s canal. Entrapment neuropathies are one of the common conditions handled by physiatrists. Ulnar nerve lesions at the wrist should be kept in mind in the differential diagnosis of patients with wrist or hand pain. Magnetic resonance imaging is a useful method in the anatomical evaluation of acute focal neuropathies.     

Cubital tunnel syndrome in patients with haemophilia

Summary. Elbow is the second most common joint involved in patients with haemophilia; however, there is little data about the involvement of ulnar nerve at elbow in patients with haemophilic arthropathy. The purpose of this study was to address this problem in the elbow and evaluate the results of anterior subcutaneous transposition of the ulnar nerve in a small group of patients with haemophilia who had been managed in two institutions. Information on six patients who were diagnosed with tardy ulnar nerve palsy in two institutions was retrospectively collected. All patients suffered form severe haemophilia A. Anterior subcutaneous transposition of the ulnar nerve had been performed in all except one. The mean age of the patients at the time of procedure was 45.8 years and the mean duration of follow‐up was 60.2 months. No postoperative complication or recurrence was observed. No additional surgery was required in operated patients. Evaluation was performed using subjective and objective measures, and a modified Bishop score. After operation, subjective sensory and motor disturbances were improved or resolved in all of the operated patients, while objective measures improved less well. Ulnar nerve can be involved in cubital tunnel in patients with haemophilia. Anterior subcutaneous transposition of the ulnar nerve is an effective procedure for improving patients’ symptoms, with low risk of complications.     

桡神经浅支端侧吻合修复尺神经高位损伤的研究

目的通过动物实验探讨感觉神经移位一期联合修复尺神经高位（肘关节以上）损伤,手内在肌组织学变化及吻合口神经病理学变化。方法选用成年雄性猕猴6只,以上肢为研究单位,6只动物双侧上肢随机分为3组,每组4侧上肢。 A组（实验组）：于上臂上段切断尺神经,再重新端端吻合。于远端切断桡神经浅支,移位于腕部与尺神经（外膜开窗）作端侧吻合。 B组（对照组）：于上臂上段切除尺神经3 cm,两断端分别折叠结扎,腕部处理同A组。 C组（对照组）：上臂部尺神经处理同A组,腕部不作神经移位。观察术后猴尺神经所支配的手内在肌萎缩程度。取术后1个月、3个月、6个月、12个月猴尺神经支配的手内在肌端侧吻合口、端侧吻合口以远的神经干及小鱼际肌组织,做成切片,光镜下观察其显微结构的变化。结果术后12个月观察到A组雄猴手内在肌恢复自主活动,术侧手内在肌肌肉萎缩不明显;B组术侧手内在肌肌肉萎缩,程度较C组轻;C组术侧手内在肌肌肉明显萎缩。组织学观察结果显示,术后A组神经纤维数量、密度随时间延长而逐渐增加。 B组术后神经纤维数量、密度达到一定数值后无明显变化,但未见肌肉出现变性坏死现象。 C组神经纤维数量明显减少,肌纤维数量亦明显减少,最终大部分肌纤维萎缩伴玻璃样变,间质出血,肉芽组织形成。结论感觉神经移位能有效防止手内在肌萎缩、变性、纤维化,为高位损伤修复后的尺神经的再生、长入创造了良好条件。     

Progressive nerve lesion in a disease-arrested leprosy patient. An electron microscopic study

An ulnar nerve biopsy from a patient with purely neural leprosy of the borderline tuberculoid group, who developed ulnar and median paralysis after 2.5 years of DDS therapy, was examined using light and electron microscopes. The nerve parenchyma was largely replaced by collagen fibrils. There were many onion bulbs similar to those seen in hypertrophic neuropathies. Bizarre fibroblasts such as those seen in hereditary sensory neuropathy were also demonstrated. A few Schwann cells contained M. leprae. It is suggested that considerable proliferation of fibrous tissue may be a reactive phenomenon in response to the continued presence of fragmented M. leprae and their products. Ischemia following a marked progressive increase of intraneural collagen is an important cause of atrophy of Schwann cells followed by segmental demyelination and necrosis of the axons in this healed leprosy patient.     

Systemic scleroderma associated with bilateral ulnar nerve entrapment at the elbow

SIR, Peripheral nervous system involvement in systemic sclerosis(SSc) is rare, and is usually limited to carpal tunnel syndrome[1]. Ulnar nerve involvement secondary to compression of Guyon'scanal has been reported in two patients [2, 3]. Herein, we describefor the first time an SSc patient who developed bilateral ulnarnerve sensorimotor compression at the elbow and improved markedlyafter neurolysis and surgery. A 36-yr-old woman was referred to our unit in March 1998 forinvestigation of bilateral     

Nerve entrapment at the elbow in rheumatoid arthritis.

Although there was some delay in the posterior interosseous branch of the radial nerve conduction time in six of the 152 rheumatoid elbows, in no case were there clinical signs attributable to entrapment of this nerve. Some delay in ulnar nerve conduction was recorded in 27 of the 152 elbows; this was marked in 12. These patients tended to be older and to have more severe disease of the elbow.     

Presence of carpal tunnel syndrome in diabetics: Effect of age,sex, diabetes duration and polyneuropathy

Summary Common thought is that diabetic neuropathy is a predisposing factor to entrapment syndromes. Carpal tunnel syndrome (CTS) is the most frequent entrapment neuropathy; females and old people are most frequently affected (Comi et al., 1978). Prevalence of CTS in diabetics and associated risk factors were studied in 401 patients (208 males and 193 females) with insulin-dependent and non-insulin-dependent diabetes using electrophysiological techniques. Median nerve sensory and motor conduction velocity, ulnar and peroneal nerve motor conduction velocity and sural nerve sensory conduction velocity were investigated in all patients. Diagnostic criteria for CTS were the presence of delayed median nerve sensory conduction velocity in the palm-wrist tract and of increased distal motor latency. Polyneuropathy was defined by slowing-down of conduction velocity in two or more nerves. Forty-five patients (11.2%), 36 females and 9 males, showed CTS. One-hundred-sixty-eight patients (41.8%), 74 females and 94 males, were suffering from peripheral neuropathy. The strongest risk factors for CTS, in order of importance, were: female sex, older age and presence of neuropathy. Polyneuropathy but not CTS was related to duration of diabetes.     

NERVE ENTRAPMENT AT THE ELBOW IN RHEUMATOID ARTHRITIS

Although there was some delay in the posterior interosseousbranch of the radial nerve conduction time in six of the 152rheumatoid elbows, in no case were there clinical signs attributableto entrapment of this nerve. Some delay in ulnar nerve conductionwas recorded in 27 of the 152 elbows; this was marked in 12.These patients tended to be older and to have more severe diseaseof the elbow. ^*Paper read at the Annual Meeting of the British Associationfor Rheumatology and Rehabilitation, London, April 1975.     

Electrodiagnosis in musculo-skeletal disease

Electrodiagnostic tests are an important part of musculoskeletal assessment, but must only be requested after careful clinical examination. Electromyography is of value in the investigation of muscle disease and the distribution of denervation changes. Single fibre studies may be useful in myasthenic problems. Nerve conduction studies can be helpful in defining localised entrapment or more generalised neuropathy. Advances in technology and understanding of the neurophysiological findings have enhanced the value and availability of these tests. Complex studies may be unnecessary if a well defined peripheral nerve problem has been adequately assessed. However, an underlying, more generalised, neuropathy should always be considered. An inadequate referral request may result in an unsatisfactory investigation and it is the responsibility of the referring physician to review the relevance of the electrodiagnostic findings in relation to the clinical findings.     

氟中毒动物模型尺神经应力松弛特性研究

目的研究氟中毒对肘关节尺神经应力松弛的影响,为临床提供生物力学参数。方法复制氟中毒动物模型,取动物模型肘关节尺神经进行应力松弛实验。结果得出了对照组和实验组动物肘关节尺神经应力松弛数据和曲线。结论实验组肘关节尺神经7200S应力松弛量显著低于对照组,氟中毒对动物尺神经应力松弛特性有一定影响。     

氟中毒对肘关节尺神经蠕变影响实验研究

目的研究氟中毒对肘关节尺神经蠕变的影响,为临床提供生物力学参数。方法复制氟中毒动物模型,取动物模型肘关节尺神经进行蠕变实验。结果得出了对照组和实验组动物肘关节尺神经蠕变数据和曲线。结论实验组肘关节尺神经7200S蠕变量显著低于对照组。     

Prevalence of peripheral neuropathy in patients with newly diagnosed rheumatoid arthritis

ObjectiveTo look for the frequency and pattern of neuropathy in Indian patients with rheumatoid arthritis (RA).Patients and MethodsOne hundred newly diagnosed patients with RA (ACR 1987 revised criteria) visiting our hospital, over a period of 3 years were screened. Diabetics, outstation patients, chronic alcoholics, those with any known cause for peripheral neuropathy and patients having an overlap with the other rheumatological illness were excluded. Clinical assessment included detailed history and examination with special reference to extra-articular features and neuropathy with relevant clinical parameters like tender joint count, swollen joint count, etc. Routine laboratory investigations and autoantibodies (RF, ANA, anti-CCP) were obtained on all patients. All the patients with or without clinical manifestations of neuropathy underwent nerve conduction studies. Autonomic function studies were performed in selected patients.ResultsSubjects included 66 patients (M 13:F 53) with mean age of 42 (±13.42) years and median disease duration of 36 months (IQR-13.5, 60). Sensory symptoms were present in 9 patients (13.6%). None had motor symptoms. On neurological examination, 16 patients had sensory (24.2%) and 6 (9.09%) had motor abnormalities. Nerve conduction studies showed abnormality in 25 patients (37.87%). Evidence of entrapment neuropathy was found in 6 patients (9.09%; 5 patients with median nerve involvement [unilateral, 3 and bilateral, 2] and 1 patient with unilateral ulnar nerve involvement), 3 patients had only sensory neuropathy, 5 had mixed sensory motor and 3 had only motor neuropathy. Eight patients (12.12%) had only small fibre neuropathy as detected by sympathetic skin response and quantitative sensory testing.ConclusionThis study shows high prevalence of subclinical neuropathy in Indian patients with RA. This may be an important contributor to disability.     

氟中毒对肘关节尺神经力学性质影响研究

目的研究氟中毒对肘关节尺神经力学性质的影响,为临床提供生物力学参数。方法复制氟中毒动物模型,取动物模型肘关节尺神经进行拉伸实验。结果得出了对照组和实验组动物肘关节尺神经拉伸最大载荷,最大应力,最大应变,弹性模量等数据。结论实验组肘关节尺神经各项拉伸力学性能指标显著低于对照组。     

Clinical and electrophysiological study of peripheral and central neuromuscular changes in connective tissue diseases in children

IntroductionChildren with juvenile connective tissue diseases (JCTDs) may have a wide variety of clinical features ranging from fever or a simple arthritis to complex multisystem autoimmune diseases.Aim of the workTo study clinical and electrophysiological peripheral and central neuromuscular changes in children with connective tissue diseases.Patients and methodsThirty children with different JCTDs were enrolled. Clinical and neurological examination and laboratory investigations were done. Electrophysiological evaluation was performed and included: peripheral nerve conduction studies, late responses, somatosensory evoked potential and electromyography.ResultsTwenty patients had juvenile idiopathic arthritis (JIA) (66.7%), 8 patients had juvenile systemic lupus erythematosus (JSLE) (26.7%), one patient had juvenile systemic sclerosis (JSScl), and one patient had juvenile overlap syndrome (JSScl and polymyositis). Clinical neurologic abnormalities were present in 3 patients (ulnar neuropathy, median neuropathy and polymyositis). Electrophysiological abnormalities were detected in 18 patients (clinical in 3 and subclinical in 15 patients) and included ulnar entrapment neuropathy, median axonal neuropathy, demyelinating sensory motor polyneuropathy, deep peroneal nerve entrapment at the ankle (anterior tarsal tunnel syndrome), prolonged posterior tibial somatosensory evoked potential latency and prolonged H reflex latency not explained by peripheral neuropathy, increased H/M ratio and myopathic motor units. The most common electrophysiological abnormalities were present in patients with JSLE.ConclusionClinical neurological abnormalities are not common in JCTDs whereas subclinical neurological abnormalities are common findings. Juvenile systemic lupus erythematosus had the most common abnormalities among JCTDs. Polyneuropathy in JIA is commonly of demyelinating type. Entrapment neuropathy is less frequent than in adults.     

氟骨症肘关节尺神经损害及畸形矫治的研究

目的 研究氟中毒对尺神经的损害及肘关节畸形矫治。方法 (1)流行病学调查；(2)手术矫治；(3）建立动物模型。结果 氟骨症患尺神经损害与非病区比较差异有显性(P＜0．01)。病人活体尺神经外膜明显增粗增厚、水肿。大鼠尺神经超微结构有明显病理改变。结论 高氟可直接损害尺神经组织，肘关节畸形矫治能改善运动功能。     

The significance of carpal tunnel syndrome in transthyretin Val30Met familial amyloid polyneuropathy

Carpal tunnel syndrome (CTS) is frequently reported in association with amyloidosis. We determined the significance of CTS in transthyretin Val30Met-associated familial amyloid polyneuropathy (FAP ATTR Val30Met) by comparing the electrophysiological indices of the median and ulnar nerves in 58 patients. As a whole, sensory nerve conduction velocity (SCV) was slowed and distal motor latency (DML) was prolonged to a similar extent in the median and ulnar nerves in these patients. The extent of abnormalities in the median nerve was almost similar to that in the ulnar nerve in both early-onset cases from endemic foci and late-onset cases from non-endemic areas. In age-matched idiopathic patients with CTS (20 patients, 27 hands), the slowing of SCV and the prolongation of DML in the median nerve were significant, while the slowing of motor conduction velocity was much less compared to FAP ATTR Val30Met patients. Although concomitant lesions in the ulnar nerve entrapment site at the wrist cannot be excluded, these findings indicate that CTS is not the sole distinctive feature in the majority of FAP ATTR Val30Met patients. The electrophysiological abnormality at the distal portion of the median nerve may be a consequence of polyneuropathy rather than an entrapment injury.     

A suspected case of proximal diabetic neuropathy predominantly presenting with scapulohumeral muscle weakness and deep aching pain

A 48-year-old man with a 14-year history of type 2 diabetes with proliferative diabetic retinopathy and distal symmetrical diabetic polyneuropathy visited our hospital. Eight months later, he subacutely developed difficulty in both shoulder movement and trouble standing up from a squatting position. This was accompanied by severe bilateral shoulder and thigh pain. Magnetic resonance imaging of the brain, cervical and lumbar spine, computed tomography of the shoulder and X-ray films of the cervical spine and shoulder revealed no abnormality. Cerebrospinal fluid showed a mild elevation of protein (0.93 g/l) without cell infiltration. Antiganglioside antibodies and point mutation of mitochondrial DNA at position 3243 were not found. Neuropathology of the sural nerve showed a moderate myelinated fiber loss, active axonal degeneration, but onion-bulb formation, endoneurial or epineurial vasculitis were not observed. Electromyography revealed neurogenic changes in the proximal upper limb muscles. Nerve conduction studies revealed mild bilateral slowing in nerve conduction velocity in both of the upper and lower limbs. The diagnosis of this patients was suspected to be a proximal diabetic neuropathy (diabetic amyotrophy). The pain and muscle weakness had persisted more severely in the shoulder than in the thigh throughout the clinical course. His unbearable symptoms could be partially alleviated by an administration of a selective serotonin reuptake inhibitor, fluvoxamine maleate. Proximal diabetic neuropathy is a rare disabling type of neuropathy, which is characterized with subacute bilateral muscle weakness and wasting in the proximal part of the lower limbs. The involvement of the scapulohumeral region observed in this case is very unusual in proximal diabetic neuropathy.