多灶性运动神经病5例分析及文献回顾

目的探讨多灶性运动神经病(MMN)的临床和电生理特点及诊断标准。方法分析我院收治的5例MMN患者的临床表现,电生理改变,实验室检查结果及治疗情况,并结合相关文献报道综合分析。结果5例病例均为男性患者,发病年龄在28～42岁,均以单肢无力为首发症状,远端重于近端;3例伴有肌肉萎缩;5例均无感觉障碍,腱反射减低,无病理反射;肌电图及神经传导速度检查提示神经源性损害,无巨大电位,3例出现了典型的运动神经多发传导阻滞(CB);4例经免疫球蛋白治疗后有不同程度好转。结论MMN的诊断应注意与慢性格林巴利(CIDP)及运动神经元病(MND)相鉴别,结合临床及电生理特点综合分析,以免误诊。     

Peripheral neuropathy in hypereosinophilic syndrome with vasculitis.

A 53-year-old woman with non-productive cough of unexplained aetiology for two years, developed a sub-acute symmetrical polyneuropathy involving all four limbs, accompanied by fever, cutaneous rash and myalgia in lower limbs. Laboratory studies revealed a leukocytosis with 70% eosinophils and excluded any cause for the hypereosinophilia. An echocardiogram showed increase in thickness of the atrial septum. Motor and sensory conduction velocity were reduced in ulnar and median nerve and unrecordable in peroneal and tibial nerves. A sural nerve biopsy showed an axonal degeneration involving myelinated and unmyelinated fibers as well as a vasculitis with fibrinoid necrosis and perivascular infiltration of eosinophils. There was considerable clinical and laboratory improvement with the use of steroids. The differential diagnosis between idiopathic hypereosinophilic syndrome and other disorders known to course with vasculitis and hypereosinophilia is discussed.     

Multifocal motor neuropathy

We present a review of the literature on multifocal motor neuropathy (MMN), a rare neurological disorder which has features in common with both chronic inflammatory demyelinating neuropathy and lower motor neuron disease. Clinically, MMN is characterised by slowly progressive asymmetrical limb weakness, usually most prominent in the forearms. Weakness may be associated with muscle wasting, fasciculations and decreased tendon reflexes. Serum anti-GM1 ganglioside antibody titres may be increased. The diagnostic hallmark of MMN is the electrophysiological demonstration of persistent localised motor conduction blocks, with otherwise normal or near-normal motor and sensory conduction velocities. The pathogenesis of MMN has not been elucidated completely. There is, however, substantial evidence for an autoimmune mechanism. Based on the possible involvement of the immune system in the pathogenesis of MMN the therapeutic efficacy of several immunomodulatory drugs has been tested. Treatment of MMN patients with human immunoglobulin or cyclophosphamide is usually followed by a marked improvement of strength. The finding that MMN is a potentially treatable disorder underscores the importance of distinguishing MMN from lower motor neuron disease, for which no effective therapy is currently available. Received: 14 October 1996 Accepted: 28 October 1996     

Peripheral nerve involvement in Churg-Strauss syndrome

Summary Peripheral neuropathy associated with bronchial asthma, multisystem organ dysfunction and idiopathic hypereosinophilia may be found in Churg-Strauss syndrome, hypereosinophilic syndrome and polyarteritis nodosa. Some authors have diagnosed their patients according to the presence in tissue biopsies of the three histological criteria of Churg and Strauss (necrotizing vasculitis, tissue eosinophilic infiltration, extravascular granulomas). We have observed three patients with a common history of a prodromal phase of allergic diseases (bronchial asthma and rhinitis) followed by a vasculitic phase with mononeuritis multiplex, purpura and arthritis, associated with hypereosinophilia of more than 1500 cells/mm³. All responded well to steroid treatment. Sural nerve biopsy revealed true vasculitis in two of these cases and a mild perivascular inflammatory infiltration in the other. On the basis of their characteristic clinical pattern, we think that our cases best fit the diagnosis of Churg-Strauss syndrome even though the typical histological features were not found in the sural nerves examined.     

Multifocal peripheral neuropathy in eosinophilic fasciitis

Summary Polyneuropathy may complicate eosinophilia associated connective tissue disease. We report a multifocal neuropathy in a patient with eosinophilic fasciitis proven by demonstrating an eosinophilic cellular infiltrate in a fascial biopsy specimen from the forearm. Sural nerve biopsy revealed lymphocytic cuffing of epineural arterioles. Although described in thel-tryptophan-related eosinophilia-myalgia syndrome, peripheral neuropathy with these features has not been previously noted in a patient with eosinophilic fasciitis who had not consumedl-tryptophan.     

Multifocal motor neuropathy: Clinical and electrophysiological findings

Multifocal motor neuropathy (MMN) can be differentiated from motor neuron disease by electrophysiological evidence of conduction block. To increase the probability of recording conduction block, we studied the whole nerve length including proximal segments in 84 patients with pure motor syndromes, using a special stimulation technique. In 8 patients, the diagnosis of MMN was confirmed by electrophysiological evidence of conduction block or temporal dispersion. The typical clinical picture of MMN with chronic progressive, asymmetrical, marked distal weakness was observed in our patients. Electrophysiological routine tests of distal nerves were usually normal except in nerve segments with conduction block. In 4 patients, conduction block could be recorded only in proximal nerve segments or spinal roots. All patients showed rapid improvement of clinical features and parallel reduction of conduction block during or after highdose intravenous immunoglobulin (ivIG) therapy, supporting the diagnosis of an immune-mediated neuropathy. Three of them are now in remission without any therapy, whereas 5 still receive a regular ivIG course every 2-12 weeks as long-term treatment. In all patients with pure or predominantly motor syndromes and normal findings in electrophysiological routine tests of distal nerve segments, there should be proximal conduction block studies to avoid overlooking a treatable disorder such as MMN.     

Cryoglobulinemic vasculitis in a patient with CREST syndrome

Cryoglobulinemic vasculitis is a rare entity. Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. We report a patient with cryoglobulinemic vasculitis with CREST syndrome who did not have typical clinical features of vasculitis. This 58-year-old woman presented with mild generalized weakness and a diagnosis of CREST syndrome, which included Raynaud’s syndrome, dysphagia and telangiectasias. She was positive for serum cryoglobulins, which led to a sural nerve biopsy. The biopsy results were consistent with cryoglobulinemic vasculitis. Cryoglobulinemic vasculitis has not been previously reported in CREST syndrome to our knowledge. Additionally, the patient also had limited clinical symptoms. Our patient displays the importance of checking for cryoglobulins and obtaining a nerve biopsy when the serum is positive. Both of these diagnostic tests were integral for directing appropriate treatment for this patient.     

Multifocal motor neuropathy: Electrodiagnostic features

Diagnosis of multifocal motor neuropathy (MMN), a syndrome characterized by progressive asymmetric weakness with intact sensation, is important because the disorder often responds to treatment. Multifocal partial motor conduction block (PMCB) has been emphasized as a cardinal feature in the diagnosis of this syndrome, but detailed nerve conduction studies are not available. Nine patients, ages 28–58, had chronic, progressive, asymmetric, predominantly distal limb weakness for 5–18 years. Sensation was normal and reflexes were reduced asymmetrically. Although all 9 demonstrated PMCB localized to short nerve segments, additional features of multifocal motor demyelination were present, including temporal dispersion (5 patients), segmentally reduced motor nerve conduction velocity (7 patients), prolonged distal motor latency (4 patients), and prolonged F-wave latency (9 patients). The strength of all patients improved after treatment with human immune globulin. A reduction in the degree of PMCB or an increase in the distal motor amplitude or both accompanied the clinical improvement. These studies suggest that patients with MMN demonstrate widespread evidence of motor demyelination in addition to the well-described PMCB, and that reduction of PMCB accounts for the increase in strength following therapy. © 1994 John Wiley & Sons, Inc.     

Multifocal motor neuropathy with conduction block misdiagnosed as multiple entrapment neuropathies

We describe a 58-year-old male with a few years history of multifocal weakness in the upper limbs with minimal to absent sensory complaints. He was diagnosed as having multiple compressive neuropathies, which required repeated decompressive surgeries. Electrodiagnostic studies prior to diagnosis were limited to a few nerves, evaluating only distal segments. Because of delay in making the diagnosis, his condition progressed, and possibly because of the unnecessary surgeries, he developed atrophy in some muscles, which resulted in significant motor disability. He was later diagnosed as having multifocal motor neuropathy with conduction block and has partially responded to intravenous immunoglobulin therapy. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21:813–815, 1998.     

Peripheral neuropathy in hypereosinophilic syndrome

We evaluated seven patients with the hypereosinophilic syndrome (HES) to define the clinicopathologic spectrum of the peripheral neuropathy. Clinically, three had evident polyneuropathy; the others were asymptomatic, although they had electrophysiologic evidence of neuropathy. Nerve conduction studies and EMG were compatible with axonal neuropathy. Morphometry of sural nerves from four patients ranged from normal to marked axonal loss, more prominent in large myelinated fibers. Demyelination was rare, and there was no evidence of vasculitis. Neuropathy may be produced by an eosinophil-derived neurotoxin.     

多灶性运动神经病10例分析

目的 探讨多灶性运动神经病(multifocal motor neuropathy，MMN)的临床特点和诊断标准。方法 回顾性分析1998年～2001年间本院神经内科收治的10例MMN患者的临床表现、电生理表现、化验检查、病理结果。结果 本组病例的临床特点是：10例患者均为男性，平均发病年龄42．1岁，症状均以单肢无力首发，远端重于近端，伴有肌肉萎缩，半数有肌束颤动；9／10有腱反射减低，感觉症状少，颅神经不受累；5／10脑脊液蛋白增高，5／10血CPK增高。电生理检查7／10出现了典型的运动神经多发CB，最常见的发生部位是正中神经、尺神经和胫神经；3／10病例经神经活检主要表现为脱髓鞘改变，伴有轻中度轴索损害，病程较长的可有髓球形成，无炎性细胞浸润。结论 应综合临床表现、电生理特点等作出MMN的诊断。     

Multifocal motor neuropathy without overt conduction block

One of the defining electrophysiological characteristics of multifocal motor neuropathy (MMN) is focal motor nerve conduction block. We have noted occasional patients with typical clinical features of MMN in whom there is no demonstrable conduction block. Upon review of 5 such cases, we conclude that otherwise typical MMN may present without overt conduction block. This subset of patients does not otherwise differ from patients with MMN in whom this hallmark electrodiagnostic feature is present. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21: 243–245, 1998.     

Multifocal motor neuropathy presenting as ophthalmoplegia

A 45-year-old man developed ophthalmoplegia and subsequently multiple cranial nerve palsies in association with bibrachial paresis. Investigations revealed evidence of conduction block occurring at a very proximal location (i.e., the spinal roots) and seemingly sparing sensory fibers. Other causes were ruled out and a diagnosis of multifocal motor neuropathy with conduction block (MMN) was suggested. The patient responded to cyclophosphamide. Differentiating features between MMN and chronic inflammatory demyelinating polyradioneuropathy (CIDP) are discussed. This case demonstrates that MMN may rarely present with ophthalmoparesis and also demonstrates that features of MMN and CIDP may overlap. © 1997 John Wiley & Sons, Inc. Muscle Nerve, 20, 347–351, 1997.     

多灶性运动神经病的初步研究

目的对６例诊断为多灶性运动神经病（ＭＭＮ）的病例进行研究，并就一些相关问题进行讨论。方法采用ＣｏｕｎｔｅｒｐｏｉｎｔＭＫⅡ肌电图仪进行电生理研究，ＥＬＩＳＡ法测血及脑脊液中抗神经节苷脂ＧＭ１抗体，５例病人行腓肠神经活检。结果６例病人中５例为上肢起病，上肢受累为主，远端重。５例有感觉症状，但仅２例查体有轻度感觉缺失。均表现为无力、肌萎缩和腱反射减弱或消失。所有病人都有运动传导阻滞。４例病人血抗ＧＭ１抗体滴度升高。病理示脱鞘及髓鞘再生。结论结果表明ＭＭＮ为一高度特异的脱鞘性周围神经病，电生理以运动传导阻滞为特点。多数病人对免疫治疗有效。此研究与国外报道一致，但尚需进一步的研究     

Multifocal motor neuropathy with high titers of anti‐MAG antibodies

Multifocal motor neuropathy (MMN) and anti‐myelin‐associated glycoprotein (anti‐MAG)‐associated neuropathy are clinically and electrophysiologically distinct entities. We describe a patient with characteristic features of both neuropathies, raising the possibility of an overlap syndrome. A 49‐year‐old patient reported a history of slowly progressive predominantly distal tetraparesis, with mild sensory deficits. Nerve conduction studies demonstrated persistent motor conduction blocks outside compression sites, typical of MMN. Laboratory findings revealed persistently high titers of anti‐MAG immunoglobulin Mλ (IgMλ) paraprotein in the context of a monoclonal gammapathy of unknown significance. Skin biopsy of distal lower limb revealed IgM positive terminal nerve perineurium deposits. This case suggests that the distinction between subtypes of chronic inflammatory neuropathies may not be as clear as initially thought, and that the pattern of pathogenicity of anti‐MAG antibodies may vary.     

Multifocal sensory demyelinating neuropathy: Report of a case

Introduction: Multifocal sensory demyelinating neuropathy has not been adequately reported in the literature. Methods: A 42‐year‐old man with numbness of the left hand for 3 years and of the right hand for 6 months had a pure multifocal sensory neuropathy involving both hands, most prominently affecting 2‐point discrimination, number writing, and object recognition of the left hand. Near‐nerve needle sensory and mixed nerve conduction studies were performed on the left ulnar nerve. Results: Studies of the left ulnar nerve documented a demyelinating neuropathy characterized by temporal dispersion and marked decrease in the amplitudes of the sensory and mixed compound nerve potentials in the above‐elbow‐axilla segment. With intravenous immunoglobulin treatment, there was improvement in his neuropathic condition. Conclusion: In this study I describe a case of multifocal sensory demyelinating neuropathy as a counterpart of multifocal motor neuropathy. Muscle Nerve 56 : 825–828, 2017     

Proliferation of epineurial capillaries and smooth muscle cells in angiopathic peripheral neuropathy

Summary Proliferation of epineurial capillaries and smooth muscle cells in human sural nerves has been documented. These are basically independant changes, although both can occur in the same nerve. Proliferated epineurial capillaries were seen in association with arterial stenosis or occlusion with or without granulating or granulomatous inflammatory reactions. Although non-specific they appear to be of considerable diagnostic value indicating compensatory hypervascularisation subsequent to peripheral focal ischemia. Separation and numerical increase of epineurial smooth muscle cells, on the other hand, was also recognised as a significant though non-specific alteration occurring in various inflammatory, immunogenetic, or other, non-inflammatory angiopathies. The youngest (1.3 years) and the oldest individual (104 years) studied, as well as many other documented and non-documented cases, did not show this type of change. Smooth muscle cells may survive isolated or focally separated from adjacent vessel walls in the epineurium.Dedicated to Prof. F. Seitelberger on the occasion of his seventieth birthdaySupported in part by Deutsche Forschungsgemeinschaft (Schr 195/5-6)Presented in part at the XIth Meeting of Swiss Neuropathologists with International Participation, St. Moritz, Switzerland, March 15th–19th, 1986     

Diagnosis of chronic neuropathy

Chronic neuropathy is the most frequent condition affecting the peripheral nervous system. It includes symmetrical polyneuropathies, multifocal mononeuropathies, mononeuropathies and radiculopathies. Mononeuropathies are mainly due to compression or entrapment and are not discussed in this review, which focuses on polyneuropathies and multifocal mononeuropathies with a chronic course, lasting for more than 2 months. Their diagnosis requires a systematic approach including clinical, electrophysiological, biological, and sometimes pathological investigations. The search for an etiological diagnosis may involve a very wide panel of hereditary and acquired diseases. Despite progress in the understanding and diagnosis of many chronic neuropathies, some of them remain unresolved. Received: 19 June 1998 Accepted: 27 June 1998