Anti-Hu antibody neuropathy: a clinical, electrophysiological, and pathological study.

OBJECTIVE: The objective is to report the clinical, electrophysiological, and histopathological features of 16 patients with anti-Hu antibody neuropathy. METHODS: Clinical and electrophysiological data in 16 patients (11 females and 5 males) with positive anti-Hu antibody and nerve biopsy data in 9 cases were analyzed. RESULTS: Cancer was detected in 11 patients, including 9 with small-cell lung cancer. Classical paraneoplastic subacute sensory neuronopathy (SSN) and/or encephalomyelitis (EM) was observed in 7 patients (44%), including 5 with SSN. The most common clinical feature was sensory-motor neuropathy (SMN), accounting for 50% of cases. Though sensory nerve conduction abnormality was the prominent feature in 14 (88%) cases, sensory and motor nerve conduction was abnormal in all cases. Motor nerve conduction findings were typical of axonal degeneration. The most common nerve conduction pattern was that of SMN, with a sensory neuronopathy pattern being observed in only 3 cases. Sural nerve biopsy in 9 patients showed axonal degeneration in all cases and inflammatory cells in 4 cases. CONCLUSIONS: Classical sensory neuronopathy is rarer than expected, both clinically and electrophysiologically. Motor involvement is not uncommon and motor nerve conduction abnormality is frequently seen. A diverse clinical and electrophysiological, and histopathological spectrum was observed in this neuropathy. SIGNIFICANCE: New guidelines for the selection of patients for anti-Hu antibody test are recommended.     

Acute ataxic neuropathy: a clinical, electrophysiological and morphological study

Sensory ataxia as the chief manifestation of acute neuropathy is rather rare. Of the 224 cases of acute polyneuropathy seen over 6 years (1984-1990) only 10 patients (M:F 3:7) had disabling ataxia as the presenting feature. Their ages ranged from 14-61 years. Antecedent febrile illness was present in 6 patients and the peak deficit evolved over 2-25 days. Severe ataxia, paresthesia, distal areflexia and predominant joint sense loss were common to all, motor weakness was either absent or insignificant. CSF was acellular and revealed elevated protein in 3 subjects. All patients had electrophysiological evidence of severe sensory neuropathy with mild or no motor neuropathy. Sural nerve biopsy in one patient showed loss of large, as well as small, diameter myelinated fibres, secondary demyelination, but no evidence of inflammation. At follow up marginal to moderate improvement in ataxia was noted in only 5 patients. Absence of ophthalmoplegia and motor weakness, poor prognosis and characteristic electrophysiological and histopathological observations suggest that acute ataxic neuropathy may be a distinct entity.     

Childhood diabetic neuropathy: a clinical and electrophysiological study

Clinical diabetic neuropathy in childhood is rare, but electrophysiological involvement of the peripheral nerve is more frequent. We assessed clinically and electrophysiologically the peripheral nervous system of 69 children and adolescents suffering from diabetes mellitus (DM). The mean age of the patients was 12.8 years and the mean age at onset of DM was 6.8 years with a mean disease duration of 6.3 years. Seven patients (10%) had clinical neuropathy of which ankle jerk reflex abolition was the most frequent sign. Twenty patients (29%) had a neurophysiological neuropathy prevalently affecting the lower limbs. Peripheral neuropathy was correlated with patient age, older age at onset, duration of DM, height and poor glycaemic control.     

感觉轴索神经病的临床与神经生理特点

目的探讨感觉轴索神经病的临床和神经生理特点。方法选择24例感觉轴索神经 病患者及88名年龄匹配的健康人,电生理检查用皮肤表面电极超强刺激,表面电极记录,测试尺神 经、正中神经和胫神经不同节段的运动潜伏期、运动传导速度及其CMAP波幅、时限,感觉传导速度及其波幅等。结果22例患者表现为肢体麻木和感觉性共济失调,18例患者远端的痛觉和(或)音叉振动觉减退或消失,17例以下肢为著,所有患者均不伴肌无力和肌束震颤。病因包括肺癌、糖尿病、遗传性脊髓小脑共济失调、酒精中毒和药物中毒等。神经传导显示感觉神经动作电位波幅明显下降,感觉神经传导速度减慢,两者与年龄匹配的健康人相比,差异有非常显著性意义(P<0.001、0.01);运动神经传导速度和波幅两组差异没有显著性意义(P>0.05)。结论感觉轴索神经病是一类多因素造成的临床综合征,以感觉纤维轴索损害为主,临床表现为以下肢为著的感觉症状体征,神经传导检测主要的改变是感觉神经动作电位(SNAP)波幅的减低或消失、伴或不伴感觉传导速度的改变,其常见病因有肿瘤、中毒、代谢性疾病、神经系统遗传病等。     

Misonidazole neuropathy: A clinical,electrophysiological and histological study

We studied eight patients with carcinoma of the pharynx and larynx (five cases) or lungs (three cases) who, during treatment with the radiosensitizing drug misonidazole, developed peripheral neuropathy dominated by severe sensory symptoms and signs mainly localized to the lower extremities. The symptoms partially subsided within months after cessation of therapy. Electrophysiological and histological findings indicated an axonal neuropathy with loss of large fibers and secondary demyelination. The neurotoxic property of misonidazole limits its therapeutic use.     

Lamotrigine monotherapy compared with carbamazepine, phenytoin, or valproate monotherapy in patients with epilepsy

OBJECTIVE: This open-label study evaluated the efficacy and tolerability of lamotrigine monotherapy compared with monotherapy with conventional antiepileptic drugs in patients converting from previous monotherapy because of inadequate seizure control or unacceptable side effects. METHODS: This study was conducted in 26 neurology clinics and epilepsy centers throughout the United States. The study enrolled 115 patients with epilepsy converting from previous monotherapy because of inadequate seizure control or unacceptable side effects. Patients were randomized 1:1 to receive 24 weeks of lamotrigine monotherapy or monotherapy with a conventional antiepileptic drug (carbamazepine, phenytoin, or valproate based on physician's choice). Patients were converted during an 相似文献   

Levetiracetam in patients with cortical myoclonus: a clinical and electrophysiological study.

Levetiracetam is a new antiepileptic agent that exerts antimyoclonic effects. We conducted an open-label trial to evaluate the effect of levetiracetam in chronic cortical myoclonus of diverse etiologies and to determine whether levetiracetam affects electrophysiological findings. Sixteen patients, aged between 19 and 72 years, with refractory, chronic, cortical myoclonus were recruited. We assessed myoclonus severity with the Unified Myoclonus Rating Scale (UMRS). The electrophysiological study comprised jerk-locked averaging, somatosensory evoked potentials (SEPs), and long loop reflex I. Levetiracetam was administered add-on at a starting dose of 500 mg twice per day up to the target dose of 50 mg/kg/day. Patients were reevaluated clinically and electrophysiologically 2 weeks after the titration phase. Fourteen patients completed the trial. Posttreatment UMRS scores showed an improvement of myoclonus in all cases. Pretreatment, 9 patients had "giant" SEPs. Posttreatment, the amplitude of these SEPs was reduced by more than 50% in 3 of 9 patients, and the mean N20-P25 amplitude was reduced significantly. Pre- and posttreatment SEP amplitude was not related to myoclonus severity or duration. Levetiracetam is a promising and a relatively easy-to-test antimyoclonic agent, which has the potential to improve significantly the patient's disability; however, its long-term efficacy should be verified in larger controlled studies.     

Differential effects of lacosamide,phenytoin and topiramate on peripheral nerve excitability: An ex vivo electrophysiological study

BackgroundAntiepileptic drugs (AEDs) are mainly used to control cortical hyperexcitability. Some of them (e.g. phenytoin (PHT) and topiramate (TPM)) have also effects on the peripheral nervous system (PNS). Lacosamide (LCM) is a novel AED that stabilizes hyperexcitable neuronal membranes by selectively enhancing the slow inactivation of voltage-gated sodium channels (VGSCs). Although the mechanism of action of LCM is fairly well understood, there are no in vitro data available regarding any possible PNS effects of LCM.ObjectiveTo investigate, in vitro, the effects of LCM on peripheral nerve excitability in comparison with PHT and TPM, two AEDs that act, in part, by stabilizing the fast inactivation state of VGSCs.MethodsExperiments were conducted on the isolated sciatic nerve of the adult rat using standard electrophysiological methods. The effects of LCM on the amplitude and latency of the evoked compound action potential (CAP) during a 48 h period of drug exposure were recorded and compared with the effects of PHT and TPM.ResultsLCM produced inhibitory effects on CAP at concentrations significantly higher than the therapeutic levels (>25 μg/ml). At these concentrations (62.57–125.15 μg/ml), an acute and immediate increment of the latency and decrement of the amplitude of the CAP were observed. In contrast to LCM, PHT caused an acute decrement in the amplitude as well as an increment in the latency of the CAP even at subtherapeutic levels (5 μg/ml). With regard to TPM, the amplitude of the CAP was not affected at the supratherapeutic concentrations but at the therapeutic concentration of 33.94 μg/ml a reduced decrement of the CAP amplitude compared to the controls was observed.ConclusionsLCM, PHT and TPM exert differential effects on peripheral nerve excitability. PHT inhibited the sciatic nerve CAP even at subtherapeutic levels whereas LCM was safe within the therapeutic concentration range. TPM did not affect the CAP amplitude even at high supratherapeutic concentrations whereas in the therapeutic range a neuroprotective effect was observed. Possible underlying mechanisms and the clinical implications of these findings are discussed.     

Peripheral neuropathy in chronic occupational inorganic lead exposure: a clinical and electrophysiological study.

BACKGROUND AND OBJECTIVES: Traditionally the neuromuscular disorder associated with lead poisoning has been purely motor. This study assessed peripheral nerve function clinically and electrophysiologically in 46 patients with neuropathic features out of a total population of 151 workers with raised blood and/or urinary lead concentrations. RESULTS: Average duration of occupational exposure for the neuropathic group ranged from 8-47 years (mean 21.7). Their mean blood lead concentration (SD) was 63.9 (18.3) microg/dl (normal <40), urinary lead 8.6 (3.3) microg/dl (normal<5.0), urinary coproporphyrins 66.7 (38.4) microg/g creatinine (20-80), urinary aminolaevulinic acid 1.54 (0.39) mg/g creatinine (0.5-2.5). All 46 had distal paraesthesiae, pain, impaired pin prick sensation, diminished or absent ankle jerks, and autonomic vasomotor or sudomotor disturbances. Reduced vibration sensation and postural hypotension were present in all 20 studied. None of these 46 patients had motor abnormalities. Motor conduction velocity and compound muscle action potential amplitudes were normal, with marginally prolonged distal motor latencies. Sensory nerve action potential amplitudes lay at the lower end of the normal range, and the distal sensory latencies were prolonged. No direct correlation was found between the biochemical variables, and the clinical or electrophysiological data. CONCLUSIONS: One additional patient was seen with shorter term exposure to lead fumes with subacute development of colicky abdominal pain, severe limb weakness, and only minor sensory symptoms. Unlike the patients chronically exposed to lead, he had massively raised porphyrins (aminolaevulinic acid 21 mg/g creatinine, coproporphyrins 2102 microg/g creatinine). Patients with unusually long term inorganic lead exposure showed mild sensory and autonomic neuropathic features rather than the motor neuropathy classically attributed to lead toxicity. It is proposed that the traditional motor syndrome associated with subacute lead poisoning is more likely to be a form of lead induced porphyria rather than a direct neurotoxic effect of lead.     

Subacute arsenic neuropathy: clinical and electrophysiological observations.

Two patients with subacute arsenic neuropathy are described and the results of serial conduction velocity determinations from the very early stages of the illness to partial recovery are reported. Sensory and motor deficits were maximal within four weeks of the estimated time of exposure. Recovery was slow, with only partial improvement of the neurological deficits two years after onset of the illness. Progressive slowing of motor conduction velocity was observed in the first three months followed by a gradual increase in velocity thereafter. The possible mechanisms underlying the temporal profile of the electrophysiological changes are considered. The need for initiating chelation therapy before the development of the neuropathy is emphasised.     

Follow-up of patients with suspected multiple sclerosis: a clinical and electrophysiological study.

We re-examined 21 patients with suspected multiple sclerosis, seven classified as probable and 14 as possible cases. At the first investigation all patients but two had abnormal visual evoked potentials and somatosensory evoked potentials, or both. All but three had an increased intracerebral production of immunoglobulin G expressed by the cerebrospinal fluid IgG index. At follow-up two to four years later, 13 of 16 patients (81%) in whom both evoked potentials and IgG index were abnormal initially had entered into a higher multiple sclerosis diagnostic class. In the five patients in whom either evoked potentials or IgG index were normal the original diagnosis was unchanged.     

Peripheral neuropathy associated with; plasma cell dyscrasia: a clinical and electrophysiological follow-up study

Thirteen patients with polyneuropathy associated with plasma cell dyscrasia had serial electrophysiological studies. Five patients with monoclonal IgG had motor and/or sensory symptoms of which 4 correlated with slow motor and sensory nerve conduction. The 4 patients with monoclonal IgM reactive with myelin-associated glycoprotein (MAG), had predominantly motor symptoms, demyelination in the nerve biopsy and slow motor and sensory nerve conduction. Four patients with monoclonal IgM without anti-MAG activity had mainly sensory symptoms, axonal neuropathy on nerve pathology and slow or absent sensory nerve conduction. After treatment with plasmapheresis and chemotherapy 9 patients improved clinically and 4 were unchanged. Criteria for electrophysiologic improvement were presence of sensory or motor responses that were absent before treatment, conduction velocity increased by more than 10 m/s and increase of amplitude by more than 100%. Electrophysiological studies showed improvement in 7, were unchanged in 4, and worse in 2. Sensory velocities in ulnar and sural nerves were significantly improved following treatment (P less than 0.002) and the same trend was noted for the sensory velocity in the median nerve (P less than 0.19). We conclude that nerve conduction studies in combination with clinical examinations are useful in documenting the effects of treatment in these neuropathies.     

Thalidomide neuropathy: clinical, electrophysiological and neuroradiological features

OBJECTIVE: Thalidomide is a promising therapy for multiple myeloma. Sensory neuropathy is a side effect of thalidomide and resulted to be partially reversible in 50% of cases, suggesting a sensory ganglionopathy. Spinal cord magnetic resonance imaging (MRI) was found to be useful in the diagnosis of sensory ganglionopathies and we use it to determine if thalidomide neuropathy has features of a ganglionopathy. MATERIAL AND METHODS: Six patients with multiple myeloma developed thalidomide-induced polyneuropathy. Nerve conduction studies, somatosensory-evoked potentials (SEPs) and cervical and dorsal spinal cord MRI were obtained in all. RESULTS: All patients had a sensory neuropathy, with clinical or electrophysiological abnormalities involving all four limbs. Spinal cord MRI showed high signal intensity in the posterior columns in only one patient, with abnormal central conduction time at SEPs. CONCLUSION: Our results suggest that thalidomide can induce either an axonal length-dependent neuropathy or, less frequently, a ganglionopathy.     

Rheumatoid neuropathy: a histological and electrophysiological study

Peripheral nerves in five patients with rheumatoid neuropathy were examined electrophysiologically and by sural nerve biopsy. There was close correlation between the clinical severity of the disease and the degree of nerve damage found histologically and by EMG. Group 1 patients with a mild distal sensory neuropathy showed varying degrees of axonal degeneration in the large myelinated fibres and some segmental demyelination. Group 2 patients with a severe, rapidly progressive sensori-motor neuropathy had extensive loss of myelinated fibres. In one case all the large fibres had degenerated. The second case had lost both large and small myelinated fibres together with many of the non-myelinated axons. The major nerve damage in both groups appeared to be axonal degeneration but some segmental demyelination was detected. Occlusive vascular disease in the vasa nervorum was considered to be the major cause of the nerve damage.     

Painful dysaesthesias following peripheral nerve injury: a clinical and electrophysiological study

Thirty-three patients with complete median, ulnar or digital nerve transections were studied 4 months to 13 years subsequent to suture or nerve grafting. In all cases, sensory disturbances, in terms of paraesthesia or hypaesthesia, were encountered. Painful or unpleasant symptoms, allodynia or hyperpathia, were observed most frequently in patients with poor recovery. The clinical findings and the patients' subjective complaints were correlated to microneurographic single fibre recordings of regenerated cutaneous mechanoreceptors. In more than 80% of the recordings, discharge properties of regenerated receptors, thresholds and a variety of other electrophysiological data were similar or equal to normal controls. Less than 20% of the receptors exhibited atypical properties suggesting defective steady-state regeneration. The ratio of rapidly adapting (RA-units) to slowly adapting mechanoreceptors (SA-units) was inverse in relation to normals. The density of regenerated RA-receptors was higher in the proximal than in the distal part of the reinnervated area. This paralleled the clinical finding of reduced sensory discrimination in these cases and suggests that SA-units may regenerate preferentially. In painful conditions no single fibres could be recorded, reflecting the relative paucity of fibres and probably the atrophy of the nerve. The results of the microstimulation experiments, although less reliable, revealed some evidence that the central processing of regenerated units is abnormal. Clinical and electrophysiological data supported this concept of central changes underlying some of the phenomena observed during peripheral nerve regeneration.     

Delayed neuropathy after organophosphorus insecticide (Dipterex) poisoning: a clinical, electrophysiological and nerve biopsy study.

Clinical, electrophysiological and histological findings in four patients accidentally poisoned with the organophosphorus insecticide Dipterex are reported. Three to five weeks after insecticide ingestion signs of a distal sensorimotor (preponderantly motor) neuropathy occurred. The patients complained of paraesthesia in the lower limbs, and two of them of very disagreeable pricking sensation in the soles of the feet, responsive to carbamazepine. They showed distal weakness mainly of the legs, footdrop , difficult gait and muscle hypotonia. Ankle jerk was abolished while other tendon reflexes persisted. Two months or even later after poisoning, knee jerks in all the patients were very brisk and more and less accompanied by other pyramidal signs (patellar clonus, abolishment of abdominal cutaneous reflexes, Babinski's sign). Clinical, electrophysiological and nerve biopsy data revealed a "dying-back" neuropathy in our patients. Distal muscle fatigue was confirmed by failure of neuromuscular transmission on repetitive nerve stimulation.     

Progressive sensory neuropathy in patients without carcinoma: a disorder with distinctive clinical and electrophysiological findings

Seven patients with severe progressive impairment of kinesthetic sense, mild dysfunction of cutaneous sense, and sparing of motor function were examined during a 3-year period. The clinical and electrophysiological findings are described in detail. None of these seven has had evidence of cancer despite a thorough investigation and a 3- to 16-year (average, 7 years) period of symptoms. These patients' symptoms were indistinguishable from those of patients with sensory neuropathy and coexistent carcinoma, suggesting that progressive sensory neuropathy is not invariably associated with carcinoma.     

Impairment,disability, or handicap in peripheral neuropathy: analysis of the use of outcome measures in clinical trials in patients with peripheral neuropathies.

Outcome measures can be classified into measures of impairment, disability, and handicap. To investigate the biological effect of treatment, measures of impairment are appropriate. Studies investigating whether patients benefit from treatment in terms of improvement of functional health, however, require disability or handicap measures. In a review of the medical literature between 1978 and 1993, 73 controlled intervention studies in patients with peripheral neuropathies were found. Disability or handicap measures were used in two of 54 studies in patients with diabetic neuropathy, in two of six studies in patients with chronic inflammatory demyelinating polyneuropathy, in none of five studies in a mixed group of patients, and in all eight studies in patients with Guillain-Barré syndrome. The limited use of disability and handicap measures in patients with diabetic and mixed neuropathies can be explained by the experimental nature of most studies. In four of six studies, however, in patients with chronic inflammatory demyelinating polyneuropathy or neuropathy associated with monoclonal gammopathy that were designed to assess effectiveness of treatment, the choice of outcome measures was not appropriate. It is concluded that in the design of intervention studies in patients with peripheral neuropathy more attention should be paid to a proper choice of suitable outcome measures to assess the effectiveness of treatment.