摘要:

Erythromelaigia is a poorly understood clinical syndrome characterized by painful, hot, red extremities. We assessed the frequency and types of abnormalities observed during tests of vascular, peripheral neurophysiologic, and autonomic function in patients with erythromelalgia.Methods Of“ 163 charts of patients fulfilling the clinical diagnosis of erythromelalgia. 93 patients underwent vascular studies Five of them had detailed vascular studies in 10 affected lower extremities performed before and during symptoms, fifty-four patients underwent neurophysiologic testing, 27 had autonomic reflex screening (ARS). and two had recordings of peripheral autonomic surface potentials (PASP).Results. Measurements in the toes during symptoms revealed a mean temperature increase of 11.6oC (P= 0,00011 along with a laser flow increase from a mean of 6.8 mL/min per 100 g tissue to 76.5 mL/min per 100 g tissue (P<.0.0001). Baseline TcPO2;in the feet decreased by 6.7 mmHg (P= 0.032) during symptoms. Twenty-one of 54 electromyographic recordings were abnormal: all fulfilled the criteria for axonal neuropathy. Seventeen of 27 ARSs and one PASP showed severe postganglionic sudomotor impairment; five of 17 additionally had peripheral adrenergic dysfunction.Conclusions During symptoms, an increase in flow and temperature is accompanied paradoxically by a decrease in oxygenation of the affected area; a high proportion of patients have a distal small fiber neuropathy with selective involvement of cutaneous sympathetic fibers; in addition, large fiber neuropathy is often present

ISSN:1522-0443    

出版商:OVID    

年代:1999

数据来源: OVID

摘要:

Needle electromyography (EMG) findings in patients with myopathy can be classified as “irritable” or “non-irritable,” depending on the presence or absence of “abnormal spontaneous activity” in the form of fibrillation potentials, positive sharp waves, or complex, repetitive discharges. An irritable myopathy suggests either an active necrotizing myopathic process and/or unstable muscle membrane potential. Although inflammatory myopathies are traditionally considered to show irritable myopathic changes on EMG, the precise incidence of these changes is not known. We retrospectively reviewed the EMG findings in 178 consecutive patients with biopsy-confirmed myopathy. Inflammatory myopathy was defined on the basis of biopsy findings combined with clinical features. Needle EMG showed “irritable” myopathic features in 66% of the patients in the inflammatory histology myopathic group and in 30% patients with non-inflammatory histology (p <0.00l). Overall, irritable EMG findings predicted an inflammatory histology in only 44% of patients. The degree and distribution of irritability on EMG did not improve the probability of finding inflammatory myopathy. By the same token, non-irritable EMG findings also did not exclude inflammatory histology. We conclude that irritable myopathy on EMG as defined above is not very sensitive and not very specific for predicting an inflammatory histology.

ISSN:1522-0443    

出版商:OVID    

年代:1999

数据来源: OVID

摘要:

Leprosy still remains a major cause of peripheral neuropathy, particularly in Asia, Africa, and immigrants from these endemic regions of the world. Lepromatous neuropathy can manifest as mononeuropathies, mononeuropachy multiplex, or as a generalized sensorimotor polyneuropathy. The electrophysiologic studies reveal a primarily axoral process, although there may be demyeltnating features as well. Long-term treatment with dapsone, rifampin, and clofazimine is required, A potential complication of therapy, particularly in the borderline patient, is the reversal reaction, a condition caused by upregulation of the cellular immune response. In this condition, patients may develop an exacerbation of their skin lesions and neuropathy, Because lepromatous neuropathy is not rare, neurologists need to be aware of the complex nature of the clinical manifestations, pathogenesis, and treatment of leprosy

ISSN:1522-0443    

出版商:OVID    

年代:1999

数据来源: OVID

摘要:

We report a 63-year-old woman with common variable immunodeficiency (CVID) and an indolent distal sensory neuropathy. Despite having negative serologic tests for Sjögren's syndrome, evaluation of the neuropathy led to a new diagnosis of Sjögren's syndrome based on the presence of sicca symptoms, an abnormal Schirmer's test, and histologic evidence of sialadenitis. In addition, a thymoma was discovered. We conclude that the occurrence of thymoma, CVID, and Sjogren's syndrome reflect a state of systemic autoimmune dysregulation in this patient. We also reiterate the diagnostic importance of salivary gland biopsy in patients with sicca symptoms and sensory neuropathy.

ISSN:1522-0443    

出版商:OVID    

年代:1999

数据来源: OVID

摘要:

We describe a patient with Guillain-Barré syndrome in whom electrophysiological studies performed 9 days after onset demonstrated multiple focal motor and sensory conduction blocks. Repeat studies 8 weeks later demonstrated complete absence of motor and sensory responses in the arm or leg. These findings suggest that early focal conduction block in sensory nerves might account for sensory symptoms and that there is a spectrum of immune-mediated injury that can proceed from myelin damage to axonal destruction.

ISSN:1522-0443    

出版商:OVID    

年代:1999

数据来源: OVID

摘要:

We report three non-inbred patients with Val 107 transthyretin (TTR) amyloidosis. Clinical features were remarkable by the combination of peripheral polyneuropathy, carpal tunnel syndrome, cardiomyopathy, and epilepsy. Pathologic examination disclosed unusual striking systemic amyloid angiopathy in all studied tissues including nerve, muscle, gut, lung, salivary glands, and synovial membrane. It appears that the rare TTR Val 107 variant causes a peculiar familial amyloid syndrome characterized by both widespread systemic TTR amyloidosis and central nervous system deposition sufficient to cause seizures, pointing out the extent of TTR amyloidosis phenotypic heterogeneity.

ISSN:1522-0443    

出版商:OVID    

年代:1999

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:1999

数据来源: OVID

摘要:

Chronic inflammatory demyelinating polyradiculoneuropathy is an immune-mediated disorder which usually responds to treatment Accurate diagnosis is essential and depends on the clinician's ability to synthesize a variety of clinical, electrodiagnostic, and laboratory data. The prognosis is generally good, This review summarizes the pathogenesis, clinical presentation, evaluation, treatment, and prognosis of adults and children with CIDP with practical guidelines for selection of the most appropriate diagnostic tests and treatment modalities.

ISSN:1522-0443    

出版商:OVID    

年代:1999

数据来源: OVID

摘要:

Paraneoplastic neuropathies are a clinically diverse group of disorders associated with a variety of neoplasms. This article reviews the clinical features, autoimmune aspects, diagnostic approach, and treatment options for patients with paraneoplastic neuropathies

ISSN:1522-0443    

出版商:OVID    

年代:1999

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:1999

数据来源: OVID