摘要:

Objectives-To evaluate the diagnostic value of nerve and muscle biopsy in suspected cases of vasculitis and their correlation with the clinical and electrophysiological data.MethodWe conducted a retrospective review of I 15 nerve and muscle biopsy specimens from cases in the past 20 years at the University of Alabama at Birmingham (UAB) Muscle and Nerve Histopathology Laboratory. Clinical and electromyography data in available cases were analyzed to evaluate the histopathologic correlation,:Results:The diagnostic sensitivity of nerve biopsy was 39%, Nerve biopsy showed a statistically higher diagnostic yield (P= 0–0001) than muscle biopsy (17%), although muscle biopsies resulted in a more definite diagnosis m 3%. of cases. The highest diagnostic yield (73%) of vasculitis on nerve biopsy was observed in patients with known rheumatologic disease and accompanying neuropathy or myopathy. Nerve conduction study was able to identify diffuse neuropathy in the majority of patients with vasculitis, including asymptomatic neuropathy. Abnormal sural nerve conduction was highly correlated (P= 0.03) with positive nerve biopsy. There was a wide spectrum of neurologic manifestations in vasculitic neuropathy, with the most common clinical manifestation of vasculitic neuropathy being polyneuropathy.Conclusions-Nerve biopsy is superior to muscle biopsy for the diagnosis of vasculitis among suspected cases of vasculitis. The highest diagnostic yield of nerve biopsy is observed when patients with known rheumatologic diseases have neuropathy or myopathy. Abnormal sural nerve conduction can be used as a guide for nerve biopsy.

ISSN:1522-0443    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Objectives:To assess the presence of mitochondria! dysfunction in 18 patients with multiple symmetric lipomatosis (MSL).Methods:Electromyography and nerve conduction study were performed in 15 patients with MSL and autonomic tests in 14. Nerve biopsy was done in four patients and muscle biopsy in six. Mitochondrial enzyme activities were measured in six muscle biopsies. We investigated myoclonic epilepsy ragged red fibers (MERRF) point mutation and multiple deletions in mtDNA with PCR, enzyme restriction digestion, and Southern blot analysis in lymphocyte DNA, or in muscle DNA when available.Results:Clinical or electrophysiological signs of polyneuropathy were found in 12 patients. Peroneal nerve biopsy showed decreased myelinated fibers. In muscle biopsies there were hyporeactive areas and subsar-colemmal rims of mitochondria. Respiratory chain enzymes levels showed a significant decrease of cyto-chrome-c oxidase (COX), succinic dehydrogenase (SDH), and citrate synthetase activity. Lymphocyte mtDNA showed the MERRF point-mutation in only one patient with MSL.Conclusions:The mitochondrial dysfunction in MSL seems to be consistent with a reduced number of mitochondria and reduced mitochondrial enzyme activities; this could represent the pathogenetic basis of lipoma formation, as well as of other multisystemic clinical manifestations.

ISSN:1522-0443    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

A 30-year-old woman developed severe bilateral radial neuropathies during vaginal delivery of twins, likely secondary to positioning and muscular effort. Subsequent evaluation led to the diagnosis of hereditary neuropathy with predisposition to pressure palsies. Avoidance of prolonged muscular effort in the arms in conjunction with medial intervention to shorten the second stage of labor may help prevent debilitating radial nerve injury in women with this disorder.

ISSN:1522-0443    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

A 72-year-old man developed Lambert-Eaton myasthenic syndrome (LEMS) with typical electrodiagnostic findings and elevated serum antibodies to the P/Q-type voltage-gated calcium channels. Two years after diagnosis, he had transitional cell carcinoma of the bladder (TCCB), There was no evidence of small cell lung cancer (SCLC) 5,5 years after the onset of the symptoms. In this article I review the association of LEMS with cancer other than SCLC, and discuss the paraneoplastic characteristics of TCCB.

ISSN:1522-0443    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Lymphomas may cause polyradiculopathies by several pathogenic mechanisms. However, such a presentation of a nasal-type NK (natural killer) cell lymphoma is rare. We report a previously healthy man who developed acute, axon-loss radiculopathies and cranial neuropathies. Despite the lack of a nasal or midline facial mass, cerebrospinal fluid cytologic and flow cytometric immurtophenotypic studies disclosed that the patient's rapid demise was the result of a nasal-type NK cell lymphoma with leptomeningeal involvement

ISSN:1522-0443    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

We report the first study in which the repetitive nerve stimulation (RNS) test was performed in conjunction with the ice-pack test in three patients with myasthenia gravis. All three patients showed an unequivocal improvement in ptosis on the side where an ice pack was placed. RNS test in the facial nerve revealed a definite improvement in the decremental response. From this we conclude that the ice-pack test produces a clinical and electrophysiological improvement in myasthenia gravis.

ISSN:1522-0443    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

We report a case of recurrent critical illness myopathy in an asthmatic patient who was treated for respiratory failure with high doses of intravenous corticosteroids alone without exposure to nondepolarizing neuronmscular-bloclong agents. Each episode was followed by substantial symptomatic improvement. Our experience indicates that intravenous corticosteroids should be used with special caution in patients with a history of critical illness myopathy

ISSN:1522-0443    

出版商:OVID    

年代:2000

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Neuromuscular disorders developing m critically ill patients often result in increased morbidity and prolonged intensive care hospitalization Detection and assessment of affected patients are frequently hindered by the severe nature of the underlying medical illness. The various syndromes have overlapping clinical and electrodiagnostic features, presenting further obstacles to prompt classification. Nonetheless, an accurate diagnosis can usually be attained through an organized approach to the evaluation and management of critically ill patients with acquired weakness.

ISSN:1522-0443    

出版商:OVID    

年代:2000

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:2000

数据来源: OVID