Monomelic muscle atrophy

Takemitsu, Masakazu; Murayama, Keiko; Saga, Takashi; Michihiro, Narumi; Shiihara, Hiroaki; Kimizuka, Mamori; Nonaka, Ikuya

doi:10.1016/0960-8966(93)90024-e

Cited by 8 publications

(2 citation statements)

References 21 publications

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“…Four apparently discrete syndromes have been reported (Table 4), presenting with proximal or distal upper limb or lower limb weakness and wasting. In Japan and India, where a benign, unilateral, distal upper limb syndrome of wasting and weakness is relatively more frequent than in Europe, EMG and muscle biopsy have confirmed a neurogenic cause [5,7,9,[41][42][43]. In Asia, juvenile-onset cases monomelic weakness represents 11-23% of all cases of MND, [2,22,24] with a male predominance and a very slow course, and not progressing to widespread lower motor neuron involvement even over a period of 10 to 20 years [2,4,22,24].…”

Section: Discussionmentioning

confidence: 89%

Monomelic neurogenic syndromes: A prospective study

Carvalho¹,

Swash²

2007

Journal of the Neurological Sciences

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Section: Discussionmentioning

confidence: 89%

Monomelic neurogenic syndromes: A prospective study

Carvalho¹,

Swash²

2007

Journal of the Neurological Sciences

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“…■ Key words EMG · amyotrophic lateral sclerosis · anterior horn cell disease However, in a minority of patients weakness remains focal and prognosis is consequently much better [12,19,33,34,37].…”

Section: Introductionmentioning

confidence: 99%

Interpretation of electrodiagnostic findings in sporadic progressive muscular atrophy

Visser

Berg-Vos

et al. 2008

J Neurol

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The spectrum of lower motor neuron syndromes

Berg-Vos

Berg

Visser

et al. 2003

Journal of Neurology

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This review discusses the most important lower motor neuron syndromes. This relatively rare group of syndromes has not been well described clinically. Two subgroups can be distinguished: patients in whom motor neurons (lower motor neuron disease (LMND)) are primarily affected or motor axons and their surrounding myelin (multifocal motor neuropathy (MMN)), both leading to muscle atrophy and weakness. Both hereditary and sporadic forms of LMND have been described. The discussion of recent advances in the genetic knowledge of several hereditary forms of LMND may lead to a better understanding of the pathophysiology and the development of therapeutic strategies. By contrast, the pathogenesis of sporadic LMND is largely unknown. It is, therefore, difficult to consider the various sporadic forms of LMND, discussed in this review, as separate diseases. Because the diagnostic and therapeutic options may differ, it would seem rational to consider sporadic LMND as a spectrum of syndromes which can be distinguished from each other on the basis of clinical presentation.MMN is a lower motor neuron syndrome with presumed immunemediated pathogenesis. Evidence of motor conduction block on nerve conduction studies and a positive response to treatment with intravenous immunoglobulins (IVIg) are considered the most relevant criteria for the diagnosis of MMN. As it is treatable, it is important to distinguish MMN from LMND. Careful electrophysiological analysis in the search for conduction block is, therefore, required in all adult patients with pure lower motor neuron syndromes. For the individual patient, distinction between the various lower motor neuron syndromes is important as it enables the physician to provide adequate information over the disease course in LMND and to facilitate early treatment in MMN.

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Monomelic muscle atrophy

Cited by 8 publications

References 21 publications

Monomelic neurogenic syndromes: A prospective study

Monomelic neurogenic syndromes: A prospective study

Interpretation of electrodiagnostic findings in sporadic progressive muscular atrophy

The spectrum of lower motor neuron syndromes

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