Pathological findings in a patient with amyotrophic lateral sclerosis and multifocal motor neuropathy with conduction block

Veugelers, Bastiaan; Theys, Paul; Lammens, Martin; Hees, J. Van; Robberecht, Wim

doi:10.1016/0022-510x(95)00295-d

Cited by 38 publications

(31 citation statements)

References 25 publications

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“…Recently, Veugelers et al 34 reported signs of spi-nal motoneuronal loss and axonal degeneration of the corticospinal tract in the postmortem examination of a patient with electrophysiologically confirmed MMN, and Pelliccioni and Scarpino 25 reported 2 patients with MND who had electrophysiological findings that suggested MMN. In some instances, therefore, the electrophysiological finding of CB may be misleading, and physicians should be cautious in establishing the diagnosis of MMN on the basis of a single electrodiagnostic examination.…”

Section: Accepted 10 March 1999mentioning

confidence: 97%

Central motor conduction time in patients with multifocal motor conduction block

et al. 1999

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Section: Accepted 10 March 1999mentioning

confidence: 97%

Central motor conduction time in patients with multifocal motor conduction block

et al. 1999

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“…Furthermore, although it is generally accepted that peripheral nerve conduction velocity is normal in ALS, detailed studies have shown slowing of motor nerve conduction [4,7,28]. Exceptional cases have been reported with severe slowing of the motor nerve conduction, even with conduction block [36]. It is not known whether the conduction slowing is due to a relatively seAbstract Although amyotrophic lateral sclerosis is a degenerative disease of the upper and lower motor neurons, there is evidence that the disease can affect other systems, including the sensory system.…”

Section: Introductionmentioning

confidence: 97%

Evolution of motor and sensory deficits in amyotrophic lateral sclerosis estimated by neurophysiological techniques

Theys¹,

Peeters²,

Robberecht³

1999

Journal of Neurology

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Although amyotrophic lateral sclerosis is a degenerative disease of the upper and lower motor neurons, there is evidence that the disease can affect other systems, including the sensory system. On the other hand, within the motor neuron pool there is possibly a predilection of the degenerative process for the motor neurons fibers with the fastest conduction velocity (MNFCV). We studied these two aspects of the disease in a group of 50 patients by prospectively assessing several sensory indices and by studying the selectivity of the spinal motor neuron loss. At baseline, nerve conduction studies and somatosensory evoked potentials showed abnormal slowing in the peripheral and central sensory pathways. Thermal thresholds for heating were elevated but were normal for cooling. In more than 60% of the patients at least one of the sensory tests studied was abnormal. However, except for a significant decrease in the amplitude of the sensory nerve action potentials of the sural nerves, these afferent dysfunctions were not progressive over the follow-up period of 6 months, in contrast to the marked deterioration in motor functions. Three different statistical models were applied to evaluate the presence of demyelination, selective loss of MNFCV, or the purely random degeneration of fast- and slow-conducting motor neurons. These data indicate a selective loss of the MNFCV and suggest that subclinical abnormalities of the sensory system in ALS are often present but almost nonprogressive. Furthermore, the amyotrophic lateral sclerosis disease process seems preferentially to affect MNFCV.

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“…Experimental paranodal demyelination in rodents severely impaired saltatoric nerve conduction suggesting that focal demyelination is the pathological basis of CB [23,39]. This hypothesis was further strengthened by morphological nerve studies confirming circumscribed demyelination in biopsies from MMN patients [38,39,40,41]. Beyond focal demyelination, generalized axonal dysfunction might be present in MMN [42].…”

Section: Pathophysiology Of Mmnmentioning

confidence: 74%

“…Beyond focal demyelination, generalized axonal dysfunction might be present in MMN [42]. Pathological and electrophysiological findings have highlighted the functional role of axonal disintegration and impaired axon-myelin interactions [41,43,44,45,46]. The question whether axonal degeneration is an intrinsic pathophysiological feature of MMN or caused by persistent CBs is still under debate.…”

Section: Pathophysiology Of Mmnmentioning

confidence: 99%

Multifocal Motor Neuropathy: Update on Clinical Characteristics, Pathophysiological Concepts and Therapeutic Options

Meuth¹,

Kleinschnitz²

2010

Eur Neurol

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Multifocal motor neuropathy (MMN) is an acquired immune-mediated neuropathy characterized by chronic or stepwise progressive asymmetrical limb weakness without sensory deficits. The upper extremities are more often affected than the lower extremities with distal paresis dominating over proximal paresis. Important diagnostic features are persistent multifocal partial conduction blocks (CBs) and the presence of high-titer anti-GM1 serum antibodies. Motor neuron disease, other chronic dysimmune neuropathies, such as chronic inflammatory demyelinating polyneuropathy and the Lewis-Sumner syndrome (MADSAM neuropathy), are important differential diagnoses. While corticosteroids and plasma exchange are largely ineffective, high-dose intravenous immunoglobulins are regarded as first-line treatment. In spite of significant success in elucidating the underlying disease mechanisms in MMN during the past few years, important pathophysiological issues and the optimum long-term therapy remain to be clarified. The present review summarizes the clinical picture and current pathophysiological concepts of MMN with a special focus on the molecular and electrophysiological basis of CBs and highlights established therapies as well as possible novel treatment options.

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Pathological findings in a patient with amyotrophic lateral sclerosis and multifocal motor neuropathy with conduction block

Cited by 38 publications

References 25 publications

Central motor conduction time in patients with multifocal motor conduction block

Central motor conduction time in patients with multifocal motor conduction block

Evolution of motor and sensory deficits in amyotrophic lateral sclerosis estimated by neurophysiological techniques

Multifocal Motor Neuropathy: Update on Clinical Characteristics, Pathophysiological Concepts and Therapeutic Options

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