Motor neuron rigidity

A 28-year-old male with generalized muscle stiffness and widespread muscle twitching is described. Continuous electrical activity was present at rest and could be abolished only by succinylcholine, curare, and block of the distal part of the peripheral nerve. Muscle biopsy revealed mild myopathic changes. In addition glycogen depletion was found in muscle fibres which were type IIb in one region of the biopsy and type I in another. With carbamazepine treatment marked improvement occurred both clinically and electromyographically. It is concluded that the abnormal electrical activity originated in the terminal branches of the peripheral motor nerve and that this activity was confined to muscle fibres belonging to single motor unit territories.     

Motor neuron diseases

Presidential Symposium

Motor neuron diseases     

Motor neuron diseases

Journal of Neurology -     

Regional pain syndromes in cancer patients

Pain is one of the cardinal presenting symptoms in cancer patients and often seems to progress in the natural history of the disease. In light of the past neglect of this problem, it is becoming crucial for clinicians and researchers in the fields of oncology, pain management, and others dealing with this patient population to have a thorough understanding of the often complex pain syndromes that are relevant to managing cancer. In this article we briefly review the important topics related to cancer pain syndromes with some emphasis on those that typically generate localized pain.     

Motor neuron disease in Cantabria

The electroencephalograms (EEG) of patients with Parkinson's disease (PD) were evaluated in relation to age, mental status and motor disability. Abnormalities, particularly mild slowing, were observed in 34% of 128 patients and more frequently in the elderly. While a slowing in occipital background activity was more frequent in demented patients, a statistically significant association was also observed between the degree of motor disability and the frequency of slowing in occipital background activity in mentally intact patients. This correlation may suggest that subcortical structures (involved in motor control) influence occipital background activity.     

Neurologic pain syndromes in patients with cancer

Recognizing and treating the cause of pain in the patient with cancer should be the initial approach to the management of this common symptom. Careful analysis of patients with cancer and pain has led to the elucidation of common neurologic pain syndromes unique to this disease process. Pain in patients with cancer is commonly the presenting symptom of neurologic involvement. Changes in the pattern of pain or neurologic deficits should be carefully evaluated to rule out the possibility that metastatic disease may be exacerbating existing neuropathic pain. This chronic intractable pain syndrome can markedly debilitate the patient and needs an aggressive approach with drug therapy as the first-line approach.     

Stretch reflexes of triceps surae in patients with upper motor neuron syndromes.

Electromyographic responses of triceps surae to dorsiflexion stretch were studied in 47 patients with a variety of lesions producing an upper motor neuron syndrome. The short latency spinal reflexes, both when the patient was at rest and when he was exerting a voluntary plantarflexion, were frequently enhanced in magnitude and the rate of increase with acceleration was also enhanced. Long-latency reflexes were uncommon at rest. With background force long-latency reflexes were present unless the short latency reflex was very large. Long latency reflexes often were normal, but in some patients they were either excessively larger or even of abnormal shape with prolonged continuous activity. The clinical assessment of the ankle jerk correlated with the magnitude of the short latency reflex. The clinical assessment of tone correlated with the magnitude of the short latency reflex, the magnitude of the long latency reflex and the duration of the long latency reflex. There appear to be multiple physiological mechanisms underlying the clinical phenomenon of spasticity.     

Motor neuron disease and paraproteinemia

We studied two patients with motor neuron disease and paraproteinemia. One had amyotrophic lateral sclerosis (ALS) and IgG lambda monoclonal gammopathy. The second had slowly progressive muscular atrophy and an IgM kappa paraprotein, followed by a biclonal gammopathy when an IgA kappa paraprotein appeared. Treatment with immunosuppressive agents and plasmapheresis lowered the serum concentration of the paraproteins. The ALS syndrome progressed despite therapy. The other patient improved, was stable for several years, but then deteriorated despite continued therapy.     

The spectrum of lower motor neuron syndromes

Abstract. 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, dist inction 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.Supported by a grant from the Prinses Beatrix Fonds.     

Motor neuron disease in textile factory workers

Five cases of motor neuron disease occurred during the period 1983-1987 in 4 small and medium-sized textile factories. These cases were 45% of 11 motor neuron disease patients diagnosed in this period in a population of about 300,000 persons. This observations may be relevant to toxic occupational hazards in the pathogenesis of motor neuron disease.