Pain in motor neuron disease.

Twenty-seven of 42 patients with motor neuron disease had significant pain. The nature and duration of the pain are described along with an illustrative case-report. The aetiology and most effective treatment of this common complication of motor neuron disease remain unclear.     

Stimulation of motor tracts in motor neuron disease.

The muscle responses evoked by cortical and cervical stimulation in 11 patients with motor neuron disease were studied. The muscle potential in the abductor pollicis brevis, evoked by median nerve stimulation and the somatosensory potential evoked by wrist stimulation were also studied. In eight of 11 patients there was absence or increased central delay of the responses evoked by cortical stimulation. In four patients muscle responses on cervical stimulation and muscle action potentials on median nerve stimulation were also altered, indicating peripheral abnormalities. Somatosensory responses evoked by wrist stimulation were normal. Electrophysiological techniques are helpful in estimating the site of motor involvement in motor neuron disease.     

Transcranial magnetic stimulation identifies upper motor neuron involvement in motor neuron disease.

OBJECTIVE: To evaluate the sensitivity of transcranial magnetic stimulation (TMS) to identify upper motor neuron involvement in patients with motor neuron disease. BACKGROUND: Diagnosis of ALS depends on upper and lower motor neuron involvement. Lower motor neuron involvement may be documented with electromyography, whereas definite evidence of upper motor neuron involvement may be elusive. A sensitive, noninvasive test of upper motor neuron function would be useful. METHODS: TMS and clinical assessment in 121 patients with motor neuron disease. RESULTS: TMS revealed evidence of upper motor neuron dysfunction in 84 of 121 (69%) patients, including 30 of 40 (75%) patients with only probable upper motor neuron signs and unsuspected upper motor neuron involvement in 6 of 22 (27%) patients who had purely lower motor neuron syndromes clinically. In selected cases, upper motor neuron involvement identified with TMS was verified in postmortem examination. Increased motor evoked potential threshold was the abnormality observed most frequently and was only weakly related to peripheral compound muscle action potential amplitude. In a subset of 12 patients reexamined after 11+/-6 months, TMS showed progression of abnormalities, including progressive inexcitability of central motor pathways and loss of the normal inhibitory cortical stimulation silent period. CONCLUSIONS: TMS provides a sensitive means for the assessment and monitoring of excitatory and inhibitory upper motor neuron function in motor neuron disease.     

Haptoglobin groups in motor neuron disease.

Haptoglobin (Hp) groups were investigated in 81 patients with motor neuron disease. A significant excess of heterozygotes was observed, accentuated among males and in the progressive spinal muscular atrophy subgroup. The results are discussed in terms of a possible influence of Hp in the immunological response.     

Antecedent events in motor neuron disease.

Sixty-three patients with motor neuron disease and sixty-one controls matched for age and sex were interviewed concerning life events. An antecedent history of back injuries was found more often and there was an increased incidence of prior electric shock amongst the patients. No increase of head injuries, fractures, malignancy or previous poliomyelitis was detected.     

Post-poliomyelitic motor neuron disease. Clinical aspects and its relation to typical motor neuron disease

Eighteen cases of post-poliomyelitic motor neuron disease (PPMND) were found in a series of 869 subjects affected by motor neuron disease (MND). The mean age of onset of acute anterior poliomyelitis (AAP) was 43.6 months. The mean age of onset of MND was 45.9 years. No case had a bulbar onset and hyperreflexia was found in only 2 subjects, one of them having also bilateral extensor plantar response. The survival curve showed a better course of these cases, compared to typical MND (TMND). The present study seems to indicate that PPMND and TMND are different diseases.     

运动神经元病患者的记忆功能研究

目的观察运动神经元病(motor neuron disease,MND)患者是否存在记忆功能障碍;并研究患者的病程对其记忆功能的影响。方法对比分析了32例MND患者与60例正常对照的临床记忆量表评分,并比较不同病程的MND患者的记忆功能评分。结果MND患者记忆商、临床记忆量表总等值量表分、指向记忆等值量表分、无意义图形再认等值量表分评分均明显低于对照组(P<0.05),且不同病程的MND患者临床记忆量表各项评分比较无显著性差异(P>0.05)。结论MND患者大多存在记忆障碍,提示MND存在运动区域以外的脑组织受累。MND患者的记忆障碍与病程长短无明显关系。     

Central motor conduction is abnormal in motor neuron disease.

Conduction in the central motor pathways of the brain and spinal cord was studied in 12 patients with motor neuron disease. Six healthy volunteers served as controls. Transcutaneous electrical stimulation of the cortex, cervical cord, thoracic cord and conus medullaris was used to determine motor latencies to the biceps brachii, thenar eminence and tibialis anterior muscles. Prominent, and often asymmetrical, slowing of central motor conduction was demonstrated in seven of the 12 patients; these findings were most marked in the spinal cord and in most cases correlated with clinical features of corticospinal involvement. In general it was more difficult to excite motor pathways in the central nervous system in the patients with motor neuron disease than in control subjects. Evidence of subclinical involvement of central motor pathways was found in five patients. The central lesion in motor neuron disease may thus contribute more significantly to the clinical deficit than has been realised, since the clinical signs of the upper motor neuron lesion are often masked by the more obvious lower motor neuron features.     

Central motor conduction in motor neuron disease

Central motor conduction was assessed in 13 patients with motor neuron disease and in 15 control subjects. All patients with motor neuron disease, even those without clinical pyramidal signs, had slowed central motor conduction, and in some the delays were asymmetrical. Evoked motor potentials represent a new and reliable method to detect physiological abnormalities of central motor pathways early in the course of motor neuron disease.     

Flaviviruses in motor neuron disease

Sporadic motor neuron disease (MND) causes a progressive loss of motor neurons. West Nile virus can attack motor neurons, so we examined whether flavivirus infection could be detected in MND cases. Spinal cord sections from 22 MND cases were stained immunohistochemically with a flavivirus-specific antibody. No staining for flavivirus was seen in any case. Sporadic MND does not appear to arise from a recent infection with a flavivirus.     

Intrafamilial heterogeneity in hereditary motor neuron disease.

Although there are varied inheritance patterns in motor neuron disease (MND), the phenotype of MND is reported to be constant within these families, ie, cases of amyotrophic lateral sclerosis or primary lateral sclerosis do not occur in pedigrees with cases of spinal muscular atrophy. We describe four pedigrees whose members diverged in the phenotype of MND expressed. The intrafamilial variation of phenotype suggests a similar pathogenesis for some of the varied types of familial MND and the need for careful inquiry of family history in all patients with MND.     

Extrapyramidal system involvement in motor neuron disease.

Three cases of motor neuron disease (MND), in which neuropathological findings were atypical, are reported. The first case manifested widespread and severe degeneration of the spinal cord, as in spinal fibrosis. Case 2 revealed severe degeneration of the pyramidal tract with many spheroids, which made it difficult to differentiate from primary amyotrophic lateral sclerosis. The last case revealed degeneration of the nigro-pallido-luysian system, even though no clinical manifestation of extrapyramidal and/or cerebellar symptoms had been noted throughout the clinical course. In MND, degeneration might occur in various locations other than the motor system.     

Cerebral glucose utilization in motor neuron disease.

Positron emission tomography with fluorodeoxyglucose F 18 (18F-fluorodeoxyglucose) was used to examine regional cerebral glucose metabolism in individuals with motor neuron disease. Motor neuron disease involves selective loss of motor neurons, large pyramidal cells in the motor cortex, and corticospinal tract degeneration. We postulated that the local cerebral metabolic rate of glucose should correlate with this regional neuronal cell loss. Glucose metabolism values in patients with motor neuron disease were reduced compared with those of controls in several regions; however, when corrected for multiple comparisons, no significant difference was observed between patients with motor neuron disease and age-matched controls. No correlation was noted between the local cerebral metabolic rate of glucose and duration or severity of illness. Correlation between metabolic changes with objective findings on neurologic examination, including motor weakness and tendon reflexes, provided interesting results, including a decline in glucose metabolism with progressive weakness and upper motor neuron dysfunction. Moreover, in supplementary motor areas, there appears to be an increase in regional glucose metabolism as the neurologic condition deteriorates, possibly representing increased metabolic activity of the motor association cortex in response to primary loss of pyramidal cells.     

Subacute, reversible motor neuron disease.

Four patients with a clinical syndrome closely resembling amyotrophic lateral sclerosis recovered completely, without treatment, 5 to 12 months after onset. Electrodiagnostic tests revealed acute and chronic denervation, with normal motor and sensory nerve conduction studies. The CSF was normal, and tests for paraproteinemia, heavy metal intoxication, and systemic illness were negative. Although such cases are rare, the possibility of spontaneous recovery should always be considered when counseling patients with suspected ALS.