All cases of motor neuron disease (MND), encompassing amyotrophic lateral sclerosis (ALS), progressive bulbar paralysis (PBP) and progressive spinal muscular atrophy (PSMA), in northern Sweden, diagnosed between 1969-1980 have been analysed. 128 cases were found, corresponding to an average annual incidence rate of 1.67 per 100,000. The prevalence on December 31, 1980 was 4.8 per 100,000. Age-specific incidence rates were higher in the high age groups with a maximum at 60-64 years for males, at 70-74 years for females and at 65-69 years for the sexes combined. The median age at onset was 61 years. Clustering was not found in mining districts and overrepresentation of miners and stone treaters was not observed. Minor differences in incidence rates, as measured by the standardized morbidity ratio, SMR, were found between the inland, coastal and mountain areas. The median survival time after onset of disease was 32 months for ALS, 30 months for PBP and 70 months for PSMA. The combined survival rate for all MND cases was 28% after 5 years and 15% after 10 years. The male to female ratio was 1.1:1, and 4.7% were familial cases.

A series of 37 patients with chronic pain was investigated with regard to neurologic and psychiatric variables. Twenty of the patients were classified as having mainly organic (= somatogenic) pain syndromes while 17 patients were rather suffering from psychogenic pain syndromes. Samples of lumbar cerebrospinal fluid (CSF) were obtained from the patients and analyzed for the presence of opiate receptor-active material, here called endorphins. Patients classified as having mainly organic pain syndromes were found to have significantly lower endorphin levels than patients with predominantly psychogenic pain syndromes. In the total group of patients as well as in the two subgroups, there was a significant correlation between CSF endorphin levels and the depth of depressive symptomatology as reported by the patients. On the other hand, there was no correlation between CSF endorphin levels and extent of anxiety or motor retardation. It is concluded that CSF endorphins reflect central processes involved in chronic pain syndromes.

Immunoreactive substance P was determined in lumbar CSF of 35 healthy volunteers and 60 patients with chronic pain syndromes of at least 6 months duration. No significant relationships were found between substance P levels and age, sex or body height. Substance P levels were lower in chronic pain patients, with either neurogenic (n = 23) or idiopathic pain (n = 37) syndromes, than in the healthy volunteers. Substance P levels were especially low in patients with neurogenic pain with lesions involving the extremities and in those with polyneuropathy, while patients with central pain or pain of the head or face had higher values. Substance P levels were related to depressive symptomatology as determined by means of visual analogue scales and to stable personality traits as determined by means of the Karolinska Scales of Personality (KSP). The most consistent (and inverse) relationship was found between substance P levels and the symptom 'inner tension' and between substance P levels and the personality trait 'psychic anxiety.'

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A hereditary myopathy with lactic acidosis

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The frequency of depressive symptomatology as estimated by means of self-rating on a visual analogue scale and the pain drawings by patients were compared between healthy volunteers, patients with neurogenic pain syndromes and patients with idiopathic pain syndromes. All patients with chronic pain syndromes had significantly more depressive symptomatology than the healthy volunteers. Patients with idiopathic pain syndromes had significantly more inhibition symptoms--memory disturbances and concentration difficulties--than patients with neurogenic pain syndromes. In the pain drawings, estimated by means of the technique suggested by Margolis et al. [10], the idiopathic pain patients had significantly higher scores on both raw scores and weighted body surface scores than the patients with neurogenic pain syndromes. Thus, both self-rating of depressive symptomatology and pain drawings can be of some help in the difficult clinical delineation between patients with idiopathic and neurogenic pain syndromes, respectively, but used as single measures, both methods have low discriminative power.

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