[Lower limb pain and leaping progressive paraparesis].
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Biomedical subjects
Publications and source records attributed to M Jokelainen.
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Amyotrophic lateral sclerosis (ALS) appears to be more prevalent in the southeastern part of Finland and among the war evacuees displaced from this area after the World War II than elsewhere in the country. A random sample of 31 ALS patients was chosen and the birthplaces of their ancestors traced back in two generations to find out whether there would be any tendency of clustering in certain regions of the country. No such trend was found. This finding speaks against a genetically determined tendency for the disease.
After World War II the southeastern part of Finland was ceded to the Soviet Union and its entire population evacuated to other areas of the country. The prevalences of amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS) were studied among the evacuees and compared to the corresponding data among the nonevacuated population. The prevalence of ALS among the war evacuees was two times higher than among the nonevacuated population (18.0 and 8.8 per 100,000, respectively). The prevalence of MS among the evacuees was only half of that found among the nonevacuated population, 38.3 and 73.0 per 100,000, respectively. The findings for ALS indicate that birthplace may have an effect on the later development of the disease and that there may have existed some environmental factor(s) which have made the evacuees more liable to contract the disease later in their lives. The low figure of MS for evacuees supports our previous results of an uneven geographic distribution of MS in Finland with the high-risk areas in the western and southwestern parts of the country. No accumulation of MS was found among the evacuees living in the high-risk areas.
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A pair of identical female twins were discordant for amyotrophic lateral sclerosis. The affected twin was not breast-fed; was bitten by a poisonous snake, and was operated on for struma. She had more infections than her sister but no fractures. Her plasma insulin response to glucose loading was also higher. The twins lived separated in their early childhood.
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Amyotrophic lateral sclerosis (ALS) is a fatal, progressive disease of the central nervous system. Its possible association with poliomyelitis was studied by measuring neutralizing antibodies against polio virus types 1, 2 and 3 in the sera and cerebrospinal fluids of 11 ALS-patients, but antibody titers did not markedly differ from those of the controls. The HLA antigens of 12 ALS patients were also determined, in order to reveal any possible genetically-determined susceptibility to the disease. Possible association of ALS with HLA-Bw40 was noted. In addition, the Bw40 antigen seemed to be associated with milder progression of the disease. The lymphocytes of the ALS patients seemed defective in their capacity to stimulate allogenic lymphocytes, possibly due to a relative decrease of B cells in the peripheral blood. Joint efforts of study groups of neuroepidemiology, immunology and genetics should be mobilized to reveal the true nature of these findings.
The prevalence of ALS and its distribution in Finland was investigated on the basis of prevalence numbers by counties. On the prevalence day, 1 January 1973, 168 ALS patients were found alive. They were collected from hospital records and the registers of the National Pension Institute. The prevalence was 3.56 cases per 100,000 populations, 4.25/100,000 for men and 1.92/100,000 for women, which gave a male to female prevalence ration 1.5 to 1. The distribution of the disease was uneven in the country being more frequent in the southeastern counties. The disease was contracted earlier (at 51.9 years of age) and its course was slower (3.7 years up to the prevalence day) in the prevalence material than in cases derived from the mortality statistics (58.0 years at the onset and duration of 2.6 years, respectively). This is explained by the dropping out of some of the younger and/or more benign cases from the mortality statistics. This seems to be true also in other countries.
The clinical characteristics of ALS in Finland were investigated. The study was based on the hospital records of 255 patients. No correlation was found between the disease and other previous illnesses. Muscular weakness was the most frequent complaint of the patients as their first symptom (149/255, i.e. 58 per cent). The duration of the disease was shortest (1.9 years) in male cases with the first symptoms in the bulbar area, and it was longest (3.4 years) in females with a spinal onset of the symptoms. The duration of the disease was in inverse relation to the age when it was contracted. Progressive muscular atrophy with fasciculations was considered the most important clinical sign of ALS. It was most frequently observed in the upper extremities including shoulders. Sensory disturbances were absent, sphincter abnormalities were reported in only three cases, bed sores did not exist, and there were no reports of impotence in the male patients. Two families with two familial cases in each were found, altogether two men and two women. Their disease did not differ from that of the rest of the series. The ALS patients soon became too disabled to work; 56 per cent bacame permanently disabled in less than 1 year's time, and only 9 per cent retained their working capacity for more than 2 years. The average length of stay at hospital was 32 days for ALS and 37 days for MS patients, but the later group was hospitalized 2-3 times more frequently than the former. Gastrostomy or tracheostomy and artificial respiration did not lenghten the lives of the patients.