Combined Sclerosis

COMBINED SCLEROSIS, a chronic disease of the central nervousous system with varied symptomatology depending on the lesion of several long systems of the brain and spinal cord, primarily the posterior and lateral columns. In the broad sense of the word, this also includes some familial endogenous diseases, such as Friedreich's disease or other "hereditary degenerations" affecting several systems of the spinal cord. However, usually under C. s. is meant such a combined disease of several systems of the spinal cord, in which exogenous (in relation to the nervous system) factors play a predominant role. In this sense, synonyms of C. s. are: pseudosystemic C. s., funicular myelitis, funicular sclerosis, subacute combined degeneration of the spinal cord, diffuse degeneration of the central nervous system, progressive funicular myelopathy.

In the etiology of C. s., great importance is attached to malignant anemia (Biermer's disease). Symptoms of C. s. were found in 80.6% or 77% of cases of Biermer's disease. Conversely, Hurst and Lauterbach believe that in all cases of C. s., symptoms of Biermer's disease appear sooner or later. Cases of C. s. have also been described with other, especially lethal forms of anemia, as well as after malaria, in alcoholism, cancer, syphilis, pellagra, Addison's disease and other diseases leading to cachexia. Importance is also attached to helminths, especially Bothriocephalus latus, and gastrointestinal diseases, especially achilia. The significance of endogenous factors is evidenced by the fact that sometimes in the same family some people develop malignant anemia, others achlorhydria, and others C. s. (Hurst)! In recent years, C. s. has become more common. According to Long, C. s. occurs less frequently in Germany and France than in England. In Byelorussia it is more common than in other places in the USSR, but there are no exact statistics. According to Henneberg, among organic nervous diseases, C. s. in frequency comes after syphilis and multiple sclerosis.

Pathological anatomy. In fresh stages of C. s., no macroscopic changes are visible. In prolonged cases, degenerations of long pathways, especially of the posterior columns and pyramidal pathways, are visible to the naked eye on cross-sections of the spinal cord. Unlike tabes dorsalis, the posterior roots are not affected in C. s. Histological changes consist of focal granular disintegration of myelin sheaths and swelling and gradual death of axis cylinders. Primary foci are initially isolated from each other. Then the resulting "fields of extinction" gradually merge. To the primary process, secondary degeneration of long pathways is subsequently added. Thus, in the cervical part of the spinal cord, the most compact degenerations are found in the ascending pathways, while in the lumbar part, in the descending ones. Reactive phenomena from the glia, inflammatory changes from the vessels usually do not occur. Fresh foci are usually grouped around the branches of aa. internuniculares. In old foci, the vessel walls are found to be degenerated. Ganglion cells in the spinal cord are rarely affected. In the brain, mainly in the cortex, such changes occur especially when complicated by mental disorders. Thus, the former view of C. s. as a primary degeneration of long systems is now abandoned. The basis of secondary degeneration is the peculiar foci described above, appearing along the long pathways (pseudosystemic disease).-The pathogenesis apparently comes down to the action on the spinal cord through the blood of a toxic agent. With its prolonged action, in addition to nerve tissue, the vessel walls themselves may be damaged, which enhances the degeneration of nerve elements. Little is known about the nature of the toxin, all the more so since C. sclerosis occurs with different diseases. A toxin is known that is secreted by Bothriocephalus and leads to pernicious anemia. The opinion of English authors about the significance of oral sepsis in the pathogenesis of C. s. deserves attention: bacteria from the oral cavity enter the gastrointestinal tract, where in the absence of hydrochloric acid they multiply and the resulting toxins act on the one hand on the blood and hematopoietic organs, and on the other on the nervous system. - The symptomatology of C. s. consists mainly of spastic paraparesis and ataxia. Babinski's and Oppenheim's symptoms are not always present. Tendon reflexes are increased, but may also be absent. Deep sensitivity is usually impaired. Superficial sensitivity is disturbed more often than is generally thought. Especially often the disease begins with segmental paresthesias, which corresponds to the initial foci of extinction. Combinations with inflammation and atrophy of the optic nerves (optico-myelitis) are sometimes observed. Sometimes mental disorders appear: hallucinations, delusional states, dementia, disorientation.-The diagnosis of C. s. can be made in the presence of a combination of spastic paralysis with ataxia, when it is possible to exclude typical focal diseases, such as spondylitis, syphilitic meningo-myelitis, multiple sclerosis or any infectious myelitis. But also segmental paresthesias in different parts of the trunk and extremities, optic neuritis, mental disorders should suggest the diagnosis of C. s., if the patient has symptoms suggestive of pernicious anemia (color index > 1), achilia, Bothriocephalus, cachexia or similar conditions.-The prognosis is based on the underlying disease. It is often doubtful. The disease then lasts 1-2 years. But there are also long remissions and even recovery.-Treatment coincides with the treatment of the underlying disease (helminths, malaria, syphilis, anemia, etc.). Perhaps preventive value has the treatment with hydrochloric acid, which changes the reaction of gastric juice. The use of liver has not given great benefit. Prevention should also include the fight against oral sepsis.