Schilder's Disease

SCHILDER'S DISEASE (morbus Schilderi) (syn. aplasia axialis extracorticalis diffusa, sclerosis interlobularis symmetrica, encephalo-leuko-pathia sclerotica progressiva, necrosis perivascularis et sclerosis cerebri infantilis, la sclerose cerebrale centro-lobaire, sclerosierende Entzündung des Hemisphärenmarkes, leucopathia cerebri progressiva, encephalitis scleroticans periaxialis diffusa progressiva), or diffuse sclerosis of the nervous system, is a collective concept encompassing heterogeneous pathological forms; characterized by the presence of extensive demyelinated foci located in the white matter of the cerebral hemispheres, and sometimes of the cerebellum. Schilder (1912) deserves credit for the detailed critical comparison of his observations with cases of other authors and for the more precise description of the histopathological changes in the brain. According to Neuburger, all cases of Schilder's disease can be divided into three groups: 1) blastomatous, 2) exogenous-inflammatory, representing the classic Schilder's disease, and 3) endogenous-degenerative. According to Belsowsky, the blastomatous group should be excluded from cases of Schilder's disease, as it represents a transition to gliomas; thus only two groups remain: inflammatory and degenerative. In recent times, some authors have spoken in favor of combining both groups together. In terms of age differences, all described observations can be attributed to three groups: 1) childhood form, or Krabbe type, 2) juvenile form, or Scholz type, and 3) form of mature age. The childhood form of Schilder's disease in turn breaks down into two subspecies, one of which is observed in early childhood, the other in late childhood. Exogenous factors and constitutional instability may play an important role in the etiology of Schilder's disease (Belsowsky and Hennoberg).-The clinical symptom complex in Schilder's disease in each individual case depends on the localization of the process in the central nervous system and differs in its variety. In early childhood, Schilder's disease manifests as spastic paralyses and mental disorders, sometimes with the presence of optic neuritis, sometimes without it. In addition, in Schilder's disease the following may be observed: tonic spasms and muscle contractures, sometimes reaching the degree of decerebrate rigidity, Babinski's symptom, amimia, opisthotonus, disorders of innervation of eye muscles, strabismus, diplopia, nystagmus, visual disorders (often in the form of hemianopia), sometimes reaching complete blindness, deafness, epileptic seizures (sometimes in the form of Jacksonian epilepsy, sometimes in the form of screaming seizures); such seizures can be up to 150-200 per day; further noted are anosmia, dysarthria, cerebellar phenomena (ataxia, adiadochokinesia, intentional tremor). There may also be changes in the psyche, among which speech disorders, euphoria or apathy, depression, hallucinations, decreased memory, lack of initiative, mental weakness, often reaching the degree of profound dementia predominate. In individual cases, general cerebral phenomena have also been noted: vomiting, headaches, loss of consciousness, etc.; before death, symptoms from the medulla oblongata are usually observed. The cerebrospinal fluid in most cases is normal, only sometimes an increase in protein and cellular elements is noted in it, indicating an inflammatory nature of the process. The Wassermann reaction is usually negative. Differential diagnosis presents great difficulties (especially in adults) due to the diversity of the clinical picture. Most cases of Schilder's disease received proper evaluation only after histopathological study. It should be borne in mind that Schilder's disease is a relatively rare disease. The pathogenesis of Schilder's disease still causes much controversy among most authors; among the factors contributing to the development of Schilder's disease, the literature notes: infectious diseases (influenza, measles, angina, tuberculosis, acute diarrhea), endocarditis verrucosa, trauma, asphyxia at birth, hereditary predisposition, congenital syphilis in children.-According to the localization of the pathological process in Schilder's disease, two forms are distinguished: 1) occipital with transition to the parietal lobe of the brain and 2) fronto-central. Some authors also describe fronto-occipital localization, others fronto-temporo-occipital, others temporal, etc. Further, the basal ganglia, optic nerves, pyramidal tracts, white matter of the cerebellum, pons, corpus callosum, etc. may be affected. Microscopic changes, according to Schilder, are characterized [see separate table (pp. 399-410), fig. 3]: 1) limitation of the pathological process only to the white matter of the brain, 2) damage to myelin along with secondary decay of axis cylinders, 3) proliferation of glial elements and 4) adventitial infiltration, consisting of granular spheres and numerous lymphocytes. In some cases of Schilder's disease, the presence of numerous 'fatty' and fibrous glial cells along with the formation of a powerful network of gliofibrils has been noted. Products of fatty decay are encountered, as is usually the case with myelin degeneration; there are numerous 'Abraumproducte' - granular and reticular cells of mesodermal and glial origin, corpora amylacea. In the vessels, thickening of connective tissue fibers, proliferation of fibroblasts, phenomena of endarteritis in the intima, swelling of the endothelium of not only large but also small vessels are observed.-The course of Schilder's disease is progressive despite remissions. The duration of the disease is usually from several months to 2-5 years. Cases of lightning-fast nature (several days) and, conversely, with a history of up to 10-15 years have been described. Therapy usually gives no results and is unable to stop the course of the disease. Exploratory surgery usually accelerates the outcome, sometimes it gave a positive result in terms of reducing the number and changing the quality of seizures.