Erba Disease

ERBA DISEASE, synonym spinal paraplegia, lateral sclerosis, Erba spinal paraplegia, Charcot's tabes spasmodique. Besides this disease, Erba described several other diseases bearing his name affecting both the central and peripheral nervous systems (see Bulbar Paralysis, Myasthenia, Duchenne-Erba Paralysis, Childhood Paralysis). As an independent entity, spinal paraplegia was first identified by Erba in 1903; it is characterized by exclusive or almost exclusive 'systemic' lesion of the pyramidal pathways in the lateral columns of the spinal cord. The cardinal symptom is spastic paraplegia of the lower extremities, accompanied by other pronounced manifestations of pyramidal system lesion. The course is typically chronic, slowly progressive, and extremely prolonged. Erba traced cases lasting up to 20 years or more. In adults, onset in most cases occurs between the ages of 20 and 40, but later onset has also been noted. At the beginning of the disease, complaints of weakness and fatigue when walking and stiffness of the lower extremities due to muscle hypertrophy are noted. At this time, the actual paresis recedes significantly before the stiffness due to hypertrophy ('spastic pseudoparesis'). Subsequently, as hypertrophy increases, a typical spastic gait develops, becoming more pronounced. However, despite the fact that spasticity can reach an extremely high degree, patients even in far-advanced cases mostly do not completely lose the ability to walk. On examination, marked signs of pyramidal system lesion are found in the form of increased tendon reflexes, pathological reflexes, clonus, and pyramidal synkinesias. In later stages, manifestations of pyramidal system lesion may also appear in the upper extremities. Finally, cases have been noted in which the lesion extended higher to the muscles of mastication and articulation, giving a picture of the spastic form of bulbar paralysis, thus joining the spastic paralysis of the extremities. In 'pure' cases of spinal paraplegia, there were no disorders of the pelvic organs, coordination, peripheral nervous system, or sensitivity, except for minor disorders of superficial (mainly temperature) sensitivity observed in far-advanced cases. The syndrome described above, characteristic of the 'pure' form of spinal paraplegia, however, often serves only as the initial stage or residual manifestation of many other diseases of the central nervous system, when among the symptoms characteristic of the latter, lesions of the pyramidal system come to the forefront. Here one must consider multiple sclerosis, chronic myelitis, combined lateral sclerosis or funicular myelitis, compression of the spinal cord by tumors or in lesions of the meninges and spine, then amyotrophic lateral sclerosis, certain intoxications, certain forms of anemia. According to Oppenheim's indications, cases of hysteria may also cause confusion. In view of the above, the differential diagnosis of E.d. always presents great difficulties and requires great caution, and it is especially necessary to pay attention, besides the duration and gradually progressive course, to whether there are symptoms of lesions of other systems besides the pyramidal. Pathological anatomy is still little studied. This is mainly due to the rarity of 'pure' forms coming to autopsy. In only a few cases was it possible to confirm pathoanatomically the lesion of the lateral columns over a greater or lesser extent, not complicated by lesions of other systems of the spinal cord. Combined lesions were more frequently noted, with greater or lesser involvement of the posterior columns (Goll's bundles). The pathogenesis has not yet been clarified with certainty. Syphilis of the central nervous system is placed first, followed by trauma, certain infections and intoxications (e.g., lead) in the anamnesis. However, in all these cases, even rare 'pure' forms of spinal paraplegia appear not as an independent nosological entity, but rather as a syndrome caused by various etiological factors. Therefore, the viewpoint of Davidenkov, which brings clarity to this question, is valuable; based on the similarity of both the clinical and patho-anatomical picture of E.d. with familial spastic paraplegia of Strümpel, he is inclined to consider spinal paraplegia in adults as sporadic, late-appearing cases of familial diplegia and sees in both diseases manifestations of the same genotypic structure. Treatment is exclusively symptomatic, mainly physiotherapy is applied: warm baths, massage and passive gymnastics. Of medicinal preparations, bromine, belladonna, scopolamine, and iodine preparations are used.