Erba disease

Erba disease (W. Erb; synonym: spastic spinal ERB's palsy, spastic paraplegia) is a slowly progressive spastic spinal paralysis. Previously it was thought that ERB's palsy is developed on the ground of syphilis. Currently, this view is not supported, although in some cases syphilis etiology is quite possible. Most likely, ERB's palsy is sporadically appearing hereditary disease with system degeneration mainly pyramidal tract outstanding pathogenesis. This is confirmed by the fact that in some cases the so-called clean spastic paraplegia (see Paraplegia) occurs in combination with this, undoubtedly, a hereditary disease, as the FA. In addition, the clinical picture of ERB's palsy is almost identical to that in family spastic paraplegia of Strumpel.
Postmortem ERB's palsy is essentially a symmetric degenerative changes of the pyramidal tract in the side columns of the spinal cord in its entirety, from a lumbar Department.
The clinical picture and over. ERB's palsy develops in childhood and adolescence, there are known cases of this disease in later life. Men suffer more often, and more than women. The disease develops very slowly, gradually progressing. First, due to the significant increase muscle tone in the legs gait changes: it becomes shuffling, patients go small steps, clinging to the earth toes, knees are to each other. Tendon reflexes are higher, there clonus patellas and feet, abnormal reflexes. Spastic phenomena prevail over paritychecking, especially early in the disease. Characteristic of ERB's palsy are full safety of all kinds of sensitivity and normal function of the sphincter. The disease usually affects only my feet, but there are cases when there is increased muscle tone and tendon reflexes and hand. ERB's palsy may occur over a period of many years and, increases gradually lead to a complete obezbijede patient.
The diagnosis put on the basis of the slow development of bilateral pyramidal syndrome in the absence of any disease, which can give a picture of bilateral bottom of the pyramid paraphrase. From this kind of disease it should be borne in mind include: spinal form of multiple sclerosis, amyotrophic lateral sclerosis, flowing with predominant symptoms from the side of the pyramid systems (see), progressive spinal compression (for example, tumors, spondylitis, cervical disc myelopathy), little's disease, in which spastic prepares due to birth trauma.
Treatment of ERB's palsy, and other hereditary degenerations, is ineffective. Use vitamins E, B1, B6, adenosine triphosphate; to reduce spastic condition of the muscles - pahikarpin, etc.