Marie's Disease

MARIE'S DISEASE (Pierre Marie). In pathology, two distinctive forms of skeletal lesions described by Marie are associated with his name, the origin of which still remains in many respects unexplained. 1. The so-called osteoarthropathie hypertrophiante pneumique (periostitis hyperplastica, acroelephantiasis ossea et mollis). This condition involves subperiosteal proliferations of bone tissue on the bones of the forearms, shins, and phalanges of the fingers, less frequently on the humerus and femur. The resulting thickenings are usually not uniform, but nodular or club-shaped, and typically occupy the distal ends of the mentioned bones, only rarely extending along their entire length. With significant deformation of the articular ends, deformation of the corresponding joints may occur, although the joints themselves as a rule do not suffer. In the area of the terminal phalanges of the fingers, the bone thickenings are in most cases small, but here the soft parts become markedly thickened, as a result of which the fingers acquire the characteristic form of "drumsticks" (see Drumstick fingers). The basis of these hyperplastic processes apparently lies on the one hand in the influence of frequent blood stagnation, and on the other in the irritating effect of those toxic products which circulate in the body in certain diseases [heart defects (especially congenital ones associated with severe blood stagnation), putrid bronchitis, bronchiectasis, certain forms of pulmonary tuberculosis, chronic extrapulmonary purulent processes in the chest cavity, chronic jaundice, chronic leukemias, some malignant tumors, mainly tumors of the lungs]. 2. Spondylose rhizomelique (spondyloarthritis ankylopoetica), a disease that was initially classified in the group of chronic deforming arthritis (spondylitis deformans), but was later separated from them on the basis of a number of significant differences. In deforming spondylitis, the primary factor is the degeneration of the intervertebral cartilages, which, due to loss of elasticity, become flattened and, bulging over the anterior and lateral surfaces of the vertebral bodies, give rise to the formation of osteophytes that fuse the vertebrae together, and sometimes also to the ossification of the anterior spinal ligament. Along with this, due partly to atrophic and partly to hypertrophic processes occurring in the vertebral bodies themselves, kyphosis or kyphoscoliosis develops. Usually not the entire spine is affected, but mainly its upper or lower part. In ankylosing spondylitis (spondylose rhizomelique), according to the opinion of most authors, the true joints of the vertebrae are primarily affected, in which an ulcerative process develops, leading to the fusion of the contacting surfaces of the articular processes, sclerosis of the capsule, and finally to complete fibrous or bony ankylosis of the joints, usually with an arcuate kyphotic curvature of the spine. The same ankylosing process can also develop in the joints between the vertebrae and ribs, which is particularly severe as it impedes thoracic respiration. At the same time, ossification of the ligamentous apparatus occurs, first of all of the capsular ligaments and the so-called ligamenta flava (some consider this process even primary). In contrast to senile deforming spondylitis, spondyloarthritis ankylopoetica often begins at a young age and gradually involves the entire spine. Two types of this disease are distinguished: the Bechterew type and the P. Marie-Strümpel type. They differ mainly in that in the first type only the spine is affected, while in the second type the large joints (hip joints, less frequently shoulder and knee joints) are also involved in the process. This latter feature gave Marie the basis to designate the disease as spondylose rhizomelique (from Greek rhiza - root, base). Initially, the difference between these types was drawn quite sharply, and in addition to the distinguishing feature mentioned, a whole series of other differences was pointed out, but later, as descriptions of a number of transitional and mixed cases accumulated in the literature, the boundaries between them largely blurred, and at present most authors treat them as accidental individual varieties of one disease form. As factors predisposing to the development of ankylosing spondylitis, heredity, trauma, syphilis, gonorrhea, rheumatism, and some acute infections (especially abdominal typhoid) are mentioned.

M. Skvortsov. Nervous syndromes in spondylitis ankylopoetica. Both of the indicated forms—Bechterew's and Marie-Strümpel's—usually come to the attention of clinicians (surgeons and neurologists), because at all stages of their development they are accompanied by varying degrees of impairment of the motor sphere and may also give rise to disturbances of sensitivity, mainly in the form of pains that sometimes arise independently, sometimes at movements of the spine and ribs. But one cannot speak of any specific complex of nervous phenomena in these forms; the pathological phenomena of this category belong to secondary symptoms, presenting diverse pictures in individual cases. In theoretical considerations regarding the nervous disorders that may arise, one must proceed from anatomical data and the topographical relationships between the spine and the parts of the nervous system located within it. It is quite obvious that in ankylosing spondylitis, when the true joints of the vertebrae are primarily affected, there is no basis in this initial stage to expect the appearance of symptoms from the nervous system directly dependent on changes in the joints of the vertebrae, but of course the gradual spread of the process to the surrounding parts with subsequent sclerosis of the ligamentous apparatus, the formation of thick bony plates on the sides of the vertebrae, reduction of the intervertebral foramina, etc., may affect the adjacent parts of the nervous system. Mainly one can expect pathological phenomena from the intervertebral ganglia and spinal nerves located in the foramina intervertebralia; these symptoms may be of both sensory and motor order and in the initial stages may be expressed by an increase (stage of irritation) of functions: pains, paresthesias, muscle twitchings, tremors, increased reflexes, but later (stage of depression) their weakening. These so-called peripheral lesions are of course concentrated in the segments corresponding to the affected parts of the spine, but may have a character of considerable spread and be scattered in different segments. From the point of view of the generally accepted anatomical principle of division of peripheral lesions, the indicated symptoms can be attributed to the radicular type. However, greater clarity must be introduced into this area, because in everyday practice lesions of the roots and spinal nerves are usually not sufficiently precisely differentiated. In ankylosing spondylitis the spinal nerves, composed of elements of both roots, are involved in the process, and therefore the anatomical type of the peripheral lesion will be radicular, but according to the symptom-complex it will differ from a purely radicular lesion. First of all, both motor and sensory disturbances will always be present (sometimes with predominance of one or the other and more often sensory), then the pains do not have an independent character and constancy, like radicular pains; they are caused by palpation or percussion of the paravertebral area, and also arise at movements of the spine; in addition, one can often note contractures of the paravertebral musculature, arising reflexively for the protection and immobilization of the vertebrae. As for the symptoms from the spinal cord, it is usually considered the rule that they are absent in ankylosing and deforming spondylitides; but one must not lose sight of the fact that in cases that have gone far, they may occur and develop according to the compressive type. Moreover, since in the etiology of ankylosing and deforming spondylitides, still sufficiently obscure, acute and chronic infections play a role, and first of all syphilis and gonorrhea, one cannot close one's eyes to the fact that in the overall picture on this basis symptoms from the nervous system, both peripheral and central, may appear that have no direct connection with changes in the spine. As for the differential diagnosis between the forms of Bechterew and Marie-Strümpel, the nervous syndromes cannot be of help, although formerly neurologists were convinced that the Bechterew form is predominantly accompanied by nervous phenomena, but as is evident from the above, the nervous syndrome is determined by the duration of the process and the individual peculiarities of the case. From the diagnostic point of view, one should beware of confusing with these forms the so-called myogenic stiffness of the spine (described by Schuster, Nonne), in which there is a slight kyphosis of the cervical part and tension of m. erector trunci, especially in the lumbar part; this form is observed in persons of neuropathic constitution after injuries to the back or from overstrain of the back muscles (lifting heavy weights). In the statics and locomotion of patients with ankylosing spondylitis there are many features resembling parkinsonians (paralysis agitans and parkinsonism after epidemic encephalitis) and myopathics, namely: when standing they often support the trunk with their hands, resting them on the thighs; gait is accomplished with small steps, cautiously; in movements there are features of stiffness. However, confusion with these forms is possible only with a superficial glance; the decisive word belongs to radiography. Treatment—see Spondylitis.

a. Surikov.