Paraplegia

PARAPLEGIA (from the Greek para meaning transversely and plesso meaning I strike), paralysis of both lower limbs (paraplegia inferior) or both upper limbs (paraplegia superior). The combination of upper and lower paraplegia is termed quadriplegia. In cases where the paralysis is incomplete, where movements are only weakened and limited in scope, one speaks of paraparesis. Paraplegia may be the result of organic lesions of the nervous system—organic paraplegia, and the result of purely dynamic disorders—functional paraplegia. Organic paraplegia is characterized either by an increase in the tone of tendon and periosteal reflexes or is accompanied by atonia and the extinction of deep reflexes. In the former case, it is termed spastic paraplegia; in the latter, flaccid paraplegia. Pathological processes causing paraplegia affect either the peripheral motor pathways, the central pathways, or both simultaneously. Because the central motor neuron runs along the entire cerebrospinal axis, beginning from the cerebral cortex and ending with the ganglion cells of the anterior horns of the spinal cord, the occurrence of paraplegia can be caused by an organic lesion at any level of the central nervous system, provided the motor pathway is damaged on both sides. In the vast majority of cases, however, this involves a lesion of the spinal cord. The localization of peripheral nervous system lesions in paraplegia can be equally varied. Here it may be a matter of bilateral damage to the cells of the anterior horns of the spinal cord, or bilateral damage to the anterior roots, plexuses, or peripheral nerves. In these cases, paraplegia is always flaccid, whereas in cases with involvement of the central motor systems, it is generally spastic. Symptomatology. Spastic paraplegia, apart from the phenomena of paralysis—that is, the inability to perform voluntary movements or the weakening of this ability (paraparesis)—is characterized primarily by an increase in tone and the development of spastic hypertonia. Passive movement causes a resilient, springy resistance, after which movement proceeds relatively freely. Hypertonia generally affects the adductors and extensors of the thigh, the extensors of the leg, and the flexors of the foot. The lower limbs are extended and, if the patient has not yet entirely lost the ability to move about, he walks by swinging his legs in a semicircle at each step. Much less common is paraplegia in which the lower limbs are, conversely, in a state of constant shortening due to the development of flexor contractures of the hip and knee (paraplégie en flexion described by Babinski). Usually, this paraplegia develops in cases of particularly extensive spinal cord lesions, further in very emaciated subjects, and in cases with the development of severe bedsores. Prognostically, it is more unfavorable than paraplegia with extensor contractures. In cases of extensor-type paraplegia, there is always a sharp increase in the knee and Achilles reflexes, patellar and ankle clonus are frequently elicited, direct and crossed periosteal reflexes are increased, and the crossed Marie reflex is well-expressed. Normal plantar reflexes disappear, just as cremasteric and abdominal reflexes fade, unless the lesion is located below the corresponding reflex centers (D_VI-D_XII for abdominal reflexes, L_I-L_II for the cremasteric reflex). So-called pathological reflexes appear, that is, reflexes that do not normally occur and whose presence indicates damage to the pyramidal tract. Stimulation of the sole instead of the normal plantar reflex with flexion of the toes causes slow tonic extension of the great toe and fanning of the others (Babinski reflex). Deep stroking irritation of the tibia periosteum (Oppenheim reflex), squeezing of the Achilles tendon (Schäffer reflex) or the calf muscle (Gordon reflex) lead to the same result. Along with these extensor reflexes, abnormal flexor reflexes also appear. Flexion of the toes is caused by a strike on their plantar side (Rossolimo reflex), percussion in the region of the fourth metatarsal bone (Mendel-Bechterew reflex) and the heel (Bechterew reflex), and a strike on the middle of the sole (Zhukovsky reflex). In cases of paraplegia with a more or less extensive transverse lesion of the spinal cord, protective reflexes are usually well-expressed. Painful, thermal, or cold stimuli cause a slow, tonic shortening of the lower limb (flexion of the thigh and leg, extension of the foot and toes, especially the big toe); strong passive flexion of the toes leads to the same effect (Marie-Foix reflex). The same synergies appear in associated movements during so-called global synkinesias: any voluntary exertion leads to extension of the thigh and leg and flexion of the feet. Alongside this, coordinative associated movements are also observed, of which the tibial phenomenon of Strümpell (extension of the foot upon knee flexion) and Raimist's symptoms are of particularly great clinical significance. When the lesion is localized high up (above the cervical enlargement), when upper paraplegia exists alongside lower paraplegia, analogous symptoms are present, of course, in the upper limbs as well. Here too, there is an increase in tendon and periosteal reflexes, the appearance of pathological reflexes (Rossolimo and Mendel-Bechterew reflexes are often excellently expressed), protective reflexes, and associated movements. As a special symptom for this region, one should note the abolition of normally existing Mayer's phenomena (passive forceful flexion of the proximal phalanx of the third finger leads to opposition of the thumb) and Leri's sign (see Leri's sign). In some cases, the picture of spastic paraplegia is limited to the indicated symptoms concerning motor function and reflexes. More often, disorders of sensitivity, coordination (spastic-ataxic paraplegia), sphincters, and trophic functions are added, which is easily explained by anatomical relationships: the cross-section of the spinal cord is so small in size that a lesion here can only relatively rarely be restricted to the motor pathways without affecting other systems. Paraplegia dependent on lesions of the central motor neurons (pyramidal and extrapyramidal pathways: rubrospinal, tectospinal, etc.) may remain spastic the entire time. However, it can also be flaccid, either permanently or during certain phases of its development. Paraplegia proceeding throughout in a flaccid form, with atonia, with the extinction of tendon and periosteal reflexes, is always a sign of total or near-total transection of the spinal cord cross-section (usually traumatic cases) and is accompanied by m PARAPLEGIA

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also by the complete extinction of sensitivity in the area below the lesion site and profound disorders of the bladder and rectum functions (incontinence of urine and feces). Trophic functions are also deeply disturbed, and despite all precautions, severe bedsores develop. The development of bedsores and urinary incontinence, to which cystitis usually soon joins, highly burdens the prognosis in this form. In cases where flaccid paraplegia is inconstant, it takes place either in the initial phase of the disease or in the final one. The sudden development of flaccid paraplegia after trauma does not necessarily speak for the presence of a complete transection of the spinal cord cross-section. The extinction of tendon reflexes and the drop in tone can also be a consequence only of diaschisis (see), developing due to the suddenness of the lesion, even if the cross-section of the spinal cord was affected only partially. In such cases, over time, with the overcoming of diaschisis, the symptoms of flaccid paraplegia begin to yield to the symptoms of spastic paraplegia, pathological reflexes appear, tendon and periosteal reflexes, protective reflexes and syncinesias appear and increase, and tone increases. In the final phase, flaccid paraplegia develops in cases where the process that initially caused the development of spastic paraplegia spreads increasingly across the cross-section of the spinal cord or compresses it more and more. This is especially observed in spinal cord tumors. In such cases, the reflexes initially increased fade away, spastic hypertonia is replaced by atonia, sensory and trophic disorders increase, urinary and fecal incontinence appears, in other words, a picture of flaccid paraplegia develops in the form already described above. Paraplegias arising due to a lesion of the peripheral motor neurons [poliomyelitic processes, paresie analgesique in syringomyelia (see), lesions of the cauda equina, especially its traumatic injuries and tumors, deep radiculitis and plexitis, polyneuritis, especially alcoholic, but also of other intoxicational and infectious origin], are always flaccid, i. e., accompanied by atonia and the extinction of tendon and periosteal reflexes. In this they are symptomatologically similar to flaccid paraplegias of central origin. But there are also major differences. On the one hand, sphincter disorders, so typical of flaccid spinal paraplegia, are usually absent here. On the other hand, there are symptoms that do not occur in paraplegia of central origin, namely, muscle atrophies with qualitative changes in electrical excitability, with the reaction of degeneration. In paraplegia of peripheral origin, such phenomena as pathological reflexes, protective reflexes, and syncinesias are absent. For the appearance of these symptoms, a lesion of the pyramidal system is necessary, and it is not involved in the pathological process in these cases. Finally, cases of paraplegia are possible where both peripheral and central conductors turn out to be affected simultaneously, as can, for example, take place in meningomyelitis with the involvement of the anterior roots in the process, in extramedullary tumors of a special localization, in amyotrophic lateral sclerosis. The clinical picture here consists of symptoms of both spastic paraplegia and flaccid paraplegia of the peripheral type in various combinations, although the phenomena of spastic paraplegia usually come far to the fore. Diagnosis. The first task of differential diagnosis is to distinguish organic paraplegia from hysterical paraplegia (pseudoparaplegia, pseudoparesis). Of primary importance is the absence in hysterical paraplegia of symptoms whose occurrence is possible only with an organic lesion: protective reflexes, pathological reflexes, foot and patellar clonus, syncinesias, sphincter disorders, muscle atrophy with the reaction of degeneration. But in addition, the analysis of the general condition and character of the patient, as well as the analysis of other symptoms from the nervous system, is of great importance for the diagnosis. Distinguishing organic paraplegia of central origin from paraplegia of peripheral origin usually presents no difficulties. True, paraplegia of central origin can also be flaccid, accompanied by a drop in tone and the extinction of reflexes. However, qualitative changes in excitability characteristic of lesions of the peripheral nervous system are not observed here, and on the other hand, there are severe disorders of urination and defecation, which are absent there. The diagnosis of spastic paraplegia as such does not encounter difficulties, since as a rule there are always symptoms indicating a lesion of the pyramidal tract, and these symptoms are very characteristic and are established without difficulty. However, it must be remembered that paraplegia is only a symptom or symptom complex, that the diseases that can be expressed by this symptom complex are very numerous and that the diagnosis should strive to elucidate their nature, and not be limited to the statement of this syndrome. First of all, it is necessary to keep in mind the following diseases: compression myelitis (especially in tuberculous spondylitis and extramedullary tumors), syphilitic meningomyelitis, traumatic lesions of the spinal cord (compression, crushing, hematomyelia), multiple sclerosis, intramedullary tumors, syringomyelia, systemic diseases of the spinal cord (amyotrophic lateral sclerosis, lateral sclerosis, so-called combined scleroses). But a number of other diseases are also possible. In cerebral lesions, the case in the majority of cases is bilateral softening of the brain (specific arteritis, arteriosclerosis). The diagnosis of the disease must be based, of course, on the totality of all its symptoms and on the analysis of the course of the disease. Of great importance is the determination of the level of the lesion. In cases where there is only lower paraplegia, the case is in the overwhelming majority of cases a lesion localized below the cervical enlargement of the spinal cord. Where there is also upper paraplegia, the process is invariably localized not lower than the cervical enlargement. A more precise diagnosis is determined by the state of reflexes (especially the extinction of skin reflexes), the level of anesthesia, and the zone from which protective reflexes are elicited (reflexogenic zone). Especially valuable here is the method of Babinski and Jarkovsky: the upper border of anesthesia corresponds to the upper level of the lesion, the upper border of the reflexogenic zone to the lower level of the lesion, the height of the lesion to the gap between these boundaries. The method is especially valuable for recognizing the position of extramedullary tumors, since an exact topical diagnosis is a necessary condition for surgical intervention. The question of the prevalence of the process across the cross-section of the spinal cord is decided by the depth of paralytic symptoms, by participation in the picture of the disease of sensory disorders, etc. Complete transection of the spinal cord invariably gives flaccid paraplegia. Conversely, the presence of flaccid paraplegia in the initial phase of the disease does not yet exclude the possibility of partial preservation of the spinal cord cross-section. The prognosis is entirely determined by the nature of the underlying disease. Other things being equal, flaccid paraplegia gives a worse prognosis than spastic, and paraplegia with extensor contractures gives a better prognosis than flexor paraplegia (paraplegie en flexion). Treatment should be directed primarily to the underlying disease: specific treatment of syphilitic meningitis, orthopedic treatment of spondylitis, surgical treatment of extramedullary tumors, etc. But symptomatic treatment is also not devoid of significance. Electrotherapy is indicated in cases of paraplegia of peripheral origin, while in spastic paraplegia its use is associated with the risk of increasing contractures. The use of massage, passive and active gymnastics is appropriate. Warm baths often have a good effect. In some cases, the use of mud procedures works well, especially in cases of specific meningomyelitis (in combination with specific treatment) and residual phenomena of traumatic injuries of the spinal cord. Of course, contraindications from the internal organs (cardiovascular system, lungs!) must be absent in this case. It must be remembered that mud therapy is absolutely contraindicated in multiple sclerosis, in systemic scleroses, in tuberculous spondylitis, and in paraplegia with a major disorder of temperature and pain sensitivity (syringomyelia, hematomyelia).