Cone syndrome is a paraplegic syndrome caused by pressure damage to the lower spinal cord at the level of the conus medullaris and is associated with voiding dysfunction. It most commonly occurs in the setting of a herniated disc. The syndrome is a medical emergency and provides an immediate indication for surgical decompression to prevent irreversible damage.
What is cone syndrome?
The brain and spinal cord make up the central nervous system. All motor activity is controlled via the nerve tissue located here. The spinal cord, in particular, assumes an essential role in controlling movement. Particularly important in this context are the pyramidal tracts, from which all voluntary and reflex movement commands are transported via efferent nerve tracts to the muscular success organs. Damage to the spinal cord has correspondingly serious consequences. Lesions in the area of the entire spinal cord diameter, for example, are called paraplegic syndromes. All cross-sectional syndromes are accompanied by paralysis. Depending on the type of spinal cord damage, the paralysis symptoms are either flaccid or spastic paralysis. The so-called conus syndrome is a cross-sectional syndrome with damage location at the level of the conus medullaris, affecting spinal cord segments S3 to S5. Leading symptoms of the syndrome are incontinence and disturbances in sexual function.
Causes
The cause of conus medulla syndrome is compression of the lower spinal cord. Pressure on the conus medullaris at the end of the caudal spinal cord causes the disorders associated with the syndrome. More specifically, the sacral spinal cord segments from S3 to S5 are predominantly affected by the associated compression. The coccygeal spinal cord at the level of the lumbar vertebral bodies may also be involved. In most cases, cone syndrome is the result of a medial disc herniation. Less commonly, circulatory disturbances or even tumors are responsible for compression of the corresponding spinal cord segments. The syndrome has also been reported in cases where the conus medullaris is congenitally attached to its surrounding structures. In such a case, however, the syndrome is referred to as tethered cord syndrome rather than conus syndrome, which damages the conus medullaris during growth. In almost all cases, conus syndrome is associated with the so-called cauda syndrome and is sometimes referred to as conus-cauda syndrome in this context.
Symptoms, complaints, and signs
Like all other paraplegic syndromes, cone syndrome is predominantly associated with movement disorders. In the case of conus syndrome, these are primarily movement disorders of the bladder and anus sphincters. Stool can often no longer be held by patients. The same applies to urine. Micturition disorders such as permanently dribbling urine are a leading symptom of the syndrome. Micturition disturbance may also be overflow incontinence, in which the patient’s bladder is constantly full but cannot be emptied voluntarily. In most cases, no movement disorders are present in the early course. Initially, the cone syndrome usually manifests itself in a so-called breeches anesthesia, which is characterized by symmetrical sensory disturbances on the posterior inner side of both thighs. This skin area is supplied by the nerves of segments S1 to S3. The male in particular often suffers from erectile or ejaculatory dysfunction in later stages of the cone syndrome. As a rule, at least slight pain in the hip area occurs due to the compression, since sensitive nerves are also damaged by the pressure condition on the lower spinal cord. Paralysis of the legs usually does not occur.
Diagnosis and course of the disease
The physician makes the diagnosis of cone syndrome by history and imaging. The patient’s anus is essentially flaccid because the sphincter muscle is not working adequately. As part of the diagnostic process, a failed anal or bulbar cavernous reflex may provide clues to conus syndrome. Patellar and Achilles tendon reflexes are preserved in isolated conus syndrome. Imaging that confirms the diagnosis and clarifies the cause may include CT or MRI. Imaging may reveal, for example, a causative disc herniation. For patients with cone syndrome, the prognosis depends largely on the timing of diagnosis. If the compression persists too long, the affected nerves die irreversibly.
Complications
Cone syndrome represents a very serious and dangerous condition for the affected person’s body. If treatment is not initiated immediately, it can usually result in irreversible secondary damage and paralysis throughout the body. The affected person suffers from micturition disorders and from paralysis and tingling sensations. The quality of life is considerably restricted and reduced by the cone syndrome. Not infrequently, this also leads to movement disorders, so that the patient may also be dependent on a wheelchair. The function of the bladder is also impaired, so that urination can no longer be controlled properly. It is not uncommon for this to lead to depression and other psychological complaints or moods. Sensory disturbances can occur all over the body, which have an extreme effect on everyday life. The genitals are usually affected by erectile dysfunction. The patient’s quality of life is drastically reduced by the conus syndrome. It is not uncommon for the hip to also experience severe pain. Treatment of the conus syndrome must take place immediately. Complications occur mainly when only delayed treatment is initiated. This can result in irreversible damage, so that the affected person suffers from the consequences and paralysis for the rest of his life. No general statement can be made about the limitation of life expectancy.
When should one go to the doctor?
Cone syndrome always represents a medical emergency. Treatment must be initiated immediately when symptoms such as paralysis or numbness in the legs are noticed. After initial treatment by the emergency physician, the patient must be examined in a specialized clinic. Long-term therapy is planned individually depending on the severity of the paraplegia and is carried out by different specialists. Individuals suffering from cone syndrome must have close consultation with the responsible physician. The paraplegia syndrome can be treated well, however, during the course of the condition, various symptoms may develop that require individualized treatment. Patients who suddenly notice signs of movement disorders of the bladder and anus sphincters should inform the physician. Unusual sensory disturbances or pain should also be clarified if they occur suddenly and a medical complication is suspected. The conus syndrome considerably restricts the quality of life, which is why those affected often need therapeutic support. If depressive moods or other problems become apparent, it is best to inform the medical professional. He or she can examine the patient to rule out an organic cause and consult a therapist if necessary.
Treatment and therapy
Cone syndrome is always a medical emergency that requires immediate intervention. The longer the pressure on the lower spinal cord persists, the more likely there will be permanent damage to the nerves. After diagnosis, surgical decompression is performed at the earliest possible time. Conservative medicinal measures are not suitable for treatment. The cause of the present disturbance must be eliminated, in this case the pressure condition close to the spinal cord. In individual cases, a so-called nucleoplasty is used to relieve the pressure on the lower spinal cord. This procedure is a minimally invasive treatment for the therapy of herniated discs. In this case, the intervention takes place under local anesthesia. During the procedure, the surgeon works with a special probe that is inserted into the spinal cord. Under certain circumstances, patients require specific physiotherapy after the intervention. The symptoms caused by the compression usually improve as soon as the spinal cord is decompressed. However, unloading usually does not fully restore the physiologic baseline condition. Physiotherapeutic measures are particularly important in cone syndrome that was recognized and corrected late.
Outlook and prognosis
The prospect of recovery in conus syndrome depends on the promptness of medical treatment. Because the condition is an intensive care emergency, emergency medical services must be alerted as soon as possible. In addition, the taking of first aid measures by persons present is mandatory for a good prognosis.If emergency or medical care is refused, the prospect of recovery deteriorates immensely. Normally, it can no longer be achieved in the further course of life. The damage suffered by the organism is irreversible and usually triggers consequential damage. The later comprehensive therapy can be initiated, the more severe the long-term effects of the cone syndrome. In many patients, freedom from symptoms cannot be achieved despite all efforts, because full treatment did not take place immediately after the triggering event. Paralysis or limitations of movement often remain for life. In addition, psychological sequelae are to be expected due to the emotional burden of the disease. Initiated therapies, the administration of medication and invasive treatments can also trigger complications or side effects. These lead to a need to optimize the treatment plan and can delay the healing process. Many patients report impaired quality of life months or years after the triggering event because of lingering symptoms.
Prevention
Because disc herniation is the most common cause of cone syndrome, the same preventive measures that apply to herniated discs apply largely to prophylaxis of the syndrome. Back training or regular visits to a back school, for example, have been shown to be effective preventive measures associated with herniated discs.
Aftercare
In most cases of cone syndrome, the measures of an aftercare are very limited. Usually, the disease cannot be fully treated either. First and foremost, the affected person depends on a quick diagnosis to prevent further paralysis of the body. In many cases, the paralyses themselves can no longer be reversed, so that in these cases the affected person no longer has any aftercare measures available. Most of those affected are therefore dependent on the help of other people in their lives and need support in their everyday lives. In this context, the help and care provided by one’s own family and friends has a particularly positive effect on the further course of the disease. Depression or other psychological upsets can also often be alleviated or even prevented as a result. Physiotherapy measures are also often necessary for cone syndrome. The affected person can repeat the exercises at home and thus accelerate the healing process. Further measures of an aftercare are usually not possible. However, the syndrome does not usually reduce the life expectancy of the affected person.
What you can do yourself
Cone syndrome is always a medical emergency. As soon as the first signs of the paraplegic syndrome appear, an emergency medical service must be called. Until they arrive, first aid measures must be given or the affected person should lie down and try to breathe calmly. After the operation, the affected person must take it easy. Sports and other physical activities should be avoided for the first few days. After a week, gentle sports may be started again in consultation with the doctor. If the cone syndrome is only detected at a late stage, the damage that has already occurred must be corrected. This is achieved primarily through physiotherapeutic measures, which can be supported by the affected person through individual training. The physician will refer the patient to a suitable physiotherapist for this purpose. Determining and eliminating the cause is an important treatment step. Many patients with cone syndrome suffer from obesity or work in an occupation in which the back is under heavy strain. These triggers must be found and corrected as soon as possible. This can be achieved by changing the workplace, changing lifestyle habits or accompanying therapy. Regular back training or attendance at a back school can also alleviate the symptoms and prevent a recurrence of cone syndrome.
