Elsberg syndrome is an inflammatory disease of the lumbar spine and is equivalent to polyradiculitis. Inflammation often affects the sacral nerve roots as well as the conus medullaris and cauda equina of the lower spinal cord. The syndrome is considered to have a favorable prognosis.
What is Elsberg syndrome?
In the polyradiculitis group of diseases, multiple nerve roots are affected by inflammation. Pathohistologic changes in nerve tissue occur as part of the inflammation. A polyradiculitis is also the Elsberg syndrome. The symptom complex is named after the U.S. neurosurgeon Charles Elsberg, who first described the disease in the 20th century. In the disease, the lower spinal cord, including the sacral nerve roots, becomes inflamed. These are the nerve roots of the sacrum at the back of the bony pelvis. Also affected by inflammation are the conus medullaris and cauda equina in the lower spinal cord. The medical terms polyradiculitis sacralis and radiculitis sacralis are used synonymously with Elsberg syndrome. To date, Elsberg syndrome has not been conclusively studied. The original description by Elsberg has also been modified. For example, it no longer refers to a chronically progressive disease but to a mostly regressive disease.
Causes
The exact cause of Elsberg syndrome has not yet been determined. It is probably a variant of Guillain-Barré syndrome. This is an acute clinical picture associated with inflammatory changes in the peripheral nervous system. The cause of Guillain-Barré syndrome also remains unknown. However, the neurological disease probably arises from an autoimmune pathological mechanism that causes the body to produce autoantibodies against gangliosides and myelin. In the case of Elsberg syndrome, there is an association with certain viral infections, such as herpes simplex type 2, cytomegalovirus, HIV, and Epstein-Barr virus. However, Elsberg syndrome can equally occur within the larger context of neuroborreliosis. Other sources speak of an outbreak following circulatory disorders, in the context of vascular inflammation, or in worm infections.
Symptoms, complaints, and signs
The clinical picture in patients with Elsberg syndrome corresponds to the so-called cone cauda syndrome. A loss of sensibility in the distal of the L3 spinal cord segment is accompanied by radicular pain, often severe, and may result in flaccid paralysis of the legs. Typically, bladder emptying or sexual dysfunction also occurs. Rectal dysfunction may also result from Elsberg syndrome. Sensory disturbances are often structured into lumbar and sacral dermatomes. Most often, they are paresthesias associated with unpleasant, painful body sensation and accompanied by tingling, numbness or falling asleep, as well as cold and heat perception disorders. In most cases, the symptoms occur in a relatively short time and with suddenness. Reflective disorders such as the so-called pyramidal tract signs are rather atypical for Elsberg syndrome. Instead of a lesion of the pyramidal tracts, there is myelitis of the caudal portions of the spinal cord.
Diagnosis and course
For diagnosis, if Elsberg syndrome is suspected, the neurologist performs a cerebrospinal fluid (CSF) examination and punctures the spinal cord to give cerebrospinal fluid from the external ventricle for laboratory diagnostic testing. In Elsberg syndrome, laboratory diagnostic CSF analysis demonstrates cytoalbumin dissociation, pleocytosis, and IgG elevation. Magnetic resonance imaging may be required to rule out space-occupying lesions. Differential diagnosis should also be considered for conus cauda syndrome, which is often associated with herniated discs of the lumbar spine or tumor space involvement. In Elsberg syndrome, medical opinion on the course of the disease has changed since it was first described. Elsberg described the disease as chronically progressive and indicated a rather unfavorable course. However, the past has proven that it is usually not a progressive disease. Symptoms have completely resolved in many of the cases observed.
When should one go to the doctor?
In the event of sudden onset of sensory disturbances, bladder dysfunction, or rectal dysfunction, a physician should be consulted in any case. Although the symptoms do not necessarily indicate Elsberg syndrome, they are almost always based on a serious underlying condition that must be medically clarified and, if necessary, treated. If symptoms such as tingling, numbness or a slackening of the legs are added, Elsberg’s syndrome is likely – at the latest then a medical clarification is necessary. If symptoms are severe, Elsberg’s syndrome should be treated by consulting an emergency physician or the nearest hospital. This is especially true for people who are already physically weakened by another illness. If they suddenly notice symptoms of Elsberg syndrome, immediate emergency medical clarification is required in any case. Otherwise, serious complications such as a herniated disc or sensitive cold and sensory disturbances may develop. Further risk groups are patients with an existing neuroborreliosis, circulatory disorders, vascular inflammations or worm infections. Patients with these and similar conditions should speak with their primary care physician expeditiously if any of the above symptoms are noticed.
Treatment and therapy
In Elsberg syndrome, therapy depends primarily on the underlying disease. Administration of immunoglobulins and corticosteroids may promote symptom relief. As a variant of Guillain-Barré syndrome, Elsberg syndrome can be completely cured. Timely diagnosis favors cure. As with Guillain-Barré syndrome, the main concern in mild forms of the disease is to prevent thrombosis and infection. Physiotherapy can help the patient regain muscle control after leg paresis. A disease of unexplained etiology is often perceived by patients as a great shock. Psychological distress can interfere with the success of physical therapy. Therefore, psychotherapeutic care can improve the prospect of recovery. Immunotherapy is available for Guillain-Barré syndrome in acute and severe cases. This type of treatment may also be considered for patients with Elsberg syndrome. Either immunoglobulins are administered to the affected person as part of the therapy or plasmapheresis is used. Therapy with immunoglobulins is much gentler and has fewer side effects than plasmapheresis. However, this treatment is associated with high costs and turns out to be less effective in Guillain-Barré syndrome. Plasmapheresis is particularly suitable for rapidly progressing, long-lasting courses. A catheter is placed in the blood system. A machine pumps the patient’s blood out of and back into the body to purify immunoglobulins.
Outlook and prognosis
Controversial as its own entity, Elsberg syndrome usually shows a favorable course. It was originally described by New York neurosurgeon Charles Elsberg as a progressive disease. Today, the picture is more nuanced. In most cases, a regressive symptomatology is observed. However, the cause of Elsberg’s syndrome is not yet clear. Thus, it is also not certain whether it is only a symptom complex with several possible causes. However, the prognosis of the disease depends on this in each individual case. Even the possible underlying diseases can differ in their course and prognosis. Thus, a connection of Elsberg’s syndrome with the infections of herpes simplex, the cytomegalovirus or the HIV virus as well as with neuroborreliosis, but also with worm infections, vascular inflammations or circulatory disorders is described. Each of these conditions has its own course and thus influences the prognosis of the resulting Elsberg syndrome. Clinically, Elsberg syndrome is similar to cone cauda syndrome, which is characterized by paralysis in herniated discs or space-occupying processes in tumors. Elsberg syndrome is treated symptomatically with corticosteroids. However, permanent regression of symptoms can only be achieved by treating the underlying disease. If irreversible changes have not yet occurred, resolution of symptoms is possible even without therapy after the underlying disease has healed. More rarely, as in the case described by Elsberg, there is a progressive chronic development of symptoms.
Prevention
The primary cause of Elsberg syndrome has not yet been conclusively determined. For this reason, the symptom complex is difficult to prevent. However, because prompt diagnosis increases the chances of a favorable course, affected individuals should seek medical attention when times arise and have even the smallest sensory disturbances in the lumbar spine clarified.
Aftercare
In most cases, the options for follow-up care in Elsberg syndrome are severely limited. In this case, the affected person depends on treatment by a physician primarily to relieve the symptoms and prevent further complications. Since this cannot result in self-healing, a medical examination and treatment must always be performed. The treatment of Elsberg’s syndrome is usually carried out with the help of medication. Patients are dependent on taking the medication regularly in order to alleviate the symptoms. In the case of children, parents should check that the medication is being taken correctly and contact a doctor if there is any doubt or uncertainty. Furthermore, infections should generally be prevented. Since it is not uncommon for psychological treatment of the affected person to be necessary, the help and support of parents and one’s own family can also have a very positive effect on the further course of Elsberg’s syndrome and prevent further psychological upsets. In general, the affected person should take it easy and avoid exertion or stressful situations. Whether the syndrome will result in a decreased life expectancy for the patient cannot be universally predicted.
Here’s what you can do yourself
For better healing or alleviation of inflammatory symptoms, it is especially helpful if the patient has a healthy and stable immune system. For this, a balanced diet, sufficient exercise and avoidance of harmful substances such as nicotine or drugs are important. A good awareness of health promotes the physical resources needed to improve the symptoms. Movement patterns should be optimized to prevent damage to the skeletal system. One-sided physical stress or misalignment of the bones should be avoided. Postures should not be held in the same position for a long time. This puts unnecessary strain on the body. Countermovements are helpful to compensate. In addition, sporting activities support muscle development and general well-being. For emotional relief, it is advisable to have conversations with friends, relatives or people who are also affected. In a joint exchange, the challenges of the day can be discussed and tips for better handling of the complaints can be given. If this is not enough, it is advisable for the patient to seek psychological help. In addition, as a balance in everyday life, the applications of relaxation methods help to alleviate the mental stress. With techniques such as meditation or yoga, the patient can stabilize his psyche and reduce stress from everyday life.
