Baló disease is a neurological disorder also known as concentric sclerosis, which is the acute variant of multiple sclerosis. Damage in the white matter, which forms a highly visible ring pattern due to demyelination, is the main feature of Baló disease. Treatment is usually with medication.
What is Baló disease?
Spiral demyelination of the white matter in the central nervous system (CNS) is the main feature of Baló disease. The white matter is the myelinated (and thus electrically insulated) fibers of the neurons. In 1928, the Hungarian József Baló described the disease for the first time in deceased patients. Today, with the help of magnetic resonance imaging (MRI), physicians can visualize the characteristic clinical pattern in the cerebrum: The neurodegenerative disease leaves concentric circles in the white matter that resemble the annual rings of a tree or a sliced onion. They occur because demyelination does not affect myelin cells uniformly, but causes alternating stronger and weaker atrophy. Baró disease represents a variant of multiple sclerosis that is also characterized by demyelination of the white matter and often occurs in episodes. Synonyms for the disease include Baló concentric sclerosis, encephalitis periaxilis concentrica, and Baló disease.
Causes
Specific causes of concentric sclerosis have not been adequately studied; they are due to more than one influence and are thus multifactorial. Baló disease is the acute variant of multiple sclerosis and therefore may trace back to similar risk factors. These factors include genetic predisposition and environmental factors. In the literature, scientists discuss dietary influences such as vitamin D deficiency and Epstein-Barr virus infections, among others. According to current knowledge, demyelination results from inflammation in the CNS, which in turn is due to an autoimmune reaction. Multiple sclerosis, the chronic form, involves multiple foci of inflammation. Researchers explain the concentric circles by a substance that originates outside the brain and migrates through the blood–brain barrier into the tissues, spreading rhythmically by diffusion. This type of diffusion is due to properties of the tissue and is not specific to this substance, which damages myelin as it spreads, leaving concentric circles in the tissue. Depending on which areas of the nervous system are damaged, the disease can manifest itself in different symptoms.
Symptoms, complaints, and signs
The outwardly visible and noticeable symptoms of Baló disease can vary greatly depending on the patient. Depression, ataxia, cognitive performance impairment, movement disorders, spasticity, paralysis, and pain with no apparent physical cause are complaints that may potentially occur in multiple and concentric sclerosis. Sensory disorders, in which the physical sensation of certain stimuli is limited or absent, are also conceivable. Medicine distinguishes four basic types of sensitivity disorders, each of which can manifest itself in all qualities of sensation: decreased sensitivity (hypesthesia), increased sensitivity (hyperesthesia), misperception of stimuli (dysesthesia; for example, pain from light touch), and perception of stimuli that are not present in the true sense (paresthesia; for example, tingling in the fingers). These symptoms can occur in numerous neurologic conditions and can include sensitivity to temperature, touch, movement, position, vibration, force, and pain.
Diagnosis and disease progression
Imaging is required to make the diagnosis; magnetic resonance imaging (MRI) is the most commonly considered method because of its high spatial resolution. Baló disease is considered confirmed when MRI reveals the typical concentric pattern in the white matter of the cerebrum. In addition, the MRI image shows demyelination signs suggestive of multiple sclerosis in almost half of the patients. Among those affected, young adults are particularly well represented.Doctors cannot yet say with certainty which course is actually typical for Baló disease: early detection was not possible for a long time, and until 2004 many findings about the clinical picture were based on examinations that took place after the death of the affected patients. Reliable predictions are also difficult to make at the individual level. However, Baló disease probably progresses in a single, severe phase.
Complications
In most cases of this disease, severe paralysis occurs over the entire body. In all cases, paralysis usually leads to severe limitations in movement and disturbances in sensitivity, resulting in a significant reduction in quality of life. In some cases, the affected person is dependent on a wheelchair or on the help of other people in everyday life. Spasticity may also occur. It is not uncommon for disorders of mental and motor development to occur in the course of development. This can lead to teasing and bullying, especially in children. Also stimuli can usually not be perceived correctly and the affected persons have a strong sensitivity to temperatures. It is not uncommon for severe pain to occur. Parents may also suffer from mental illness or depression as a result of the symptoms. The treatment of this disease is carried out with the help of medications. There are no further complications. However, not all complaints can be limited. If necessary, it comes to a reduced life expectancy. The affected person is thereby dependent on various therapies.
When should one go to the doctor?
When movement disorders, paralysis, and other signs of Baló concentric sclerosis are noticed, a physician should be consulted. The condition must be treated before serious complications develop. If health problems have already occurred, a medical professional should be consulted immediately. The same applies if the physical symptoms are accompanied by psychological complaints, such as depression or changes in personality. If severe paralysis develops, medical advice is also required. Parents who notice disturbances in their child’s mental and motor development should consult a pediatrician. He or she can diagnose Baló’s disease and, if necessary, initiate treatment directly. This is necessary at the latest when the child complains of pain. Since the disease can be a great psychological burden for both child and parents, a therapist must also be consulted to accompany the medical treatment. Further contacts are internists and orthopedists. A nutritionist can work out an individual diet in consultation with the parents and thereby alleviate the symptoms.
Treatment and therapy
To treat Baló disease, physicians often use glucocorticoids in practice, although they do not improve all cases. The steroid hormone also occurs naturally in the human body; the adrenal cortex produces it to break down fats and proteins and synthesize carbohydrates. In high doses, as found in drugs, they inhibit the formation of proteins. These include the immune system antibodies that falsely trigger an inflammatory response in Baló’s disease and multiple sclerosis. Mitoxantrone may also be considered as an agent, which is also used in multiple sclerosis; however, to date, there are only single-case reports of its efficacy in concentric sclerosis. The same applies to other forms of treatment such as the exchange of blood plasma (plasmapheresis). The possibilities and risks of various options must be assessed individually by the treating physician in each case.
Outlook and prognosis
Baló concentric sclerosis is one of the variants of multiple sclerosis, a so-called borderline form. It affects the central nervous system and, unlike MS, does not usually occur in relapses. In Baló concentric sclerosis, typical lesions form in the demyelinated tissue and are arranged concentrically. The prognosis of Baló concentric sclerosis is somewhat better than that of multiple sclerosis. In some cases, spontaneous remission even occurs. In others, an asymptomatic case may follow an individual course. In many monophasic courses, the symptoms of acute inflammatory demyelinating disease can be improved.Also called encephalitis periaxialis concentrica, this disease of the CNS occurs preferentially in young adults. The symptoms depend on which region of the brain is affected. While in the past this disease was always fatal and could only be diagnosed post mortem, the outlook is better today. Treatment can be initiated with gluco-corticoids, immunosuppressants or plasmapheresis. If Baló lesions are present in concentric sclerosis as in MS, the disease courses may also be similar and relapsing. This is the case in about one third to one half of those affected. In this case, MS drugs are also administered for prophylaxis and relapse prevention. In a monophasic course of the disease, the prognosis is generally better.
Prevention
Because the causes of Baló disease are still largely unknown and its development is based on the interaction of many complex factors, targeted and reliable prevention is not possible.
Aftercare
In most cases of Baló sclerosis, only a few measures of direct aftercare are available to the patient. Here, a diagnosis of the disease with subsequent treatment must take place at a very early stage to prevent further complications. If the disease is not treated properly by a physician, there are usually significant limitations in movement as the disease progresses. These can have a very negative effect on the quality of life of the affected person. Patients with this disease are usually dependent on taking various medications. Care should always be taken to ensure that the correct dosage is used and that the medication is taken regularly in order to provide lasting relief from the symptoms. Many patients are dependent on the support and help of other people in their everyday lives, whereby the help and care of one’s own family in particular can have a very positive effect on the further course of this disease. In this context, intensive and loving conversations with the affected person are also very important, so that depression and other psychological upsets can be prevented. Whether the sclerosis Baló leads to a reduced life expectancy of the affected person, can not be universally predicted in this case.
Here’s what you can do yourself
Patients with Baló concentric sclerosis usually suffer from severe limitations in everyday activities as well as quality of life. The reason for this is, on the one hand, the motor disturbances and movement restrictions, whereby long-term physiotherapy makes a positive contribution to the patient’s body image. Patients also perform certain physiotherapeutic exercises at home and thus support the maintenance of the musculature. If paralysis occurs or mobility restrictions are too great, many patients use a wheelchair. It makes sense to adapt the patient’s home to the use of a wheelchair. In some cases, a move to a barrier-free environment is necessary to allow patients more independence. In addition, care facilities come into question, in which patients additionally benefit from the social aspect through contact with other sufferers. An important factor for the quality of life of the patients is the proper intake of the prescribed medication. However, the life expectancy of those affected is reduced even with adequate therapy. In conjunction with the pain and other symptoms, some patients develop depression. Psychotherapeutic treatment helps affected individuals alleviate their mental suffering.