Baló’s concentric sclerosis is a disease that destroys the myelin of neurons.
Baló’s disease is a rare neurological disease, similar to multiple sclerosis, that affects the myelin of the brain and causes symptoms such as muscle spasms, paralysis or seizures. It is a very disabling disease and today there is no cure.
In this article we explain in more detail what the disease consists of, what its causes are, the symptoms it causes and the usual treatment indicated.
What is Baló’s disease?
Baló’s disease or Baló’s concentric sclerosis was described in 1928 by the Hungarian doctor Jozsef Baló. It is a rare demyelinating disease (in which the myelin, the protective layer of the axons, is damaged), considered a variant of multiple sclerosis.
The term “concentric sclerosis” is due to the presence of a pattern of concentric (circular) areas of damaged myelin alternating with areas of relatively intact myelin in various areas of the brain and spinal cord.
Generally, Baló’s disease presents acutely and affects, as occurs with multiple sclerosis, young adults, with rapid progression until the person dies. However, cases have also been reported in which there has been a progressive course, partial and even total remissions, both spontaneously and followed by conventional therapeutic treatments.
This rare disease affects men and women equally, and its incidence seems to be more common in people of oriental origin, especially from countries such as China and the Philippines.
Causes and diagnosis
Although the causes of Baló’s disease and its variants are unknown today, there are studies that indicate that autoimmune factors may play a prominent role in its development.
Autoimmune disorders occur when the body’s natural defenses, against foreign or invading organisms, begin to attack healthy tissue for unknown reasons, thus causing inflammation (swelling).
The causes of the recovery observed in some patients who have received, or not, the indicated treatment for Baló’s disease are also not currently known, so much remains to be investigated in this regard .
Years ago, the diagnosis of this disease was obtained after performing an autopsy on the deceased patient. However, today, with new neuroimaging techniques, earlier detection of the disorder is already possible.
Professionals usually rely on consistent and specific clinical signs and symptoms, trying to exclude other neurological diseases. Concentric rings characteristic of this disease can be seen in magnetic resonance images.
Symptoms of the disease
The characteristic symptoms of Baló’s disease vary depending on the areas of the brain that are affected. Demyelinating lesions in the brain can be located in any area (brain, cerebellum, or brainstem).
The sessions usually consist of irregular demyelination plaques that extend in a series of concentric circles, as we discussed at the beginning. The symptoms caused by the disease are very varied: persistent headache, progressive paralysis, involuntary muscle spasms, seizures, intellectual disability and cognitive loss or deterioration.
The symptoms caused by Baló’s disease can be very disabling for the person who suffers them and can be seriously life threatening, progressing rapidly in a few weeks or, conversely, evolving more slowly over 2 or 3 years.
Due to the low incidence of a disorder such as Baló’s disease and the limited number of cases described, no systematic studies have been carried out for the treatment of the disease.
The usual treatment is the same as that applied to people suffering from flare-ups in multiple sclerosis ; that is, consumption of corticosteroids at high doses to reduce severity in acute presentations, through their anti-inflammatory actions. The use of immunosuppressive drugs seems to be indicated by the associated poor prognosis.
Treatment to relieve symptoms such as spasticity, weakness, pain, or ataxia includes drug and rehabilitation modalities. However, Baló’s disease has a fatal course and lacks episodes of exacerbation and remission, as it does in multiple sclerosis.
Baló’s disease shares symptoms with another series of neurological diseases, which is why it is important to know which ones they are to make a proper diagnosis.
It is a rare inherited metabolic disorder characterized by brain demyelination and progressive degeneration of the adrenal gland.
Symptoms of this disorder include: generalized muscle weakness (hypotonia), exaggerated reflex responses (hyperreflexia), impaired ability to coordinate movements (ataxia), spastic partial paralysis, and / or tingling or burning sensations in the arms or legs.
2. Multiple sclerosis
Multiple sclerosis is a disease of the central nervous system that causes destruction of myelin or brain demyelination.
The course of the disorder is variable, since the patient may relapse, remit symptoms, or stabilize. Symptoms of this disease include double vision (diplopia), involuntary rhythmic movements of the eyes (nystagmus), impaired speech, numbness of the arms and legs, difficulty walking, etc.
3. Canavan leukodystrophy
It is a rare hereditary type of leukodystrophy characterized by progressive degeneration of the central nervous system. Symptoms include progressive mental decline accompanied by increased muscle tone (hypertonia), an enlarged brain (megalocephaly), poor head control, and / or blindness.
Symptoms usually begin during childhood and can include a general lack of interest in daily life (apathy), muscle weakness (hypotonia), and the loss of previously acquired mental and motor skills. As the disease progresses, there may be spastic muscle contractions in the arms and legs, lack of muscle strength in the neck, swelling of the brain (megalocephaly), and paralysis.
4. Alexander disease
Alexander disease is an extremely rare progressive metabolic disorder, which is often inherited. It is one of the subtypes of leukodystrophy. This disorder is characterized by demyelination and the formation of abnormal fibers (Rosenthal fibers) in the brain.
Symptoms of this disease can include muscle spasms, mental decline, and / or delayed growth. Most babies with Alexander disease have an abnormally large head (megalencephaly), lack of growth, and seizures.
- Karaaslan E, Altintas A, Senol U, et al. Balo’s concentric sclerosis: clinical and radiological characteristics of five cases. AJNR Am J Neuroradiol. 2001; 22: 1362-67
- Shankar SK, Rao TV, Srivastas VK, Narula S, Asha T, Das S. Balo’s concentric sclerosis: a variant of multiple sclerosis associated with oligodendroglioma. Neurosurgery 1989; 25: 982-986