Balo Disease

Disease Overview

Balo Disease is a rare and progressive variant of multiple sclerosis. It usually first appears in adulthood, but childhood cases have also been reported. While multiple sclerosis typically is a disease that waxes and wanes, Balo Disease is different in that it tends to be rapidly progressive. Symptoms may include headache, seizures, gradual paralysis, involuntary muscle spasms, and cognitive loss. The alternative names for Balo Disease, concentric sclerosis or Balo concentric sclerosis, refer to the fact that Balo Disease is characterized by bands of intact myelin (the sheath of fatty substances surrounding nerve fibers), alternating with rings of loss of myelin (demyelination), in various parts of the brain and brain stem. The symptoms of Balo Disease vary, according to the areas of the brain that are affected. Symptoms may progress rapidly over several weeks or more slowly over two to three years.

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Synonyms

Concentric Sclerosis
Encephalitis Periaxialis Concentrica
Leukoencephalitis Periaxialis Concentric

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Signs & Symptoms

Most cases are characterized by the gradual onset of symptoms that might be found in the more common type of MS, including muscle spasms and paralysis. Other neurological symptoms develop depending on the areas of the brain that are affected and may include intellectual impairment and/or physiological abnormalities. However, in its most serious form, Balo Disease may also suggest the presence of an infectious disease, starting with a high fever and painful headaches.

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Causes

The cause of MS and its variants remains unknown. However, some studies indicate that autoimmune factors may play a role in the development of Balo Disease. Autoimmune disorders are caused when the body’s natural defenses against “foreign” or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons.

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Affected populations

Balo Disease is a rare disorder that affects males and females in equal numbers. More cases have been reported from China and the Philippines than elsewhere.

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Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of Balo Disease. Comparisons may be useful for a differential diagnosis:

Adrenoleukodystrophy is a rare inherited metabolic disorder characterized by the loss of the fatty covering (myelin sheath) around nerve fibers in the brain (cerebral demyelination) and the progressive degeneration of the adrenal gland. The symptoms of this disorder may include generalized muscle weakness (hypotonia), exaggerated reflex responses (hyperreflexia), impaired ability to coordinate movement (ataxia), spastic partial paralysis, and/or tingling or burning sensations in the arms or legs. (For more information on this disorder, choose “Adrenoleukodystrophy” as your search term in the Rare Disease Database.)

Multiple Sclerosis is a chronic disorder of the central nervous system (CNS) that causes the destruction of the covering that surrounds nerve fibers (myelin sheath). The course of the disease is variable. It may advance, relapse, remit, and/or stabilize. Symptoms may include double vision (diplopia), involuntary rhythmic movements of the eyes (nystagmus), speech impairment, numbness in the arms and legs, and/or difficulty walking. Impaired function of the bowel and bladder may also be present. (For more information on this disorder, choose “Multiple Sclerosis” as your search term in the Rare Disease Database.)

Canavan’s Leukodystrophy is a rare inherited type of leukodystrophy characterized by the progressive degeneration of the central nervous system. Symptoms may include progressive mental deterioration accompanied by increased muscle tone (hypertonia), poor head control, an enlargement of the brain (megalocephaly), and/or blindness. Symptoms typically begin during infancy. Early symptoms of Canavan’s Leukodystrophy may include general lack of interest in daily living (apathy), muscle weakness and floppiness (hypotonia), and the loss of previously acquired mental and motor skills. As the disease progresses, there may be spastic muscle contractions of the arms and legs, lack of muscle strength in the neck, swelling of the brain (megalocephaly), and paralysis. (For more information on this disorder, choose “Canavan” as your search term in the Rare Disease Database.)

Metachromatic Leukodystrophy (MLD) is a rare inherited leukodystrophy characterized by the abnormal accumulation of a fatty-like substance known as sulfatide in the tissues of the nervous system and other organs. This results in the loss of the coverings on nerve fibers (myelin sheath). Symptoms may include blindness, convulsions, muscle rigidity hypertonia) and/or motor disturbances that may lead to paralysis and dementia. (For more information on this disorder, choose “Metachromatic Leukodystrophy” as your search term in the Rare Disease Database.)

Krabbe’s Leukodystrophy is a rare inherited metabolic disorder characterized by the abnormal accumulation of a fatty substance (ceremide galactoside) in the brain. Symptoms develop due to a deficiency of the enzyme galactoside beta-galactosidase. These may include irritability, vomiting, episodes of partial unconsciousness, and/or seizures. There may also be spastic contractions of the legs, difficulty swallowing, and mental deterioration. (For more information on this disorder, choose “Krabbe” as your search term in the Rare Disease Database.)

Alexander’s Disease is an extremely rare, progressive metabolic disorder which is frequently inherited. It is one of the sub-types of Leukodystrophy. Alexander’s Disease is characterized by the loss of fatty layers that cover nerve fibers (demyelination) and the formation of abnormal fibers (Rosenthal) in the brain. The symptoms may include muscle spasms, mental impairment, and/or growth delays. Most infants with Alexander’s Disease have an abnormally large head (megalencephaly), failure to thrive, and seizures. (For more information on this disorder, choose “Alexander” as your search term in the Rare Disease Database.)

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Standard Therapies

Treatment is symptomatic and supportive. Corticosteroids are usually useful in decreasing severity of acute presentations through their anti-inflammatory actions. Treatment to relieve symptoms, such as spasticity, weakness, pain, or ataxia, includes pharmacologic and rehabilitative modalities.

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Clinical Trials and Studies

Therapies Investigational

Information current clinical trials is posted on the Internet at www.clinicaltrials.gov. All

studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

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References

TEXTBOOKS

Giesser BS. Rare Variants of Multiple Sclerosis. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:560.

Adams RD, Victor M, Ropper AA. Eds. Principles of Neurology. 6th ed. McGraw-Hill Companies. New York, NY; 1997:915.

Menkes JH, Pine Jr JW, et al. Eds. Textbook of Child Neurology. 5th ed. Williams & Wilkins. Baltimore, MD; 1995:535.

REVIEW ARTICLES

Dupel-Pottier C. [Diagnostic criteria of borderline forms of multiple sclerosis] Rev Neurol (Paris). 2001;157(8-9 Pt 2):935-43. French

Fontaine B. [Borderline forms of multiple sclerosis] Rev Neurol (Paris). 2001;157(8-9 Pt 2):929-34. French

Moser HW. Neurometabolic disease. Curr Opin Neurol. 1998;11:91-95.

JOURNAL ARTICLES

Moore GR, Berry K, Oger JJ, et al. Balo’s concentric sclerosis: surviving normal myelin in a patient with relapsing-remitting clinical course. Mult Scler. 2001;7:375-82.

Kastrup O, Stude P, Limmroth V. Balo’s concentric sclerosis. Evolution of active demyelination demonstrated by serial contrast-enhanced MRI. J Neurol. 2002;249:811-14.

Karaaslan E, Altintas A, Senol U, et al. Balo’s concentric sclerosis: clinical and radiological features of five cases. AJNR Am J Neuroradiol. 2001;22:1362-67.

Chen CJ. Serial proton magnetic resonance spectroscopy in lesions of Balo’s concentric sclerosis. J Comput Assist Tomogr. 2001;25:713-18.

Caracciolo JT, Murtagh RD, Rojiani AM, et al. Pathognomic MR imaging in Balo concentric sclerosis. AJNR Am J Neuroradiol. 2001;22:292-93.

Iannucci G, Mascalchi M, Salvi F, et al. Vanishing Balo-like lesions in multiple sclerosis. J Neurol Neurosurg Psychiatry. 2000;69:399-400.

Singh S, Kuruvilla A, Alexander M, et al. Balo’s concentric sclerosis: value of magnetic resonance imaging in diagnosis. Australas Radiol. 1999;43:400-04.

Chen CJ, Chu NS, Lu CS, et al. Serial magnetic resonance imaging in patients with Balo’s concentric sclerosis: natural history of lesion development. Ann Neurol. 1999;46:651-56.

FROM THE INTERNET

Balo Disease. nd. 1p.

www.kovaidoctors.com/diseases/b/b4.php

What is Multiple Sclerosis? Last Modified; 12/20/2002:4pp.

www.mult-sclerosis.org/whatisms.html

How does Multiple Sclerosis do its damage? Last Modified; 11/27/2002:4pp.

www.mult-sclerosis.org/howms.html

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Programs & Resources

RareCare^® Assistance Programs

NORD strives to open new assistance programs as funding allows. If we don’t have a program for you now, please continue to check back with us.

Additional Assistance Programs

MedicAlert Assistance Program

NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations.

Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/

Rare Disease Educational Support Program

Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.

Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/