Balo Disease: Symptoms, Types, Causes and Treatment

Balo's disease, or Baló’s concentric sclerosis (BCS), is a rare and intriguing neurological condition that continues to puzzle scientists and clinicians alike. Distinguished by its striking concentric rings of demyelination in the brain, Balo's disease stands apart from other demyelinating disorders both in its clinical presentation and imaging features. This article explores the symptoms, variants, underlying causes, and treatment options for Balo disease, weaving together the latest research and expert insights.

Symptoms of Balo Disease

Balo's disease often presents with a constellation of neurological and behavioral symptoms that can appear suddenly or progress over weeks to months. Recognizing these symptoms is crucial for timely diagnosis and effective management.

Symptom	Description	Frequency/Onset	Sources
Behavioral Change	Personality disturbance, confusion	Early/subacute	1 3
Motor Deficits	Weakness, hemiplegia, gait disturbance	Acute or progressive	2 3 5 6
Sensory Loss	Numbness, hypoesthesia	Acute/subacute	2 5
Incontinence	Double (urinary and fecal)	Subacute	1
Emotional Lability	Uncontrollable laughing/crying	Subacute	3
Speech Issues	Hyporeactivity, hypologia	Early	3
Visual Disturbance	Gaze palsy	Acute	5
Fatigue	General tiredness	Common	3
Coma	Severe, late-stage	Rare, late	5

Table 1: Key Symptoms

Behavioral and Cognitive Changes

One of the most striking features of Balo’s disease is the alteration in behavior and mental status. Patients may show indifference, reduced reactivity, or even frank personality changes such as social withdrawal or difficulty communicating. These changes can precede or accompany more classic neurological symptoms and may be mistaken for psychiatric disorders in the early stages 1 3.

Motor and Sensory Deficits

Acute or progressive weakness—often on one side of the body (hemiplegia)—is a common presenting complaint. Gait disturbances, loss of coordination, and difficulties with fine motor skills may also occur. Sensory symptoms, including numbness and loss of feeling, can develop alongside motor deficits 2 3 5 6.

Other Neurological Features

• Double Incontinence: Loss of bladder and bowel control has been observed, particularly in subacute or advanced disease 1.
• Emotional Lability: Patients may experience episodes of uncontrollable laughing or crying, reflecting involvement of brain regions controlling emotion 3.
• Speech and Language: Hyporeactivity (diminished response) and hypologia (reduced speech output) are sometimes noted 3.
• Visual Disturbances: Gaze palsy and other visual symptoms may occur, though less commonly 5.
• Fatigue: Generalized tiredness is almost universally reported by patients 3.
• Severe Complications: In rare, rapidly progressive cases, patients can lapse into coma due to extensive brain involvement 5.

Types of Balo Disease

While Balo’s disease is most often described as a variant of multiple sclerosis (MS), it displays its own unique characteristics. Recent advances have expanded our understanding of its different types and presentations.

Type	Defining Features	Patient Profile	Sources
Classical Balo	Concentric rings, solitary lesions	Young adults	4 6 7
MS-Associated Balo	Balo lesions + typical MS plaques	MS patients	4 7
Fulminant Balo	Rapid progression, poor outcome	All ages	4 5
Benign/Relapsing Balo	Slow/relapsing course, recovery possible	Young to middle-aged	2 3 8

Table 2: Types of Balo Disease

Classical Balo’s Disease

The hallmark of classical Balo’s disease is the appearance of concentric, ring-like lesions in the white matter of the brain, seen on MRI as alternating bands of normal and demyelinated tissue. These typically occur as solitary or few large lesions and are most commonly seen in young adults 4 6.

MS-Associated Balo Variant

Some patients with Balo’s disease also show typical MS lesions elsewhere in the brain or spinal cord. This has led to the view of Balo’s disease as a rare variant or subtype of multiple sclerosis. In these cases, patients may fulfill diagnostic criteria for MS and experience both Balo-type and typical MS relapses 4 7.

Fulminant and Benign/Relapsing Types

• Fulminant Balo: Some cases are rapidly progressive, leading to severe disability or death within weeks or months, as seen in several historical reports 4 5.
• Benign/Relapsing Balo: Other patients show a much milder or relapsing course, sometimes even recovering completely or experiencing only minor long-term deficits 2 3 8.

Radiological and Histological Features

Balo’s lesions are characterized by:

• MRI: “Fried egg” or multilayered “onion bulb” appearance, with alternating rings of enhancement and hypodensity.
• Histology: Concentric bands of demyelination, astrocytic proliferation, and relative axonal preservation 3 5 7.

Causes of Balo Disease

Despite decades of study, the exact causes of Balo’s disease remain elusive. However, recent research has provided important insights into the mechanisms that might underlie its distinctive pathology.

Cause/Factor	Mechanism or Role	Evidence Level	Sources
Immune-mediated	Autoimmune attack on myelin	Strong	4 7 8
Astrocytopathy	Astrocyte dysfunction, AQP4 loss	Emerging	7 8
Hypoxia-like Injury	Tissue preconditioning, cytokine release	Moderate	8
Genetic/Unknown	Potential predisposing factors	Limited	4 6

Table 3: Causes and Mechanisms

Immune-Mediated Demyelination

Like multiple sclerosis, Balo’s disease is fundamentally a demyelinating disorder driven by an abnormal immune response. The immune system targets myelin, the insulating sheath around nerve fibers, leading to the formation of characteristic concentric plaques 4 7.

Astrocytopathy and AQP4 Loss

Recent studies have highlighted the role of astrocytes—support cells in the brain—in Balo’s disease. Hypertrophic (enlarged) astrocytes are found in and around Balo lesions. Notably, these astrocytes show loss of aquaporin-4 (AQP4), a water channel protein, even though the classic antibodies found in neuromyelitis optica (NMO) are absent. This suggests a unique, possibly antibody-independent astrocytopathy in Balo’s disease 7.

Hypoxia-Like Tissue Injury

Research indicates that the formation of Balo’s concentric rings may involve a process similar to tissue preconditioning seen in response to low oxygen (hypoxia). Proteins such as hypoxia-inducible factor-1α are upregulated in glial cells near the lesions. These glial cells then release cytokines like interleukin-1β, which can promote demyelination and lesion formation 8.

Genetic and Other Factors

While no specific genetic mutation has been linked to Balo’s disease, its occurrence in young adults and occasional familial clustering suggest that genetic or environmental predispositions may play a role. The precise triggers remain unknown 4 6.

Treatment of Balo Disease

The rarity and variability of Balo’s disease make treatment challenging. However, several therapies have shown promise, particularly when initiated early in the disease course.

Treatment	Mechanism/Approach	Typical Outcome	Sources
High-dose Steroids	Reduce inflammation	Rapid improvement	2 4
MS Disease-Modifying	Immunomodulation	For MS-associated	4
Supportive Care	Symptom management	Varies	1 3 5
Experimental/Other	Plasma exchange, other agents	Limited evidence	4

Table 4: Treatment Options

High-Dose Steroids

The mainstay of acute treatment for Balo’s disease is high-dose corticosteroids, such as methylprednisolone. These agents rapidly suppress inflammation and can lead to significant improvement in neurological symptoms, especially when started early 2 4.

MS Disease-Modifying Therapies

For patients with Balo lesions who also meet criteria for multiple sclerosis, disease-modifying therapies (DMTs) such as interferons or other immunomodulators may be considered. These medications aim to reduce the risk of future relapses and slow disease progression, although their specific effectiveness in Balo’s disease remains to be fully established 4.

Supportive and Symptomatic Care

Given the potential for severe neurological impairment, supportive therapies are essential. This can include:

• Physical and occupational therapy
• Management of incontinence
• Psychological support for behavioral and emotional symptoms
• Treatment of complications such as infections or aspiration pneumonia 1 3 5

Experimental and Other Therapies

In refractory or fulminant cases, interventions such as plasma exchange (plasmapheresis) or immunosuppressive agents may be tried. However, the evidence for these treatments in Balo’s disease is limited and largely anecdotal 4.

Prognosis

The clinical course of Balo’s disease is highly variable. Some patients recover fully or improve significantly with treatment, while others may experience rapid deterioration and fatal outcomes, especially without prompt intervention 2 3 4 5.

Conclusion

Balo’s disease is a rare, distinctive, and often challenging demyelinating disorder. Improved imaging and a deeper understanding of its mechanisms have enhanced diagnosis and management, but many questions remain. Early recognition and aggressive treatment offer the best chance for recovery.

Key Points:

• Balo’s disease presents with behavioral, motor, and sensory symptoms, often with a subacute or acute onset.
• It is considered a variant of multiple sclerosis but has unique radiological and pathological features.
• The disease involves immune-mediated demyelination, astrocyte dysfunction, and possibly hypoxia-like tissue injury.
• High-dose corticosteroids remain the cornerstone of acute treatment, with MS therapies considered for associated cases.
• Prognosis ranges from full recovery to rapid fatality, underlining the importance of early diagnosis and intervention.

Balo’s disease remains a frontier in neurology—by recognizing its signs and understanding its mechanisms, clinicians can offer the best care for those affected by this rare disorder.