|Year : 2021 | Volume
| Issue : 5 | Page : 37-38
Balo's concentric sclerosis with bilateral cerebral hemisphere involvement
Manisha Sharma1, Bindu Menon1, Gayatri Manam2
1 Department of Neurology, Apollo Speciality Hospital, Nellore, Andhra Pradesh, India
2 Department of Radiology, Apollo Speciality Hospital, Nellore, Andhra Pradesh, India
|Date of Submission||04-Sep-2020|
|Date of Decision||22-May-2021|
|Date of Acceptance||21-Jun-2021|
|Date of Web Publication||19-Jul-2021|
Department of Neurology, Apollo Speciality Hospital, Pinakini Nagar, Nellore - 524 004, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Balo's concentric sclerosis (BCS) is a variant of multiple sclerosis which differs in terms of clinical presentation, neuroimaging, and neuropathology. It is known for its characteristic “concentric ring” appearance on radioimaging. We present an interesting case of BCS with bilateral cerebral hemisphere involvement.
Keywords: Atypical inflammatory demyelinating syndromes, Balo's concentric sclerosis, multiple sclerosis
|How to cite this article:|
Sharma M, Menon B, Manam G. Balo's concentric sclerosis with bilateral cerebral hemisphere involvement. Apollo Med 2021;18, Suppl S1:37-8
| Introduction|| |
Multiple sclerosis (MS) has evolved as a disease spectrum in the last two centuries. Over a period of time, multiple variants have come into the picture. Neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, tumefactive demyelination, Balo's concentric sclerosis (BCS), Schilder's disease, and Marburg's MS are collectively labeled as “atypical inflammatory demyelinating syndromes” which differ in terms of clinical presentation, neuroimaging, and neuropathology. BCS is a MS variant that is known for its characteristic magnetic resonance imaging (MRI) appearance. Initially, it was considered as rare, acute, aggressive, fatal, monophasic, refractory to treatment and limited by biopsy or autopsy diagnosis. But with recent MRI advances, more cases are being diagnosed. It has become clear that MS variants are no longer so rare, can have acute or insidious onset, benign or fulminant course, independent or co-existent with relapsing MS, monophasic or relapsing, easy to diagnose by neuroimaging, and can be fatal or spontaneously recoverable. Early recognition is crucial as they have different prognosis and management.,
We report a case of a middle-aged male with typical imaging findings of BCS but lesions in bilateral cerebral hemispheres.
| Case Report|| |
A 43-year-old male patient came to the emergency department with acute-onset paresthesias in the left lower limb for 20 days followed by similar complaints in the left upper limb for the last 10 days. There was no history of weakness, slurring of speech, neck pain, headache, and vomiting. Past history of psoriasis, urticaria, and allergic rhinitis was present. On examination, there was no focal motor deficit. Bilateral plantar reflexes were flexor. Deep tendon reflexes were 2+ in all limbs. Hyperesthesia was present on the left half of the body on exteroceptive sensory system examination. On proprioceptive sensory examination, joint position and vibration sense were impaired in the left upper and lower limbs. Romberg's test was negative. The anatomical localization of the lesion was made to either the cervical spine or parietal lobe. MRI of the brain [Figure 1] was showing two heterogeneous intensity lesions of different sizes in bilateral centrum semiovale and frontoparietal deep white matter. Both the lesions had concentric rings as hypointense and isointense on T1-weighted images, corresponding hyperintense and isointense on T2-weighted images, and the T1 hypointense regions showed enhancement on contrast study. Diffusion-weighted imaging (DWI) was showing diffusion restriction without ADC fall. There was no surrounding edema or mass effect. Possibility of demyelinating or infectious etiology was kept. The right side lesion was bigger in size than the left one correlating with the patient's symptoms on the left side. MRI of the spine was a normal study. To confirm the nature of illness, magnetic resonance spectroscopy (MRS) was done which showed decreased N-acetylaspartate peak, elevated lactate peak, and elevated choline and lipid peaks. VEP was suggestive of prolonged P100 peak latencies bilaterally. Lumbar puncture was done. Routine CSF examination was normal. CSF culture sensitivity was sterile. CSF TB PCR and cryptococcal antigen were negative. CSF oligoclonal bands were positive. Aquaporin-4 antibodies and anti-MOG antibodies were negative. Blood viral markers including HIV were negative. Vasculitic panel including ANA, dsDNA, pANCA, cANCA, anti-SSA, and SSB were negative. Based on a typical concentric pattern on MRI, MRS findings, and positive CSF OCBs, the final diagnosis of BCS was kept. The patient was managed with high-dose intravenous steroids for 5 days followed by tapering doses of oral steroids which were switched to mycophenolate mofetil after partial resolution of MRI findings on 3-month follow-up scan.
|Figure 1: Magnetic resonance imaging of the brain T1 axial images (a and b) showing a concentric ring pattern of the lesion (hypointense center with isointense periphery) within the bilateral parietal subcortical region. Axial fluid-attenuated inversion recovery images (c and d) showing corresponding lesions with alternating hypointense and hyperintense concentric rings|
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| Discussion|| |
BCS has a predilection for young males with an average age at onset of 34 years (range: 3–62 years) and Southeast Asian ethnicity. Clinically, it presents as acute or subacute onset of focal neurological deficit as per the location of lesions such as hemiparesis, dysarthria, aphasia, or mass effect such as headache, vomiting, and seizures.
BCS has a distinct radiological picture of concentric rings which can be correlated pathologically. Computed tomography of the brain may or may not show low attenuation lesions. On MRI of the brain, T1 findings correlate with alternating rings of demyelinated (hypointense) and myelinated (isointense) axons. Concentric ring enhancement in demyelinated areas is seen after contrast. T2 findings correlate with alternating rings of demyelinated (hyperintense) and myelinated (isointense) axons. On DWI, there are alternating rings of demyelinated (high signal intensity or restriction diffusion) and myelinated (low signal intensity) axons. Typical location for BCS is cerebral white matter, although brainstem, cerebellum, and spinal cord involvement has also been reported. Bilateral hemisphere involvement in BCS has been seen rarely. BCS with multifocal lesions is not uncommon in children <20 years of age but rarely reported in adults.
The first line of therapy is a course of intravenous steroids at a high dosage. In those cases where no improvement is observed after steroid therapy, plasma exchange and immunosuppressive therapy are considered as the second line of treatment.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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