Appearance of Balo's Concentric Sclerosis Eighteen Years after Acute Psychosis

Background:  Balo’s concentric sclerosis (BCS) is a rare variant of multiple sclerosis (MS) and is characterized by findings of alternating bands of demyelination and remyelination on brain magnetic resonance imaging (MRI). BCS is classically viewed as an acute and often life threatening disease. Although MS can present as acute psychosis, BCS has been rarely described in psychotic patients.    

Objectives: To describe a case of relapsing remitting MS with initial presentation as acute psychosis and later development of a BCS lesion on brain MRI. 

Methods: Case report and literature review. 

Results: A 15-year-old right handed boy was institutionalized for acute unexplained psychosis. This consisted of auditory and visual hallucinations in addition to grandiose delusions (e.g. body is made of gold, ability to fly, and claims of prophecy). Patient had no known psychiatric disease, drug or substance use.  At that time, cerebrospinal fluid (CSF) analysis was negative for infection or inflammation, electroencephalogram (EEG) was normal, but MRI showed white matter disease within the internal capsule and the right parieto-occipital area. Tests for Wilson disease, porphyria, and lymphoma were all negative. Patient was then placed on lithium and discharged.  Six months later he presented with a second episode of psychosis for which he was admitted and successfully treated. Ten years later he developed unprovoked seizures. At that time, EEG showed mild slowing over bilateral frontal head region and MRI was highly suggestive of multiple sclerosis. CSF analysis revealed pleocytosis, elevated unmatched oligoclonal bands and immunoglobulin G index. Workup for metachromatic leukodystrophy, sarcoidosis, and syphilis was negative. Thus a diagnosis of MS was confirmed and patient was initiated on interferon beta. Eight years later patient presented with right homonymous hemianopia and brain MRI showed multiple demyelinating lesions with one lesion consistent with BCS. Clinical follow up data will be revealed at CMSC conference. 

Conclusions: BCS lesions can appear later in life in patients diagnosed with MS and can co-exist with typical MS lesions. In agreement with recent reports, BCS can have a relatively benign clinical course and is not always fatal. Our patient unusual clinical presentation of acute psychosis followed by epilepsy syndrome was suggestive of an atypical form of MS many years before confirming the diagnosis.