Nov, 2001 we report a 43yearold man with balos concentric sclerosis. Balo concentric sclerosis is an infrequent variant of a demyelinating disease related to multiple sclerosis, initially thought to have an acute presentation and a fatal outcome. Clinically, he had recurring episodes of memory deficits and rightsided hemiparesis. Tumefactive multiple sclerosis is characterized by a tumorlike lesion larger than two centimeters and signs and symptoms similar to those of a brain tumor. Balo concentric sclerosis is a unique and rare phenomenon in demyelinating disease. Advances in tumefactive cns demyelinating disease and balos. Balo concentric sclerosis is a rare and severe monophasic demyelinating disease, considered a subtype of multiple sclerosis, appearing as a rounded lesion with alternating layers of high and low signal intensity on mri, giving it a characteristic. Balo s concentric sclerosis is a rare variant of ms with characteristic lesions. Bcs is often regarded as a rare variant of multiple sclerosis ms. Balos concectric sclerosis bcs is a rare demyelinating disease, usually considered as a multiple sclerosis ms variant with an acute clinical course and fatal progression, although recently it has been reported spontaneous remissions and relapsing. Balos concentric sclerosis is immunologically distinct from multiple. Balos concentric sclerosis presenting as a strokelike. Balos concentric sclerosis in a patient with spontaneous remission.
The clinical and radiological features can mimic other diseases such as primary central nervous system lymphoma, lowgrade glioma or stroke. Balo s disease is a rare form of multiple sclerosis that can cause paralysis and problems communicating with others. Balo s disease is also known as balo disease, encephalitis periaxialis concentrica, leukoencephalitis periaxialis concentrica and concentric sclerosis. Balo concentric sclerosis bcs is an atypical idiopathic inflammatory demyelinating disorder initially described by josef balo in 1928. Balos concentric sclerosis bcs is a demyelinating disorder believed to be a rare variant of multiple sclerosis ms. Balos concentric sclerosis is a rare demyelinating disease that was first described by joszef balo in 1928.
Balos concentric sclerosis presenting with benign clinical course. Is there a cure for balos disease concentric sclerosis. However, the advent of magnetic resonance imaging mri has changed this, and longterm survival has been reported with bcs 1,2,3. These centrifugal rings reflect concentric layers of active demyelination, typical for balo concentric sclerosis. Pdf balo concentric sclerosis bcs is a rare disease considered as a variant of multiple sclerosis ms with a clinical presentation similar to. Primary infection with hhv6 usually occurs in early. However, mri showed a striking feature, reminiscent of histopathology of balos concentric sclerosis, and played an important role in antemortem diagnosis of this rare demyelinating. Balos concentric sclerosis is immunologically distinct from. Balos concentric sclerosis presenting with benign clinical. Balos concentric sclerosis is a histological variant of ms which used to be regarded as a distinct form of the disease. Balos concentric sclerosis is often regarded as a rare variant of multiple sclerosis. In the past, the diagnosis was usually made post mortem.
Balos concentric sclerosis is a rare disease, which makes precise estimates of prevalence di. Pdf balos concentric sclerosis with acute presentation and co. Balos concentric sclerosis bcs is a rare demyelinating disease considered to be a variant of multiple sclerosis. Concentric sclerosis is a rare variant of multiple sclerosis, first described by balo in 1928 balo, 1928. It may present as clinically similar to multiple sclerosis, affecting young adults with mild cognitive impairment without frank dementia, or be associated with altered behavior and focal cns deficits. Pdf balos disease also known as balos concentric sclerosis bcs is a rare demyelinating disorder which is characterized pathologically and. Pediatric balo concentric sclerosis response to dimethyl fumarate sophia french, md, daniel crowder, md oregon health and science university, pediatric neurology, portland, oregon article info article history. Balos concentric sclerosis with acute presentation and co. Balos concentric sclerosis presenting as a strokelike syndrome. Human herpesvirus 6 hhv6 is a highly neurotropic virus causing severe central nervous system cns infections predominantly following reactivation of latent hhv6 in immunocompromised individuals. Balos concentric sclerosis is a disease in which the white matter of the brain appears damaged in concentric layers, leaving the axis cylinder intact. Balos concentric sclerosis request pdf researchgate. Case report a 52yearold lefthanded engineer reported three weeks of progressive left leg weakness.
Patients with this disorder present with acute or subacute neurological deterioration, with mri showing one or more concentrically multilayered ringlike lesions usually in the cerebral white matter. There are stripes of high or low signal depending on the sequence, most likely due to areas of demyelination and myelination onion bulb appearance. Pediatric balo concentric sclerosis response to dimethyl fumarate. Balo concentric sclerosis an overview sciencedirect topics.
Balos concentric sclerosis bcs is a rare inflammatory demyelinating disorder of the central nervous system characterised by concentric. Balos concentric sclerosis bcs differs from multiple sclerosis ms in several aspects. Mri, mrs, dti, and asl perfusion imaging over 1 year. We describe the case of a 25yearold female presenting with strokelike symptoms who was diagnosed with bcs on.
Pdf balo concentric sclerosis in children a case series. Balos concentric sclerosis presenting with benign clinical course and multiple sclerosislike lesions on magnetic resonance images. Basic and advanced imaging of a case of balo concentric sclerosis. A 45yearold man presented with progressive broca aphasia and right hemiparesis. Mri scans of the brain revealed a large lesion with a concentric ring pattern. Balos concentric sclerosis bcs is a rare demyelinating disorder of the central nervous system cns characterised by concentric layers of demyelination as detected by mri or histopathology, and named after the hungarian pathologist jozsef balo 18951979 1, 2. Basic and advanced imaging of a case of balos concentric sclerosis paolo ripellino,1,2 alessandro stecco,3 maurizio a leone,2 roberto cantello2 1department of neurology, university of turin, torino, italy 2department of neurology, university of eastern piedmont, aou maggiore della carita, novara, italy 3department of radiology. Inicio neurologia english edition balo concentric sclerosis. Balos concentric sclerosis bcs is a rare demyelinating disease that was first described by the hungarian neuropathologist josef balo in 1928. Advanced neuroimaging in balos concentric sclerosis. Five bcs cases were diagnosed antemortem based on their typical concentric mass patterns on mr images and based on clinical and. A 46yearold man noticed numbness and tingling in his right upper extremity and left hand, followed by left eye visual impairment. Learn about the diseases causes, symptoms, diagnosis, and treatment. Balo s concentric sclerosis is a rare and a relatively acute condition.
The american autoimmune related diseases association web site reports that symptoms include headache, gradual paralysis, seizure, involuntary muscle spasm, and loss of cognitive function. Balo s concentric sclerosis is a ms variant characterized pathologically by concentric rings of alternating demyelinated and undemyelinated tissue, which are visible macroscopically. Balos concentric sclerosis mimicking cerebral tuberculoma. This case study presents neuroimaging findings, differential diagnosis. Most cases of bcs show acute encephalopathy with a fulminant course that can rapidly become fatal.
Balos concentric sclerosis is a demyelinating disorder in which bands of demyelination alternate with concentric bands of myelin preservation. Csf analysis was not done due to recent neurosurgery, but the diagnosis of multiple sclerosis ms, and specifically of balos concentric sclerosis. Mri after application of gadolinium showed alternating bands. Pdf balos concentric sclerosis in a young female patient. Examination revealed pyramidal weakness, hyperreflexia and spasticity in the left leg. Balos concentric sclerosis balo, 1928 is a rare and severe variant of ms. May 31, 2018 to report a case of balo s concentric sclerosis which resolved completely after treatment with steroid and plasmapharesis. Balo s disease also known as balo s concentric sclerosis bcs is a rare demyelinating disorder which is characterized pathologically and radiologically by concentric rings of demyelinated and relatively myelinpreserved white matter. Histological examination of the brain shows sharply defined concentric rings of demyelination alternating with bands of unaffected myelin, giving an onion skin appearance fig. Patients with this disorder present with acute or subacute neurological deterioration, with mri showing one.
Pathognomonic mr imaging findings in balo concentric sclerosis article pdf available in american journal of neuroradiology 222. Feb 28, 2015 balo s disease also known as balo s concentric sclerosis bcs is a rare demyelinating disorder which is characterized pathologically and radiologically by concentric rings of demyelinated and relatively myelinpreserved white matter. It was described by jozsef matyas balo who initially named it leukoencephalitis periaxialis concentrica from the previous definition, and it is currently considered one of the borderline forms of multiple sclerosis. Balo concentric sclerosis bcs is a rare type of inflammatorydemyelinating disease first described by joszef balo in the early 19th century.
Balos concentric sclerosis with acute presentation and coexisting. Typically thought of as a subtype of multiple sclerosis, balo concentric sclerosis is characterized pathologically by striking rings of demyelination alternating with areas of preserved myelination. Mri demonstrated a large white matter lesion with multiple rings figure. A brain biopsy should be obtained whenever possible. Balos concentric sclerosis associated with primary human. Jun 17, 2015 balo s concentric sclerosis bcs is a rare and recognized variant of multiple sclerosis ms. Balo concentric sclerosis radiology reference article. Partial recovery followed 5 days of treatment with iv. Balo s disease, or concentric sclerosis, is an unusual variant that tends to progress rapidly.
Jan 18, 2018 balos concentric sclerosis bcs is a rare demyelinating disorder of the central nervous system cns characterised by concentric layers of demyelination as detected by mri or histopathology, and named after the hungarian pathologist jozsef balo 18951979 1, 2. Balo concentric sclerosis bcs is widely believed to be a rare variant of multiple sclerosis. Received 22 january 2019 accepted 30 march 2019 available online 5 april 2019 balo concentric sclerosis bcs is an atypical idiopathic. The initial terminology for this entity was leukoencephalitis periaxialis concentrica, which is based on its early definition of a disease in the course of which the white matter of the brain is destroyed in concentric layers in a manner that leaves the axis cylinders intact. Balos concentric sclerosis in a patient with spontaneous remission based on magnetic resonance imaging. Balos concentric sclerosis demonstrated by contrast mri. The average age of onset of balos concentric sclerosis is 34 years range 362 years. Establishing preconditions for balos concentric sclerosis. This case study presents neuroimaging findings, differential diagnosis, and management of bcs in a 37yearold woman. Tumefactive multiple sclerosis genetic and rare diseases. Balos concentric sclerosis an overview sciencedirect topics. Balos concentric sclerosis is a rare demyelinating disease presenting with a concentric ring in the white matter.
Pediatric balo concentric sclerosis response to dimethyl. Marburg type and balos concentric sclerosis are considered particular variants of multiple sclerosis, clinically characterized by a severe, rapidly evolving clinical course, and by unusual neuropathological changes, often large tumorlike demyelinating plaques in marburg type and concentric layers of partial demyelination alternating with demyelinating bands in balos concentric. It is characterized by the development of alternating bands of demyelinated and myelinated white matter, forming concentric rings or irregular stripes. In this case, we found that ct demonstrated only lowdensity lesions, but not their concentric pattern. Balos concentric sclerosis bcs is a rare demyelinating disease considered to be. Historically, balos concentric sclerosis was thought fatal in all cases. The term concentric sclerosis comes from a pattern of concentric circular areas of damaged myelin alternating with areas of relatively undamaged myelin in various parts of the brain and. Balo first described the histological features, alternating bands of loss and partially preserved myelin, of this clinical enitity, balos concentric scleriosis bcs.
Clinical senior lecturer, university of sydney may 28th, 2015 disclosures honoraria for talks from novartis, genzyme, merck honoraria for chairing meetings from genzyme conference travel from novartis, biogen and. Balo s concentric sclerosis is a very rare variant of multiple sclerosis with rapid procession, alternatively it may be a different entity altogether. Basic and advanced imaging of a case of balos concentric. Clinically it can present similarly to ms or may be associated with cognitive or behavioral dysfunction, focal. In comparison to classic ms symptoms, bcs patients tend to present with symptoms that most closely resemble those of intracranial mass lesions 1.
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