Serial proton mr spectroscopy and diffusion tensor imaging in infantile balos concentric sclerosis by steffi f. Media in category balo concentric sclerosis this category contains only the following file. Neuromyelitis optica nmo, also known as devics disease or devics syndrome, is an autoimmune, inflammatory disorder in which a persons own immune system attacks the optic nerves and spinal cord. Phase iii randomized, doubleblind, shamcontrolled study. As a result, treatment decisions are largely based on a doctors experience and information from single case reports. To date there have been no controlled treatment studies for tumefactive multiple sclerosis. Although inflammation may also affect the brain, the lesions are different from those observed in the related.
Ring sign on diffusionweighted imaging radiology jama. Balos concentric sclerosis is a rare demyelinating disorder characterised pathologically by concentric rings of alternating demyelinated and relatively myelin preserved white matter. Balos concentric sclerosisa case report, psychiatry and. This is a report of balos concentric sclerosis in a 23. Any information contained in this pdf file is automatically generated from digital material. We describe the case of a 25yearold female presenting with strokelike symptoms who was diagnosed with bcs on. Pdf balos concentric sclerosis with acute presentation and co. Five bcs cases were diagnosed antemortem based on their typical concentric mass patterns on mr images and based on clinical and. As supported in previously reported clinical trials, bcs is not always a fatal disease and supports the definition that it may be a selflimiting disease. Balos concentric sclerosislike lesion in the brainstem of. Bcs is often regarded as a rare variant of multiple sclerosis ms. This paper describes a case of balo s concentric sclerosis, a rare demyelinating disease of the central nervous system cns, which is currently classified as multiple sclerosis. Multiple sclerosis wikipedia, the free encyclopedia. Effect of aqueous extract of phyllanthus fraternus leaf against cyclophosphamide induced dyslipidemia and aortitis in wistar albino rats rakesh singh moirangthem, singh shailendra vikram jitendra, heikham chandani devi, kh.
Get a printable copy pdf file of the complete article 1. Balos concentric sclerosis is a disease in which the white matter of the brain appears damaged in concentric layers, leaving the axis cylinder intact. Balo concentric sclerosis multiple concentric layers onion skin lesions, as a mosaic, or as a floral configuration t2 hiperintense bands dm and thinner isointense bands preserved myelin the center of the lesion usually shows no layering because of massive demyelination. Balos concentric sclerosis was diagnosed antemortem in 2 patients, by magnetic resonance mr imaging showing striking concentric alternating rings in 1 patient and by characteristic. Although 85% of mscases develop a clinically isolated syndrome involving the optic nerves, brainstem, or spinal cord, ms lesions are also able to mimic a number of cerebral malignancies, particularly in the presence of gradual mass growth tumefactive ms. Phase iii randomized, doubleblind, shamcontrolled study of. Multiple sclerosis ms is a demyelinating disease with complex clinical and radiological features. Pitfalls in the diagnosis of a tumefactive demyelinating. Balo s concentric sclerosis was diagnosed antemortem in 2 patients, by magnetic resonance mr imaging showing striking concentric alternating rings in 1 patient and by characteristic. A challenging case of concurrent multiple sclerosis and. Balo sclerosis comes under the ms lesion type iii pattern distal oligodendrogliopathy. Industrysponsored, randomized, multicenter, raterblinded, controlled trial to compare the effects of two cycles of intravenous alemtuzumab either 24 mg a dose that was discontinued or 12 mg per day, once a day for 5 consecutive days at baseline and once a day for 3 days at 12 months with interferon beta1a 44 mcg given subcutaneously three times a week on relapse rate and time to 6.
Structural and metabolic features of two different. Together, over the past 15 years we have made netforum into a vibrant global community where more than 30,000 of you have been able to learn, share and grow. How do i view different file formats pdf, doc, ppt, mpeg on this site. Treatment of balos concentric sclerosis with immunosuppressive drugs followed by multimodality evoked potentials and mri. Balo concentric sclerosis bcs is a rare type of inflammatorydemyelinating disease first described by joszef balo in the early 19th century. The changes are felt to represent cytotoxic edema in association with acute. Balos concentric sclerosis bcs is considered a rare demyelinating disease and regarded as an aggressive variant of multiple sclerosis ms. Characteristic appearance can be seen as bulb eye or onion bulb. Full text get a printable copy pdf file of the complete article 1. Assessment of lesions on magnetic resonance imaging in. Tumefactive multiple sclerosis genetic and rare diseases. 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. Five autopsy cases of balos concentric sclerosis in. Histological examination of the brain shows sharply defined concentric rings of demyelination alternating with bands of unaffected myelin, giving an onion skin appearance fig.
The generally accepted pathologic criteria of a demyelinating disease are 1 destruction of the myelin sheaths of nerve fibers with relative sparing of the other elements of nervous tissue, i. To release radiological findings of tumefactive multiple sclerosis lesions. Pdf balos disease also known as balos concentric sclerosis bcs is a rare. To diference these lesions from other spaceoccupying lesions in the central nervous system. Balos concentric sclerosis an overview sciencedirect. Multiple sclerosis and other inflammatory demyelinating. However, this characterization implies that the primary event is an aberrant immune response directed at cns antigens, promoting inflammation and later. The first group of patients receives a true plasma exchange using continuousflow centrifugation with serum albumin and crystalloid replacement every 2. Case or problembased learning is a mainstay of acquiring knowledge in medical education.
Mean age of the patients was 31 years old and the total duration of illness was 4 months. Balo concentric sclerosis is a demyelinating disease similar to standard multiple sclerosis, but with the particularity that the demyelinated tissues form concentric layers. Mri has shown that the natural history of the disease varies but with acute treatment recovery from symptomatic lesions is possible. After his death, histologic examination of the affected tissue revealed bands of. Cases with nonstandard behavior have also been described.
Janina r behrens charite universitatsmedizin berlin corporate member of freie universitat berlin, humboldtuniversitat zu berlin, and berlin institute of health neurocure cluster of excellence neurocure clinical research center berlin germany. Histopathologic investigation was also performed in one case. 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. Serial proton mr spectroscopy and diffusion tensor imaging in infantile balos concentric sclerosis by download pdf 40 kb. Restricted diffusion in the outer rings was demonstrated in axial diffusionweighted imaging figure 1d.
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. Balo s concentric sclerosis is a rare demyelinating disorder characterised pathologically by concentric rings of alternating demyelinated and relatively myelin preserved 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. Balos concentric sclerosis is a rare subtype of demyelinating disease with characteristic radiological and pathological features.
Balos concentric sclerosis an overview sciencedirect topics. Balos concentric sclerosis bcs is a rare demyelinating disease that. In a small number of cases, people with tumefactive multiple sclerosis have minimal to no symptoms. In the past 3 years, 5 typical cases 2males and 3 females of balos concentric sclerosis have been encountered in manila philippines. The first group of patients receives a true plasma exchange using continuousflow centrifugation with serum albumin and crystalloid replacement every 2 days for a total of 7 exchanges. In recent years, there has been a more favorable clinical course of balo s sclerosis. Acute multiple sclerosis marburg disease and tumefactive multiple sclerosis diffuse cerebral sclerosis schilder disease and concentric sclerosis of balo neuromyelitis optica devic disease, nmo and progressive necrotic myelopathy. Mri of the brain showed a wellcircumscribed 2cm sized concentric. Balos disease also known as balos concentric sclerosis bcs is a rare demyelinating disorder which is characterized pathologically and radiologically by concentric rings of demyelinated and relatively myelinpreserved white matter. Csf analysis was not done due to recent neurosurgery, but the diagnosis of multiple sclerosis of balos concentric sclerosis, was strongly suggested based on.
It seems that this is the first report of 1hmrs findings in balos concentric sclerosis. Csf analysis was not done due to recent neurosurgery, but the diagnosis of multiple sclerosis of balos concentric sclerosis, was strongly suggested based on the clinical history, pathology report, and imaging findings. Balo s 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. Sometimes referred to as borderline forms of multiple sclerosis, 38 these include devics disease, balo concentric sclerosis, schilders diffuse sclerosis and marburg multiple sclerosis. Serial proton mr spectroscopy and diffusion tensor imaging in infantile balos concentric sclerosis. Our case report summarizes the pathology, clinical. We describe the case of a 25yearold female presenting with strokelike symptoms who was diagnosed with bcs on mri. Balos concentric sclerosis with acute presentation and co. Highdose steroid treatment in a patient with balo disease diagnosed by means of magnetic resonance imaging. Balo concentric sclerosis is one of the borderline forms of multiple sclerosis. Balos concentric sclerosis with acute presentation and coexisting. Balos concentric sclerosis is a histological variant of ms which used to be regarded as a distinct form of the disease. Balos concentric sclerosislike lesion in the brainstem of a multiple sclerosis patient article in journal of neurology 2555. This was previously only diagnosed at postmortem examination and was thought to be.
Highdose steroid treatment in a patient with balo disease. Five bcs cases were diagnosed antemortem based on their typical concentric mass patterns on mr images and based on clinical and csf findings. Balos concentric sclerosis international journal of contemporary. In the past 3 years, 5 typical cases 2males and 3 females of balo s concentric sclerosis have been encountered in manila philippines. Clinical and radiologic features of five cases, american journal of neuroradiology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. In rare instances, demyelinating disorders manifest as tumefactive lesions that simulate brain tumors.
Balo disease nord national organization for rare disorders. Csf analysis was not done due to recent neurosurgery, but the diagnosis of multiple sclerosis ms, and specifically of balos concentric sclerosis. This produces an inflammation of the optic nerve optic neuritis and the spinal cord. This young male patient suffered progressive neurologic deterioration that was thought to be caused by a brain tumor. Multiple sclerosis ms is traditionally considered an autoimmune inflammatory demyelinating disease of the central nervous system cns with much knowledge available to support this view. Balos concentric sclerosis presenting with benign clinical course. In recent years, there has been a more favorable clinical course of balos sclerosis. We report a patient with a spaceoccupying lesion in the parietal lobe, which presented a serious diagnostic dilemma, between a rare tumefactive demyelinating disease, such as balo concentric sclerosis and a glioma. Dear netforum user, at philips, we believe in putting trust, transparency and control at the heart of our customer experience. The typical pattern is consistent with a diagnosis of balos concentric sclerosis bcs, which was first described by marburg in 1906, and was advanced by joszef balo as a rare variant of multiple sclerosis in 1928 1.
Balo instrument, an african musical instrument balo concentric sclerosis, a borderline form of multiple sclerosis malka balo, a woreda administrative division in the oromia region of ethiopia. Aug 15, 2015 restricted diffusion in the outer rings was demonstrated in axial diffusionweighted imaging figure 1d. Multimodality imaging such as proton magnetic resonance spectroscopy mrs and positron emission tomography pet have provided information specific to the underlying mechanisms of many brain diseases, including multiple sclerosis ms. The foci of demyelination increase in waterfurther subtle hypodensity within the hypodense bg of the white matter low diagnostic yield of ct in multiple sclerosis. Pdf balo concentric sclerosis bcs is a rare disease considered as a variant of multiple sclerosis ms with a clinical presentation similar to.
Balos concentric sclerosis is immunologically distinct. Each presented behavioral changes,marked motor symptoms and double incontinence. This case report demonstrates that balos concentric sclerosis bcs a patient with a mass lesion containing concentric rings, bcs diagnosis was reported by magnetic resonance imaging. We describe three cases one male and two females with neuroimaging features suggestive of bcs and heterogeneous symptoms, with benign longterm clinical course upon treatment with natalizumab and. Recently, dwi features with balo concentric sclerosis were described in 3 separate case reports, 2 of which manifested a ring pattern. Balos concentric sclerosis bcs is a rare demyelinating disease considered to be a variant of multiple sclerosis. 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. To understand clinical and radiological appearance of bale concetric sclerosis.
Balos concentric sclerosis bcs is a rare demyelinating disease considered to be. Balo first described the histological features, alternating bands of loss and partially preserved myelin, of this clinical enitity, balos concentric scleriosis bcs. 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. Balos concentric sclerosis todd a hardy, david h miller balo s concentric sclerosis is often regarded as a rare variant of multiple sclerosis. Balo s concentric sclerosis bcs is a rare monophasic demyelinating disease known as multiple sclerosis subtype and seen as a round lesion with variable hyper and hypodetoxification layers. The antemortem diagnosis of balo s concentric sclerosis was made in a 52 year old woman with subacute right hemiparesis on the basis of brain mri and stereotactic brain biopsy, which showed multiple ringlike lesions of lamellated demyelination alternating with spared white matter. To determine the structural and metabolic characterization of two particular variants of ms, namely tumefactive ms and balos.
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