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Концентричний склероз Бало (КСБ) — рідкісне демієлінізуюче захворювання, що характеризується появою як специфічного патоморфологічного субстрату концентричних вогнищ демієлінізації за типом кілець на спилі дерева або зрізі цибулини з шарами ремієлінізації і демієлінізації, що чергуються. Патогенез КСБ вірогідно відповідає варіанту дистальної олігодендроцитопатії. Також висловлюються припущення про гіпоксичний принцип причинності демієлінізуючих уражень при КСБ і розсіяному склерозі. Справжня частота КСБ невідома, проте вважається, що ця патологія належить до рідкісних захворювань нервової системи з дещо більшою поширеністю в країнах Азії. Більшість випадків захворювання припадає на вік 20–50 років. Симптоматика найчастіше розвивається підгостро, протягом декількох днів або тижнів. Клінічні прояви КСБ зазвичай представлені вираженою вогнищевою неврологічною симптоматикою (пірамідні, мозочкові симптоми, ретробульбарний неврит, когнітивні порушення) у поєднанні із загальномозковою (головний біль, епілептичний синдром), часто з когнітивним дефіцитом. У цереброспінальній рідині при КСБ можуть виявлятися невеликий лімфоцитарний плеоцитоз, нормальний рівень білка, облігатні для розсіяного склерозу олігоклональні IgG відзначаються в 10 % хворих. До типових нейровізуалізаційних ознак цієї патології при проведенні магнітно-резонансної томографії відносять концентричні кільця, що нагадують завитки або спил дерева, на Т2- і Т1-зважених зображеннях із чергуванням структур, змінених і незмінених у відповідному режимі MP-сигналу. У лікуванні КСБ зазначаються дані лише IV класу доказовості. Основними методами вибору терапії цієї патології є: пульс-терапія глюкокортикостероїдними препаратами, високооб’ємний плазмаферез або цитостатики (мітоксантрон). У статті подано клінічний розбір діагностики цієї патології в чоловіка 52 років і результати секційного дослідження.

A previously healthy 40-year-old man developed acute left-sided weakness and numbness that progressed over 2 weeks. Physical examination revealed decreased sensation to pinprick in the left arm and leg and left leg weakness. MRI supported a diagnosis of Balo concentric sclerosis (BCS), a rare demyelinating disease (figure). The concentric bands of differing intensities on MRI are due to alternating layers of normal and demyelinated tissue that is classically observed with BCS.1,2 Patients with BCS commonly present with acute neurologic symptoms, which can be due to a solitary lesion. The clinical course can be fulminant and progressive or benign. In the case presented, the patient's symptoms improved within 1 week of starting high-dose glucocorticoids.

ObjectiveTo describe clinical, radiologic, and pathologic features of Baló concentric sclerosis (BCS) and assess overlap between BCS and other CNS inflammatory demyelinating diseases.MethodsRetrospective review of BCS cases from US and Australian tertiary care centers.ResultsWe identified 40 BCS cases with 38 available MRIs. Solitary MRI lesions were present in 26% (10/38). We saw >1 active concurrent BCS lesion in 45% (17/38). A third (13/38) had multiple sclerosis–suggestive lesions on the index MRI, of which 10 fulfilled Barkhof criteria. In patients with serial MRI performed within 1 month of the index MRI, lesions expanded radially with sequentially increased numbers of T2 hyperintense rings 52% (14/27). Initially nonenhancing or centrally enhancing lesions subsequently developed single or multiple enhancing rings (41%; 9/22) and incomplete enhancing rings (14%; 3/22). Discordance between rings as they appear on apparent diffusion coefficient, diffusion-weighted imaging, and gadolinium-enhanced imaging was observed in 67% (22/33). Aquaporin-4 immunoglobulin G (n = 26) and myelin oligodendrocyte glycoprotein immunoglobulin G (n = 21) were negative in all patients with serum available. Clinical response to steroid treatment was seen in 46% (13/28). A monophasic clinical course was present in 56% (18/32) at last follow-up (median 27.5 months; range 3–100 months). The initial attack was fatal in 10% (4/40). Median time from symptom onset to death was 23 days (range 19–49 days). All 17 patients with pathology available demonstrated typical findings of multiple sclerosis. Patients with active demyelinating lesions all demonstrated oligodendrocytopathy (pattern III).ConclusionsBCS may be a distinct subtype of multiple sclerosis characterized by pattern III immunopathology.

Hintergrund: Die Konzentrische Sklerose Baló (Baló concentric sclerosis, BCS) ist eine seltene, atypisch demyelinisierende Erkrankung, die durch Läsionen der weißen Substanz mit alternierenden Schichten von hoher und geringer Signalintensität in der Magnetresonanztomographie (MRT) gekennzeichnet ist. Bisher wurde angenommen, dass BCS eine Variante der Multiplen Sklerose (MS) ist. Des Weiteren wird vermutet, dass neben der BCS Läsion auftretende MS typische Läsionen einen Hinweis auf weitere Krankheitsaktivität geben. Ziel dieser Arbeit ist es, die Ähnlichkeiten und Unterschiede zwischen BCS und MS durch Beschreibung der Läsionsmorphologie und -lokalisierung in hochauflösenden 7 Tesla (7T) MRT Scans darzustellen. Außerdem wird von den charakteristischen klinischen Verläufen von BCS Patienten berichtet und diese mit denen von MS Patienten verglichen, um die bisherigen Annahmen einer Zuordnung zur MS wissenschaftlich zu prüfen und bildmorphologische Parameter zum prospektiv beobachteten klinischen und paraklinischen Krankheitsverlauf zu untersuchen und zu vergleichen. Methoden: Zehn Patienten mit Läsionen vom Baló-Typ und zehn Patienten mit schubförmig remittierender MS erhielten eine 7T MRT Untersuchung. Zusätzlich wurden neun BCS Patienten mittels 3T MRT untersucht und bei vier von diesen erfolgte ein 3T MRT Follow-up nach einem Jahr. Des Weiteren wurden von allen Patienten klinische Daten erhoben. Ergebnisse: In neun von zehn Baló-Typ Läsionen waren im Zentrum intraläsionale Venen zu finden. Im Gegensatz zu den MS Läsionen konnten in vier Läsionen vom Baló-Typ inhomogene intraläsionale T2*w-Signalintensitäten gefunden werden, welche auf Mikroblutungen oder kleine ektatische Venulen hindeuten. Neben der Baló-Typ Läsion konnten in acht von zehn BCS-Patienten 97 weitere Läsionen gefunden werden, von denen 36 (37%) eine zentrale Vene hatten und damit MS typisch sind. Im Gegensatz dazu wiesen 77% aller MS Läsionen eine zentrale Vene auf. Bei BCS Patienten wurden im Vergleich zu MS Patienten keine Läsionen der kortikalen grauen Substanz und der U-Faser festgestellt. Vier von zehn BCS Patienten erfüllten die MS Kriterien nach McDonald, wobei alle drei BCS Patienten mit schubförmigen Verlauf enthalten waren. Schlussfolgerung: Die Ergebnisse zeigen Gemeinsamkeiten, aber auch starke bildmorphologische und klinische Unterschiede von BCS und MS. Die zentrale Vene, die sowohl in Baló-Typ Läsionen als auch in MS Läsionen zu finden ist, spricht für eine mögliche ähnliche pathophysiologische Initiierung der entzündlichen Läsionen. Die Läsionsausbreitung ist jedoch verschieden. Eine mögliche Hypothese für diese aufgewiesenen Ähnlichkeiten sowie auch differenten Läsionen kann die Hypoxie-induzierte Gewebsverletzung sein, welche die Läsionsmorphologie von MS in BCS Läsionen transformieren kann. Des Weiteren könnten erfüllte MS Kriterien nach McDonald bei BCS Patienten ein möglicher Indikator für weitere Krankheitsaktivität sein., Background: Concentric sclerosis Baló (Baló concentric sclerosis, BCS) is a rare, atypical demyelinating disease characterized by white matter lesions of the cortex with alternating layers of high and low signal intensity in magnetic resonance imaging (MRI). So far, it has been assumed that BCS is a variant of multiple sclerosis (MS). Furthermore, it is suggested that typical MS lesions in addition to the BCS lesion give an indication of further disease activity. The aim of this work is to demonstrate the similarities and differences between BCS and MS by describing the lesion morphology and localization in high resolution 7 Tesla (7T) MRI scans. In addition, clinical courses of BCS are reported and compared with those of MS patients to validate previous assumptions of association with MS and to examine and compare morphological parameters of the prospectively observed clinical and paraclinical disease course. Methods: Ten patients with Baló-type lesions and ten patients with relapsing-remitting MS received a 7T MRI scan. In addition, nine BCS patients were examined by 3T MRI and four of these were followed up at one year. Furthermore, clinical data were collected from all patients. Results: In nine of ten Baló-type lesions intralesional veins were found in the center. In contrast to the MS lesions, inhomogeneous intralesional T2*w signal intensities were found in four lesions of the Baló type, indicating microhemorrhages or small ectatic venules. In addition to the Baló-type lesion, 97 additional lesions were found in eight out of ten BCS patients, of which 36 (37%) had a central vein and are therefore typical of MS. In contrast, 77% of all MS lesions had a central vein. In BCS patients, no lesions of cortical gray matter and U-fiber were detected compared to MS patients. Four of ten BCS patients met McDonald's MS criteria, with all three BCS patients being relapsing-remitting. Conclusion: The results show similarities, but also strong morphological and clinical differences between BCS and MS. The central vein, which can be found both in Baló-type lesions and in MS lesions, suggests a possible similar pathophysiological initiation of the lesions. However, the spread of lesions is different. A possible hypothesis for these demonstrated similarities as well as different lesions may be hypoxia-induced tissue injury, which may transform the lesion morphology of MS into BCS lesions. In addition, fulfilled McDonald MS criteria in BCS patients may be a possible indicator of further disease activity.

Notable in Neurology This Week This issue features an article that compares the rate of remission associated with different surgical procedures for thymectomy in patients with myasthenia gravis; another describes the clinical, radiologic, and pathologic features of patients with Balo concentric sclerosis. A Historical Neurology article looks at the role of microbiologist Clarence Joseph Gibbs on the adoption and use of the “Creutzfeldt-Jakob disease” eponym. ### Accuracy of a Deep Learning System for Classification of Papilledema Severity on Ocular Fundus Photographs ![Graphic][1] Patients with severe papilledema from raised … [1]: /embed/inline-graphic-1.gif

Balo concentric sclerosis (BCS) is a rare, atypical demyelinating disease, which may rapidly progress to become severe and fatal. Advanced neuroimaging has proven helpful for early diagnosis, classification, prognostication, and monitoring of progression in multiple sclerosis, but has not been fully explored in BCS. We present the case of a 27-year-old woman with BCS in whom advanced neuroimaging was used to correlate the evolution of disease with clinical findings over the course of 1 year. Magnetic resonance imaging, magnetic resonance spectroscopy (MRS), diffusion tensor imaging (DTI), and arterial spin labeling cerebral perfusion were obtained at presentation (Day 0), and at Day 67 and Day 252. Imaging features include multilayered concentric ring lesion, reduced diffusion along the rim, hypoperfusion with possible mild central hyperperfusion, and MRS findings of increased choline, decreased N-acetylaspartate (NAA), and possible presence of lactate and/or lipid peak. DTI tractography and relative apparent diffusion coefficient analyses correlated with clinical symptoms and may help to determine extent of white matter tract injury and prognosis.

Acute disseminated encephalomyelitis (ADEM) has been linked to immunizations, viral infections, and occasionally to arthropods stings. Its association with scorpion stings has not been described. A 29-year-old man, who after being stung by a scorpion presented right hemiparesis and language impairment. Brain MRI showed subcortical bihemispheric lesion characteristics of ADEM and a bigger one compatible with Balo concentric sclerosis (BCS). Patient was managed with steroids, showing complete clinical improvement. Magnetic resonance imaging (MRI) after 6 months showed cavitation of the bigger lesion with no new findings. In 2 years of follow-up, there is no clinical relapse or new lesions in MRI. Although cases of ADEM have been reported due to venom of chilopoda and hymenoptera, the scorpion sting can be considered as another new cause; this case being even more relevant due to present a Balo-like lesion in MRI.

A 23-year-old man presented to our clinic with acute left-sided numbness for one week. He had been given a diagnosis of lymphoma as a child and had been in remission for more than 10 years. The neurologic examination showed decreased sensation to pinprick over the left side of the patient’s face

Fulminant demyelinating disease is a heading that covers acute disseminated encephalomyelitis and its variant acute hemorrhagic leukoencephalitis (Hurst disease), severe relapses of multiple sclerosis (MS), variants of MS (tumefactive MS, Marburg variant, Balo concentric sclerosis, myelinoclastic diffuse sclerosis), and neuromyelitis optica-spectrum disorders associated with aquaporin autoimmunity. These categories of inflammatory demyelinating disease often prompt hospital admission and many necessitate intensive care monitoring due to the aggressive nature of the illness and associated neurologic morbidity. In this review, we highlight the discriminating clinical, radiographic, and pathologic features of these disorders. Acute management is often accomplished with use of high-dose intravenous steroids and plasma exchange. Aggressive disease may respond to immunosuppression. Prognosis for recovery varies among the disorders but most patients improve. Factors influencing outcome are also discussed.

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. Recent studies have reported non-fatal forms of Balo concentric sclerosis, focusing on the importance of early diagnosis using magnetic resonance imaging (MRI), along with spectroscopy and diffusion/perfusion sequences. Recently, we have been able to draw a three-dimensional image of a specific bundle of fibers by means of a diffusion tensor technique of the magnetic resonance imaging tractography (t-MRI). We report the case of a young woman presenting with acute and progressive focal neurological symptoms, including right body paresis, whose diagnosis was suggested by MRI and confirmed by pathology to be Balo concentric sclerosis. She was treated with boluses of methylprednisolone, achieving full neurological remission one year after admission. This is, to our knowledge, the first report describing the use of t-MRI for diagnosing BCS. We consider that t-MRI will allow, in a near future, early diagnosis of the disease, its prompt treatment, and establishing new classification criteria. This case confirms the existence of benign forms of Balo concentric sclerosis with a good response to steroid therapy, where functional recovery is possible.La esclerosis concéntrica de Baló es una variante infrecuente de enfermedad desmielinizante relacionada con la esclerosis múltiple, inicialmente considerada de progresión fatal. En estudios recientes se reportan variantes no fatales de esclerosis concéntrica de Baló en los que se enfatiza la importancia del diagnóstico por medio de la imagen por resonancia magnética, utilizando además la espectroscopia y las secuencias de difusión y perfusión. En los últimos años se ha logrado reproducir la imagen tridimensional de un fascículo en particular y observar la presencia de lesiones por medio de la tractografía por imagen por resonancia magnética mediante la técnica de tensor de difusión. Presentamos el caso de una mujer joven con síntomas neurológicos focales agudos, incluyendo paresia de extremidades derechas, cuyo diagnóstico por biopsia fue de esclerosis concéntrica de Baló, confirmando el resultado de los estudios de imagen. La paciente recibió tratamiento con bolos de metilprednisolona, obteniendo remisión clínica completa a largo plazo. A nuestro entender, este es el primer reporte que describe los hallazgos de la esclerosis concéntrica de Baló utilizando la técnica de tensor de difusión. Consideramos que dicha técnica permitirá en el futuro la detección temprana de la enfermedad, su tratamiento oportuno y permitirá establecer nuevos criterios de clasificación y estratificación. Este caso demuestra la existencia de variantes benignas de esclerosis concéntrica de Baló, que tienen buena respuesta a la terapia con glucocorticoides y donde se logra la recuperación funcional.

The authors present the results of an 8-year retrospective-prospective follow-up of a patient with Balo concentric sclerosis. The disease meets the diagnostic criteria of remitting-relapsing multiple sclerosis.Представлены результаты восьмилетнего ретроспективно-проспективного наблюдения клинического случая концентрического склероза Бало. Установлено соответствие заболевания диагностическим критериям ремиттирующе-рецидивирующего рассеянного склероза.

The present report is a rare case of Balo Concentric Sclerosis. Most cases have either been diagnosed post mortem or have succumbed to the disease after being diagnosed ante mortem. In our case, the patient showed a dramatic response to treatment, and after a one-year follow-up, he was asymptomatic, with no relapses or residual effect of the illness.

In the Clinical/Scientific Note “Balo concentric sclerosis evolving from apparent tumefactive demyelination” by T.A. Hardy et …

A 45-year-old man presented with progressive Broca aphasia and right hemiparesis. MRI demonstrated a large white matter lesion with multiple rings (figure). These centrifugal rings reflect concentric layers of active demyelination, typical for Balo concentric sclerosis.1 CSF showed oligoclonal bands and a mild pleocytosis. Partial recovery followed 5 days of treatment with IV methylprednisolone (1 g daily). Balo concentric sclerosis, a rare variant of multiple sclerosis, was long thought to have a progressive and fatal course. The present case confirms the possibility of a more benign course in some cases.2

A 26-year-old man of Portuguese descent with no significant past medical history presents with subacute onset of right-sided hemiparesis and aphasia, with marked expressive aphasia, word-finding difficulty, and rare paraphrasic errors. Examination demonstrated right central facial weakness, 2–3/5 strength in the right arm and leg, and dysmetria in the right upper and lower extremities. Reflexes were hyperactive on the right with a right-sided Babinski response. MRI of the brain showed multiple bilateral concentric ring-like structures in the centrum semiovale and the corona radiata on T2 imaging (figure,A), consistent with the pattern of Balo concentric sclerosis. There was associated restricted diffusion in 3 lesions and incomplete ring enhancement in 1 lesion. CSF analysis showed 30 leukocytes (79% lymphocytes, 4% monocytes), erythrocyte count was 850, and oligoclonal bands were absent. He was treated with 1 g of IV methylprednisolone daily for 5 days, with little improvement. He then underwent 5 plasmapheresis exchanges, with significant improvement of motor, sensory, speech deficits, and gait. Forty-five days after discharge, his examination showed 4+/5 strength in the right intrinsic muscles of the hand and a right-sided positional tremor. Gait and tandem gait were normal. He was placed …

Introduction In rare instances, demyelinating disorders manifest as tumefactive lesions that simulate brain tumors. We report a patient with a space-occupying 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. This case report highlights important diagnostic clues in the differential diagnosis of Balo concentric sclerosis. Case presentation A 20-year-old Caucasian woman with acute onset of left-sided weakness and numbness was admitted to hospital with neurologic signs of left-sided hemiparesis and hypoesthesia. Brain magnetic resonance imaging showed a mass lesion of abnormal signal intensity with concentric enhancing rings in the right parietal lobe, without perifocal edema. The characteristic concentric pattern detected on the magnetic resonance images was highly suggestive of Balo disease, and corticosteroids were administered. Evoked potentials, cerebrospinal fluid analysis, and magnetic spectroscopy findings were not specific, and glioma was also included in the differential diagnosis. A stereotactic biopsy was not diagnostic. After one month the patient showed moderate clinical improvement, and during 12 months follow-up, no further relapses occurred. In the follow-up magnetic resonance imaging, the concentric pattern had completely disappeared, and only a low-signal, gliotic lesion remained. Conclusion We hope this case presentation will advance our understanding of clinical and radiologic appearance of Balo concentric sclerosis, which is a rare demyelinating disease. Although this is a specific entity, it has a broader clinical impact across medicine, because it must be differentiated from other space-occupying lesions in the central nervous system.