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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.

We aim to review the imaging appearance of fulminant demyelinating disorders of central nervous system that have different pathological features, clinical course, clinical features, and imaging findings different from classic multiple sclerosis. Routine magnetic resonance imaging (MRI) can help in accurate localization of the lesions, detection of associated lesions, and monitoring of these patients. Advanced MRI combined with routine MRI can aid in differentiation fulminant demyelinating lesions from simulating malignancy. Tumefactive demyelination lesions are located in supratentorial white matter mainly frontal and parietal regions with incomplete rim enhancement. Baló concentric sclerosis shows characteristic concentric onion skin appearance. Schilder disease is subacute or acute demyelinating disorders with one or more lesions commonly involving the centrum semiovale. Marburg disease is the most severe demyelinating disorder with diffuse infiltrative lesions and massive edema involving both the cerebral hemisphere and brain stem.

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.

Idiopathic inflammatory-demyelinating diseases (IIDDs) represent a broad spectrum of central nervous system (CNS) disorders, including monophasic, multiphasic, and progressive disorders that range from highly localized forms to multifocal or diffuse variants. In addition to the classic multiple sclerosis (MS) phenotypes, several MS variants have been described, which can be differentiated on the basis of severity, clinical course, and lesion distribution. Other forms of IIDD are now recognized as distinct entities and not MS variants, such as acute disseminated encephalomyelitis, and neuromyelitis optica spectrum disorders. The CNS can also be affected by a variety of inflammatory diseases. These include primary angiitis of the CNS (PACNS), a rare disorder specifically targeting the CNS vasculature, and various systemic conditions which, among other organs and systems, can also affect the CNS, such as systemic vasculitis and sarcoidosis. The diagnosis of PACNS is difficult, as this condition may be confused with reversible cerebral vasoconstriction syndrome (RCVS), a term comprising a group of conditions characterized by prolonged but reversible vasoconstriction of the cerebral arteries. Magnetic resonance imaging of the brain and spine is the radiologic technique of choice for diagnosing these disorders, and, together with the clinical and laboratory findings, enables a prompt and accurate diagnosis.

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.

Studies in adult patients with multiple sclerosis (MS) suggest significant benefit of early treatment initiation. However, there are no approved therapies for children and adolescents with MS. For adult MS, tolerability and efficacy of several immunomodulatory and immunosuppressive drugs have been demonstrated. Guidelines for the use of these MS therapies in children do not exist. Several small cohort studies of the safety and tolerability of disease-modifying therapies (DMT) in children and adolescents with MS have been recently reported. The side effects of interferon beta (IFNB) and glatiramer acetate (GA) appear to be similar to those reported by adults. The long-term tolerability and safety have yet to be established and efficacy data have yet to be studied. In view of the potential for significant long-term physical and cognitive disability in children with MS, and recent evidence that initiation of immunomodulatory therapy early in the course of MS improves long-term prognosis, an increasing number of children and adolescents with MS are being offered the DMT approved for adults. This review summarizes current knowledge of DMT in pediatric MS and experience in several centers treating pediatric MS and MS variants such as neuromyelitis optica or Devic disease, Balo concentric sclerosis, Marburg acute MS, and Schilder disease (myelinoclastic diffuse sclerosis). Finally, an overview of symptomatic MS therapies and experiences with these treatments in pediatric patients is provided.

Multiple sclerosis (MS) is, by far, the most common inflammatory demyelinating disease of the central nervous system (CNS), but it is not the only CNS inflammatory demyelinating disease and there is a broad spectrum of disorders with varied clinical course, imaging features, epidemiological characteristics, regional distribution of pathological abnormalities, and pathology. Other syndromes associated to MS are clinically isolated syndromes and radiologically isolated syndromes. MS variants included active MS (Marburg type), Schilder’s disease, and Balo lesions. Neuromyelitis optica spectrum, idiopathic acute transverse myelitis, acute disseminated encephalomyelitis, chronic inflammatory myelopathy, and chronic relapsing inflammatory optic neuritis must be distinguished from MS.

The presentation of acute-onset hemiparesis in a teenager can be challenging and offers a wide differential diagnosis. We discuss the approach to the patient (which should begin with thorough history taking and physical examination) and advanced imaging as directed by the patient's signs and symptoms. We report the case of an otherwise well 17-year-old girl who presented to the pediatric emergency department with a 2-day history of left-sided weakness and difficulty ambulating. Her eventual diagnosis of Balo concentric sclerosis, a rare form of multiple sclerosis, is discussed.

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.

The use of magnetic resonance imaging as a surrogate outcome measure in clinical trials, or even as a prognosticator in the assessment of the natural evolution, assumes a close relationship between extent and rate of development of magnetic resonance imaging abnormalities with the clinical status and rate of development of disability. While it may seem obvious that patients who develop new lesions are worse off than those without new lesions, the association between clinical findings and radiological extent of involvement is generally poor. In this review, various confounders are discussed, including inappropriate clinical rating, lack of histopathological specificity (especially for axonal loss), neglect of spinal cord involvement, underestimation of damage to the normal appearing brain tissue (both white and gray matter), and masking effects of cortical adaptation. It is concluded that much progression has been made in magnetic resonance techniques so that the clinico-radiological dissociation has indeed proved to be a paradox. Thus, the relevance of normal appearing brain tissue damage, residual brain volume, spinal cord damage and cerebral plasticity had to be reiterated. The increased awareness of the subtle interplay between these dimensions should be kept in mind when magnetic resonance is used as a surrogate outcome measure. This corroborates with conventional wisdom that one should not rely on a single magnetic resonance measure, but take full advantage of the fact that magnetic resonance is able to provide multidimensional information.

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 …

Vascular pathology and, more specifically, compromised capillaries have long been implicated in the pathogenesis of Balo concentric sclerosis (BCS).1 More recently, hypoxia-like tissue injury and corresponding counter-regulatory mechanisms, such as upregulation of heat shock proteins and HIF1α in oligodendrocytes, have been proposed as underlying the conspicuous concentric lesion formation.2 And although classic BCS is clearly …

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.

Background/purpose Target lesions on diffusion-weighted imaging are uncommon and their significance not well appreciated. To assess the diagnostic value of this neuroimaging finding, a case of cerebral aspergillosis is presented and the literature reviewed. Methods The diffusion-weighted magnetic resonance imaging features of target lesions in a case of cerebral aspergillosis with neuropathologic correlate is presented and 8 other cases manifesting this neuroimaging finding are reviewed for etiology, patient immune status, lesion number, enhancement, and location. Results The etiologies included cerebral aspergillosis, Balo concentric sclerosis, and acute necrotizing encephalopathy. The cerebral aspergillosis cases were immunocompromised with multiple lesions in 4 of 5 patients. The acute necrotizing encephalopathy and Balo concentric sclerosis patients were immunocompetent with bilateral thalamic lesions in the former and multiple random or solitary lesions in the later. Enhancement was seen in 5 patients. Conclusion Target lesions on diffusion-weighted imaging are compelling for a diagnosis of cerebral aspergillosis in immunocompromised patients and for acute necrotizing encephalopathy in immunocompetent patients when lesions are bilateral thalamic and Balo concentric sclerosis when white matter is involved.

Balo concentric sclerosis is a rare demyelinating disease. Pathognomonic features have been previously described. Diffusion-wighted imaging findings have not been previously described in Balo concentric sclerosis. We describe the diffusion-weighted imaging findings in a 45-year-old lady with Balo concentric sclerosis. Diffusion-weighted imaging offers insight into the possible pathophysiology of this rare disease.

Immune-mediated CNS disorders include relapsing forms of multiple sclerosis (RRMS), primary progressive MS (PPMS), Balo concentric sclerosis, Marburg disease, transverse myelitis (TM), longitudinally extensive myelitis (LETM), optic neuritis (ON), neuromyelitis optica (NMO), acute disseminated encephalomyelitis (ADEM), and acute hemorrhagic leukoencephalopathy (AHLE). Others should probably be included in this list: postinfectious movement and behavioral disorders, paraneoplastic disorders, stiff person syndrome, and Rasmussen encephalitis—even collagen-vascular disorders such as systemic lupus erythematosus and Sjogren, sarcoidosis, and vasculitis. Each of these disorders has some distinctive spatial, temporal, imaging, biologic, or pathologic feature that separates it from the others,1 but how should we classify and diagnose these disorders? Are they variations of a single theme that warrant lumping together? Or are they distinct disorders, linked only by the presence of abnormal inflammation in the CNS, more appropriately split into individual entities? Equally important, does it really matter? Once we name a specific disorder in a given patient, does that indicate the most appropriate treatment and predict prognosis for that patient? The answer is probably yes on both counts. It is increasingly clear that the risk of recurrence, …

Objective: Balo concentric sclerosis is a rare demyelinating disorder. Serial proton magnetic resonance spectroscopic ( 1 H-MRS) studies were carried out to better understand the biochemical changes within concentric lesions. Materials and Methods: Five concentric lesions in four patients with Balo concentric sclerosis were chosen as the objects of serial observation. They included two early acute lesions (showing as concentric ring enhancement on magnetic resonance imaging (MRI) after gadolinium administration), two late acute lesions (showing as marginal enhancement on MRI), and one early subacute lesion (showing as edematous concentric lesions without enhancement on MRI). The duration of follow-up ranged from 2-23 months (mean 10 months). A total of 20 1 H-MRS studies were performed. On each 'H-MRS study, short-echo (30 ms) and long-echo (136 ms) spectra were obtained. The peaks of N-acetyl-asparate (NAA), choline-containing compounds (Cho), creatine and phosphocreatine (Cr), lactate, and mobile lipid were observed and compared. Results: Generally, a decrease of NAA/Cr ratio and an increase of Cho/Cr ratio were seen on all the spectra. Observing longitudinally, a trend of decreasing NAA/Cr ratio first and then partially recovering later was noted. The lowest level of NAA/Cr ratio was noted at the late acute stage or early subacute stage. The Cho/Cr ratio and amplitude of the lactate peak showed a similar trend as that of NAA/Cr, but in an opposite direction. It was rising first and descending later. The highest levels of Cho/Cr ratio and lactate peak were also observed at the late acute or early subacute stage. In addition, lactate peaks could be detected as long as 7 months after onset of symptoms. Lipid metabolite (two broad peaks at 0.9-1.5 ppm) was seen at the initial study of each group, but fluctuated in size on follow-up. Conclusion: The characteristic biochemical changes of concentric sclerosis were a decreased NAA/Cr ratio, an increased Cho/Cr ratio, two broad peaks at 0.9-1.5 ppm, lactate production, and a reversible NAA/Cr ratio on follow-up. The serial 'H-MRS studies revealed a strong biochemical association between NAA, Cho, and lactate, which may be caused by the same pathogenetic process of demyelination and inflammatory cellular infiltration. The specificity of the serial changes may provide information about the stage of the concentric lesion and perhaps aid in monitoring progression of concentric lesions and evaluating therapy.

We describe an unsuccessful treatment of Balo concentric sclerosis (BCS), review the current evidence directing therapy, and propose next steps for exploring this rare and commonly fatal condition. Case Report A 52–year–old left–handed engineer reported three weeks of progressive left leg weakness. Examination revealed pyramidal weakness, hyperreflexia and spasticity in the left leg. An MRI head scan showed concentric sclerosis in the right frontoparietal region (Figure 1A). He was diagnosed with Balo concentric sclerosis (BCS). Sequential courses of oral and intravenous steroids, plasma exchange and pulsed cyclophosphamide failed to slow his decline with spread to the left side of his face and arm after six months (Figure 1B). Given its high efficacy on relapse rate and disability accumulation in relapsing–remitting Multiple Sclerosis we administered a standard protocol of alemtuzumab (Campath–1H). At the time of administration his expanded disability status scale (EDSS) score was 6.5. He experienced no immediate or delayed complications. He continued to accrue clinical and radiological evidence of disease progression at the preceding rate including left hemispheric involvement (Figure 1C). 6 months after alemtuzumab administration he was bedbound, totally dependent and fed via nasogastric tube (EDSS score 9.5); 4 months later he died from aspiration pneumonia, 16 months after his first symptoms. Discussion This first use of alemtuzumab in Balo9s concentric sclerosis produced no therapeutic effect. BCS was previously a pathological diagnosis; the advent of MRI technology revealed lesions comprising rings of demyelination with alternating intact myelin bands throughout the neuroaxis (though most commonly in the brain). Pathologically the intact myelin rings are thought to arise through upregulation of ischaemia–tolerance factors induced by immediately adjacent inflammatory demyelination; the lesion eventually progresses to demyelinate tissue beyond this protected band giving rise to the characteristic concentric appearance. BCS may follow a monophasic, relapsing–remitting or relentlessly progressive course; thus far the scarcity of cases has precluded identifying early features that predict subtype. An asymptomatic case found at post–mortem has also been reported. Determining whether clinical improvement reflects the natural history or a response to immunomodulatory therapy is therefore challenging. Some patients with BCS have improved without treatment or following corticosteroids, plasma exchange, cyclophosphamide, mitoxantrone or combined intravenous immunoglobulin with interferon–beta, giving some support to immunomodulatory treatment avenues. Alemtuzumab, a humanised monoclonal antibody, has shown high efficacy in relapsing–remitting Multiple Sclerosis reducing both the risk of relapse and disability accumulation; but, like other immunotherapies, it has no effect on progressive Multiple Sclerosis. No clinical or radiological improvement followed this first use in BCS. It is hard for the clinician, aware of the inflammatory pathology of Balo9s, to withhold immunotherapy, and at least high–dose steroids seem appropriate and in many cases effective. But the rarity of the condition, and its unpredictable natural history, mean there is no robust evidence that immunotherapies alter its long–term course. A practical solution would be a systematic register of cases of Balo9s, collecting information on disease course and treatment.