Treatment of balo's concentric sclerosis with immunosuppressive drugs followed by multimodality evoked potentials and MRI

Louboutin, Jean‐Pierre; Elie, B.

doi:10.1002/mus.880181221

Cited by 13 publications

(5 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Courses of oral (500 mg for 5 days at diagnosis) and intravenous (1 g for 3 days 1 month later) methylprednisolone 1 and a 5-day course of plasma exchange 1 month later 2 produced no improvement. Over the next 10 months the weakness progressed to his left arm and face and he developed language difficulties, at first aphasia for low-frequency words, in keeping with radiological progression throughout the right hemisphere (Figures 1(b) and 1(c)); six pulses of cyclophosphamide 3 failed to slow his decline. Given alemtuzumab's proven high efficacy in relapsing-remitting multiple sclerosis 4 we administered a standard protocol of alemtuzumab (Campath-1H, five intravenous infusions of 12 mg over 5 days, with methylprednisolone to ameliorate the associated cytokine release syndrome).…”

Section: Case Reportmentioning

confidence: 75%

Section: Case Reportmentioning

confidence: 75%

“…An asymptomatic case found at post-mortem has also been reported. 6 Determining whether clinical improvement reflects the natural history or a response to immunomodulatory therapy is challenging: some patients with progressive BCS have improved spontaneously while improvement in others follows corticosteroids, 1 plasma exchange, 2 cyclophosphamide, 3 mitoxantrone 7 and combined intravenous immunoglobulin and interferon-beta, 8 lending support to immunomodulatory treatment avenues.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

First use of alemtuzumab in Balo’s concentric sclerosis: a case report

2013

View full text Add to dashboard Cite

Balo's concentric sclerosis (BCS) is a rare demyelinating disorder of the central nervous system. The humanised monoclonal antibody alemtuzumab has shown efficacy in another demyelinating disorder, relapsing-remitting multiple sclerosis. We aimed to explore its efficacy in treatment-refractory BCS. A 52-year-old male with radiologically confirmed progressive BCS resistant to steroids, plasmapharesis and cyclophosphamide was administered a standard protocol of alemtuzumab. Treatment failed to slow his decline; he died 6 months after administration. Why alemtuzumab induced no clinical or radiological impact may be multifactorial. We review the evidence directing BCS therapy and propose the next steps for exploring this potentially fatal condition.

show abstract

Section: Case Reportmentioning

confidence: 75%

Section: Case Reportmentioning

confidence: 75%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

First use of alemtuzumab in Balo’s concentric sclerosis: a case report

2013

View full text Add to dashboard Cite

show abstract

“…The next step is less clear since there have been only anecdotal reports of cases refractory to steroid treatment and plasmapheresis in which long-term treatments including interferons, cyclophosphamide, [33][34][35][36] and mitoxantrone 37 were administered, together with support treatment for complications typical of this severe and extensive involvement, such as intracranial hypertension.…”

Section: Figurementioning

confidence: 99%

Lesser-known myelin-related disorders: Focal tumour-like demyelinating lesions

Arango

Uribe

González

2015

Neurología (English Edition)

View full text Add to dashboard Cite

Introduction: Focal tumour-like demyelinating lesions are defined as solitary demyelinating lesions with a diameter greater than 2 cm. In imaging studies, these lesions may mimic a neoplasm or brain abscess; as a result, invasive diagnostic and therapeutic measures may be performed that will in some cases increase morbidity. Our aim was to analyse and characterise these lesions according to their clinical, radiological, and pathological characteristics, and these data in addition to our literature review will contribute to a better understanding of these lesions.Methods: This descriptive study includes five cases with pathological diagnoses. We provide subject characteristics gathered through reviewing their clinical, radiology, and pathology reports.Results: Patients' ages ranged from 12 to 60 years; three patients were female. The time delay between symptom onset and hospital admission was 3-120 days. Clinical manifestations were diverse and dependent on the location of the lesion, pyramidal signs were found in 80% of patients, there were no clinical or radiological signs of spinal cord involvement, and follow-up times ranged from 1 to 15 years.Conclusion: Brain biopsy is the gold standard for the diagnosis of demyelinating tumour-like lesions; however, their clinical features, along with several magnetic resonance imaging features such as open ring enhancement, venular enhancement, the presence of glutamate in spectroscopy, and others may be sufficient to differentiate neoplastic lesions from focal tumourlike demyelinating lesions.Enfermedades autoinmunes desmielinizantes; Esclerosis múltiple; Lesiones desmielinizantes focales seudotumorales Enfermedades menos conocidas de la mielina: lesiones desmielinizantes focales seudotumorales ResumenIntroducción: Las lesiones desmielinizantes focales seudotumorales se definen como lesiones solitarias, desmielinizantes, con diámetro superior de 2 cm. Estas pueden imitar mediante el estudio imagenológico una neoplasia o absceso cerebral, lo que lleva a medidas diagnósticas y terapéuticas invasivas en algunos casos, incrementando la morbilidad. Nuestro objetivo fue analizar y caracterizar estas lesiones clínica, radiológica y patológicamente, lo que, sumado a la revisión de la literatura, aportará al entendimiento de este tipo de trastornos.Métodos: En este estudio descriptivo, se reportan 5 casos con diagnóstico patológico.Mediante la revisión de informes relacionados, clínicos, radiológicos y patológicos, se resumen las características de los sujetos.Resultados: La edad de los pacientes osciló entre los 12 y los 60 años, 3 pacientes fueron de género femenino. La latencia de los síntomas hasta admisión hospitalaria fue entre 3 y 120 días, las manifestaciones clínicas fueron diversas y dependientes de la localización de la lesión, en el 80% de los pacientes se encontraron signos piramidales y no se encontraron clínica o imagenológicamente lesiones de la médula espinal; el seguimiento de los pacientes abarca desde un año hasta 15 años.Conclusión: La biopsia cerebral es el e...

show abstract

“…[1][2][3] Although the conclusive diagnosis of BCS is confirmed via histopathology, MRI has made it possible to diagnose BCS intra vitam, demonstrating the characteristic onion bulb-like structure of the lesions. [4][5][6][7][8][9][10][11][12][13][14][15] Treatment of BCS with corticosteroids, [3][4][5][6][7][8][9][10][11][12][13] other immunosuppressants, [10][11][12] and plasmapheresis 13 is of varying efficacy.…”

mentioning

confidence: 99%

Balo's concentric sclerosis associated with primary human herpesvirus 6 infection

Pohl

2005

Journal of Neurology, Neurosurgery & Psychiatry

View full text Add to dashboard Cite

Treatment of balo's concentric sclerosis with immunosuppressive drugs followed by multimodality evoked potentials and MRI

Cited by 13 publications

References 12 publications

First use of alemtuzumab in Balo’s concentric sclerosis: a case report

First use of alemtuzumab in Balo’s concentric sclerosis: a case report

Lesser-known myelin-related disorders: Focal tumour-like demyelinating lesions

Balo's concentric sclerosis associated with primary human herpesvirus 6 infection

Contact Info

Product

Resources

About