Lublin Medical University
Neurology

Author Of 2 Presentations

Disease Modifying Therapies – Risk Management Poster Presentation

P0342 - Immune reconstitution inflammatory syndrome in a patient treated with alemtuzumab a few years earlier (ID 1627)

Presentation Number
P0342
Presentation Topic
Disease Modifying Therapies – Risk Management

Abstract

Background

Alemtuzumab is a monoclonal antibody directed against antigen CD52 on cells of the immune system. It is used in the treatment of highly active multiple sclerosis. Immune reconstitution inflammatory syndrome (IRIS) develops due to reconstruction of cellular immunity and inflammation in the central nervous system, in most cases after progressive multifocal leukoencephalopathy (PML). Sometimes IRIS can also occur in the absence of PML.

Objectives

IRIS in a patient treated with alemtuzumab eight years earlier is described. A series of images of MR of the brain will be presented.

Methods

A 38-year-old male patient was admitted because of dysphasia, bulbar syndrome and severe agitation. In 2011 and 2012 he was treated with alemtuzumab because of highly active MS. After 7 years of remission, in 2019 he had a relapse, and in February 2020 he developed severe neurological exacerbation.

Results

MRI of the brain showed multiple big confluencing lesions hyperintensive in T2 weighted images, in the white matter, both supra- and infratentorially, associated with mass effect and contrast enhancement. The patient was treated with prolonged steroids therapy and plasma exchange. JCV-DNA was negative in the CSF. On series of control MRI there was gradual regression of lesions. Neurological state of the patient improved.

Conclusions

IRIS can be a complication of alemtuzumab treatment and arise from the restoration of the previously suppressed immune response, in the absence of active infection i.e. PML.

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Neuro-Ophthalmology Poster Presentation

P0771 - Susac syndrome – a differential diagnosis of demyelinating process (ID 1608)

Presentation Number
P0771
Presentation Topic
Neuro-Ophthalmology

Abstract

Background

Susac syndrome is a retinocochleocerebral autoimmune microangiopathy manifesting as vision disorders (due to branch retinal artery occlusion), encephalopathy and sensory neural hearing loss. In the MRI of the brain in the white matter focal disseminated lesions are present, especially along the corpus callosum.

Objectives

To present a case of Susac syndrome and to underline importance of differential diagnosis in demyelinating process in case of characteristic triad of symptoms.

Methods

A 28-year-old female patient was admitted due to sudden visual impairment of the left eye. Additionally, she had had progressive hearing loss lasting about 2 years and was also treated due to psychiatric disorders.

Results

Additional examinations (MRI of the brain, audiogram, fluorescein angiography of retina) confirmed the diagnosis. Steroids were used in the treatment and azathioprine was introduced. About 7 months later retinal arterial occlusions occurred in the right eye.

Conclusions

In the differential diagnosis of demyelinating process Susac syndrome should be included. It is a multidisciplinary problem, often misdiagnosed as it mimics many other diseases.

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Presenter Of 2 Presentations

Disease Modifying Therapies – Risk Management Poster Presentation

P0342 - Immune reconstitution inflammatory syndrome in a patient treated with alemtuzumab a few years earlier (ID 1627)

Presentation Number
P0342
Presentation Topic
Disease Modifying Therapies – Risk Management

Abstract

Background

Alemtuzumab is a monoclonal antibody directed against antigen CD52 on cells of the immune system. It is used in the treatment of highly active multiple sclerosis. Immune reconstitution inflammatory syndrome (IRIS) develops due to reconstruction of cellular immunity and inflammation in the central nervous system, in most cases after progressive multifocal leukoencephalopathy (PML). Sometimes IRIS can also occur in the absence of PML.

Objectives

IRIS in a patient treated with alemtuzumab eight years earlier is described. A series of images of MR of the brain will be presented.

Methods

A 38-year-old male patient was admitted because of dysphasia, bulbar syndrome and severe agitation. In 2011 and 2012 he was treated with alemtuzumab because of highly active MS. After 7 years of remission, in 2019 he had a relapse, and in February 2020 he developed severe neurological exacerbation.

Results

MRI of the brain showed multiple big confluencing lesions hyperintensive in T2 weighted images, in the white matter, both supra- and infratentorially, associated with mass effect and contrast enhancement. The patient was treated with prolonged steroids therapy and plasma exchange. JCV-DNA was negative in the CSF. On series of control MRI there was gradual regression of lesions. Neurological state of the patient improved.

Conclusions

IRIS can be a complication of alemtuzumab treatment and arise from the restoration of the previously suppressed immune response, in the absence of active infection i.e. PML.

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Neuro-Ophthalmology Poster Presentation

P0771 - Susac syndrome – a differential diagnosis of demyelinating process (ID 1608)

Presentation Number
P0771
Presentation Topic
Neuro-Ophthalmology

Abstract

Background

Susac syndrome is a retinocochleocerebral autoimmune microangiopathy manifesting as vision disorders (due to branch retinal artery occlusion), encephalopathy and sensory neural hearing loss. In the MRI of the brain in the white matter focal disseminated lesions are present, especially along the corpus callosum.

Objectives

To present a case of Susac syndrome and to underline importance of differential diagnosis in demyelinating process in case of characteristic triad of symptoms.

Methods

A 28-year-old female patient was admitted due to sudden visual impairment of the left eye. Additionally, she had had progressive hearing loss lasting about 2 years and was also treated due to psychiatric disorders.

Results

Additional examinations (MRI of the brain, audiogram, fluorescein angiography of retina) confirmed the diagnosis. Steroids were used in the treatment and azathioprine was introduced. About 7 months later retinal arterial occlusions occurred in the right eye.

Conclusions

In the differential diagnosis of demyelinating process Susac syndrome should be included. It is a multidisciplinary problem, often misdiagnosed as it mimics many other diseases.

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