Contributions
Abstract: EP1413
Type: ePoster
Abstract Category: Pathology and pathogenesis of MS - Pathology
Background: Idiopathic inflammatory demyelinating disorders (IDD) encompass several distinct disorders including Multiple sclerosis, Neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis and other entities including tumefactive demyelination. Those disorders can present with atypical MRI findings, sometimes leading to brain biopsy to exclude differential diagnoses. Luchinetti et al., reported in 2015 a series of patients with atypical demyelinating lesions with an extensive pathologic workup suggestive of NMOSD.
Objectives: To characterize radiological and pathological data of patients with atypical demyelinating lesions undergoing brain biopsy in a retrospective monocentric study
Methods: Al the patients with brain biopsy suggestive of a demyelination were included. Clinical, pathological and radiological findings were analyzed.
Results: After exclusion of 64 patients with alternative diagnosis (n=39 tumor) 6 patients (4 males/2 females; age at onset 37 years) were analyzed. Two patients had marked hemiparesis, 2 had brainstem syndrome, one had myelitis and visual disturbance and one had headache and seizures. Three had 1 to 3 recurrences, always under 3 years of follow (mean follow-up: 6.5 years). One patient died. CSF study was normal in all, but one (oligoclonal bands and marked pleiocytosis). MRI findings included juxtacortical and cortical lesions in 3, brainstem and/or diencephalon involvement in 1 and spinal cord lesions in 3. Gadolinium enhancement was mainly punctate and curvilinear (in 4). Histological analysis consisted of predominant macrophagic infiltrate with myelin phagocytosis and/or myelin loss. Dystrophic astrocytes were noted in 1 case. Aquaporin-4 immunohistochemistry was negative in 3 patients suggestive of a NMO (2 had negative NMO IgG).
Conclusion: Atypical central nervous system demyelinating lesions can present with various brain MRI features that can sometimes resemble those seen in NMOSD (especially for patient 1)2.
Our data suggest that: - Patients share similar pathological data in favor of a demyelinating disease rather than autoimmune astrocytopathy. - AQP4 immunoreactivity is increased in the majority of the patients.
Disclosure: Xavier Ayrignac: nothing to disclose
Sonia Hebbadj: nothing to disclose
Benoit Lhermitte: nothing to disclose
Valérie Rigau: nothing to disclose
Thierry Vincent: nothing to disclose
Cédric Raoul: nothing to disclose
Jerome de seze: nothing to disclose
Beatrice Lannes: nothing to disclose
Stephane Kremer: nothing to disclose
Pierre Labauge: nothing to disclose
Abstract: EP1413
Type: ePoster
Abstract Category: Pathology and pathogenesis of MS - Pathology
Background: Idiopathic inflammatory demyelinating disorders (IDD) encompass several distinct disorders including Multiple sclerosis, Neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis and other entities including tumefactive demyelination. Those disorders can present with atypical MRI findings, sometimes leading to brain biopsy to exclude differential diagnoses. Luchinetti et al., reported in 2015 a series of patients with atypical demyelinating lesions with an extensive pathologic workup suggestive of NMOSD.
Objectives: To characterize radiological and pathological data of patients with atypical demyelinating lesions undergoing brain biopsy in a retrospective monocentric study
Methods: Al the patients with brain biopsy suggestive of a demyelination were included. Clinical, pathological and radiological findings were analyzed.
Results: After exclusion of 64 patients with alternative diagnosis (n=39 tumor) 6 patients (4 males/2 females; age at onset 37 years) were analyzed. Two patients had marked hemiparesis, 2 had brainstem syndrome, one had myelitis and visual disturbance and one had headache and seizures. Three had 1 to 3 recurrences, always under 3 years of follow (mean follow-up: 6.5 years). One patient died. CSF study was normal in all, but one (oligoclonal bands and marked pleiocytosis). MRI findings included juxtacortical and cortical lesions in 3, brainstem and/or diencephalon involvement in 1 and spinal cord lesions in 3. Gadolinium enhancement was mainly punctate and curvilinear (in 4). Histological analysis consisted of predominant macrophagic infiltrate with myelin phagocytosis and/or myelin loss. Dystrophic astrocytes were noted in 1 case. Aquaporin-4 immunohistochemistry was negative in 3 patients suggestive of a NMO (2 had negative NMO IgG).
Conclusion: Atypical central nervous system demyelinating lesions can present with various brain MRI features that can sometimes resemble those seen in NMOSD (especially for patient 1)2.
Our data suggest that: - Patients share similar pathological data in favor of a demyelinating disease rather than autoimmune astrocytopathy. - AQP4 immunoreactivity is increased in the majority of the patients.
Disclosure: Xavier Ayrignac: nothing to disclose
Sonia Hebbadj: nothing to disclose
Benoit Lhermitte: nothing to disclose
Valérie Rigau: nothing to disclose
Thierry Vincent: nothing to disclose
Cédric Raoul: nothing to disclose
Jerome de seze: nothing to disclose
Beatrice Lannes: nothing to disclose
Stephane Kremer: nothing to disclose
Pierre Labauge: nothing to disclose