Hanne Pernille Bro Skejoe

Aleris Hamlet Hospital

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Nasrin Asgari

University of Southern Denmark

Friedemann Paul

Max Delbrück Center

Søren Thue Lillevang

Odense University Hospital

Jakob Grauslund

University of Southern Denmark

Vanda A. Lennon

Mayo Clinic

Kerstin Soelberg

University of Southern Denmark

Brian G. Weinshenker

Mayo Clinic

Jens Wuerfel

Humboldt-Universität zu Berlin

Troels Steenstrup

University of Southern Denmark

Terry J. Smith

University of Michigan–Ann Arbor

Cooperative Institutions

University of Southern Denmark

Odense University Hospital

Charité - Universitätsmedizin Berlin

Humboldt-Universität zu Berlin

Freie Universität Berlin

Max Delbrück Center

Lillebaelt Hospital

Slagelse Hospital

Rigshospitalet

University of Copenhagen

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Modifications of longitudinally extensive transverse myelitis and brainstem lesions in the course of neuromyelitis optica (NMO): a population-based, descriptive study

BMC Neurology (2013)

Nasrin Asgari Hanne Pernille Bro Skejoe Søren Thue Lillevang Troels Steenstrup Egon Stenager

Neuromyelitis optica (NMO) includes transverse myelitis, optic neuritis and brain lesions. Recent studies have indicated that the brainstem is an important site of attack in NMO. Longitudinally extensive transverse myelitis (LETM) is an important component of the clinical diagnosis of NMO. The frequency of brainstem and LETM lesions, changes over time of LETM and the clinical consequences in the course of NMO have only been sparsely studied. The study was a population-based retrospective case series with clinical and magnetic resonance imaging (MRI) follow-up of 35 patients with definite NMO and a relapsing-remitting course. Brainstem lesions were observed in 25 patients, 18 in medulla oblongata (11 in area postrema). Lesions in the pons, mesencephalon and diencephalon occurred in 10, 7 and 7 patients, respectively. Lesions were symptomatic in medulla oblongata and pons, asymptomatic in mesencephalon and diencephalon. Brainstem lesions were observed significantly more often in anti-aquaporin-4 (AQP-4) antibody positive than in seronegative patients (p < 0.002). LETM was demonstrated by MRI of the spinal cord in 30/36 patients, 23/30 of whom had follow-up MRI of the spinal cord. Recurrent LETM was observed in five patients. In nine patients the LETM changed into multiple lesions during remission or treatment. Spinal cord atrophy was observed in 12/23 (52%) patients, correlating to Expanded Disability Status Scale (r = 0.88, p < 0.001). NMO patients had frequent occurrence of brainstem lesions and LETM. Brainstem lesions were associated with anti-AQP4 antibody positivity. LETM lesions differentiated over time and the outcome included relapses, fragmentation and atrophy. Correlation was observed between spinal cord atrophy and neurological disability.

Neuromyelitis Optica

Pons

10.1186/1471-2377-13-33

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Citations (94)

Signal abnormalities on magnetic resonance imaging in acute optic neuritis. A prospective study

Kerstin Soelberg Hanne Pernille Bro Skejoe Jesper Mehlsen Jakob Grauslund Friedemann Paul

Optic neuritis

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Evolution of longitudinally extensive transverse myelitis in an aquaporin-4 IgG-positive patient

Neurology (2013)

Nasrin Asgari Hanne Pernille Bro Skejoe Vanda A. Lennon

A 36-year-old woman presented with hemiplegia and loss of pain and temperature sensation 1 day after the subacute onset of intractable vomiting. Paresthesia followed. She was tetraplegic at day 12. Initial MRI revealed a lesion in the medulla oblongata, involving primarily the area postrema (figure). The lesion progressively extended into the upper cervical cord. Forebrain MRI was normal. CSF contained 11 leukocytes/mm3. Brainstem tumor and multiple sclerosis were early diagnostic considerations. Aquaporin-4 immunoglobulin (Ig)G was detected in serum. This case supports the concept that fenestrated capillaries in the area postrema are an important initial CNS entry site for pathogenic neuromyelitis optica–IgG in neuromyelitis optica spectrum disorders.1,2

Area postrema

Neuromyelitis Optica

Fourth ventricle

10.1212/wnl.0b013e318297ef07

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Leptomeningeal and Intraparenchymal Blood Barrier Disruption in a MOG-IgG-Positive Patient

Case Reports in Neurological Medicine (2018)

Seyed Hamidreza Mohseni Hanne Pernille Bro Skejoe Jens Wuerfel Friedemann Paul Markus Reindl

Recently, pathogenic serum immunoglobulin G (IgG) autoantibodies to myelin oligodendrocyte glycoprotein (MOG) have been detected in a subgroup of patients with central nervous system (CNS) demyelination, including in patients with myelitis. Relatively little is known so far about leptomeningeal involvement in MOG-IgG-positive myelitis.We report the case of a 30-year-old previously healthy woman presenting with longitudinally extensive transverse myelitis and tetraparesis, in whom both the leptomeningeal barrier and the blood-brain barrier (BBB) were altered, as demonstrated by gadolinium-enhanced MRI during relapse. Blood samples taken at onset and four years later were retrospectively found positive for MOG-IgG.Our findings demonstrate that spinal leptomeningeal enhancement (LME) can occur in MOG-IgG-positive encephalomyelitis (EM) and may accompany intraparenchymal BBB breakdown.

Myelin oligodendrocyte glycoprotein

Encephalomyelitis

10.1155/2018/1365175

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Citations (13)