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Neuromyelitis Optica Spectrum Disorder with Tumefactive Demyelination mimicking Multiple Sclerosis: A Rare Case.

Roy U, Saini DS, Pan K, Pandit A, Ganguly G, Panwar A - Front Neurol (2016)

Bottom Line: Neuromyelitis optica spectrum disorder (NMOSD) is a diverse condition which not only encompasses isolated longitudinally extensive transverse myelitis (LETM) and optic neuritis but also includes area postrema syndrome, acute brainstem syndrome, symptomatic narcolepsy or acute diencephalic clinical syndrome, and symptomatic cerebral syndrome.Imaging may reveal periependymal lesions surrounding the ventricular system or involvement of corticospinal tracts, area postrema, diencephalon, and corpus callosum.Here, we present a case of aquaporin-4 positive relapsing NMOSD who presented to us with recurrent episodes of paraparesis with LETM and tumefactive lesions of brain on imaging, which enhanced in an incomplete ring like pattern resembling multiple sclerosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Bangur Institute of Neurosciences, The Institute of Post-Graduate Medical Education and Research , Kolkata , India.

ABSTRACT
Neuromyelitis optica spectrum disorder (NMOSD) is a diverse condition which not only encompasses isolated longitudinally extensive transverse myelitis (LETM) and optic neuritis but also includes area postrema syndrome, acute brainstem syndrome, symptomatic narcolepsy or acute diencephalic clinical syndrome, and symptomatic cerebral syndrome. Imaging may reveal periependymal lesions surrounding the ventricular system or involvement of corticospinal tracts, area postrema, diencephalon, and corpus callosum. Rarely, there may be hemispheric tumefactive lesions that enhance in a "Cloud-like" fashion on gadolinium injection unlike in tumefactive multiple sclerosis where there is incomplete ring enhancement. Here, we present a case of aquaporin-4 positive relapsing NMOSD who presented to us with recurrent episodes of paraparesis with LETM and tumefactive lesions of brain on imaging, which enhanced in an incomplete ring like pattern resembling multiple sclerosis.

No MeSH data available.


Related in: MedlinePlus

Repeat MRI brain showing bilateral frontal–parietal hyperintensities on T2 sequence (single arrow) with central hypointensity on FLAIR (double arrow) (A,B). On contrast injection incomplete ring enhancement was seen (triple arrow) (C,D).
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Figure 3: Repeat MRI brain showing bilateral frontal–parietal hyperintensities on T2 sequence (single arrow) with central hypointensity on FLAIR (double arrow) (A,B). On contrast injection incomplete ring enhancement was seen (triple arrow) (C,D).

Mentions: The patient’s neurological status remained unchanged over for next 5 weeks when she developed a left focal motor seizure, neck weakness, and bilateral facial and bulbar palsy along with increased weakness in all four limbs. A repeat MRI of the brain revealed bilateral frontal–parietal hyperintensities on T2 sequence with central hypointensity on FLAIR (Figures 3A,B). Furthermore, on contrast injection incomplete ring enhancement was noted (Figures 3C,D). Cervical MRI showed patchy enhancing long TR hyperintensities in cervical and dorsal cord favoring demyelination (Figures 4A,B). We decided to treat the patient with five cycles of therapeutic plasma exchange (TPE) along with IVMP. On the fourth day of TPE, she again started recovering with regard to extremity strength and with significant improvement in facial and bulbar weakness as well. She was discharged on azathioprine and continued physiotherapy at home. At her 4 months follow–up, she was ambulatory, had re-joined her college, and was doing fine otherwise. Subsequently, OPN was tapered off and azathioprine maintained at a dose of 125 mg/day.


Neuromyelitis Optica Spectrum Disorder with Tumefactive Demyelination mimicking Multiple Sclerosis: A Rare Case.

Roy U, Saini DS, Pan K, Pandit A, Ganguly G, Panwar A - Front Neurol (2016)

Repeat MRI brain showing bilateral frontal–parietal hyperintensities on T2 sequence (single arrow) with central hypointensity on FLAIR (double arrow) (A,B). On contrast injection incomplete ring enhancement was seen (triple arrow) (C,D).
© Copyright Policy
Related In: Results  -  Collection

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Show All Figures
getmorefigures.php?uid=PMC4862986&req=5

Figure 3: Repeat MRI brain showing bilateral frontal–parietal hyperintensities on T2 sequence (single arrow) with central hypointensity on FLAIR (double arrow) (A,B). On contrast injection incomplete ring enhancement was seen (triple arrow) (C,D).
Mentions: The patient’s neurological status remained unchanged over for next 5 weeks when she developed a left focal motor seizure, neck weakness, and bilateral facial and bulbar palsy along with increased weakness in all four limbs. A repeat MRI of the brain revealed bilateral frontal–parietal hyperintensities on T2 sequence with central hypointensity on FLAIR (Figures 3A,B). Furthermore, on contrast injection incomplete ring enhancement was noted (Figures 3C,D). Cervical MRI showed patchy enhancing long TR hyperintensities in cervical and dorsal cord favoring demyelination (Figures 4A,B). We decided to treat the patient with five cycles of therapeutic plasma exchange (TPE) along with IVMP. On the fourth day of TPE, she again started recovering with regard to extremity strength and with significant improvement in facial and bulbar weakness as well. She was discharged on azathioprine and continued physiotherapy at home. At her 4 months follow–up, she was ambulatory, had re-joined her college, and was doing fine otherwise. Subsequently, OPN was tapered off and azathioprine maintained at a dose of 125 mg/day.

Bottom Line: Neuromyelitis optica spectrum disorder (NMOSD) is a diverse condition which not only encompasses isolated longitudinally extensive transverse myelitis (LETM) and optic neuritis but also includes area postrema syndrome, acute brainstem syndrome, symptomatic narcolepsy or acute diencephalic clinical syndrome, and symptomatic cerebral syndrome.Imaging may reveal periependymal lesions surrounding the ventricular system or involvement of corticospinal tracts, area postrema, diencephalon, and corpus callosum.Here, we present a case of aquaporin-4 positive relapsing NMOSD who presented to us with recurrent episodes of paraparesis with LETM and tumefactive lesions of brain on imaging, which enhanced in an incomplete ring like pattern resembling multiple sclerosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Bangur Institute of Neurosciences, The Institute of Post-Graduate Medical Education and Research , Kolkata , India.

ABSTRACT
Neuromyelitis optica spectrum disorder (NMOSD) is a diverse condition which not only encompasses isolated longitudinally extensive transverse myelitis (LETM) and optic neuritis but also includes area postrema syndrome, acute brainstem syndrome, symptomatic narcolepsy or acute diencephalic clinical syndrome, and symptomatic cerebral syndrome. Imaging may reveal periependymal lesions surrounding the ventricular system or involvement of corticospinal tracts, area postrema, diencephalon, and corpus callosum. Rarely, there may be hemispheric tumefactive lesions that enhance in a "Cloud-like" fashion on gadolinium injection unlike in tumefactive multiple sclerosis where there is incomplete ring enhancement. Here, we present a case of aquaporin-4 positive relapsing NMOSD who presented to us with recurrent episodes of paraparesis with LETM and tumefactive lesions of brain on imaging, which enhanced in an incomplete ring like pattern resembling multiple sclerosis.

No MeSH data available.


Related in: MedlinePlus