Author Of 1 Presentation
P0738 - Onset of neuromyelitis optica spectrum disorder with limbic encephalitis or coexistence of those conditions in a single patient? (ID 343)
Abstract
Background
Limbic encephalitis (LE) has been rarely reported to precede, follow or coexist with neuromyelitis optica spectrum disorder (NMOSD). In addition, NMOSD lesions can rarely mimic LE. LE and NMOSD may occur in association with ovarian teratoma.
Objectives
To report a case of NMO-IgG positive NMOSD patient with ovarian teratoma who presented with clinical and magnetic resonance imaging (MRI) findings suggestive of both LE and NMOSD. The patient was finally diagnosed with NMO-IgG positive NMOSD fourteen years following the disease onset.
Methods
Case report: A 23-year-old previously healthy female presents with subacute encephalopathy, ataxia and memory impairment and is diagnosed with herpes simplex virus encephalitis. Fourteen years later she presents with new left arm and bilateral leg weakness and is diagnosed with seropositive NMOSD.
Results
A 23-year-old female had a persistent fever and dizziness three weeks prior to presenting with somnolence, disconjugate gaze, ataxia, seizure and short-term memory impairment in December 2003. Initial brain MRI revealed T2/FLAIR hyperintensities in the medial frontal lobes, interhemispheric fissure, head of the caudate nucleus on the left, left thalamus, diencephalon, around the aqueduct of Sylvius, within the inferior pons, midbrain and middle cerebellar peduncle on the left. An extensive abnormal signal intensity in bilateral medial temporal lobes showed a subtle patchy enhancement. Cerebrospinal fluid (CSF) studies showed mild pleocytosis and mild hyperproteinorachia, negative viral studies and negative CSF culture. Antinuclear antibodies were positive (titer, 1:160), while screening for extractable nuclear antigen, syphilis, lyme disease and human immunodeficiency virus were negative. The patient was diagnosed and treated for HSV encephalitis despite negative CSF viral studies. She was also diagnosed with ovarian teratoma that was surgically removed a year later after which she slowly recovered, but with residual mild short-term memory problems. In 2005 she developed new left-sided optic neuritis. In 2017 she presented with subacute onset of asymmetrical cervical myelopathy associated with longitudinally-extensive transverse myelitis on cervical spine MRI study and positive NMO-IgG (titer, > 1:100000). Autoimmune encephalopathy panel was negative in serum and CSF. The patient was treated with high dose steroids intravenously for five days, followed by a prolonged steroid taper and recovered to her baseline. She started treatment with Rituximab in March 2018 and has been relapse-free since then.
Conclusions
Our case further support the notion that NMOSD may co-exist or mimic LE. Ovarian teratoma might have triggered an initial anti-aquaporin-4 autoimmune response in our patient.
Presenter Of 1 Presentation
P0738 - Onset of neuromyelitis optica spectrum disorder with limbic encephalitis or coexistence of those conditions in a single patient? (ID 343)
Abstract
Background
Limbic encephalitis (LE) has been rarely reported to precede, follow or coexist with neuromyelitis optica spectrum disorder (NMOSD). In addition, NMOSD lesions can rarely mimic LE. LE and NMOSD may occur in association with ovarian teratoma.
Objectives
To report a case of NMO-IgG positive NMOSD patient with ovarian teratoma who presented with clinical and magnetic resonance imaging (MRI) findings suggestive of both LE and NMOSD. The patient was finally diagnosed with NMO-IgG positive NMOSD fourteen years following the disease onset.
Methods
Case report: A 23-year-old previously healthy female presents with subacute encephalopathy, ataxia and memory impairment and is diagnosed with herpes simplex virus encephalitis. Fourteen years later she presents with new left arm and bilateral leg weakness and is diagnosed with seropositive NMOSD.
Results
A 23-year-old female had a persistent fever and dizziness three weeks prior to presenting with somnolence, disconjugate gaze, ataxia, seizure and short-term memory impairment in December 2003. Initial brain MRI revealed T2/FLAIR hyperintensities in the medial frontal lobes, interhemispheric fissure, head of the caudate nucleus on the left, left thalamus, diencephalon, around the aqueduct of Sylvius, within the inferior pons, midbrain and middle cerebellar peduncle on the left. An extensive abnormal signal intensity in bilateral medial temporal lobes showed a subtle patchy enhancement. Cerebrospinal fluid (CSF) studies showed mild pleocytosis and mild hyperproteinorachia, negative viral studies and negative CSF culture. Antinuclear antibodies were positive (titer, 1:160), while screening for extractable nuclear antigen, syphilis, lyme disease and human immunodeficiency virus were negative. The patient was diagnosed and treated for HSV encephalitis despite negative CSF viral studies. She was also diagnosed with ovarian teratoma that was surgically removed a year later after which she slowly recovered, but with residual mild short-term memory problems. In 2005 she developed new left-sided optic neuritis. In 2017 she presented with subacute onset of asymmetrical cervical myelopathy associated with longitudinally-extensive transverse myelitis on cervical spine MRI study and positive NMO-IgG (titer, > 1:100000). Autoimmune encephalopathy panel was negative in serum and CSF. The patient was treated with high dose steroids intravenously for five days, followed by a prolonged steroid taper and recovered to her baseline. She started treatment with Rituximab in March 2018 and has been relapse-free since then.
Conclusions
Our case further support the notion that NMOSD may co-exist or mimic LE. Ovarian teratoma might have triggered an initial anti-aquaporin-4 autoimmune response in our patient.