Teaching NeuroImages: NMDA encephalomyelitis with MRI abnormalities isolated to ventral spinal cord gray matter

Published on Aug 11, 2015in Neurology9.91
路 DOI :10.1212/WNL.0000000000001839
Sarah Zubkov2
Estimated H-index: 2
(NYU: New York University),
Puja Aggarwal Joshi2
Estimated H-index: 2
(NYU: New York University)
+ 1 AuthorsSanjeev V. Kothare13
Estimated H-index: 13
(NYU: New York University)
Sources
Abstract
A 17-year-old girl presented with a month of anxiety followed by acute psychosis, catatonia, choreoathetosis, seizures, autonomic instability, lower extremity spasticity, and hyperreflexia. CSF was positive for the NMDA antibody; additional antibodies and infectious etiologies tested negative. MRI brain was normal. MRI spine revealed symmetric T2 hyperintensities in ventral gray matter (figure). EEG showed extreme delta brush. She received the following sequentially: IV gammaglobulin, methylprednisolone, ovarian teratoma resection, and plasmapheresis, only improving after rituximab. At 3 months, repeat MRI spine and motor examination results were normal. NMDA encephalitis with myelopathy is rare1,2; spinal cord gray matter involvement has not been reported previously.
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#1Catherine Pennington (Southern General Hospital)H-Index: 1
#2Shona Livingstone (Southern General Hospital)H-Index: 1
Last. Saif Razvi (Southern General Hospital)H-Index: 12
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Abstract Encephalitis associated with antibodies to the N-methyl d -aspartate receptor (NMDA-R) was first described in young women with ovarian teratoma [ 1 ]. It has subsequently been described in men, children and in those without an underlying tumour [2]. Characteristic clinical features include neuropsychiatric symptoms, seizures, movement disorders, hypoventilation and autonomic instability. Spinal cord disease in association with other typical clinical features has been described in only o...
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#1Michael C. Kruer (OHSU: Oregon Health & Science University)H-Index: 30
#2T.K. KochH-Index: 1
Last. Grazyna AdamusH-Index: 57
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NMDA receptor antibody encephalitis typically begins as a fulminant encephalopathy, with prominent neuropsychiatric manifestations, seizures, dyskinesias, and autonomic instability. After this often dramatic presentation, 1-3 relapses may occur. Most patients either die or recover from the disease.1 We describe a 15-year-old girl who initially presented with encephalopathy, hypoventilation, dyskinesias, and seizures. Her subsequent course was atypical, with more than 10 relapses during the next ...
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#3Thomas C. Varkey (KP: Kaiser Permanente)H-Index: 2
#4Robert P. Lisak (KP: Kaiser Permanente)H-Index: 1
Last. Scott Newsome (KP: Kaiser Permanente)H-Index: 1
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A 31-year-old woman presented with fever, ataxia, double vision, confusion, and auditory hallucinations, preceded by 2 weeks of cough, increased oral secretions, and dermatomal rash. Her medical history was unremarkable. Her family history was notable for first-degree relatives with autoimmune
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#1Agnese SuppiejH-Index: 25
#2Margherita NosadiniH-Index: 16
Last. Stefano SartoriH-Index: 26
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Abstract Objective To clarify the most frequent modalities of use of plasma exchange (PE) in pediatric anti-N-methyl- d -aspartate receptor (anti-NMDAR) encephalitis and to establish the most effective association with other immunotherapies. Methods Systematic literature review on PE in pediatric anti-NMDAR encephalitis (2007鈥2015). Results Seventy-one articles were included (mostly retrospective), reporting a total of 242 subjects (73.2%, 93/127 females; median age at onset 12聽years, range 1鈥18...
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