Overlapping anti-N-methyl-D-aspartate receptor (NMDAR) Encephalitis With Neuromyelitis Optica Spectrum Disorders: A Case Report

Abstract BackgroundAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis can coexist with neuromyelitis optica spectrum disorder (NMOSD). Patients with overlapping anti-NMDAR encephalitis with positive NMDAR antibodies and aquaporin 4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD are rare but should not be ignored.Case presentationThis report describes a unique case of anti-NMDAR encephalitis coexisting with NMOSD is presented. A 27-year-old male presented with blurred vision, cognitive impairment, psychosis, dysphagia, gait instability and urinary incontinence. Brain magnetic resonance imaging (MRI) showed abnormal signals in the right cerebellar hemisphere, temporal lobe, and corpus callosum. NMDAR antibodies were positive in the CSF. AQP4-IgG antibodies were positive in the serum. The patient's condition was stable following intravenous gamma globulin, corticosteroids, immunosuppressants and symptomatic treatments. ConclusionsThis case provides further evidence for the occurrence of anti-NMDAR encephalitis overlapping NMOSD with AQP4-IgG-seropositive in a Chinese patient. However, the mechanisms underlying the occurrence of double positive antibodies remain elusive.

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Meningitis as a recurrent manifestation of anti-AQP4/anti-MOG negative neuromyelitis optica spectrum disorder: a case report

BMC Neurology ◽

10.1186/s12883-021-02133-8 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Chenyang Zhang ◽

Kang Zhang ◽

Bing Chen ◽

Jiao Yin ◽

Miaomiao Dong ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Area Postrema ◽

Neurological Diseases ◽

Brain Magnetic Resonance Imaging ◽

Transverse Myelitis ◽

Corticosteroid Treatment ◽

Neck Stiffness ◽

Mixed Cell ◽

Glucose Levels ◽

The Right

Abstract Background Neuromyelitis optica spectrum disorders (NMOSD), a group of autoimmune neurological diseases, involve the optic nerve, spinal cord, and brain. Meningitis is rarely reported as the primary clinical manifestation of both anti-aquaporin-4 (AQP4)/ anti-myelin oligodendrocyte glycoprotein (MOG) antibody-negative NMOSD (NMOSDneg). Case presentation A 30-year-old man initially presented with fever, headache, and neck stiffness. Lumbar puncture revealed mixed cell reaction and decreased glucose levels. As a result, tuberculous meningitis was suspected. After 1 month, the patient developed longitudinally extensive transverse myelitis and area postrema syndrome. This was followed by the presentation of meningitis-like symptoms once again in the third attack, but his condition eventually improved after corticosteroid treatment without relapse for 2 years. However, he was readmitted to our hospital owing to symptoms of diplopia, hiccup, and numbness in the right hand. Brain magnetic resonance imaging (MRI) revealed that the area postrema still contained lesions. Spinal MRI revealed several segmental enhancements at the C4–C5, T1, and T5 levels. Anti-AQP4 and anti-MOG antibodies were persistently absent in the serum and cerebrospinal fluid (CSF). The patient was finally diagnosed with NMOSDneg. Conclusions Meningitis could be a recurrent manifestation of NMOSDneg and requires more careful evaluation.

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Paroxysmal kinesigenic dyskinesia–like phenotype in multiple sclerosis

Multiple Sclerosis Journal ◽

10.1177/1352458517702535 ◽

2017 ◽

Vol 23 (13) ◽

pp. 1795-1797 ◽

Cited By ~ 4

Author(s):

Roxana Pop ◽

Stefan Kipfer

Keyword(s):

Multiple Sclerosis ◽

Gene Mutations ◽

Brain Magnetic Resonance Imaging ◽

Acute Onset ◽

Intravenous Steroid ◽

Paroxysmal Kinesigenic Dyskinesia ◽

Hyperkinetic Movement Disorder ◽

Magnetic Resonance Imaging Mri ◽

The Right ◽

Rapid Regression

In April 2015, a 20-year-old woman with multiple sclerosis (MS) presented with acute onset of repetitive abnormal postures and choreatic movements of the right arm, precipitated by voluntary movements (online video 1 and 2). Brain magnetic resonance imaging (MRI) showed a new active MS lesion involving the basal ganglia on the left side (Figure 1(a)). Intravenous steroid treatment resulted in rapid regression of this paroxysmal kinesigenic dyskinesia (PKD)-like hyperkinetic movement disorder. The patient became asymptomatic within 3 months. PKD is characterized by recurrent uni- or bilateral choreoathetosis and usually represents an autosomal dominant inherited disorder caused by PRRT2 gene mutations. As in the present case, a PKD-like phenotype may be associated with MS relapses in presumably genetic negative cases.

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A Conjunctival Vascular Malformation as a Rare Presenting Sign of Wyburn–Mason Syndrome

Journal of Pediatric Neurology ◽

10.1055/s-0037-1607996 ◽

2017 ◽

Vol 16 (04) ◽

pp. 239-242

Author(s):

Gunnar Buyse ◽

Lieven Lagae ◽

Philippe Demaerel ◽

Frank Kesteloot ◽

Ingele Casteels ◽

...

Keyword(s):

Vascular Malformation ◽

Arteriovenous Malformations ◽

Brain Magnetic Resonance Imaging ◽

Rare Condition ◽

Vascular Lesion ◽

Facial Skin ◽

Sudden Appearance ◽

Magnetic Resonance Imaging Mri ◽

The Right ◽

The Brain

AbstractWyburn–Mason syndrome is a rare condition that is characterized by ipsilateral arteriovenous malformations affecting the eye, brain, and facial skin. A conjunctival vascular dilation can be a rare ocular presenting sign. We report a 6-year-old boy who attended the hospital because of the sudden appearance of a conjunctival vascular lesion in his right eye. Inspection of his facial skin showed a subtle discoloration along the right trigeminal nerve and a vascular structure of the conjunctiva. Fundoscopy showed dilated and tortuous retinal vessels. Brain magnetic resonance imaging (MRI) revealed a large arteriovenous malformation involving the thalamus and perimesencephalic area. Ophthalmologic and neuroradiologic findings were consistent with the diagnosis of Wyburn–Mason syndrome. The sudden emergence of a vascular malformation in the conjunctiva should alert the clinician to perform an ophthalmoscopy, and in our patient, this finding was the clue to diagnosis of Wyburn–Mason syndrome. Because of the association between retinal and intracranial arteriovenous malformations, an MRI of the brain is strongly recommended in all patients with ocular arteriovenous malformations.

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Case Report: Anti-NMDAR Encephalitis With Anti-MOG CNS Demyelination After Recurrent CNS Demyelination

Frontiers in Neurology ◽

10.3389/fneur.2021.639265 ◽

2021 ◽

Vol 12 ◽

Author(s):

Bing-Yan Ren ◽

Yi Guo ◽

Jing Han ◽

Qian Wang ◽

Zai-Wang Li

Keyword(s):

Case Report ◽

Brain Mri ◽

Clinical Manifestations ◽

Epileptic Seizures ◽

Autoimmune Disorder ◽

Pulse Therapy ◽

Nmdar Encephalitis ◽

Cns Demyelination ◽

Magnetic Resonance Imaging Mri ◽

The Right

Introduction: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, a serious neurological autoimmune disorder caused by autoantibodies with diverse clinical manifestations, may simultaneously onset with antimyelin oligodendrocyte glycoprotein (MOG) demyelination after recurrent central nervous system (CNS) demyelination.Case Report: We present a case of anti-NMDAR encephalitis combining with anti-MOG CNS demyelination following recurrent CNS demyelination. A 38-year-old man admitted to hospital developed epileptic seizures following recurrent episodes of cross-sensory disturbance and dizziness. Magnetic resonance imaging (MRI) showed a demyelinating lesion in the right brainstem initially. Despite a good response to methylprednisolone pulse therapy at the beginning, the patient still had relapses and progression after corticosteroid reduction or withdrawal. Then brain MRI discovered new serpentine lesions involving extensive cerebral cortex on his second relapse. Repeat autoantibodies test indicated cerebrospinal fluid (CSF) NMDAR antibodies coexisted with MOG-Abs simultaneously, suggesting the diagnosis of anti-NMDAR encephalitis with anti-MOG CNS demyelination.Results: After a definite diagnosis, the patient was treated with mycophenolate mofetil (MMF) and corticosteroid. He was discharged after his symptoms ameliorated. No neurological sequels remained, and there were no effects on his activities of daily living after 6 months of immunoregulatory therapy of MMF and corticosteroid.Conclusion: For individuals with recurrent CNS demyelination, especially combining with cortical encephalitis, repeated detection of autoantibodies against AE, and demyelination in CSF/serum can be helpful to enable a definite early diagnosis. For patients who suffer from anti-NMDAR encephalitis combining with anti-MOG CNS demyelination, second-line immunotherapy is recommended when first-line treatment such as steroids, intravenous immunoglobulin G (IVIG) and plasma exchange has been proven ineffective to prevent the relapse of disease.

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Surgical Resection of a Cavernous Malformation of the Anterior Perforated Substance: 2-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opy354 ◽

2018 ◽

Vol 17 (2) ◽

pp. E64-E64

Author(s):

Alejandro Enriquez-Marulanda ◽

Abdulrahman Y Alturki ◽

Luis C Ascanio ◽

Ajith J Thomas ◽

Christopher S Ogilvy

Keyword(s):

Optic Tract ◽

Brain Magnetic Resonance Imaging ◽

Mass Effect ◽

Brain Structures ◽

Acute Onset ◽

Elective Resection ◽

Cavernous Malformations ◽

Patient Consent ◽

Magnetic Resonance Imaging Mri ◽

The Right

Abstract Cavernous malformations (CMs) are intracranial vascular anomalies of the brain blood vessels which are usually asymptomatic but sometimes may cause headache, seizures, or focal neurologic symptoms.1 The latter may be attributed to either hemorrhage of the lesion or due to associated mass effect.2 Here, we describe the case of a 27-yr-old woman who had an acute onset of headache and occasional left sided peripheral visual disturbances. A brain computed tomography scan revealed intraparenchymal hemorrhage near the right hypothalamus and a brain magnetic resonance imaging (MRI) was consistent with an anterior perforated substance CM. Medical management was pursued but severe headache persisted for several weeks to the point of interfering with normal daily function. Given the evidence of past hemorrhage and the persistence of symptoms, a microsurgical resection was indicated. She was scheduled for an elective resection through a right frontotemporal craniotomy. The lesion was found in the right anterior perforated substance, just above the optic tract and was resected without complications. The patient was neurologically stable at discharge. At 4-mo follow-up, the patient had a significantly decreased intensity of headache and an adequate resection cavity on MRI. Otherwise, she was neurologically intact. Cavernous malformations of deep brain structures, such as the anterior perforated substance can be surgically treated but carry a challenging procedure with a risk of visual deficits. In the following video illustration, we narrate this operative case and highlight the nuances of this approach. Patient consent was obtained for the submission of the video to this journal.

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NMO-DBr: the Brazilian Neuromyelitis Optica Database System

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2011000500021 ◽

2011 ◽

Vol 69 (4) ◽

pp. 687-692 ◽

Cited By ~ 8

Author(s):

Marco A. Lana-Peixoto ◽

Lívia Edwiges Talim ◽

Alessandra C. Faria-Campos ◽

Sérgio V.A. Campos ◽

Cristiane F. Rocha ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Brain Magnetic Resonance Imaging ◽

Database System ◽

Optic Coherence Tomography ◽

History Record ◽

Information Management Systems ◽

Associated Conditions ◽

Laboratory Information Management Systems ◽

Spectrum Disorders ◽

Magnetic Resonance Imaging Mri

OBJECTIVE: To present the Brazilian Neuromyelitis Optica Database System (NMO-DBr), a database system which collects, stores, retrieves, and analyzes information from patients with NMO and NMO-related disorders. METHOD: NMO-DBr uses Flux, a LIMS (Laboratory Information Management Systems) for data management. We used information from medical records of patients with NMO spectrum disorders, and NMO variants, the latter defined by the presence of neurological symptoms associated with typical lesions on brain magnetic resonance imaging (MRI) or aquaporin-4 antibody seropositivity. RESULTS: NMO-DBr contains data related to patient's identification, symptoms, associated conditions, index events, recurrences, family history, visual and spinal cord evaluation, disability, cerebrospinal fluid and blood tests, MRI, optic coherence tomography, diagnosis and treatment. It guarantees confidentiality, performs cross-checking and statistical analysis. CONCLUSION: NMO-DBr is a tool which guides professionals to take the history, record and analyze information making medical practice more consistent and improving research in the area.

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Complete Microsurgical Resection of Large Retrochiasmatic Hypothalamic Craniopharyngioma by Transpetrosal Approach: 2-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opz250 ◽

2019 ◽

Author(s):

Qazi Zeeshan ◽

Juan P Carrasco Hernandez ◽

Nina T Yoh ◽

Laligam N Sekhar

Keyword(s):

Frozen Section ◽

Brain Magnetic Resonance Imaging ◽

Optic Chiasm ◽

Pituitary Stalk ◽

Excessive Sleepiness ◽

Microsurgical Resection ◽

Magnetic Resonance Imaging Mri ◽

Prefixed Chiasm ◽

The Right ◽

Retrochiasmatic Region

Abstract This two-dimensional video shows the technical nuances of complete microsurgical resection of a hypothalamic craniopharyngioma located in the retrochiasmatic region by the transpetrosal approach. This 49-yr-old man presented with progressive fatigue, excessive sleepiness, and difficulty in vision in both eyes. He was found to have right CN 3 paralysis and bitemporal hemianopsia on neurological examination. Further workup revealed panhypopituitarism. Brain magnetic resonance imaging (MRI) demonstrated a large solid retrochiasmatic hypothalamic lesion with homogeneous contrast enhancement, measuring 2.1 × 2.6 × 2.4 cm. Optic chiasm was prefixed, and the tumor was just posterior to the pituitary stalk area. The preoperative differential diagnosis included hypothalamic astrocytoma, craniopharyngioma, germinoma, and histiocytosis. Because of the prefixed chiasm, a presigmoid, transpetrosal approach was performed. Our initial plan was a large biopsy, but based on frozen section histology, we decided to excise the tumor completely. The tumor had a pseudocapsule, which was firm and yellowish. It was debulked, dissected from the surrounding hypothalamus, and removed completely. The pituitary stalk was found at the anterior and inferior ends of the tumor and was preserved. Postoperatively, the patient developed diabetes insipidus and requires desmopressin replacement, which was gradually tapered. For panhypopituitarism, he is receiving thyroxine, hydrocortisone, and testosterone. Postoperatively, patient had an improvement in vision in his left eye and ptosis was improving in the right eye with mRs 1- at 10-wk follow-up. An informed consent was obtained from the patient prior to the surgery, which included videotaping of the procedure and its distribution for educational purposes. All relevant patient identifiers have also been removed from the video and accompanying radiology slides.

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Neuromyelitis optica in Brazil: a study on clinical and prognostic factors

Multiple Sclerosis Journal ◽

10.1177/1352458508101935 ◽

2009 ◽

Vol 15 (5) ◽

pp. 613-619 ◽

Cited By ~ 46

Author(s):

DB Bichuetti ◽

EML Oliveira ◽

NA Souza ◽

RLM Rivero ◽

AA Gabbai

Keyword(s):

Neuromyelitis Optica ◽

Age Of Onset ◽

Care Center ◽

Brain Mri ◽

Tertiary Care ◽

Brain Magnetic Resonance Imaging ◽

Brain Lesions ◽

Relapsing Remitting ◽

First Relapse ◽

Magnetic Resonance Imaging Mri

Objectives To describe the clinical characteristics of patients with relapsing neuromyelitis optica (NMO) from a tertiary care center in Brazil and compare the groups with normal and abnormal brain magnetic resonance imaging (MRI). Methods Retrospective review of 41 patients followed at the Neuroimmunology Clinic of the Federal University of São Paulo, Brazil, from 1994 to 2007. Results All patients had relapsing-remitting optic-spinal disease, long extending spinal cord lesions, and brain MRI not meeting Barkhof criteria for multiple sclerosis (MS), thus fulfilling the 1999 and 2006 Wingerchuck criteria for NMO. Mean follow-up time was 52 months; mean age of onset was 32.6 years. The mean relapse rate (RR) and progression index (PI) were 1.0 and 0.9, respectively. Twenty-four patients had brain lesions not compatible with MS on MRI, and there were no statistical differences on PI and RR between patients who had brain lesions and patients who did not. Incomplete recovery, but not the type of first relapse, correlated with a worse prognosis. Seventeen patients were tested for NMO-IgG (anti-aquaporin-4 antibody) with 41% positivity. Conclusions In this series, we did not find a statistical difference of disease progression between patients with and without brain lesions, suggesting that the presence of brain abnormalities is not a marker of disease severity.

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Primary multiple cerebral hydatid disease in a young patient with surgically-treated intracerebral haemorrhage

Romanian Neurosurgery ◽

10.33962/roneuro-2021-011 ◽

2021 ◽

pp. 71-74

Author(s):

Anwar N. Hafedh ◽

Awfa A. Aktham ◽

Zahraa F. Al-Sharshahi ◽

Ahmed Ibrahim Al-Jorani ◽

Sama Albairamani ◽

...

Keyword(s):

Hydatid Disease ◽

Intracerebral Haemorrhage ◽

Right Hemisphere ◽

Brain Magnetic Resonance Imaging ◽

Future Research ◽

New Findings ◽

Mri Scans ◽

Magnetic Resonance Imaging Mri ◽

One Year ◽

The Right

Introduction: Cerebral hydatid disease (CHD) is rare and the multiple-cystic variety is even rarer. In this paper, we report a case of multiple CHD and explore a possible link with a preceding spontaneous intracerebral haemorrhage (ICH). Case presentation: A 27-year old gentleman with a history of surgically-evacuated, spontaneous ICH presented with severe headache, left-sided weakness - Medical Research Council (MRC) grade II - and recurrent tonic-clonic seizures, while on a full dose of anti-epileptic medication. Brain magnetic resonance imaging (MRI) scans showed multiple intra-axial cystic lesions in the right hemisphere. The cysts were removed intact using Dowling’s technique through a large temporoparietal craniotomy. The surgery went uneventful and the patient recovered as expected. Post-operatively, a prophylactic course of albendazole (200 mg) was prescribed. On his one-year follow-up visit, the patient was symptom-free and his weakness had improved (left upper limb: MRC grade IV and full power of the left lower limb). The computed tomography (CT) scan showed no new findings. Conclusion: Primary cerebral hydatid disease is rare and the multiple-cyst variety is even rare. In this case, a peculiar association with a surgically-treated ICH was explored with possible theories to suggest future research directions.

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Cluster-like headache secondary to maxillary sinusitis by Aspergillus

Cephalalgia Reports ◽

10.1177/2515816318814226 ◽

2018 ◽

Vol 1 ◽

pp. 251581631881422

Author(s):

Laura Martinez Rodriguez ◽

Angel Ignacio Perez Alvarez ◽

Jose Maria Sanchez Alvarez

Keyword(s):

Maxillary Sinus ◽

Maxillary Sinusitis ◽

International Headache Society ◽

Brain Magnetic Resonance Imaging ◽

Imaging Study ◽

Resonance Imaging ◽

Symptomatic Cluster Headache ◽

Magnetic Resonance Imaging Mri ◽

Atypical Feature ◽

The Right

A symptomatic cluster headache (CH), caused by fungal maxillary sinus infection fulfilling the criteria of International Headache Society (ICDH-3) for CH is presented. A 35-year-old man without previous headaches reported episodes of pain in the right fronto-orbitary region, with severe eyelid oedema, photophobia, frontal sweating, lacrimation, conjunctival injection and rhinorrhoea, which lasted for 2–3 h. A brain magnetic resonance imaging (MRI) showed occupation of the right maxillary sinus, a mycetoma due to Aspergillus fumigatus. Although rare, secondary causes must be discarded before the diagnosis of a primary CH is made. Imaging study should always be performed, even though no atypical feature was present and ICDH-3 were fulfilled.

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