Hemicraniectomy and externalized ventricular drain placement in a pediatric patient with myelin oligodendrocyte glycoprotein-associated tumefactive demyelinating disease

Sinha, Saurabh; Banwell, Brenda; Tucker, Alexander M.; Storm, Phillip B.; Huh, Jimmy W.; Lang, Shih-Shan

doi:10.1007/s00381-021-05139-2

Cited by 12 publications

(8 citation statements)

References 20 publications

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“…[4][5][6] They can be accompanied by severe clinical manifestations (with potential for intensive care unit admission) and often represent a diagnostic challenge for neurologists, especially when encountered in isolation as the initial manifestation of the disease. 7,8 These lesions have been described in up to 2% of patients with MS, 9 3% of patients with AQP4+NMOSD, 10 and during MOGAD attacks. 5,11 However, the frequency of this manifestation in MOGAD is still unknown because no studies have systematically evaluated tumefactive demyelination in this disease.…”

Section: Discussionmentioning

confidence: 99%

“…evaluated images in an anonymous fashion for consensus. The definition and pictorial explanation of features evaluated are reported in Figure 1 and included the presence of the following: (1) T2-hypointense rim on T2-weighted image, 22 (2) T1-hypointensity on T1-weighted image, 4 (3) Baló-like appearance, 23 (4) cystic component, 24 (5) poorly demarcated borders (i.e., fluffy appearance), 25 (6) restricted diffusion within the lesion, 26 (7) arc/ring of peripheral restricted diffusion, 12 (8) enhancement on postcontrast T1weighted images, (9) pattern of enhancement on postcontrast T1-weighted images including ring (closed if complete; otherwise, open) 22 and cloud-like, 27 and (10) mass effect (i.e., if the lesion exerted a secondary effect on adjacent structures such as sulci, ventricles, or midline).…”

Section: Index Lesion Characteristicsmentioning

confidence: 99%

“…4-6 They can be accompanied by severe clinical manifestations (with potential for intensive care unit admission) and often represent a diagnostic challenge for neurologists, especially when encountered in isolation as the initial manifestation of the disease. 7,8…”

mentioning

confidence: 99%

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Tumefactive Demyelination in MOG Ab–Associated Disease, Multiple Sclerosis, and AQP-4-IgG–Positive Neuromyelitis Optica Spectrum Disorder

et al. 2023

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Background and Objectives.Studies on tumefactive brain lesions in myelin-oligodendrocyte-glycoprotein-IgG-associated disease (MOGAD) are lacking. We sought to characterize the frequency, clinical, laboratory, and MRI features of these lesions in MOGAD and compare them to multiple sclerosis (MS) and aquaporin-4-IgG-positive-neuromyelitis-optica-spectrum-disorder (AQP4+NMOSD).Methods.We retrospectively searched 194 MOGAD and 359 AQP4+NMOSD patients with clinical/MRI details available from the Mayo Clinic databases and included those with ≥1 tumefactive brain lesion (maximum transverse diameter ≥2 cm) on MRI. Tumefactive MS patients were identified using the Mayo Clinic medical-record-linkage-system. Binary multivariable stepwise logistic regression identified independent predictors of MOGAD diagnosis; Cox proportional regression models were used to assess the risk of relapsing disease and gait aid in tumefactivevs. non-tumefactive MOGAD patients.Results.We included 108 patients with tumefactive demyelination (MOGAD=43; AQP4+NMOSD=16; MS=49). Tumefactive lesions were more frequent among MOGAD (43/194[22%]) than AQP4+NMOSD (16/359[5%]) (p<0.001). Risk of relapse and need for gait aid were similar in tumefactive and non-tumefactive MOGAD. Clinical features more frequent in MOGAD than MS included headache (18/43[42%]vs.10/49[20%]; p=0.03) and somnolence (12/43[28%]vs.2/49[4%]; p=0.003), the latter also more frequent than AQP4+NMOSD (0/16[0%]; p=0.02). The presence of: peripheral T2-hypointense rim, T1-hypointensity, diffusion restriction (particularly an arc pattern), ring enhancement, Balo’-like or cystic appearance favored MS over MOGAD (p<0.001). MRI features were broadly similar in MOGAD and AQP4+NMOSD, except for more frequent diffusion restriction in AQP4+NMOSD (10/15[67%]) than MOGAD (11/42[26%], p=0.005). Cerebrospinal fluid analysis revealed less frequent positive oligoclonal bands in MOGAD (2/37[5%]) than MS (30/43[70%]) (p<0.001) and higher median white cell count in MOGAD than MS (33vs.6 cells/µl, p<0.001). At baseline, independent predictors of MOGAD diagnosis were presence of somnolence/headache, absence of T2-hypointense rim, lack of T1-hypointensity, and no diffusion restriction (Nagelkerke R Squared=0.67). Tumefactive lesion resolution was more common in MOGAD than MS or AQP4+NMOSD and improved model performance.Discussion.Tumefactive lesions are frequent in MOGAD but are not associated with a worse prognosis. The clinical, MRI, and CSF attributes of tumefactive MOGAD differ from tumefactive MS, and are more similar to tumefactive AQP4+NMOSD with the exception of lesion resolution, which favors MOGAD.

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Section: Discussionmentioning

confidence: 99%

Section: Index Lesion Characteristicsmentioning

confidence: 99%

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Tumefactive Demyelination in MOG Ab–Associated Disease, Multiple Sclerosis, and AQP-4-IgG–Positive Neuromyelitis Optica Spectrum Disorder

et al. 2023

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“…There have been few reports of MOGAD presenting with isolated ICH. 5,6 In our first case, the identification of papilledema could have been due to ICH, bilateral ON, or both. If not fully worked up, particularly with CSF analysis or neuroimaging, this clinical presentation could be mistaken for idiopathic intracranial hypertension.…”

Section: Discussionmentioning

confidence: 82%

Perplexing Initial Presentations of MOGAD in Two Children: Intracranial Hypertension and New-Onset Seizure

Zhou,

Powers,

Cave

et al. 2023

The Neurohospitalist

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We report two distinct challenging initial presentations of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Case 1 describes a 12-year-old boy who developed headaches refractory to pain medication followed by cranial neuropathies and intracranial hypertension, confirmed by lumbar puncture with an opening pressure >36 cm H2O. Case 2 describes a 3-year-old boy who developed new-onset seizures refractory to antiseizure medications, a presentation of FLAIR-hyperintense lesions in MOG-antibody associated encephalitis with seizures (FLAMES). On repeat magnetic resonance imaging, both patients were found to have cortical T2 hyperintensities, leptomeningeal contrast enhancement, and bilateral optic nerve enhancement. In the cerebrospinal fluid, both patients had CSF pleocytosis with neutrophilic predominance. The patients were treated with intravenous immunoglobulins, plasma exchange, and high-dose corticosteroids. The first patient achieved disease remission, whereas the second patient required the addition of rituximab for management of seizures. The two cases highlight the pleomorphic clinical phenotypes of MOGAD.

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“…While severe cerebral edema is not a common feature of MOGAD, it has previously been reported in both children and adults. Table 1 describes published cases, including medical and surgical management and patient outcomes (11)(12)(13)(14). There are also reports of severe cerebral edema in cases of ADEM without MOG antibodies or for which MOG status was not tested or reported.…”

Section: Discussionmentioning

confidence: 99%

Invasive Multimodality Neuromonitoring to Manage Cerebral Edema in Pediatric Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

Fainberg,

Silver,

Arena

et al. 2023

Critical Care Explorations

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Background: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory disorder of the CNS with a variety of clinical manifestations, including cerebral edema. Case Summary: A 7-year-old boy presented with headaches, nausea, and somnolence. He was found to have cerebral edema that progressed to brainstem herniation. Invasive multimodality neuromonitoring was initiated to guide management of intracranial hypertension and cerebral hypoxia while he received empiric therapies for neuroinflammation. Workup revealed serum myelin oligodendrocyte glycoprotein antibodies. He survived with a favorable neurologic outcome. Conclusion: We describe a child who presented with cerebral edema and was ultimately diagnosed with MOGAD. Much of his management was guided using data from invasive multimodality neuromonitoring. Invasive multimodality neuromonitoring may have utility in managing life-threatening cerebral edema due to neuroinflammation.

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Hemicraniectomy and externalized ventricular drain placement in a pediatric patient with myelin oligodendrocyte glycoprotein-associated tumefactive demyelinating disease

Cited by 12 publications

References 20 publications

Tumefactive Demyelination in MOG Ab–Associated Disease, Multiple Sclerosis, and AQP-4-IgG–Positive Neuromyelitis Optica Spectrum Disorder

Tumefactive Demyelination in MOG Ab–Associated Disease, Multiple Sclerosis, and AQP-4-IgG–Positive Neuromyelitis Optica Spectrum Disorder

Perplexing Initial Presentations of MOGAD in Two Children: Intracranial Hypertension and New-Onset Seizure

Invasive Multimodality Neuromonitoring to Manage Cerebral Edema in Pediatric Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

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