Possible Neuro-Sweet Disease Mimicking Brain Tumor in the Medulla Oblongata  -Case Report-

Akiba, Chihiro; Esaki, Takanori; Ando, Maya; Furuya, Tsuyoshi; Noda, Kazuyuki; Nakao, Yasuaki; Yamamoto, Takuji; Okuma, Yasuyuki; Mori, Kentaro

doi:10.2176/nmc.51.140

Cited by 5 publications

(2 citation statements)

References 9 publications

(12 reference statements)

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“…However, some unusual brainstem tumors can mimic brainstem gliomas, 10 and even nontumorous diseases can exhibit radiological features similar to brainstem gliomas. 1,19 Therefore, radiological diagnosis cannot be considered definitive, and a proactive surgical biopsy can provide accurate diagnosis and prevent unnecessary treatment targeting. 10 Neuromyelitis optica (NMO) is an autoimmune inflammatory disease primarily affecting the spinal cord and optic nerves.…”

Section: ©Aans 2014mentioning

confidence: 99%

“…These reported diseases include cavernous angioma 9 and some rare diseases, such as Alexander disease and neuro-Sweet disease. 1,19 Their imaging features, exact lesion location, and clinical course are usually atypical for brainstem gliomas and prompt surgical biopsies and other ancillary tests. Neuromyelitis optica is classically known as a disease that characteristically involves only the optic nerve and spinal cord.…”

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confidence: 99%

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Aquaporin-4 autoimmunity masquerading as a brainstem tumor

Lim

Chae

Kim

et al. 2014

PED

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Brainstem glioma is a highly devastating disease, and any mass-like lesion in the brainstem can raise suspicion of this diagnosis. However, other inflammatory, demyelinating, or degenerative diseases can mimic brainstem glioma in clinical presentation and imaging features. Therefore, diagnosis based solely on imaging is often insufficient for brainstem lesions and may lead to incorrect diagnosis and treatment. This case report is the first description of central nervous system aquaporin-4 (AQP4) autoimmunity confined mainly to the brainstem. It demonstrates the wide spectrum of neuroinflammatory diseases in children and highlights the utility of surgical biopsy for suspicious brainstem lesions with atypical imaging features for glioma.

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Section: ©Aans 2014mentioning

confidence: 99%

mentioning

confidence: 99%

Aquaporin-4 autoimmunity masquerading as a brainstem tumor

Lim

Chae

Kim

et al. 2014

PED

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Protean Neurologic Manifestations of Two Rare Dermatologic Disorders: Sweet Disease and Localized Craniofacial Scleroderma

Wallach

Magro

Franks

et al. 2019

Curr Neurol Neurosci Rep

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Neuro sweet syndrome: a systematic review. A rare complication of Sweet syndrome

et al. 2016

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Sweet's syndrome (SS) is an inflammatory disease characterized by fever, leucocytosis and distinctive skin lesions that histologically consist of a dermal infiltrate of neutrophils with nuclear fragmentation. Aseptic neutrophilic inflammation may occur also in other organs. Central nervous system involvement in SS, Neuro-Sweet's syndrome (NSS), is rare and reported especially among Asian patients. A systematic review of the literature has been performed to find articles reporting cases of SS with neurological involvement. The search terms: "Sweet's syndrome/disease with neurological involvement, Neuro Sweet Syndrome/Disease" were used in the Pubmed Database. Sixty-nine NSS patients including 46 males and 23 females, more Asian than Caucasian, have been described from 1983 to date. The average age was 48.7 year-old. The most representative neurologic symptom was the altered state of consciousness, followed by headache and memory disorders. Differently from SS with skin or other district involvement, NSS appears to be more common in Asian patients than in Caucasian ones and affects mainly the male sex in the third or fourth decade of life. A very wide range of symptoms and signs can occur, depending on which part of the nervous system is affected. Initial presentation is usually with the SS typical skin lesions followed by neurological involvement. However, also an opposite presentation or a simultaneous skin and nervous involvement may happen. Awareness of the possible neurological complications in SS is important to avoid unnecessary therapies for other forms of meningoencephalitis and lead to successful treatment with systemic corticosteroids.

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Possible Neuro-Sweet Disease Mimicking Brain Tumor in the Medulla Oblongata -Case Report-

Cited by 5 publications

References 9 publications

Aquaporin-4 autoimmunity masquerading as a brainstem tumor

Aquaporin-4 autoimmunity masquerading as a brainstem tumor

Protean Neurologic Manifestations of Two Rare Dermatologic Disorders: Sweet Disease and Localized Craniofacial Scleroderma

Neuro sweet syndrome: a systematic review. A rare complication of Sweet syndrome

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