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논문KCI 등재

급성 실어증으로 발현한 신경세포핵내봉입체병

Neuronal Intranuclear Inclusion Disease Presented with Acute Aphasia

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주제분류신경과학

초록

Neuronal intranuclear inclusion disease (NIID) is a rare disorder with slowly progressive neurodegeneration. Major clinical presentation is dementia, but some patients can have episodic symptoms. This is a rare case of NIID presented with acute aphasia. A 62-yearsold male visited emergency room presenting with aphasia. On brain magnetic resonance imaging, a U-shaped diffusion high signal intensity lesion in bilateral frontal corticomedullary junction was observed. A skin biopsy showed intranuclear cytoplasmic inclusion bodies, so he was diagnosed as NIID.

Abstract

Neuronal intranuclear inclusion disease (NIID) is a rare disorder with slowly progressive neurodegeneration. Major clinical presentation is dementia, but some patients can have episodic symptoms. This is a rare case of NIID presented with acute aphasia. A 62-yearsold male visited emergency room presenting with aphasia. On brain magnetic resonance imaging, a U-shaped diffusion high signal intensity lesion in bilateral frontal corticomedullary junction was observed. A skin biopsy showed intranuclear cytoplasmic inclusion bodies, so he was diagnosed as NIID.

참고문헌 (9)

  1. Sone J, "Clinicopathological features of adult-onset neuronal intranuclear inclusion disease", Brain, 139, pp.3170~3186 (2016)
  2. Liu Y, "Clinical and mechanism advances of neuronal intranuclear inclusion disease", Front Aging Neurosci, 14, pp.934725~ (2022)
  3. Huang Y, "Adult-onset neuronal intranuclear inclusion disease, with both stroke-like onset and encephalitic attacks : a case report", BMC Neurol, 21, pp.142~ (2021)
  4. Yang TW, "Neuronal intranuclear inclusion disease presenting with recurrent headache, confusion and progressive cognitive decline", J Korean Neurol Assoc, 38, pp.342~345 (2020)
  5. Zhang J, "Clinical features of neuronal intranuclear inclusion disease with seizures : a systematic literature review", Front Neurol, 15, pp.1387399~ (2024)
  6. Tai H, "Clinical features and classification of neuronal intranuclear inclusion disease", Neurol Genet, 9, pp.e200057~ (2023)
  7. Mori K, "Imaging findings and pathological correlations of subacute encephalopathy with neuronal intranuclear inclusion disease-case report", Radiol Case Rep, 17, pp.4481~4486 (2022)
  8. Kutsuna F, "Perfusion abnormality in neuronal intranuclear inclusion disease with stroke-like episode : a case report", Cereb Circ Cogn Behav, 3, pp.100127~ (2022)
  9. Liang H, "Clinical and pathological features in adult-onset NIID patients with cortical enhancement", J Neurol, 267, pp.3187~3198 (2020)

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