繰り返す脳梗塞に閉塞性水頭症と脳幹萎縮を併発した神経サルコイドーシスの1例  [in Japanese] A case of neurosarcoidosis with recurrent brainstem infarction, obstructive hydrocephalus and brainstem atrophy  [in Japanese]

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<p>症例は42歳女性.2011年に垂直方向の回転性めまいが出現し,1週間程持続した.2013年左片麻痺出現し,右橋上部梗塞と診断された.2017年左下肢麻痺が出現し右橋中部梗塞を認めた.この際,脳室拡大および脳幹の萎縮も認め,当院を紹介受診した.神経学的には下眼瞼向き眼振,左片麻痺,痙性及び逆ハの字型のすり足歩行を認めた.髄液検査では細胞数増多,蛋白高値と糖低下を認め,頭部造影MRIでは脳底部髄膜の増強効果と一部に癒着を認めた.右鼠径部リンパ節腫脹もあり,生検でサルコイドーシスと診断した.本症例は脳底部髄膜炎から脳幹梗塞を多発し,閉塞性水頭症と脳萎縮を併発したことが病態と考えられた.</p>

<p>We report the case of a 42-year-old female with neurosarcoidosis who was hospitalized in year 2017 for gait disturbance. In 2011, she suddenly had vertigo that lasted for a few days. In 2013, she noticed left hemiplegia. A brain MRI revealed an acute infarction on the right side of the upper pons extending longitudinally from the ventral surface. In 2017, she again had left lower limb paralysis. A Brain MRI showed another infarction on the right side of the mid-pons. Hydrocephalus and brainstem atrophy were also noted. The patient was referred to our hospital. Upon neurological examination, she presented with down beat nystagmus, muscle weakness on the left side, and a broad-based spastic gait. CSF findings included an increased number of cells and protein levels with decreased glucose levels. A contrast-enhanced MRI revealed basilar meningitis causing hydrocephalus. A contrast CT scan revealed inguinal lymph node swelling, and scintigram found gallium accumulation. We diagnosed sarcoidosis via a lymph node biopsy. We speculate that chronic basilar meningitis obstructed the patient's branching penetrating arteries inducing infarction together with obstruction of the spinal fluid flow causing hydrocephalus and cerebral atrophy.</p>

Journal

  • Rinsho Shinkeigaku

    Rinsho Shinkeigaku 58(7), 445-450, 2018

    Societas Neurologica Japonica

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