99m-Tc-HM-PAO SPECTで広範な集積低下と髄液neuron-specific enolaseの著高を呈した病初期のCreutzfeldt-Jacob病

Diffuse Reduced Uptake in 99m-Tc-HM-PAO SPECT and High Level of Neuron-specific Enolase in Cerebrospinal Fluid in the Early Stage of Creutzfeldt-Jakob Disease

　^99m-Tc-HM-PAO SPECTで広範な集積低下と髄液neuron-specific enolase（NSE）の著高を呈したCreutzfeldt-Jacob病（CJD）の1剖検例を経験した。症例は75歳の女性で，歩行失調と急速に進行する痴呆のため発症3カ月で受診した。発症4カ月で無動無言状態となったがCTやMRIは正常だった。精査のため入院し，脳波で，周期性同期性放電がみられた。髄液NSEは90.6ng/mlと著高を呈し，SPECTでは小脳の集積は比較的保たれていたが，テント上の集積は広範に低下していた。発症13カ月後に死亡し，病理診断はCJDだった。SPECTおよび髄液NSEは，CJDの早期診断および病態解明に有用と思われた。

　We report an autopsy-diagnosed case of Creutzfeldt-Jacob disease (CJD) showing diffuse reduced uptake in SPECT and a high level of neuron-specific enolase (NSE) in the cerebrospinal fluid (CSF). A 75-year-old woman with a 3-month history of progressive gait ataxia and dementia was examined. Four months after onset of the disease, she developed an akinetic mutism with normal CT and MRI. She was admitted for further examination, and electroencephalography showed periodic synchronous discharges. NSE in the CSF showed a high level at 90.6 ng/ml, and SPECT showed diffuse reduced uptake in the supratentorial region, with relatively spared uptake in the cerebellum. She died 13 months after onset of the disease, and the autopsy diagnosis was CJD. SPECT data and the level of NSE in CSF seem to be useful for clinical diagnosis and pathogenetic analysis in the early stage of CJD.