Decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata determined by an easy Z-score (eZIS) analysis of 99mTc-ECD-SPECT images in a case of MM2-thalamic-type sporadic Creutzfeldt-Jakob disease

Yuichi Hayashi, Yasushi Iwasaki, Nobuaki Yoshikura, Takahiko Asano, Taku Hatano, Shinsui Tatsumi, Katsuya Satoh, Akio Kimura, Tetsuyuki Kitamoto, Mari Yoshida, Takashi Inuzuka

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

We report a case of autopsy-verified MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD) in a 46-year-old patient with a 16-month history of abnormal behavior, progressive dementia, insomnia, and speech disturbances without family history. Neurological examination revealed progressive dementia, frontal signs, insomnia, speech disturbance, gait disturbance and bilaterally exaggerated tendon reflexes. Both brain MRI and cerebrospinal fluid examinations, including 14-3-3 protein, yielded normal results. An easy Z-score (eZIS) analysis for 99mTc-ethyl cysteinate dimer-single photon emission computed tomography (99mTc-ECD-SPECT) revealed decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata. PRNP gene analysis revealed methionine homozygosity at codon 129 without mutation. Neuropathological examinations revealed severe neuronal loss, gliosis, and hypertrophic astrocytosis in the medial thalamus and inferior olivary nucleus. A slight depletion of Purkinje cells was observed. PrP immunostaining showed no obvious PrP deposits in the basal ganglia, thalamus, cerebellum, or brainstem; however, mild synaptic-type PrP deposits with some smaller plaque-like structures were only partially observed in the localized region of the frontal lobe with the spongiform change. Western blot analyses of protease-resistant PrP showed a type 2 pattern. In conclusion, eZIS analysis of 99mTc-ECD-SPECT images is useful for detecting both thalamic and medullary lesions. This is the first case of medullary lesions detected in a live patient with MM2-thalamic-type sCJD using SPECT.

Original languageEnglish
Pages (from-to)447-452
Number of pages6
JournalJournal of the neurological sciences
Volume358
Issue number1-2
DOIs
Publication statusPublished - 2015 Apr 10

Keywords

  • ECD-SPECT
  • Easy Z-score analysis
  • MM2-thalamic-type
  • Medulla oblongata lesion
  • Sporadic Creutzfeldt-Jakob disease

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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