Dura mater graft‐associated Creutzfeldt‐Jakob disease with 30‐year incubation period

Abstract

Over 60% of all patients with dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) have been diagnosed in Japan. The incubation period has ranged from 1 to 30 years and the age at onset from 15 to 80 years. Here, we report a 77-year-old male Japanese autopsied dCJD case with the longest incubation period so far in Japan. He received a cadaveric dural graft at the right cranial convexity following a craniotomy for meningioma at the age of 46. At 30 years post-dural graft placement, disorientation was observed as an initial symptom of dCJD. He rapidly began to present with inconsistent speech, cognitive impairment and tremor of the left upper extremity. Occasional myoclonic jerks were predominantly observed on the left side. Brain MRI presented hyperintense signals on diffusion-weighted and T2-weighted images, at the right cerebral cortex. The most hyperintense lesion was located at the right parietal lobe, where the dura mater graft had been transplanted. Single-photon emission CT scan showed markedly decreased cerebral blood flow at the right parietal lobe. EEG revealed diffuse and slow activities with periodic sharp-wave complex discharges seen in the right parietal, temporal and occipital lobes. He died of pneumonia 9 months after onset. Brain pathology revealed non-plaque-type dCJD. Laterality of neuropathological changes, including spongiform change, neuronal loss, gliosis or PrP deposits, was not evident. Western blot analysis showed type 1 PrP . Alzheimer-type pathology and PSP-like pathology were also observed.

Case report: Primary intracranial EWs/PNET in adults: Clinical experience and literature review

“…Meningiomas are common adult central nervous system tumors, mostly benign. Meningiomas on CT are mostly isodense or high-density spherical or hemispherical masses, with a higher probability of calcification, but with less low-density cystic necrosis, and even if present, the volume of cystic necrosis is small ( 31 ). On MRI, the signal of meningiomas is relatively uniform, and the T1WI and T2WI sequences are iso-to-hyperintense, and hyperintensity is more common on DWI, while our patient was hypointense on DWI, which is different from it.…”

Differential Accumulation of Misfolded Prion Strains in Natural Hosts of Prion Diseases

“…One report on an individual with dura mater-derived iCJD states that there was Type 1 PrP Sc accumulated in the cerebellar cortex. A synaptic staining pattern was found in both the molecular and granule cell layer of the cerebellar cortex [177]. When PrP Sc accumulates in humans with sCJD, reports vary.…”

“…PrP Sc accumulates in the cerebella of humans with prion diseases [95,163,166,[175][176][177]. A summary of these differences can be found in Table 9.…”

Research and application progress on dural substitutes

“…The titer of prions in the central nervous system is much higher than that in other parts, and there is no simple and reliable detection method for prions [23]. Therefore, dural substitutes made from allogeneic dura mater were gradually withdrawn from the historical stage when the source of the dura mater was insufficient and the transmission of prions could not be completely avoided [24,25]. Domestic and foreign scholars have also tried to use amniotic membrane, dermis, and other allogenic materials to prepare dural substitutes and have achieved some positive results [26][27][28].…”