HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

Correspondence to:

BRIEF COMMUNICATIONS: C. Ishida, A. Kakishima, S. Okino, Y. Furukawa, M. Kano, Y. Oda, I. Nakanishi, T. Makifuchi, T. Kitamoto, and M. Yamada Sporadic Creutzfeldt–Jakob disease with MM1-type prion protein and plaques Neurology 2003; 60: 514-517 [Abstract] [Full text] [PDF]

Correspondence: Submit a response to this article

Correspondence published:

Sporadic Creutzfeldt-Jakob disease with MM1-type prion protein and plaques

Chiho Ishida, Tetsuyuki Kitamoto, and Masahito Yamada.   (4 September 2003)

Sporadic Creutzfeldt–Jakob disease with MM1-type prion protein and plaques

Gianfranco Puoti, Giorgio Giaccone, Lucia Limido, Roberto Cotrufo, Giuseppe Di Fede and Fabrizio Tagliavini.   (4 September 2003)

Sporadic Creutzfeldt-Jakob disease with MM1-type prion protein and plaques 4 September 2003
Chiho Ishida, Kanazawa University Graduate School of Medical Science Takara-machi, 13-1, Kanazawa, Ishikawa 920-8640, Japan, Tetsuyuki Kitamoto, and Masahito Yamada. Send Correspondence to journal: Re: Sporadic Creutzfeldt-Jakob disease with MM1-type prion protein and plaques cishida{at}noto-hospital.jp Chiho Ishida, et al.	Dr. Puoti et al [1] raise the possibility of co -occurrence of type 1 and type 2 protease-resistant prion protein (PrPSc) in the brain of our patient with Creutzfeldt-Jakob disease (CJD). However, the report of the distribution of synaptic (granular) and plaque-like prion protein (PrP) deposits in our patient was clear. We found not only the PrP granular deposits of the synaptic type but also plaque-type PrP deposits in the cerebral cortex.[1] In their study, Dr. Puoti et al concluded that the synaptic PrP deposits were associated with type 1 PrPSc and that the plaque-like PrP deposits and a perivacuolar PrP immunoreactivity were related to type 2 PrPSc in the patients with a homozygosity for methionine at codon 129.[2] According to their results, our patient should reveal co-occurrence of type 1 and type 2 PrPSc in the frontal cortex yet only type 1 PrPSc was demonstrated. Dr. Puoti et al reported two patients (Patients 3 and 4) of MM homozygote with focal perivacuolar and plaque-like PrP deposits and found the co-existence of both type 1 and type 2 PrPSc in those patients. However, the plaque-like deposits detected in our patient were unicentric plaque type as shown in our report [1] and appear morphologically different from those in Patient 4 shown in their study.[2] Our patient had different molecular and neuropathologic features of PrP than those reported by Dr. Puoti et al. Recently, we reported that a C-terminal PrP fragment of 11-12 kDa (fPrP11-12) is related to subtypes of dural graft-associated CJD (dCJD) and other prion diseases.[3] There are two subtypes of dCJD-- dCJD with plaque-type PrP deposits (p-dCJD) and dCJD without PrP plaques (np-dCJD), and both the subtypes have type 1 PrPSc.[3] Interestingly, all of the p-dCJD cases show no fPrP11-12, while the np-dCJD cases reveal fPrP11-12.[3] Our patient showed absence of fPrP11-12 as found in p-dCJD cases (data not shown). In addition, our patient and p-dCJD patients shared common neuropathologic features as described in our report.[1] Taken together, we consider that a prion strain in our patient may be similar to that in p-dCJD rather than that in the sporadic CJD patients of MM homozygote with co-occurrence of type 1 and type 2 PrPSc. References 1. Ishida C, Kakishima A, Okino S, et al. Sporadic Creutzfeldt-Jakob disease with MM1-type prion protein and plaques. Neurology 2003;60:514- 517. 2. Puoti G, Giaccone G, Rossi G, Canciani B, Bugiani O, Tagliavini F. Sporadic Creutzfeldt-Jakob disease: Co-occurrence of different types of PrPSc in the same brain. Neurology 1999;53:2173-2176. 3. Satoh K, Muramoto T, Tanaka T, et al. Association of an 11-12 kDa protease-resistant prion protein fragment with subtypes of dura graft- associated Creutzfeldt-Jakob disease and other prion diseases. J Gen Virol 2003 (in press).
Sporadic Creutzfeldt–Jakob disease with MM1-type prion protein and plaques 4 September 2003
Gianfranco Puoti, Second University of Naples - Italy Via Pansini 5, 80131 Napoli - Italy, Giorgio Giaccone, Lucia Limido, Roberto Cotrufo, Giuseppe Di Fede and Fabrizio Tagliavini. Send Correspondence to journal: Re: Sporadic Creutzfeldt–Jakob disease with MM1-type prion protein and plaques gpuoti{at}hotmail.com Gianfranco Puoti, et al.	Ishida et al [1] report a case of sporadic Creutzfeldt-Jakob disease (sCJD) characterized by homozygosity for methionine at codon 129 of the prion protein gene (PRNP) and type 1 PrPres associated with focal plaque-like deposits in the brain. They report that these deposits were mainly in the cerebellar cortex, while the cerebral cortex showed mostly diffuse, synaptic type PrP -immunoreactivity. The immunoblot analysis was performed only on homogenates from the frontal cortex. We previously found the co-occurrence of focal and synaptic PrP-immunoreactivity in the same subject, both in different brain regions and within the same region. [2] In each, we demonstrated the presence of both type 1 and type 2 PrPres in the brain, with a strict relation between PrPres type and pattern of PrP immunoreactivity. In particular, the synaptic pattern was associated with type 1 PrPres, while the focal (plaque-like, perivacuolar and perineuronal) deposits were linked to type 2 PrPres. Most of these cases were homozygous for methionine at codon 129 of PRNP. Sample selection for immunoblot analysis was based on the microdissection of discreet areas of cerebral cortex, subcortical gray structures and cerebellum from frozen brain slices adjacent to the paraffin-embedded section. In this section, immuno-histochemistry revealed either diffuse or focal PrP deposits or both. Immunoblot analysis should be extended to other brain regions, particularly to the cerebellar cortex, where focal PrP-immunoreactivity was prevalent. References 1. Ishida C, Kakishima A, Okino S, et al. Sporadic Creutzfeldt-Jakob disease with MM1-type prion protein and plaques. Neurology 2003; 60: 514- 517 2. Puoti G, Giaccone G, Rossi G, et al. Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP-Sc in the same brain. Neurology 1999; 53: 2173-2176. 3. Parchi P, Giese A, Capellari S et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999; 46:224-233. 4. Giaccone G, Canciani B, Puoti G, et al. Creutzfeldt-Jakob disease: Carnoy’s fixative improves the immunohistochemistry of proteinase-K- resistant prion protein. Brain Pathology 2000; 10:31-37. 5. Bessen R, Marsh RF. Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy J Virol 1994; 68:7859-7868.