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MM1+2C sporadic Creutzfeldt-Jakob disease presenting as rapidly progressive nonfluent aphasia

Ricardo F Allegri1, Leonardo Bartoloni2, Mónica Iturry3

  • 1Department of Cognitive Neurology, Neuropsychology and Neuropsychiatry, Institute for Neurological Research "Raul Carrea", FLENI, Buenos Aires, Argentina.

Journal of Alzheimer'S Disease : JAD
|October 15, 2013
PubMed

Insights

This study details a rare case of Creutzfeldt-Jakob disease (CJD) subtype MM1+2C, presenting initially with progressive aphasia and rapid dementia. The findings highlight a unique clinical presentation for this specific CJD subtype.

Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative prion disease.
  • Subtypes of CJD, like MM1+2C, are classified based on prion protein (PrP) characteristics and codon 129 polymorphism.
  • Understanding CJD subtypes is crucial for diagnosis and research.

Observation:

  • A 77-year-old male presented with progressive aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, a, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, aphasia, a. A 77-year-old man presented with progressive aphasia, later developing severe dementia over 20 months. Neuropathological examination revealed PrPSc type 2 (MM2C) in the frontal cortex and a mixed MM1+2C pattern in the cerebellum. This specific histopathological subtype (MM1+2C) has not previously been associated with this particular clinical presentation. The study highlights a novel association between a specific CJD subtype and its clinical manifestation, expanding our understanding of prion disease heterogeneity. This case underscores the importance of detailed neuropathological analysis in diagnosing rare CJD subtypes and their varied clinical presentations.
  • Findings

Findings:

  • Western blot of frontal cortex PrPSc was type 2, with codon 129 being MM.
  • Brain neuropathology confirmed MM2C in the cortex, characterized by vacuoles and perivacuolar PrP immunostaining.
  • Cerebellar pathology showed a mixed MM1+2C pattern, with distinct PrP deposits in the molecular and granule cell layers.

Implications:

  • This case presents a unique clinical manifestation (progressive aphasia as initial symptom) for the MM1+2C CJD subtype.
  • The findings expand the known spectrum of clinical presentations for Creutzfeldt-Jakob disease subtypes.
  • Highlights the importance of correlating clinical symptoms with detailed neuropathological and genetic analysis for accurate CJD subtyping and diagnosis.
Keywords:
AphasiaMM1+2C subtypeMM2 cortical subtypeprionsporadic Creutzfeldt-Jakob disease

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