The Importance of Rapid Consideration of Creutzfeldt- Jakob Disease in the Differential Diagnosis of Progressive Neurodegenerative Disease: A Case Report

  • Arthur Joseph Nova Southeastern University College of Osteopathic Medicine, Florida, USA
  • Jacob Core Nova Southeastern University College of Osteopathic Medicine, Florida, USA.
  • Daniel Solano Nova Southeastern University College of Osteopathic Medicine, Florida, USA.
  • Marquand Patton Palmetto General Hospital, Florida, USA.
  • Shaun Smart Palmetto General Hospital, Florida, USA.
Keywords: Creutzfeldt-Jakob Syndrome, Status Epilepticus, Diagnosis

Abstract

Background: Creutzfeldt-Jakob disease (CJD) is a prion disease characterized by misfolded proteins that lead to neurodegeneration and inevitable death. Classic sporadic CJD presents primarily with cognitive symptoms and ataxia without visual impairment at the onset of the illness. Seizure activity is a rare presentation of patients with sporadic CJD. Case: We present a rare case of rapidly progressive encephalopathy in a 57-year-old female who presented to the emergency department with bizarre behavior and vision deterioration. Imaging was unrevealing, and infectious and organic causes were ruled out. Electroencephalogram showed evidence of encephalopathy and non-convulsive status epilepticus. Magnetic resonance imaging conducted later displayed high signal intensity in centrum ovale. The patient’s history, results from diagnostic analyses, and clinical presentation suggested the diagnosis of CJD (sporadic type). Conclusion: Due to the low incidence and varying clinical presentations, it is difficult to include CJD in a differential diagnosis without specific analytic measures. However, for the benefit of the patient and healthcare resources, CJD needs to be quickly considered when rapid neurological decline or non-convulsive status epilepticus is not suggestive of another entity

Author Biography

Arthur Joseph, Nova Southeastern University College of Osteopathic Medicine, Florida, USA

Arthur Joseph is a student at Nova Southeastern University College of  Osteopathic Medicine.

References

1. González-Duarte A, Medina Z, Balaguer RR, Calleja JH. Can prion disease suspicion be supported earlier? Clinical, radiological and laboratory findings in a series of cases. Prion. 2011 Jul-Sep;5(3):201-7.
2. Head MW, Ironside JW. Review: Creutzfeldt-Jakob disease: prion protein type, disease phenotype and agent strain. Neuropathol Appl Neurobiol. 2012 Jun;38(4):296-310.
3. Appleby BS, Appleby KK, Crain BJ, Onyike CU, Wallin MT, Rabins PV. Cha¬racteristics of established and proposed sporadic Creutzfeldt-Jakob disease variants. Arch Neurol. 2009 Feb;66(2):208-15.
4. Prusiner SB. Shattuck lecture—neurodegenerative disease and prions. N Engl J Med. 2001 May 17;344(20):1516-26.
5. Zerr I, Poser S. Clinical diagnosis and differential diagnosis of CJD and vCJD. With special emphasis on laboratory tests. APMIS. 2002 Jan;110(1):88-98.
6. Espinosa PS, Bensalem-Owen MK, Fee DB. Sporadic Creutzfeldt-Jakob di¬
Published
2016-08-14
How to Cite
Joseph, A., Core, J., Solano, D., Patton, M., & Smart, S. (2016). The Importance of Rapid Consideration of Creutzfeldt- Jakob Disease in the Differential Diagnosis of Progressive Neurodegenerative Disease: A Case Report. International Journal of Medical Students, 4(2), 72-75. Retrieved from http://ijms.info/index.php/IJMS/article/view/154
Section
Case Report