Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy Follow-up

  • Author: Florian P Thomas, MD, MA, PhD, Drmed; Chief Editor: Karen L Roos, MD   more...
 
Updated: Jun 21, 2010
 

Further Inpatient Care

Patients may be admitted for an expedited workup, including lumbar puncture and biopsies. Some patients may need inpatient psychiatric care.

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Further Outpatient Care

As discussed, patients may need treatment for psychiatric and sensory symptoms in addition to the increasing need for supportive care.

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Deterrence/Prevention

Variant CJD may spread by iatrogenic means; concern is even more necessary with variant CJD compared with other prion diseases because the titer of the causative agent appears to be high in systemic organs, although not as high as in the neural tissues.

Because the prion agent is highly resistant to inactivation, routine sterilization methods such as autoclaving are not effective. Hence, electromyography and EEG needles, surgical instruments, and other equipment that has been exposed to a patient with variant CJD should not be reused. When such equipment is used in patients with suspected prionoses, it can be quarantined while the definitive studies are pending. If variant CJD is proven, used equipment must be destroyed. If the diagnosis is disproved, equipment can be reused.

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Complications

Any part of the CNS can be affected; therefore, a range of CNS complications can be expected. These include gait difficulties due to spasticity and cerebellar ataxia, choreoathetosis, startle responses, myoclonus, dysphagia due to pseudobulbar palsy, incontinence, and akinetic mute state.

Most patients succumb to bronchopneumonia, brought about by their bed-ridden state.

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Patient Education

For excellent patient education resources, visit eMedicine's Brain and Nervous System Center and Public Health Center. In addition, see eMedicine's patient education articles Mad Cow Disease and Variant Creutzfeldt-Jakob Disease and FDA Overview.

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Contributor Information and Disclosures
Author

Florian P Thomas, MD, MA, PhD, Drmed  Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University School of Medicine

Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, and National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Amy A Pruitt, MD  Associate Professor of Neurology, University of Pennsylvania School of Medicine; Attending Neurologist, Hospital of the University of Pennsylvania

Amy A Pruitt, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Neil A Busis, MD  Chief, Division of Neurology, Department of Medicine, Head, Clinical Neurophysiology Laboratory, University of Pittsburgh Medical Center-Shadyside

Neil A Busis, MD is a member of the following medical societies: American Academy of Neurology and American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Karen L Roos, MD  John and Nancy Nelson Professor of Neurology, Professor of Neurological Surgery, Department of Neurology, Indiana University School of Medicine

Karen L Roos, MD is a member of the following medical societies: American Academy of Neurology and American Neurological Association

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Chitharanjan V Rao, MD, MRCP, DM to the development and writing of this article.

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Incidence of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (CJD) in Great Britain. The BSE epidemic peaked in 1992, 4 years after the introduction of the ban on ruminant feed. The associated human disease, variant CJD, was not defined until 1996, 7 years after a ban was introduced in Britain on the use of specified offal from cattle in human food.
Geographic distribution of bovine spongiform encephalopathy (BSE) by country as of January 9, 2004. From http://www.oie.int/eng/info/en_esb.htm.
Time course of epidemic bovine spongiform encephalopathy (BSE) in the United Kingdom, 1986-2000, with dates of major precautionary interventions. SBO stands for specified bovine offal (ie, brain, spinal cord, thymus, spleen, and intestines from cattle aged >6 mo). MBM stands for meat and bone meal (protein residue produced by rendering). From Brown P, Will RG, Bradley R, et al. "Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution and current concerns". Emerging Infectious Diseases, 2001;7: 6-16.
Normal fluid-attenuated inversion recovery (FLAIR) image at the level of the basal ganglia shows that the thalamus is normally isointense or slightly hypointense relative to putamen. From Collie DA, Summers DM, Sellar RJ, et al. "Diagnosing variant Creutzfeldt-Jakob disease with the Pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases." Am J Neuroradiol, 2003;24: 1560-9.
Pulvinar sign of variant Creutzfeldt-Jakob disease. Fluid-attenuated inversion recovery (FLAIR) image shows marked symmetrical hyperintensity of the pulvinar (posterior) thalamic nuclei, and this sign is present in 100% of cases imaged with FLAIR imaging. From Collie DA, Summers DM, Sellar RJ, et al. "Diagnosing variant Creutzfeldt-Jakob disease with the Pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases." Am J Neuroradiol, 2003;24: 1560-9.
Axial fluid-attenuated inversion recovery (FLAIR) showing periaqueductal gray matter hyperintensity (arrow). Although not a specific sign, periaqueductal hyperintensity is observed in 83% of patients imaged with FLAIR imaging. From Collie DA, Summers DM, Sellar RJ, et al. "Diagnosing variant Creutzfeldt-Jakob disease with the Pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases." Am J Neuroradiol, 2003;24: 1560-9.
Hockey stick sign of variant Creutzfeldt-Jakob disease. Fluid-attenuated inversion recovery (FLAIR) image shows symmetrical pulvinar and dorsomedial thalamic nuclear hyperintensity. This combination produces a characteristic hockey stick appearance and is present in 93% of patients imaged with FLAIR imaging. From Collie DA, Summers DM, Sellar RJ, et al. "Diagnosing variant Creutzfeldt-Jakob disease with the Pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases." Am J Neuroradiol, 2003;24: 1560-9.
Prion protein (PrP) accumulation in the tonsil in variant Creutzfeldt-Jakob disease within follicular dendritic cells and macrophages in a germinal center as demonstrated by PrP immunocytochemistry. From Ironside JW, Frosch MP, Bernardino G. "Human prion diseases." In: Gray F, De Girolami U, Poirier J, eds. Escourelle & Poirier Manual of Basic Neuropathology. Philadelphia, Pa: Elsevier, 2004: 145-57.
The florid plaque in the cerebral cortex in variant Creutzfeldt-Jakob disease comprises a dense core with a paler outer layer of amyloid fibrils surrounded by spongiform change (hematoxylin and eosin stain at low magnification). From Ironside JW, Frosch MP, Bernardino G. "Human prion diseases." In: Gray F, De Girolami U, Poirier J, eds. Escourelle & Poirier Manual of Basic Neuropathology. Philadelphia, Pa: Elsevier, 2004: 145-57.
The florid plaque in the cerebral cortex in variant Creutzfeldt-Jakob disease comprises a dense core with a paler outer layer of amyloid fibrils surrounded by spongiform change (hematoxylin and eosin stain at high magnification). From Ironside JW, Frosch MP, Bernardino G. "Human prion diseases." In: Gray F, De Girolami U, Poirier J, eds. Escourelle & Poirier Manual of Basic Neuropathology. Philadelphia, Pa: Elsevier, 2004: 145-57.
Immunocytochemistry for prion protein (PrP) shows strong staining of the florid plaques and multiple smaller plaques and diffuse PrP deposits (low magnification). From Ironside JW, Frosch MP, Bernardino G. "Human prion diseases." In: Gray F, De Girolami U, Poirier J, eds. Escourelle & Poirier Manual of Basic Neuropathology. Philadelphia, Pa: Elsevier, 2004: 145-57.
Immunocytochemistry for prion protein (PrP) shows strong staining of the florid plaques and multiple smaller plaques and diffuse PrP deposits (higher magnification). From Ironside JW, Frosch MP, Bernardino G. "Human prion diseases." In: Gray F, De Girolami U, Poirier J, eds. Escourelle & Poirier Manual of Basic Neuropathology. Philadelphia, Pa: Elsevier, 2004: 145-57.
Table 1. Psychiatric Features According to Frequency and Median Time of Onset (adapted from Spencer et al, 2002[64] )
Psychiatric FeaturesEarly Onset



< 4 mo



Later Onset



4 to < 6 mo



Late Onset



≥ 6 mo



Common



n ≥ 50



Dysphoria



Withdrawal



Anxiety



Irritability



Insomnia



Loss of interest



Poor memory



Impaired concentration



Disorientation



Agitation



Less common



n = 25 to < 50



Behavioral changes



Anergia



Poor performance



Tearfulness



Weight loss



Appetite change



Hypersomnia



Confusion



Hallucinations



Impaired self care



Paranoid delusions



Inappropriate affect



Rare



n < 25



Obsessive features



Losing things



Suicidal ideation



Panic attacks



Psychomotor retardation



Diurnal mood variation



Loss of confidence



Bizarre behavior



Paranoid ideation



Recognition impairment



Confabulation



Lack of emotion



Perseveration



Impaired comprehension



Change in eating preferences



Impaired use of devices



Acalculia



Table 2. Neurologic Features According to Frequency and Median Time of Onset (adapted from Spencer et al, 2002[64] )
Neurologic FeaturesEarly Onset



< 4 mo



Later Onset



4 to < 6 mo



Late Onset



≥ 6 mo



Common



n ≥ 50



NoneGait disturbance



Slurring of speech



Hyperreflexia



Impaired coordination



Myoclonus



Incontinence



Eye features



Less common



n = 25 to < 50)



PainParesthesia



Numbness



Chorea



Extensor plantars



Dysphagia



Clonus



Hypertonia



Primitive reflexes



Rare



n < 25



Headaches



Dropping things



Sweatiness



Loss of consciousness



Tremors



Handwriting impairment



Coldness



Odd sensation



Dizziness



Cranial motor weakness



Dysdiadochokinesis



Taste disturbance



Startle response



Hypersensitivity



Peripheral motor weakness



Primitive reflexes



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