What EEG findings are characteristic of subacute spongiform encephalopathy>?

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Aguglia et al described 20 patients with subacute spongiform encephalopathy and periodic paroxysmal activities in the EEG. Evolution of clinical and EEG abnormalities were analyzed in all 20 (16 pathologically confirmed). Illness duration was less than 4 months in 65% and greater than 17 months in 10%. The early clinical stage was characterized by gradual gait disturbances, mental deterioration, and sensory or autonomic changes. In 10 EEG recordings from 7 patients examined in the early clinical stage, no periodic discharges were present.^[47]

Early periodic paroxysmal activity appeared within 12 weeks of the onset of the disease in 88% of the patients who underwent EEG recordings. This early periodic paroxysmal activity usually occurred at an intermediary stage, when the patients demonstrated marked worsening of the clinical picture. Focal, segmental, and/or generalized myoclonic jerks were observed in 15%, 53%, and 100% of cases at prodromal, intermediary, and terminal stages, respectively. Different kinds of periodic paroxysmal activity were observed:

Biphasic or triphasic periodic complexes
Periodic complexes with multiphasic configuration
Periodic polyspiking discharges

Abnormal "pacing" by slowly repeated flashes was found in 4 patients presenting with visual hallucinations or cortical blindness. Burst-suppression activity was observed frequently in the terminal stage in decorticate patients.

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Media Gallery

EEG in dementia.
Bilateral periodic epileptiform discharges (BiPEDs).
Triphasic waves, maximum amplitude bilateral frontal.
Frontal intermittent rhythmic delta activity (FIRDA).
MRI axial diffusion weighted image: Cortical ribbon sign in CJD.
Generalized periodic epileptiform discharges (GPED).
MRI axial FLAIR with gadolinium; herpes encephalitis, left temporal.
Left temporal intermittent rhythmic delta (TIRDA).
Periodic lateralized epileptiform discharges.
Anoxic encephalopathy. Burst suppression pattern with bursts of spike and wave and polyspike wave discharges with voltage suppression.

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Author

Eli S Neiman, DO, FACN Clinical Associate Professor of Neurology, Department of Neurology, Kansas City University of Medicine and Biosciences College of Osteopathic Medicine; Clinical Assistant Professor, Robert C Byrd Health Science Center, West Virginia University School of Medicine; Assistant Professor of Neurology, Hackensack Meridian School of Medicine at Seton Hall University; Neurologist and Clinical Neurophysiologist, National Neuromonitoring Services, LLC; Neurologist, Advanced Neurology Center

Eli S Neiman, DO, FACN is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Osteopathic Association

Disclosure: Nothing to disclose.

Coauthor(s)

Philip A Hanna, MD Associate Professor of Neuroscience, JFK Neuroscience Institute at JFK Medical Center, Hackensack Meridian School of Medicine at Seton Hall University

Philip A Hanna, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida Morsani 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, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Ceribell, Eisai, Greenwich, Growhealthy, LivaNova, Neuropace, SK biopharmaceuticals, Sunovion<br/>Serve(d) as a speaker or a member of a speakers bureau for: Eisai, Greenwich, LivaNova, Sunovion<br/>Received research grant from: Cavion, LivaNova, Greenwich, Sunovion, SK biopharmaceuticals, Takeda, UCB.

Acknowledgements

Leslie Huszar, MD Consulting Staff, Department of Neurology, Indian River Memorial Hospital

Leslie Huszar, MD is a member of the following medical societies: American Academy of Neurology, American Association for the Advancement of Science, and American Medical Electroencephalographic Association

Disclosure: Nothing to disclose.

Erasmo A Passaro, MD, FAAN Director, Comprehensive Epilepsy Program/Clinical Neurophysiology Lab, Bayfront Medical Center, Florida Center for Neurology

Erasmo A Passaro, MD, FAAN is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Association, and American Society of Neuroimaging

Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Forest Honoraria Speaking and teaching

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

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