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Substantial Remission in Subacute Sclerosing Panencephalitis by Following the Ketogenic Diet: A Case Report

An eight-year-old boy presented with rhythmic myoclonic jerks that stretched back to the age of four years. He was diagnosed as having subacute sclerosing panencephalitis (SSPE). This is a progressive and almost uniformly fatal disease. His condition gradually deteriorated till he was unable to spea...

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Autores principales: Nathan, Janak, Khedekar Kale, Dhanashri, Naik, Vidula D, Thakker, Forum, Bailur, Sonal
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6713239/
https://www.ncbi.nlm.nih.gov/pubmed/31489275
http://dx.doi.org/10.7759/cureus.5485
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author Nathan, Janak
Khedekar Kale, Dhanashri
Naik, Vidula D
Thakker, Forum
Bailur, Sonal
author_facet Nathan, Janak
Khedekar Kale, Dhanashri
Naik, Vidula D
Thakker, Forum
Bailur, Sonal
author_sort Nathan, Janak
collection PubMed
description An eight-year-old boy presented with rhythmic myoclonic jerks that stretched back to the age of four years. He was diagnosed as having subacute sclerosing panencephalitis (SSPE). This is a progressive and almost uniformly fatal disease. His condition gradually deteriorated till he was unable to speak or walk. He also experienced incontinence and severe cognitive decline (stage 3a in the Risk and Haddad scale). An electroencephalogram (EEG) showed myoclonic jerks with periodic, generalised, high-amplitude and slow-wave complexes. Cerebrospinal fluid (CSF) findings also were supportive of the diagnosis of SSPE. The ketogenic diet (KD) therapy was started on the patient. His myoclonic jerks stopped after 11 months. After 36 months, his cognition and physical abilities vastly improved. His EEG showed no slow-wave complexes and background activity was almost normal. SSPE is secondary to measles and causes inflammatory and neurodegenerative changes. KD has an anti-inflammatory effect and can halt and reverse neurodegenerative changes. Its neuroprotective effects could be due to the reduced oxidative stress, enhanced mitochondrial activity, and the suppression of pro-apoptotic factors. Thus, KD could control the myoclonic jerks and also reverse the cognitive and physical decline arising from SSPE.
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spelling pubmed-67132392019-09-05 Substantial Remission in Subacute Sclerosing Panencephalitis by Following the Ketogenic Diet: A Case Report Nathan, Janak Khedekar Kale, Dhanashri Naik, Vidula D Thakker, Forum Bailur, Sonal Cureus Neurology An eight-year-old boy presented with rhythmic myoclonic jerks that stretched back to the age of four years. He was diagnosed as having subacute sclerosing panencephalitis (SSPE). This is a progressive and almost uniformly fatal disease. His condition gradually deteriorated till he was unable to speak or walk. He also experienced incontinence and severe cognitive decline (stage 3a in the Risk and Haddad scale). An electroencephalogram (EEG) showed myoclonic jerks with periodic, generalised, high-amplitude and slow-wave complexes. Cerebrospinal fluid (CSF) findings also were supportive of the diagnosis of SSPE. The ketogenic diet (KD) therapy was started on the patient. His myoclonic jerks stopped after 11 months. After 36 months, his cognition and physical abilities vastly improved. His EEG showed no slow-wave complexes and background activity was almost normal. SSPE is secondary to measles and causes inflammatory and neurodegenerative changes. KD has an anti-inflammatory effect and can halt and reverse neurodegenerative changes. Its neuroprotective effects could be due to the reduced oxidative stress, enhanced mitochondrial activity, and the suppression of pro-apoptotic factors. Thus, KD could control the myoclonic jerks and also reverse the cognitive and physical decline arising from SSPE. Cureus 2019-08-25 /pmc/articles/PMC6713239/ /pubmed/31489275 http://dx.doi.org/10.7759/cureus.5485 Text en Copyright © 2019, Nathan et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Neurology
Nathan, Janak
Khedekar Kale, Dhanashri
Naik, Vidula D
Thakker, Forum
Bailur, Sonal
Substantial Remission in Subacute Sclerosing Panencephalitis by Following the Ketogenic Diet: A Case Report
title Substantial Remission in Subacute Sclerosing Panencephalitis by Following the Ketogenic Diet: A Case Report
title_full Substantial Remission in Subacute Sclerosing Panencephalitis by Following the Ketogenic Diet: A Case Report
title_fullStr Substantial Remission in Subacute Sclerosing Panencephalitis by Following the Ketogenic Diet: A Case Report
title_full_unstemmed Substantial Remission in Subacute Sclerosing Panencephalitis by Following the Ketogenic Diet: A Case Report
title_short Substantial Remission in Subacute Sclerosing Panencephalitis by Following the Ketogenic Diet: A Case Report
title_sort substantial remission in subacute sclerosing panencephalitis by following the ketogenic diet: a case report
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6713239/
https://www.ncbi.nlm.nih.gov/pubmed/31489275
http://dx.doi.org/10.7759/cureus.5485
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