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Published on: June 21, 2016
Substantial Remission in Subacute Sclerosing Panencephalitis by Following the Ketogenic Diet: A Case Report
Janak Nathan1, Dhanashri Khedekar Kale2, Vidula D Naik2
1Neurology, Shushrusha Hospital, Mumbai, IND.
Abstract:
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.
Insights
Subacute sclerosing panencephalitis (SSPE) is a fatal disease. Ketogenic diet (KD) therapy halted myoclonic jerks and reversed cognitive and physical decline in an SSPE patient.
Area of Science:
- Neurology
- Pediatrics
- Metabolic Therapies
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, and fatal neurological disease caused by measles virus infection.
- SSPE leads to severe neurodegeneration, cognitive decline, and motor deficits, with limited treatment options.
Observation:
- An eight-year-old boy with SSPE presented with severe myoclonic jerks, cognitive impairment, and motor deterioration.
- Electroencephalogram (EEG) revealed characteristic periodic, high-amplitude, slow-wave complexes associated with myoclonic jerks.
Findings:
- Ketogenic diet (KD) therapy was initiated, leading to the cessation of myoclonic jerks within 11 months.
- Remarkable improvements in cognition and physical abilities were observed after 36 months of KD therapy, with normalized EEG findings.
Implications:
- KD demonstrates significant anti-inflammatory and neuroprotective effects, potentially halting and reversing SSPE-related neurodegeneration.
- This case suggests KD as a promising therapeutic strategy for managing SSPE, offering hope for improved patient outcomes.
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