“A 3-Year-Old Mute Girl Miraculously Starts Talking After Cream Cheese Diet.”  This headline appeared in the news for several days in July, 2013 (1).  It is a true story about a little girl who lives in England. It is also an excellent starting point for a discussion of the ketogenic diet.

The little girl had been mute from birth.  When she was 15 weeks old, she had a seizure and doctors diagnosed her with epilepsy.  When she was 3 years old, doctors found she had an incurable disease called glucose transporter type 1 deficiency disease (GLUT 1-DS).  GLUT-1 is the name of an enzyme that transfers glucose across the blood-brain barrier from the blood to the brain.  Glucose normally provides all of the brain’s energy.  When GLUT-1 is not working glucose is unable to enter the brain, and when the brain gets no energy, the brain does not function.  This is why the little girl could not speak.

The Ketogenic Diet

Once the doctors discovered it was a GLUT-1 problem, they put the girl on a ketogenic diet; a high-fat, low carbohydrate, adequate protein diet.  Ketogenic diets promote the biosynthesis of ketone bodies.  Ketone bodies are chemicals that were designed by natural evolution to provide energy to the brain when blood glucose was extremely low. Ketone bodies can cross the blood-brain barrier without the help of GLUT-1.

From what has been written about this little girl, it is not apparent why her doctors selected cream cheese for her ketogenic diet because there seems to be no evidence in the medical literature that cream cheese alone has ever been used for this purpose.  In any event, the doctors made an excellent decision.  Cream cheese is simple to use and in no way harmful.  It is composed totally of dairy products which have been known for a long time to enhance the effectiveness of ketogenic diets.  Cream cheese is a ketogenic because it contains ample fat, very little carbohydrate, and protein.  The little girl was free to eat as much as she wanted and consumed two pounds of this cheese per week.  She did not gain weight.

The cream cheese alone was effective:  The little girl’s surprised mother said that within a few weeks she noticed improvement.  Her daughter was more alert and her personality began to show.  She went on to say, “after eating four tubs of cream cheese a week for three months, my baby said her first word: ‘Mum’.”  Her mother also said her little girl loved the cream cheese diet from the very first day.  The fact that she loved eating the cream cheese makes one wonder if the little girl had a sixth sense that told her that cream cheese was the kind of food that she needed.

This child’s dysfunctional GLUT-1 enzyme is a genetic problem.  She was born with it and this problem prevented energy-containing glucose from reaching her brain. Thus, she could not speak because her brain was not functioning properly. The cream cheese diet enabled her biochemistry to synthesize ketone bodies that can cross the blood-brain barrier and supply the brain with energy without the need for the GLUT-1 enzyme.

Ketone Bodies and Ketosis

At this point, we move on beyond the malfunctioning GLUT-1 enzyme because the ketogenic diet has shown promise as a safe and effective way to treat other diseases. Ketone bodies are chemical molecules.  Some years ago, scientists believed there were three different ketone body molecules, acetone, acetoacetate, and beta-hydroxybutyrate.  Today, the consensus is there is only one, beta-hydroxybutyrate.  Acetoacetate is now considered a precursor of beta-hydroxybutyrate, and acetone is a volatile ketogenic waste product that is eliminated via the lungs and urine (2).

Humans biosynthesize small amounts of ketone bodies under most conditions.  However, the production of ketone bodies increases during starvation or with a diet that is extremely rich in fats and low in carbohydrates (2).  Ketosis is a state where ketone bodies are being biosynthesized in quantity, circulating in the bloodstream, and measurable in the blood.  A ketogenic diet is a diet that induces ketosis.

The Role of Evolution

The ability to synthesize ketone bodies was built into human biochemistry eons ago to increase human resistance to starvation.  Primitive man had no certain sources of food and regularly faced periods of starvation.  Man’s large brain used 60% of his total body’s glucose requirements and because fats could not cross the blood-brain barrier, glucose was the sole energy source for man’s brain (1). During periods of starvation, the human brain quickly used up the body’s available glucose, and when this glucose was gone, man’s brain slowed or stopped working.  This condition was probably fatal for primitive man.

Scientists who have studied this issue conclude that perhaps a half-million years ago, evolutionary pressures altered man’s biochemistry so that it extended man’s starvation survival time from about 2 weeks to 2 months (3).  This evolutionary process changed human biochemistry in two ways: The body learned to convert fat into ketone bodies and the brain learned to use these ketone bodies for energy when blood glucose levels were very low (a state of imminent starvation).  It is important to remember that this modified human biochemistry has not changed. Today, it remains as a part of our biochemistry. We, like our primitive ancestors, are able to synthesize ketone bodies and our brains are able to use these ketone bodies for energy.

Apprehension about Ketosis

Physicians are taught to be seriously concerned about potentially fatal diabetic ketoacidosis, which is often mistakenly called ketosis.  Ketosis is safe and healthful. Diabetic ketoacidosis is a serious medical emergency.  It occurs only in people with type-1 diabetes.  Ketoacidosis can be fatal because individuals with type-1 diabetes, unlike normal people, cannot biosynthesize their own insulin. The failure to inject the proper amount insulin into a patient when it is needed causes the body to actively synthesize enormous amounts of ketone bodies that can cause a fatal acidosis if not promptly treated.  Absent type-1 diabetes, ketosis is a natural biochemical process that shows evidence of having positive therapeutic effects.

Early Medical Research

Ancient doctors knew that fasting was healthful and sometimes cured seizures and other ailments.  No further progress was made until about 1920 when medical researchers discovered that a low carbohydrate, high fat (ketogenic) diet benefited children with epilepsy.  This diet, with variations and improvements is still being used to treat children and adults with epilepsy and other seizures that cannot be treated with prescription drugs (4).

Ketogenic Diets

Table 1, Common Ketogenic Diets (below), lists seven ketogenic diets that have been used to treat diseases, primarily epilepsy (5).  In this table, each diet is described in terms of both grams and calories of fat, protein and carbohydrate.  When planning the use of these diets, the daily protein requirement is first estimated.  The accepted protein standard in grams per day is one half of the patient’s lean body weight in pounds.  For example, a person with a lean body weight of 140 pounds would have a daily protein need of 70 grams.  Doctors keep this protein need in mind when scaling up the daily diets shown in Table 1.  The following conversion factors will be helpful in understanding how calories relate to grams in Table 1:  One gram of fat is 9 calories, one gram of protein is 4 calories, and one gram of carbohydrates is 4 calories.

The “Classic 4 to 1” Diet

The medical literature indicates that the first successful ketogenic diet was developed in the 1920’s to treat children with epilepsy.  This diet cured about 20% of the young patients and benefited up to about 50% of them.

The diet was a 4 to 1 ratio of fat to carbohydrate plus protein.  It employed the commonly used fats of the time; animal fats, olive oil, butter and perhaps coconut oil.  It included enough protein for body growth and repair, contained minimal amounts of carbohydrate, and provided enough calories to maintain correct weight.  It was costly because it was administered in hospital settings, required close monitoring by physicians and dieticians, and was often preceded by a day or two of fasting.

When anti-epileptic drugs became available in the 1930’s, interest in ketogenic diets vanished.  In about 1991, a voluntary public interest organization, The Charlie Foundation, sparked a new medical interest in ketogenic dietary treatments for epilepsy and other seizures in children.  All of the diets listed in Table 1, below, except the Classic 4-1 Diet, were developed after 1991.

Table 1: Common Ketogenic Diets

Type of Diet	Fat, grams (% calories)	Protein, grams (% calories))	Carbohydrates, grams (% calories)
Classic 4 to 1 (common fats)	100 (90%)	17 (7%)	8 (3%)
Classic 4 to 1 (with milk fat)	78 (70%)	25 (10%)	30 (20%)
Modified Atkins	70 (70%)	60 (25%)	15 (5%)
Low Glycemic Index Diet	67 (66%)	50 (17%)	50 (17%)
Sears low carb (keto diet)	100 (60%)	125 (33%)	33 (9%)
Sears low carb (non keto diet)	50 (30%)	117 (30%)	157 (40%)
Cream Cheese Diet	72 (70%)	16 (15%)	16 (15%)

Classic Ketogenic Diet with Milk Fat

This diet is reported to be more effective and easier on the patients than the Classic 4 to 1 Diet that employed long chain fats, primarily lard, tallow, and olive oil. It is now known that milk fats are easier to digest and contain a series of beneficial medium chain fats.  The addition of milk fats to the ketogenic diet was an important step forward.

The Modified Atkins Diet

After 1990, the activities of the Charlie Foundation spurred interest in making the ketogenic diet less expensive and easier to use.  The Modified Atkins Diet was the result.  It uses regular foods that are commonly available and it is ketogenic.  There is no fasting and no requirement for a hospital setting when using this diet.  The need for supervision by doctors and dieticians is minimal depending on the abilities of the families involved.  Doctors that have utilized this diet believe it is more effective than the older diets because it costs less, needs little professional supervision, can be done at home, and uses foods that are commonly available.

The side effects associated with the Modified Atkins Diet are reported to be constipation, weight loss, and vitamin and mineral deficiencies.  Hypercholesterolemia (high cholesterol in the blood) and its associated cardiovascular diseases have also been considered to be risky side effects of ketogenic diets because of their high dietary levels of animal fats and cholesterol.  Even though this fear is not supported by scientific facts, governmental agencies and nearly the entire medical establishment believe this myth is true.  The biochemical facts however, are as follows:   The immediate high blood insulin levels caused by the dietary sugars and starches convert excess dietary sugar and starch directly to body fat and cholesterol.    Concurrently, the high blood insulin levels stimulate the biosyntheses of inflammatory eicosanoids (messenger biochemicals) that are the underlying cause cardiovascular diseases.  In short, dietary fat and cholesterol do not cause cardiovascular diseases as believed by the medical establishment.  Dietary sugar and starch cause both the high blood cholesterol and the cardiovascular diseases.

Modified Atkins Diet Study in Japan

In 2011, a Japanese research study tested the effectiveness of the Modified Atkins Diet as a treatment for GLUT 1-DS, glucose transporter type 1 deficiency disease (6).  The researchers selected six males with GLUT 1-DS.  All participants had early onset epilepsy.  Their ages ranged 7 to 16 years and the duration of the treatments required from 1 to 42 months to achieve successful results.  During treatment all participants showed significant urinary levels of ketone bodies.  Seizures decreased and cognitive function came back in all individuals.  There were no significant side effects.

This study (6) brought forth two important conclusions:  The Modified Atkins Diet is an effective treatment for GLUT 1-DS.  And dietary treatment, when extended over long enough periods, benefited all of the GLUT 1-DS patients in the study group.

Low Glycemic Index Diet

The Low Glycemic Index Diet was developed in 2002 as an alternative to the Classic 4 to 1 diet for the long-term treatment of intractable epilepsy (7).   The Classic 4 to 1 Diet was an effective treatment for epilepsy, but physician and hospital costs were unreasonably high.  The Low Glycemic Index Diet was far less costly.  It was administered at home, food quantities were estimated rather than measured, and reasonable amounts of low glycemic carbohydrates were used.  This diet is not considered to be ketogenic.

The Low Glycemic Index Diet was found to be effective for long-term use.  Seizures were reduced in a majority of patients; some were cured and many were able to reduce their use of anticonvulsant prescription medications.  Side effects were weight loss and mild acidosis.  The weight loss was considered beneficial.  The acidosis was controlled by supplementation with a bicarbonate solution with no effect on treatment efficiency (7).

The Sears Diet

In 2006, Barry Sears and co-investigators (8) carried out the first human feeding study that compared a ketogenic diet (KLC) to a low glycemic carbohydrate diet (NLC) in human population groups.  In this study, twenty obese adults with body mass indices averaging 34.4 were randomly divided into two groups of ten individuals each.  One group (KLC) was fed a ketogenic diet and the other group was fed a healthful non-ketogenic diet (NLC).  The KLC diet was 60% fat calories, 35% protein calories, and 5% carbohydrate calories.  The NLC diet was 30% fat calories, 30% protein calories, and 40% low glycemic carbohydrate calories.  This study was continued for six-weeks.  Participants were sedentary and 24-hour food intakes were strictly controlled.

Nutritional values and biological effects were documented.  Food analyses for each group showed that calorie intakes in both groups were the same, 1500 calories per day.  Except for the vitamin, niacin, the dietary intakes of vitamins and minerals were significantly higher in the NLC group.  Incidentally, the NLC diet appears to be the same as the diet recommended by Barry Sears in his 1995 book, Enter the Zone.

Laboratory Test Results

Laboratory test results at the end of the six-week study period were as follows:  BMI dropped by 2.4 in KLC and 2.7 in NLC.  AA (arachidonic acid) to EPA (eicosapentaenoic acid) ratios increased from 21.4 to 39.2 in KLC and decreased from 23.8 to 20.9 in NLC group.  This is significant because an increase in the AA to EPA ratio signifies an increase in whole body inflammation and predicts increased risk of cardiovascular diseases and other inflammatory diseases.

The amounts of ketone bodies created by each diet were determined by measuring beta-hydroxybutyrate in blood samples.  The KLC diet brought about an increase of beta-hydroxybutyrate in blood of  0.244 mmol/liter.  In the NLC diet group, beta-hydroxybutyrate increased by 0.10 mmol/liter.  These numbers mean that the KLC diet produced more ketone bodies than the NLC diet.  According to the authors, this difference was not particularly important, because both diets produced meaningful amounts of ketone bodies.

Insulin resistance, a measure of the risk of type-2 diabetes, decreased in both groups.  Hunger ratings in both groups remained the same and were considered satisfactory by the study participants.  Feelings of vigor however were significantly better in the NLC group.

The study’s authors reached the following conclusions:  Both diets were equally effective in reducing body weight and insulin resistance.  The KLC diet was associated with adverse metabolic emotional effects.  The use of the ketogenic KLC diet for weight loss is not warranted, because the NLC Diet was more effective.  In dieting aimed at weight loss, patients should know that there is no apparent metabolic advantage associated with ketosis.

The Cream Cheese Diet

There appears to be no record in the scientific literature indicating that cream cheese alone has ever before been used as a ketogenic diet.  Apparently, “the little girl” is the first patient known to use cream cheese as the sole component of a ketogenic diet.

All of the cream cheese sold in the USA is manufactured in accordance with a national standard.  It is made from dairy products only.  An 8 ounce package weighs 85 grams and contains 72 grams of milk fats, 16 grams of protein, and 16 grams of carbohydrate.

See Table 1, above, to compare the cream cheese diet with other ketogenic diets.  Note that protein intake may be somewhat low at 15% of calories.  The recommended level for normal people is about 30% of calories.  In fact the cream cheese diet does not resemble any of the diets in Table 1.  In any event, this diet was effective, appetizing, and simple to use.  It was also free of harmful side effects in the case of the “little girl.”

Special Benefits of Cream Cheese

Cream cheese is an unusually healthy food because it is rich in beneficial fats and contains essentially no harmful high glycemic carbohydrates and no damaging vegetable fats or oils.  Widely used high glycemic carbohydrates in America are sugar, high fructose corn syrup, starches, grains and grain products such as cereals, bread, and pasta.  The vegetable fats and oils are also widely used.  All are made from vegetable seeds, namely soy, corn, canola, peanut, safflower, and sunflower.  All of these fats and oils are rich in pro-inflammatory omega-6 fatty acids.

These two damaging food groups were not a part of the human evolutionary diet and therefore are foreign to human biochemistry.  The high glycemic carbohydrates increase blood insulin levels, which, in turn, stimulate a desaturase enzyme (delta 5 desaturase).  When stimulated, this enzyme increases the production inflammatory eicosanoids, messenger biochemicals that cause inflammation throughout the body.

Importantly, the omega-6 vegetable fats and oils also stimulate the delta 5 desaturase enzyme and add to the production of inflammatory eicosanoids.  The result is an increase in long-term whole body inflammation that is now known to be the underlying cause of the great epidemics of inflammatory diseases that plague this country.  The most common and most costly are cardiovascular diseases, cancers, type-2 diabetes, obesity, Alzheimer’s and Parkinson’s diseases, and a growing newcomer, now the third major cause of death in the USA, suicide (9).

Editorial Comments

When thinking about ketogenic diets, recall that “the little girl” was a victim of glucose transporter type 1 deficiency disease (GLUT 1-DS) caused by malfunctioning GLUT 1 enzymes.   The objective of a diet in this case must be to produce ketone bodies reliably to take the place of glucose as an energy source for the brain.  It is not clear if GLUT 1-DS can be cured by the long-term use a ketogenic diet.  However, the Japanese study mentioned above suggests that this may be possible.

On the other hand, recall that epilepsy and other seizures apparently do not have a GLUT-1 deficiency. In these cases a non-ketogenic diet, such as the Low Glycemic Index Diet, mentioned above is effective against epilepsy and some seizures.  This tells us that a strict ketogenic diet may not be necessary to treat epilepsy and related seizures.  And the study by Sears suggests that such a diet is easier to manage and has essentially no unwanted side effects.

The history of ketogenic diet suggests that more thought must be given to the need to supplement diets with vitamins, minerals, and omega-3 fatty acids.  Vitamin C (ascorbic acid) should always be considered a necessary supplement because is required to support the immune system.  A relevant example is the use of cream cheese as a diet.  It is made from pasteurized milk and pasteurization destroys vitamin C.   This means that a cream cheese diet must be supplement with vitamin C.  With regard to minerals, magnesium is biochemically essential to control constipation.  And, seriously important, long chain omega-3 fatty acids are essential for brain and nervous system construction and maintenance.  It seems unreasonable not to include omega -3 fatty acids supplementation especially when planning ketogenic diets for diseases that involve brain and nerve functions.

Closing Thought

Ketogenic diets resemble the uncertain human evolutionary diet that forced primitive humans to move in and out of ketosis over their entire lives.  Archeologists have shown that these ancient people were extremely healthy.  Yet, there were no doctors, no medicine, no surgery, no heart disease, no obesity, no toothpaste, no dentists, and no cavities.

References

1. NY Daily News. Little girl’s heavy cream cheese-heavy diet helps her speak her first words, 8-15-2013. http://www.nydailynews.com/life-style/health/girl-3-finally-speaks/  Downloaded August 16, 2013
2. Brandt, M. The Synthesis and Utilization of Ketone bodies. Copyright © 2000-2003 Mark Brandt, Ph.D, Professor of Biochemistry, Rose-Hulman Institute of Technology, Terra Haute, IN.  http://www.rose-hulman.edu/-brandt/chem330/ketone.bodies pdf.  Accessed August 26, 2013.
3. Veech RL, Chance B, Kashiwaya Y, Lardy, Henry A. 4 and Cahill G F Jr. Ketone Bodies, Potential Therapeutic Uses. IUBMB Life, 51: 241–247, 2001.
4. Freeman JM, Kossoff EH, Hartman AL. The Ketogenic Diet: One Decade Later DOI: 10.1542/peds. 2006-2447 Pediatrics 2007;119;535.
5. Kossoff EH, et al. Dietary Therapies for Epilepsy.  Biomed J. 2013;36:2-8.
6. Oguni, IY, Ito S, Oguni M, Osawa M.  A modified Atkins diet is promising as a treatment for glucose transporter type 1 deficiency syndrome. Dev Med Child Neurol. 2011 Jul;53(7):658-63.
7. Epilepsy, com/. The Low Glycemic Index Treatment and the Ketogenic Diet. http://www.epilepsy.com/epilepsy/keto_news_may07?print=true#, Accessed on August 22, 2013.
8. Johnston CS, Tjonn SL, Swan PD, White A, Hutchins H, Sears B.  Ketogenic low-carbohydrate diets have no metabolic advantage over non-ketogenic low-carbohydrate diets, Am J Clin Nutr 2006;83: 1055–61.
9. Ottoboni A, Ottoboni F.  The Modern Nutritional Diseases, Vincente Books, Fernly, NV, 2013.