118 American Journal of Medical Case Reports
milk, dairy, refined carbohydrates, cereals, legumes,
maize, rice and most vegetable oils. Thus the diet was
based on vegetables, fruits, meat, eggs but also contained
oilseeds, coconut oil, sugar alcohols and coconut. This
diet was however low in animal fat, red meats and offal.
Amount of fat, protein and carbohydrates were not
predefined in the popular paleolithic diet. She had been
following the diet for 20 months between Nov 2010 and
A laboratory test on 09 Nov 2011, a year after diet
onset, indicated a decrease in the level of both total and
direct bilirubin. Iron level was now in the normal range
and other parameters were normal too (Table 1).
Yellowish decoloration of the sclera disappeared. There
was a decrease in the number of migraine episodes (~6
episodes/year) and constipation resolved too. However
there was no change in feeling fatigue and in the presence
of granulomatosus dermatitis. Another concern was
weight loss. While on the popular paleolithic diet she lost
5 kilograms and so her BMI at this time was only 16.1.
2.2. Paleolithic Ketogenic Diet
In July 2012 we adviced a shift toward the paleolithic
ketogenic diet. This diet is based on animal fat, meat, eggs
and offal and to a lesser extent (less than 30%) vegetables
and fruits. Fat to protein ratio was at least 2:1 (in gram).
Fat and red meats derived from pork and cattle were
encouraged over lean meats from poultry. She consumed
offal from pork and cattle (predominantly liver, brain and
marrow) at least two times a week. She was avoiding
foods with additives including nitrites and/or nitrates.
Foods that are allowed or even encouraged in the popular
paleolithic diet such as artificial sweeteners, coconut oil,
oilseeds, oilseed flours and cocoa were excluded. She used
small amounts of honey. Ketosis was checked regularly by
urinary keton strips which showed sustained ketosis. The
four laboratory tests taken five months, 12 months, 19
months and 31 months after the onset of the paleolithic
ketogenic diet showed total bilirubin and direct bilirubin
levels below the upper reference limit (Figure 1). In these
measurements cholesterol and LDL cholesterol were
elevated but other laboratory measures were normal.
Testing for folic acid, vitamin B12 and vitamin D
(25(OH)D) in years 2014 and 2015 showed adequate
levels of these vitamins (Table 1).
Figure 1. Time course of total bilirubin levels through the normal, the
popular paleolithic and the paleolithic ketogenic diet. Note that with a
shift toward the popular paleolithic and then toward the paleolithic
ketogenic diet bilirubin levels fall below the upper reference limit
While on the paleolithic ketogenic diet her fatigue
disappeared and she experienced increased fittness both
physically and mentally. Her migraine episodes further
decreased (to ~2/year). Granulomatosus dermatitis
disappeared on both legs. Her weight was increased by 4
kilograms. Currently her BMI is 17.6. She reports no side
effects of the diet.
GS is regarded as a lifelong condition of altered
bilirubin metabolism . In our patient, however, clinical
features designating this condition have been reversed by
shifting first toward the paleolithic then toward the
paleolithic ketogenic diet. During this time serum level of
both total and directbilirubin declined below the upper
limit of the normal range. Although bilirubin levels
decreased and yellowish decoloration of the sclera
disappeared while on the popular paleolithic diet,
weakness, fatigue, migraine and granulomatosus
dermatitis improved considerably only after the shift
toward the paleolithic ketogenic diet.
While on the paleolithic ketogenic diet a laboratory
assessement indicated low level of inflammatory markers
(CRP, fibrinogen), normal level of triglicerides, uric acid,
glucose, ions, normal liver and kidney function. Total
cholesterol as well as LDL cholesterol were elevated.
Such a laboratory profile corresponds to that seen in our
previous patients with epilepsy , type 1 diabetes  and
metabolic syndrome  on the paleolithic ketogenic diet.
Supplementing vitamins on the classical ketogenic diet is
generally recommended. In the present case, however,
despite the absence of supplementing, vitamin D, folic
acid and vitamin B12, as assessed by laboratory
measurements in 2014 and 2015, were in the normal range.
Physicians generally opine that a metabolic condition
with a perceived genetic predisposition such as the GS
cannot be influenced by diet. We are not aware of studies
using dietary intervention is GS. However fasting and
glucose administration both orally and intravenously are
known to elevate bilirubin levels in GS patients [12,13].
Interestingly in a study carried out 40 years ago both
phenomena could be reversed by the addition of lipids
. Then it was concluded that both phenomena are due
to the withdrawal of lipids.
Current dietary guidelines recommend the reduction of
fat and especially saturated fat in the diet . However
there is growing evidence that the recommendation on
high carbohydrate/low fat diet may not be supported with
sufficient evidence  while carbohydrate-restricted
ketogenic diets have been shown to confer several health
benefits . This was also the case in our patient with
GS and in the three other cases on the paleolithic
ketogenic diet [7,8,9].
It is of important to emphasize that unlike the classical
ketogenic diet which is known to be associated with
several adverse effects the paleolithic ketogenic diet does
not have any side effects as also examplified our present
and previous patients [7,8,9]. A second important point is
that although in this case there were some improvements
onthe popular paleolithic diet, the remaining symptoms
were only resolved with the paleolithic ketogenic diet.