- Email: [email protected]
Electroencephalogram changes in celiac disease
Volume 62 Number 3
Brief clinical and laboratory observations
435
Electroencephalogram changes in celiac disease Benjamin Emanuel, M.D., and Allan D. Lieberman, M.D. "~ CHICAGO,
ILL.
W I T I-t the discovery of gliadin as its specific cause, celiac disease, gluten-enteropathy, was added to the growing list of diseases classified as inborn errors of metabolism? It is not surprising, therefore, to find some similarities in the signs and symptoms with those of galactosemia and phenylketonuria, specifically mental and developmental retardation. The psychologic and developmental aspects of celiac disease have received little attention despite the classic behavior and appearance of the patient. The apathy, irritability, and unhappiness is reflected in their facial expression which lacks all joy and pleasure. There is concentration of interest upon themselves with little reaction to external stimuli. Negativism is a key sign of their behavior. 2 Head banging, rocking, and grinding of teeth are common, and, should the disease have its onset in infancy, walking and talking may be delayed? Some think that emotional factors play a large part in the initiation of episodes of diarrhea and in its treatment, a Many writers consider the bizarre behavior and apathy to be primarily psychogenic? In the light of certain observations we have made, we would like to propose an organic basis for the abnormal psyche and behavior observed in the patient with celiac disease.
From the Department o[ Pediatrics, Mt. Sinai Hospital, Chicago, Ill. ~Address, Department of Pediatrics, Mr. 81nai Hospital, Calqornia Avenue at lSth Street, Chicago 8, Ill.
CASE Hx A 2-year-old Negro child was seen by us because of failure to thrive and malnutrition. He was irritable and apathetic, withdrawn, and made very little effort-to communicate. He was very small, falling into the third percentile for height and weight, with a relatively large head, thin, long extremities, atrophic gluteal muscles, and a protuberant abdomen. The stools were bulky, greasy, and foul smelling, and because of his clinical picture, a diagnosis of celiac disease was considered. The diagnosis was confirmed by the abnormal fat content of stool, flat glucose tolerance curve (68 mg. per cent, 116, 84, 81, 71), peculiar pattern of small intestine as revealed by barium roentgen studies, normal sweat electrolytes, absence of giardiasis, negative Mantoux, and clinical improvement with gluten-free diet. The absolute confirmation of the diagnosis was made much later by the histologic picture of the jejunal mucosa examined at autopsy. During the diagnostic work-up, an electroencephalogram was performed and was surprisingly found to be abnormal. There was a very atypical parietal activity during light sleep. There were 7 to 9 sharp waves per second (Fig. 1). The child was placed on a gluten-free diet. The stools became brown, well formed, and lost their offensive odor. He became more aware of his surroundings and made some effort to communicate and play. He was re-evaluated 1 month later after being on a gluten-free diet. He would speak
4 3 6 Brie[ clinical and laboratory observations
March 1963
Fig. 1. First electroencephalogram showing the 7 to 9 sharp waves per second activity in the parietal region during sleep.
a n occasional word, was more alert and playful but still unable to stand alone. Re-examination of the stools for fat content showed a complete return to normal values as did the glucose tolerance cmwe. T h e electroencephalogram was repeated and gave a normal pattern awake and during sleep (Fig. 2). H e was continued on a gluten-free diet and follow-up 3 months later continued to show a normal E E G and stool. We consider these electroencephalographic abnormalities which returned to normal on a gluten-free diet significant evidence of an organic or neurophysiologic basis of the celiac patient's abnorlnal behavior and retardation. T h e similarities with phenylketonuria
Fig. 2. The repeat normal electroencephalogram taken during sleep after child was on a gluten-free diet.
where nearly all patients show electroencephalographic abnormalities s,7 and galactosemia s where about 50 per cent show changes are of interest. We could find no previous reports of electroencephalography in celiac disease and thought this report would stimulate further investigation. After observing the abnormal E E G in our patient, we studied 6 fibrocystic patients and one patient with idiopathic malabsorption disease to see what effect malabsorption per se would have. There were no abl~ormalities in the EEG's in the fibrocystic children. T h e idiopathic malabsorption disease which manifested failure to thrive, hypopro-
Volume 62 Number 3
teinemia, hypoglycemia, and low-serum lipids revealed an a b n o r m a l l y slow E E G . Hypoglycemia classically produces a slow E E G so the significance of the change is questionable. T h a t a metabolic basis can be ascribed to a b n o r m a l b e h a v i o r is of special interest because of its possible reversibility. T h e implication that specific metabolites acting in a defective enzyme system or pathologic process m a y be the cause of some other m e n t a l disorders is challenging. We wish to acknowledge the assistance of Dr. F. Stamps for the reading of the electroencephalograms. REFERENCES
1. Weijers, H. A., and van de Kramer, J. H.: Celiac disease and wheat sensitivity, Pediatrics 25: 127, 1960.
Brie[ clinical and laboratory observations
437
2. Haas, S. V., and Haas, M. P.: Management of celiac disease, Philadelphia, 1951, J. B. Lippincott Company, pp. 37-38, 96. 3. Bakwin, H., and Bakwin, R. M.: Clinical management of behavior disorders in children, Philadelphia, 1960, W. B. Saunders Company, p. 134. 4. Prugh, D. G.: Preliminary report on role of emotional factors in celiac disease, Psychosom. Med. 12" 220, 1951. 5. Haas, S. V., and Hass, M. P.: Management of celiac disease, Philadelphia, 1951, J. B. Lippincott Company, pp. 38 and 98. 6. Low, N. L., Bosma, J. F., and Armstrong, M. D.: EEG studies on phenylketonurla, A. M. A. Arch. Neurol. & Psychlat. 77: 359, 1957. 7. Fois, A., Rosenberg, C., and Gibbs, F, A.: The EEG in phenylpyruvic oligophrenia, Electroencephalography Clinic. Neurophysiol. 7; 569, 1955. 8. Donnell, G. N., Callado, M., and Koch, R.: Growth and development of children with galactosemia, J. P~mAT. 58" 836, 1961.
Brief clinical and laboratory observations
435
Electroencephalogram changes in celiac disease Benjamin Emanuel, M.D., and Allan D. Lieberman, M.D. "~ CHICAGO,
ILL.
W I T I-t the discovery of gliadin as its specific cause, celiac disease, gluten-enteropathy, was added to the growing list of diseases classified as inborn errors of metabolism? It is not surprising, therefore, to find some similarities in the signs and symptoms with those of galactosemia and phenylketonuria, specifically mental and developmental retardation. The psychologic and developmental aspects of celiac disease have received little attention despite the classic behavior and appearance of the patient. The apathy, irritability, and unhappiness is reflected in their facial expression which lacks all joy and pleasure. There is concentration of interest upon themselves with little reaction to external stimuli. Negativism is a key sign of their behavior. 2 Head banging, rocking, and grinding of teeth are common, and, should the disease have its onset in infancy, walking and talking may be delayed? Some think that emotional factors play a large part in the initiation of episodes of diarrhea and in its treatment, a Many writers consider the bizarre behavior and apathy to be primarily psychogenic? In the light of certain observations we have made, we would like to propose an organic basis for the abnormal psyche and behavior observed in the patient with celiac disease.
From the Department o[ Pediatrics, Mt. Sinai Hospital, Chicago, Ill. ~Address, Department of Pediatrics, Mr. 81nai Hospital, Calqornia Avenue at lSth Street, Chicago 8, Ill.
CASE Hx A 2-year-old Negro child was seen by us because of failure to thrive and malnutrition. He was irritable and apathetic, withdrawn, and made very little effort-to communicate. He was very small, falling into the third percentile for height and weight, with a relatively large head, thin, long extremities, atrophic gluteal muscles, and a protuberant abdomen. The stools were bulky, greasy, and foul smelling, and because of his clinical picture, a diagnosis of celiac disease was considered. The diagnosis was confirmed by the abnormal fat content of stool, flat glucose tolerance curve (68 mg. per cent, 116, 84, 81, 71), peculiar pattern of small intestine as revealed by barium roentgen studies, normal sweat electrolytes, absence of giardiasis, negative Mantoux, and clinical improvement with gluten-free diet. The absolute confirmation of the diagnosis was made much later by the histologic picture of the jejunal mucosa examined at autopsy. During the diagnostic work-up, an electroencephalogram was performed and was surprisingly found to be abnormal. There was a very atypical parietal activity during light sleep. There were 7 to 9 sharp waves per second (Fig. 1). The child was placed on a gluten-free diet. The stools became brown, well formed, and lost their offensive odor. He became more aware of his surroundings and made some effort to communicate and play. He was re-evaluated 1 month later after being on a gluten-free diet. He would speak
4 3 6 Brie[ clinical and laboratory observations
March 1963
Fig. 1. First electroencephalogram showing the 7 to 9 sharp waves per second activity in the parietal region during sleep.
a n occasional word, was more alert and playful but still unable to stand alone. Re-examination of the stools for fat content showed a complete return to normal values as did the glucose tolerance cmwe. T h e electroencephalogram was repeated and gave a normal pattern awake and during sleep (Fig. 2). H e was continued on a gluten-free diet and follow-up 3 months later continued to show a normal E E G and stool. We consider these electroencephalographic abnormalities which returned to normal on a gluten-free diet significant evidence of an organic or neurophysiologic basis of the celiac patient's abnorlnal behavior and retardation. T h e similarities with phenylketonuria
Fig. 2. The repeat normal electroencephalogram taken during sleep after child was on a gluten-free diet.
where nearly all patients show electroencephalographic abnormalities s,7 and galactosemia s where about 50 per cent show changes are of interest. We could find no previous reports of electroencephalography in celiac disease and thought this report would stimulate further investigation. After observing the abnormal E E G in our patient, we studied 6 fibrocystic patients and one patient with idiopathic malabsorption disease to see what effect malabsorption per se would have. There were no abl~ormalities in the EEG's in the fibrocystic children. T h e idiopathic malabsorption disease which manifested failure to thrive, hypopro-
Volume 62 Number 3
teinemia, hypoglycemia, and low-serum lipids revealed an a b n o r m a l l y slow E E G . Hypoglycemia classically produces a slow E E G so the significance of the change is questionable. T h a t a metabolic basis can be ascribed to a b n o r m a l b e h a v i o r is of special interest because of its possible reversibility. T h e implication that specific metabolites acting in a defective enzyme system or pathologic process m a y be the cause of some other m e n t a l disorders is challenging. We wish to acknowledge the assistance of Dr. F. Stamps for the reading of the electroencephalograms. REFERENCES
1. Weijers, H. A., and van de Kramer, J. H.: Celiac disease and wheat sensitivity, Pediatrics 25: 127, 1960.
Brie[ clinical and laboratory observations
437
2. Haas, S. V., and Haas, M. P.: Management of celiac disease, Philadelphia, 1951, J. B. Lippincott Company, pp. 37-38, 96. 3. Bakwin, H., and Bakwin, R. M.: Clinical management of behavior disorders in children, Philadelphia, 1960, W. B. Saunders Company, p. 134. 4. Prugh, D. G.: Preliminary report on role of emotional factors in celiac disease, Psychosom. Med. 12" 220, 1951. 5. Haas, S. V., and Hass, M. P.: Management of celiac disease, Philadelphia, 1951, J. B. Lippincott Company, pp. 38 and 98. 6. Low, N. L., Bosma, J. F., and Armstrong, M. D.: EEG studies on phenylketonurla, A. M. A. Arch. Neurol. & Psychlat. 77: 359, 1957. 7. Fois, A., Rosenberg, C., and Gibbs, F, A.: The EEG in phenylpyruvic oligophrenia, Electroencephalography Clinic. Neurophysiol. 7; 569, 1955. 8. Donnell, G. N., Callado, M., and Koch, R.: Growth and development of children with galactosemia, J. P~mAT. 58" 836, 1961.