D‐lactic acidosis simulating a hypothalamic syndrome after bowel bypass

Abstract

A 36-year-old man had one year of periodic symptoms suggestive of episodic hypothalamic dysfunction: hypersomnia, thirst, ravenous hunger and gorging behavior, pallor, and irritability. However, neuroendocrine testing proved normal. A mild transient acidosis at the onset of his attacks and a history of bowel bypass five years earlier prompted metabolic screening. Markedly increased urinary D-lactic and phenolic acids were present, as were intermittent elevations of plasma D-lactic acid during two symptomatic episodes. Prompt and sustained clinical remission coincided with disappearance of abnormal organic acid excretion during oral antibiotic therapy. D-Lactic acidosis must be considered in the differential diagnosis of otherwise unexplained neurological syndromes, particularly in patients with altered bowel anatomy.

Examining clinical similarities between myalgic encephalomyelitis/chronic fatigue syndrome and d-lactic acidosis: a systematic review

“…Within d -la, several authors have proposed that the level of acidosis and associated encephalopathy may result in differing severity and either an acute or chronic presentation [27, 28, 32]. A subclinical elevation of d -lactate has been reported in SBS patients and diverse populations [94].…”

Examining clinical similarities between myalgic encephalomyelitis/chronic fatigue syndrome and d-lactic acidosis: a systematic review

“…Within d -la, several authors have proposed that the level of acidosis and associated encephalopathy may result in differing severity and either an acute or chronic presentation [27, 28, 32]. A subclinical elevation of d -lactate has been reported in SBS patients and diverse populations [94].…”

d‐Lactic Acidosis in Calves as a Consequence of Experimentally Induced Ruminal Acidosis

“…As d ‐lactate does cross the blood–brain barrier it can also accumulate in the brain, where it can cause central nervous system toxicity; encephalopathic manifestations in humans, mostly characterized by confusion, ataxia, weakness, lethargy and slurred speech, have in fact been clearly correlated with d ‐lactic acidosis (Carr et al., 1982; Dahlquist et al., 1984; Hudson et al., 1990; Koletzko et al., 1994). Data are required to determine the threshold level of d ‐lactate necessary to produce these symptoms.…”

53-years-old with mental status changes

“…13 The neurological symptoms include: changes in mental status, 4 12 or even an hypothalamic-like syndrome with hypersomnia, thirst, ravenous hunger and gorging behavior, pallor and irritability. 18 Neurological examination may be completely normal, 12,26,27 or may show nystagmus, ataxia and impaired coordination without any focal neurological deficit. 1,14 The duration and frequency of symptoms vary, and spontaneous resolution often occurs.…”

“…Rehydration is also advisable and dietary modifications including a low carbohydrate diet can be used, since it decreases substrate available for bacterial fermentation. 18 Even use of starch polymers, which are slowly metabolized, can help. Mayne et al 24 reported that strict control of simple and oligosaccharides, and use of polymeric carbohydrates is successful in treating severe recurrent acidosis which had not improved with the use of oral nonabsorbable antibiotics.…”

Examining clinical similarities between myalgic encephalomyelitis/chronic fatigue syndrome and d-lactic acidosis: a systematic review

“…Within d -la, several authors have proposed that the level of acidosis and associated encephalopathy may result in differing severity and either an acute or chronic presentation [27, 28, 32]. A subclinical elevation of d -lactate has been reported in SBS patients and diverse populations [94].…”

d‐Lactic Acidosis in Calves as a Consequence of Experimentally Induced Ruminal Acidosis

“…As d ‐lactate does cross the blood–brain barrier it can also accumulate in the brain, where it can cause central nervous system toxicity; encephalopathic manifestations in humans, mostly characterized by confusion, ataxia, weakness, lethargy and slurred speech, have in fact been clearly correlated with d ‐lactic acidosis (Carr et al., 1982; Dahlquist et al., 1984; Hudson et al., 1990; Koletzko et al., 1994). Data are required to determine the threshold level of d ‐lactate necessary to produce these symptoms.…”

53-years-old with mental status changes

“…13 The neurological symptoms include: changes in mental status, 4 12 or even an hypothalamic-like syndrome with hypersomnia, thirst, ravenous hunger and gorging behavior, pallor and irritability. 18 Neurological examination may be completely normal, 12,26,27 or may show nystagmus, ataxia and impaired coordination without any focal neurological deficit. 1,14 The duration and frequency of symptoms vary, and spontaneous resolution often occurs.…”

“…Rehydration is also advisable and dietary modifications including a low carbohydrate diet can be used, since it decreases substrate available for bacterial fermentation. 18 Even use of starch polymers, which are slowly metabolized, can help. Mayne et al 24 reported that strict control of simple and oligosaccharides, and use of polymeric carbohydrates is successful in treating severe recurrent acidosis which had not improved with the use of oral nonabsorbable antibiotics.…”

Examining clinical similarities between myalgic encephalomyelitis/chronic fatigue syndrome and d-lactic acidosis: a systematic review

“…Within d -la, several authors have proposed that the level of acidosis and associated encephalopathy may result in differing severity and either an acute or chronic presentation [27, 28, 32]. A subclinical elevation of d -lactate has been reported in SBS patients and diverse populations [94].…”

d‐Lactic Acidosis in Calves as a Consequence of Experimentally Induced Ruminal Acidosis

“…As d ‐lactate does cross the blood–brain barrier it can also accumulate in the brain, where it can cause central nervous system toxicity; encephalopathic manifestations in humans, mostly characterized by confusion, ataxia, weakness, lethargy and slurred speech, have in fact been clearly correlated with d ‐lactic acidosis (Carr et al., 1982; Dahlquist et al., 1984; Hudson et al., 1990; Koletzko et al., 1994). Data are required to determine the threshold level of d ‐lactate necessary to produce these symptoms.…”

53-years-old with mental status changes

“…13 The neurological symptoms include: changes in mental status, 4 12 or even an hypothalamic-like syndrome with hypersomnia, thirst, ravenous hunger and gorging behavior, pallor and irritability. 18 Neurological examination may be completely normal, 12,26,27 or may show nystagmus, ataxia and impaired coordination without any focal neurological deficit. 1,14 The duration and frequency of symptoms vary, and spontaneous resolution often occurs.…”

“…Rehydration is also advisable and dietary modifications including a low carbohydrate diet can be used, since it decreases substrate available for bacterial fermentation. 18 Even use of starch polymers, which are slowly metabolized, can help. Mayne et al 24 reported that strict control of simple and oligosaccharides, and use of polymeric carbohydrates is successful in treating severe recurrent acidosis which had not improved with the use of oral nonabsorbable antibiotics.…”