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The effects of thiamine on abnormal brainstem auditory evoked potentials
Abstract
Two infants with recurrent episodes of life-threatening apnea had abnormal BAEP test results. One of them received an intravenous injection of thiamine tetrahydrofurfuryl disulfide (TTFD) and BAEP test results improved greatly within 15 minutes. No further treatment was given and 5 days later the test results were again grossly abnormal. The infant was then given TTFD by mouth and sequential BAEP testing revealed slow steady improvement. The second infant had similarly abnormal BAEP test results and was treated with watersoluble thiamine hydrochloride. The BAEP returned to normal within one month.
... Of perhaps far greater importance, it must be emphasized that this test will only demonstrate erythrocyte deficiency of TPP and gives no information at all on the activity of thiamin triphosphate. In our own studies we have found at least one threatened SIDS infant with abnormal thiamin pyrophosphate percentage effect (4) who clearly showed erythrocyte deficiency of TPP, and two infants who excreted a factor in their urines which has been reported to inhibit the phosphotransferase reaction which forms thiamin triphosphate in brain (4,5). The studies by Davis et al. (6) have shown that concentrations of thiamin are higher in SIDS infants than are found in age-matched controls and Davis has suggested that this excess of thiamin was in a biologically inactive form (R. E. Davis, personal communication). ...
... Dr. Lonsdale makes some interesting observations including a cautionary comment these reasons that the conclusions of Peterson et al. (I) should not yet be accepted as a deterrent to the ideas promoted by Read (7) or by others who have evidence that thiamin may well playa vital role in the SIDS story (4,5,8,9). 1) The authors have monitored the growth of preschool children for 28 weeks as follows: a) 125 children who remained uninfected throughout (control group); b) 61 children who had ascariasis during first 14 wk and were free of infection during the second 14 wk. (They, as well as the children in the control group, were administered levamisole at 14 wk.) ...
... Of perhaps far greater importance, it must be emphasized that this test will only demonstrate erythrocyte deficiency of TPP and gives no information at all on the activity of thiamin triphosphate. In our own studies we have found at least one threatened SIDS infant with abnormal thiamin pyrophosphate percentage effect (4) who clearly showed erythrocyte deficiency of TPP, and two infants who excreted a factor in their urines which has been reported to inhibit the phosphotransferase reaction which forms thiamin triphosphate in brain (4,5). The studies by Davis et al. (6) have shown that concentrations of thiamin are higher in SIDS infants than are found in age-matched controls and Davis has suggested that this excess of thiamin was in a biologically inactive form (R. E. Davis, personal communication). ...
... Dr. Lonsdale makes some interesting observations including a cautionary comment these reasons that the conclusions of Peterson et al. (I) should not yet be accepted as a deterrent to the ideas promoted by Read (7) or by others who have evidence that thiamin may well playa vital role in the SIDS story (4,5,8,9). ...
... The ETK activity and T P P effect also returned to near normal values. Similar findings in 2 thiamine deficient infants were reported by Lonsdale et al, 1979 N o significant correlation was found when infants were fed on other regimes. ...
i) An unexpectedly high incidence of biochemical thiamine deficiency (erythrocyte transketolase) was found in groups of mothers and infants, selected for apparent health from a westernized Caucasian community in Australia.
Deficiency was common in mothers at term but not their infants, and in apparently healthy older infants but not their mothers. These findings can be explained by preferential delivery of thiamine to the fetus, at the expense of the pregnant mother; after delivery the mother recovers, and the infant becomes depleted.
ii) The incidence of thiamine deficiency was high in ‘near‐miss' sudden infant death syndrome (SIDS) infants and their mothers, and in siblings of SIDS. The thiamine deficient infants had a high familial incidence of SIDS deaths. These ‘high risk families' might reflect poor nutrition or genetic defects of thiamine uptake and metabolism.
Since apparently thriving infants with thiamine deficiency can sometimes die unexpectedly, thiamine status deserves more attention in clinical practice and research.
... Her communication indicated that high calorie malnutrition, in the shape of milled rice eaten by the breast feeding mothers of these infants was responsible. This was further explored in more recent years by Read and coworkers in Sydney, Australia (56) and Lonsdale et al. (57)(58)(59)(60)(61). Barker and Jordan (62) found that thiamine phosphate ester gradients along the phrenic nerve and recurrent laryngeal nerve in SIDS infants revealed axonal thiamine depletion with net efflux of 70% compared with fire death controls and other SIDS nerves. ...
Thiamin(e), also known as vitamin B1, is now known to play a fundamental role in energy metabolism. Its discovery followed from the original early research on the 'anti-beriberi factor' found in rice polishings. After its synthesis in 1936, it led to many years of research to find its action in treating beriberi, a lethal scourge known for thousands of years, particularly in cultures dependent on rice as a staple. This paper refers to the previously described symptomatology of beriberi, emphasizing that it differs from that in pure, experimentally induced thiamine deficiency in human subjects. Emphasis is placed on some of the more unusual manifestations of thiamine deficiency and its potential role in modern nutrition. Its biochemistry and pathophysiology are discussed and some of the less common conditions associated with thiamine deficiency are reviewed. An understanding of the role of thiamine in modern nutrition is crucial in the rapidly advancing knowledge applicable to Complementary Alternative Medicine. References are given that provide insight into the use of this vitamin in clinical conditions that are not usually associated with nutritional deficiency. The role of allithiamine and its synthetic derivatives is discussed. Thiamine plays a vital role in metabolism of glucose. Thus, emphasis is placed on the fact that ingestion of excessive simple carbohydrates automatically increases the need for this vitamin. This is referred to as high calorie malnutrition.
Evoked potentials (EPs) are a pattern of electrophysiologic activity recorded from the brain (or spinal cord) shortly after the occurrence of an event, usually a form of sensory stimulation. Because an EP pattern can be obtained under various conditions, even upon the omission of an expected sensory event, the phenomenon is sometimes referred to as an event-related potential response. It is also referred to by other descriptive phrases such as cerebral-evoked potential, cortical-evoked potential, averaged-evoked response (AER) or by the sensory modality eliciting the response. Thus we have the auditory-evoked potential (AEP), visual-evoked potential (VEP) and somatosensory-evoked potential (SEP). Other modalities (i.e., olfactory, vestibular, pain, vibratory, etc.) can be employed but seldom are because of technical difficulties. Detafls of the methodology and procedures employed to obtain EP patterns are described later in this chapter.
Mental retardation may be defined as impairment in the acquisition of psychomotor developmental skills, with onset in childhood. The causes are multiple, the severity of the disorder may be mild to severe, and other neurological and systemic dysfunctions often accompany the retardation. A problem arises in differentiating a true amentia from an acquired disorder or dementia. There is no precise correlation of intelligence quotient (IQ) with the electroencephalogram. Evoked potentials (EPs) are clinically applicable in some patients with mental retardation. EPs are at an investigative stage for many patients with multiple disorders, in whom an attempt is being made to define precise neurophysiological indicators of the nature and localization of cerebral dysfunction. The neonatal period is the most common time of onset of the events leading to subsequent retardation. Structural defects of the central nervous system, congenital inborn errors of metabolism, intrauterine infections, intrauterine teratogens, acquired head trauma or infection, severe seizure disorders, the neuroectodermal syndromes, and other dysmorphic syndromes are among the various known causes of retardation.
One of the earliest vitamins to be discovered and synthesized, thiamin was originally spelled with an "e". The terminal "e" was dropped when it was found that it was not an amine. It is still spelled with and without the "e" depending on the text. This chapter provides a brief historical review of the association of thiamin with the ancient scourge of beriberi. It emphasizes that beriberi is the model for high calorie malnutrition because of its occurrence in predominantly white rice consuming cultures. Some of the symptomatology of this ancient scourge is described, emphasizing the difference from that seen in starvation. High calorie malnutrition, due to excessive ingestion of simple carbohydrates, is widely encountered in the U.S.A. today. Thiamin deficiency is commonly associated with this, largely because of its cofactor status in the metabolism of glucose. The biochemistry of the three phosphorylated esters of thiamin and the transporters are discussed and the pathophysiology of thiamin deficiency reviewed. The role of thiamin, and particularly its synthetic derivatives as therapeutic agents, is not fully appreciated in Western civilization and a clinical section describes some of the unusual cases described in the scientific literature and some experienced by the author. The possible role of high calorie malnutrition and related thiamin deficiency in juvenile crime is hypothesized.
Dysautonomia refers to a disease where the autonomic nervous system is dysfunctional. This may be a central control mechanism, as in genetically determined familial dysautonomia (Riley-Day Syndrome), or peripherally in the distribution of the sympathetic and parasympathetic systems. There are multiple reports of a number of different diseases associated with dysautonomia. The etiology of this association has never been explained. There are also multiple publications on dysautonomia associated with specific non-caloric nutritional deficiencies. Beriberi is the prototype of autonomic dysfunction. It is the best known nutritional deficiency disease caused by an imbalance between ingested calories and the vitamins required for their oxidation, particularly thiamin. Long thought to be abolished in modern medical thinking, there are occasional isolated reports of the full-blown disease in developed Western cultures.
Apart from genetically and epigenetically determined disease, evidence is presented that marginal high calorie malnutrition, particularly with reference to simple carbohydrates, is responsible for widespread dysautonomia. The brain and heart are the organs that have a fast rate of oxidative metabolism and are affected early by any mechanism that reduces oxidative efficiency. It is hypothesized that this results in a chaotic state of the hypothalamic/autonomic/endocrine axis. Due to the lack of adequate automatic controls, this may be responsible in some cases for breakdown of organ systems through long-standing energy deficiency, thus leading eventually to organic disease.
In four infants, afflicted by life threatening dysautonomic symptoms, BAEP testing showed grossly abnormal latency measurements, particularly in latency I-V. Two of these infants had laboratory studies which suggested that they were deficient in one or more of the biologically active forms of intracellular thiamine. All four infants improved clinically after treatment with synthetic fat soluble thiamine (TTFD) given in large doses, and BAEP latency measurements either decreased bilaterally or became similar in each ear. This provides additional evidence that thiamine dependent neurochemistry plays a vital part in mechanisms involving automatic brainstem reflex pathways and may be a causative factor in some cases of SIDS.
Thirty-six infants identified as infant apnea syndrome (IAS) and 25 controls with a comparable age distribution were evaluated with Brainstem Auditory Evoked Potential (BAEP) testing. There was a significant predilection for leftsided BAEP abnormalities in IAS patients. Fifteen IAS patients had bilateral abnormalities, and of the 21 IAS patients with unilateral abnormalities, 17 had abnormalities on the left side (p less than 0.01 by McNemar's test). Significant differences (p less than 0.05 by analysis of covariance adjusting for age) between normal controls and IAS infants were found for peak latencies I, III, and V, and amplitude III. Linear regression analyses of the above parameters versus age in months for normal controls were constructed with 68% and 95% prediction interval bands to permit analysis of individual data points. Data points from the IAS infants with bilateral BAEP abnormalities have been plotted on these linear regression curves. No single measurement of latency or amplitude is abnormal in the majority of IAS infants, but many of the individual points fall outside of the 95% prediction curve.
A relatively new noninvasive technique for the diagnosis of abnormalities affecting the central nervous system is presented. It is especially useful in patients with acoustic neuroma, brain stem disorder such as tumors of the posterior fossa with neuraxis impingement, as well as demyelinating disorders such as multiple sclerosis. It is certainly useful in the verification of neurologic normalcy in patients complaining of dizziness and vertigo. © 1980, American Medical Electroencephalographic Association. All rights reserved.
Fifteen infants at risk for sudden infant death syndrome by clinical criteria were tested using brain stem auditory evoked potential (BAEP) techniques. All infants demonstrated abnormalities on two or more of the seven criteria employed to assess results. The data indicate that BAEP testing may play a significant role in the identification and monitoring of these children.
Miller Fisher syndrome is characterized by external ophthalmoplegia, ataxia, and areflexia. Most researchers favor a peripheral origin while others suggest a brainstem inflammatory lesion or a combination of central and peripheral demyelination. We report 2 cases of Miller Fisher syndrome with the typical triad of ataxia, areflexia, and ophthalmoplegia. Strong clinical evidence of central involvement included initial drowsiness, bilateral Babinski sign, and quadriparesis. Evoked potential studies showed prolongation of central conduction time. Plasmapheresis was performed to relieve respiratory failure in Patient 1 and to shorten the duration of nasogastric tube feeding due to severe bulbar palsy in Patient 2. Significant improvement of electrophysiologic parameters was recorded after plasmapheresis. Abnormal evoked potentials, together with clinical evidence of central nervous system abnormalities, support the hypothesis that there is a combination of peripheral and central involvement in Miller Fisher syndrome in our patients. Plasmapheresis is highly effective in relieving the profound neurological deficits of this atypical syndrome.
Over the past 30 years or more, the problem of sudden, unexplained death in infants (SIDS) has made little headway. Many hypotheses have been offered but the basic cause remains elusive. The only successful prevention has been made by the supine sleeping posture. There is still, however, a hard core of unexplained incidents. There is evidence that certain stress factors are involved, and there is good evidence that the tragedy has a familial or genetic tendency. The third factor necessary for the event is inefficient oxidation in brain cells induced most commonly by marginal malnutrition in pregnancy or after birth. The absence of any one or more of these three factors decreases risk to the point of extinction. Anything that impedes healthy oxidation, or accelerates energy utilization through responding to stress, increases the risk greatly. Improving the biochemical mechanisms through appropriate nutrition is by far the best defense.
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