Derrick Lonsdale

Derrick Lonsdale
Cleveland Clinic · Associate Emeritus

MB BS London

About

118
Publications
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Publications

Publications (118)
Article
Full-text available
Thiamine or vitamin B1 is an essential, water-soluble vitamin required for mitochondrial energetics-the production of adenosine triphosphate (ATP). It is a critical and rate-limiting cofactor to multiple enzymes involved in this process, including those at the entry points and at critical junctures for the glucose, fatty acid, and amino acid pathwa...
Article
The resurgence of interest in cancer metabolism has linked alterations in the regulation and exploitation of metabolic pathways with an anabolic phenotype that increases biomass production for the replication of new daughter cells. To support the increase in the metabolic rate of cancer cells, a coordinated increase in the supply of nutrients, such...
Preprint
Full-text available
A huge number of proteins that occur in the body have to be folded into a specific shape in order to become functional. Proteins are made up of chains of amino acids and the folding process is exquisitely complex. When this folding process is inhibited, the respective protein is referred to as being misfolded and nonfunctional. So the hypothesis th...
Article
A huge number of proteins that occur in the body have to be folded into a specific shape in order to become functional. Proteins are made up of chains of amino acids and the folding process is exquisitely complex. When this folding process is inhibited, the respective protein is referred to as being misfolded and nonfunctional. So the hypothesis th...
Chapter
Starting with a brief history of beriberi and the discovery that thiamin deficiency is its cause, the symptoms and signs are reviewed. None are pathognomonic. The disease has a low mortality and a long morbidity. The appearance of the patient can be deceptive, often being mistaken for psychosomatic disease in the early stages. The chemistry of thia...
Book
Thiamine Deficiency Disease, Dysautonomia, and High Calorie Malnutrition explores thiamine and how its deficiency affects the functions of the brainstem and autonomic nervous system by way of metabolic changes at the level of the mitochondria. Thiamine deficiency derails mitochondrial oxidative metabolism and gives rise to the classic disease of be...
Article
Full-text available
Although it has been generally accepted that moving the infant from the prone to the supine position has solved the problem of sudden infant death syndrome (SIDS), it has been hypothesized that this is an insufficient explanation and that a mixture of genetic risk, some form of stressful incident and marginal brain metabolism is proportionately req...
Article
Full-text available
Although there has been much research into autism or autistic spectrum disorder (ASD), there is room for considerable conjecture regarding the etiology of these developmental brain disorders. ASD is marked by a complex interaction between environmental factors and genetic predisposition, including epistasis. This manuscript argues that changes in o...
Article
Full-text available
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...
Article
Full-text available
Autism and autism spectrum disorder (ASD) are developmental brain disorders with complex, obscure, and multifactorial etiology. Our recent clinical survey of patient records from ASD children under the age of 6 years and their age-matched controls revealed evidence of abnormal markers of thiol metabolism, as well as a significant alteration in depo...
Article
Full-text available
Case histories of a mother and her two children are reported. The mother was a recovered alcoholic. She and her two children, both of whom had symptoms that are typical of autistic spectrum disorder, had dysautonomia. All had intermittently abnormal erythrocyte transketolase studies indicating abnormal thiamine pyrophosphate homeostasis. Both child...
Article
The study of vitamin biochemistry is still in a relatively early stage of development. The discovery of allithiamine in Japan gave a strong impetus to the study of thiamine as a potential therapeutic agent rather than a simple vitamin replacement. When it was discovered that allithiamine was a naturally occurring substance that is formed when thiam...
Article
This paper reports on 10 patients who were found from routine laboratory studies to have Gilbert's syndrome (GS), also known as benign hyperbilinibinemia. Each patient came to our clinic with multiple symptoms which appear to have become increasingly common in the population of the USA as a whole. Each received only nutrient therapy and symptomatic...
Article
Full-text available
Purpose: The aim of this study was to examine the clinical effect of a single dose of secretin given to patients whose disease status lay within the autistic spectrum. Design: Open, uncontrolled trial. Materials and Methods: Sixty-four patients were registered for the study. They included 3 female adults and 2 male adolescents. There was a total of...
Article
Functional dysautonomia has been reported in relation to a large number of human disorders [1,2]. Conventionally, it is usually regarded as a complication of a given disease, as for example, in diabetes [3], although it has been observed as a condition in its own right [4–8]. The case of a patient with an array of symptoms, many of them clearly dys...
Article
Full-text available
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 assoc...
Article
Full-text available
Non-caloric nutrients (NCN) are extremely numerous and it is more than obvious that they work in a team relationship. These vitally important interactions are, for the most part, poorly understood. These brief case reports illustrate this in the therapeutic use of thiamin in a clinical setting. The initially abnormal erythrocyte transketolase activ...
Article
Full-text available
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 thousand...
Conference Paper
Full-text available
During the last several years, there has been growing clinical and scientific evidence that most children with autism suffer from mercury/metal toxicity. Furthermore, there have been many reports from physicians and parents that removal of mercury and other toxic metals can be very beneficial to children with autism, sometimes resulting in a major...
Article
Full-text available
Thiamine tetrahydrofurfuryl disulfide (TTFD) is the synthetic counterpart of allithiamine, occurring naturally in garlic. Allithiamine was discovered in Japan in 1951. Its extensive research was reported by a group known as the Vitamin B Research Committee of Japan, and given this name because of its existence in the bulbs of many of the allium spe...
Article
In a Pilot Study, the clinical and biochemical effects of thiamine tetrahydrofurfuryl disulfide (TTFD) on autistic spectrum children were investigated. Ten children were studied. Diagnosis was confirmed through the use of form E2, a computer assessed symptom score. For practical reasons, TTFD was administered twice daily for two months in the form...
Article
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...
Article
The present period of medical advances may be regarded as a paradigm shift in concepts of health and disease. There is inevitably a transition where the “old” paradigm is in conflict with the “new.” Each depends upon the disease model that is in vogue at the time that it was conceived. The present model, accepted by the majority of physicians, as w...
Article
A case report is described of a child whose clinical course imitated that of the peracute form of cardiac beriberi known as Shoshin. The nature of the disorder, certain symptoms of which started in infancy, was such that it strongly indicated a metabolic rather than a nutritional etiology, involving energy metabolism. Because autopsy was refused by...
Article
Regardless of clinical diagnosis, many acutely and chronically sick patients benefit from intravenous vitamins and minerals, which are usually administered in multiple infusions before observing obvious benefit. We hypothesized this effect was due to improved cellular energy, and attempted to find laboratory evidence via this study. Two groups of p...
Article
Though only a single case report, the patient depicted represents an unusual problem, although most of the symptoms that concerned her are common in America today. Total preoccupation with hair, particularly in a woman, is a potentially dangerous indication of brain dysfunction. The author had a similar patient whose preoccupation of her closely se...
Article
A pair of identical twins was brought to our office for clinical assessment. At the age of 7 years they had a strikingly identical history. Hair analyses from each of the twins were so similar in virtually every detail that one could almost be superimposed on the other. In addition a father and son, unrelated to the twins, presented with similar hi...
Article
It is difficult to obtain an accurate and precise assessment of specific nutrient deficiency metabolism. The cases presented here are those of two middle-aged and unrelated women whose completely non-specific symptoms were those seen every day in the offices of physicians. Because of one patient's conviction that she may be suffering from perniciou...
Article
Marginal, high calorie malnutrition is widespread in the United States. One of the major causes is all the different forms of sugar, ingested with insufficient concentration of non-calorie nutrients. Although thiamin was one of the first vitamins to be recognized and synthesized, it is surprising that it is ignored to a great extent in the present...
Article
Three family members are reported with functional symptoms considered to be caused by intracellular deficiency of thiamin. Persistence of desaturation of erythrocyte transketolase in the face of megadose thiamin hydrochloride (THCl), accompanied by a balanced multivitamin and mineral formula, suggested a familial thiamin dependency state. Each of t...
Article
Erythrocyte transketolase (TKA) and thiamin pyrophosphate percentage uptake, or effect (TPPE) were performed on 1011 patients between 1983 and 1986. The subjects were drawn from a private practice specializing in nutritional correction as a major therapeutic thrust. Either TKA or TPPE or both were abnormal in 283 (28%). Out of the total number of p...
Article
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 wi...
Article
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 clinic...
Article
Weanling mice of the DBA/2J strain are spontaneously audiogenically seizure prone between 19 and 24 days. Thereafter, susceptibility declines rapidly within the next 7-10 days. It was found that thiamine tetrahydrofurfuryl disulfide (TTFD) significantly delayed the natural disappearance of seizure proneness in male animals compared with controls tr...
Article
The possibility of endorphins playing a role in the apneas and abnormal polygraphic sleep findings of IAS is an interesting hypothesis and we believe this to be the first report on levels of β-endorphins in the CSF and serum of IAS babies.
Article
Four patients with hypermethioninemia were ascertained in neonatal mass metabolic screening programs. Hypermethioninemia has persisted in all cases. There were no other abnormalities in sulfur-amino acid concentrations, and routine serum chemical determinations, including the results of "liver function" tests, were normal. Hepatic methionine adenos...
Article
A perspective on disease is proposed which treats the brain and psyche as part of the soma. The brain, operating as a computer, coordinates energy metabolism in all organs, modulating the organism's response to stress which results in a complex mixture of sympathetic, parasympathetic and endocrine drive governing behavior and host resistance. It is...
Article
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 moni...
Article
Twenty patients who had symptoms that were apparently neurotically functional are reported because they proved to have abnormal red cell transketolase activity. Although in many of the cases the etiology appeared to be associated with heavy consumption of "junk" foods, carbonated or sweet beverages, and candy, this was not so in all of them and the...
Article
The 2 children whose cases are reported here both had recurrent episodes of fever and cervical lymphadenopathy. The conventional approach had been unsuccessful in identifying the cause or therapy. In neither case was there an infectious agent demonstrated, and biopsy of a pathologically enlarged lymph gland revealed only reactive hyperplasia in eac...
Article
The thiamine status of 42 physically healthy nonalcoholic psychiatric inpatients was evaluated by measuring transketolase activity in a hemolysate of their red blood cells, before and after addition of thiamine pyrophosphate to the hemolysate. Sixteen patients (38%) showed evidence of thiamine deficiency. Five of these patients had received thiamin...
Article
Ten infants with life-threatening apneas and clinical criteria characteristic of the infant at risk for Sudden infant death syndrome (SIDS) were found to have abnormal brainstem auditory evoked potential BAEP tests. These abnormalities were in contradistinction to normal BAEP results in two infants with apneas secondary to mechanical airway obstruc...
Article
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...
Article
A sensitive, reproducible, and specific method for the determination of urinary thiamine has been established. Unique to the use of high-pressure liquid chromatography (HPLC) to separate the fluorescent thiamine derivative from interfering fluorescent compounds. Urine samples were passed through a Decalso catoin-exchange column, washed with 0.5 M K...
Article
Cerebral gigantism, or Sotos' syndrome, was first described in five patients in 1961. Thirty-eight cases have been reported subsequently, but none in the ophthalmologic literature. High birth weight, rapid growth, antimongoloid slant, mental retardation, and normal endocrine studies are characteristic of the syndrome. Other than one patient with ny...
Article
To the Editor.— The CLINICAL NOTE by Kramer and Goodwin (238:2176,1977) should draw attention to a phenomenon that seems to receive little attention in considering nutritional disturbances. In our own institution there were no less than six cases of Wernicke's encephalopathy in a short period of time two years ago. All patients were receiving supp...
Article
Transketolase is a thiamine pyrophosphate dependent enzyme, which occurs twice in the hexose monophosphate shunt.¹ A number of investigators have used it in assessing intracellular cofactor deficiency, since it occurs in red cells, which are easily obtainable for testing.2–4 Two aspects must be considered in the use of this enzyme as a clinical too...
Article
This study was designed to determine if resistance to a standard drug during the second remission of children with acute leukemia was reduced by discontinuation of therapy during the initial remission. The initial maintenance therapy was either 6-mercaptopurine (6-MP), methotrexate (MTX), or cyclophosphamide (CYC) given continuously to relapse or d...
Article
Steinschneider presented good evidence that infants who died suddenly and unexpectedly frequently have premonitory symptoms which enable the alert physician to recognize the danger (Pediat 50:646, 1972). Fehily (Br. Med. J. 2:590, 1944) described a syndrome of sudden death in Chinese infants in Hong Kong called “breast milk toxicity syndrome”. It c...
Article
The status of mitochondrial functioning in Reye's syndrome was assessed by comparing organic acid profiles from nine pathological urines with those from normal urines. It was found that Reye's syndrome urines have a normal content of succinic, oxaloacetic, aconitic, and citric acids suggesting that the enzymes of the citric acid cycle are functiona...
Article
A total of 180 children with acute leukemia was randomized to one of two induction regimens: vincristine plus prednisone, or 6-mercaptopurine plus prednisone. Of 170 patients evaluable for induction therapy, a hematologic remission was achieved in 83% (72/87) on vincristine plus prednisone, and in 93% (77/83) on 6-mercaptopurine plus prednisone. Wh...
Article
A total of 313 patients with childhood acute leukemia received a combination of vincristine (2 mg/m2/week) and prednisone (60 mg/m2/day); 86% of 276 evaluable patients achieved a complete bone marrow remission in a median of 35 days. When a complete bone marrow remission was achieved, patients were randomized to one of three oral maintenance therap...
Article
Successful treatment of phenylketonuria (PKU) depends upon neonatal diagnosis and optimum combination of dietary phenylalanine and calories, especially in the first 3 months. Head circumference increases and “catch up” growth occurs with such dietary optimum. This acceleration requires appropriate changes in dietary phenylalanine and calories. Low...
Article
We previously reported an obtunded plasma glucagon response to stimuli in fetal sheep and suggested that relative hypoglucagonemia in the neonatal period might explain the tendency to hypoglycemia. The present studies were conducted to further test this hypothesis. Glucagon responses to alanine were measured after 4 or 24 hr. fast in 10 newborn and...
Article
From 1958 through 1970 1,024 patients were entered on the 7 clinical studies of the Southwest Cancer Chemotherapy Study Group for newly diagnosed cases of acute leukemia. A review of these cases was undertaken to determine the important factors, other than therapy, influencing survival. The most important variables in terms of survival prognosis we...
Article
From 1958 through 1970 a total of 1,024 patients was entered on the 7 clinical studies of the Southwest Cancer Chemotherapy Study Group for newly diagnosed cases of acute leukemia. A review of these cases was undertaken to determine the important factors, other than therapy, influencing survival. The most important variables in terms of survival pr...
Article
Twenty-one patients in whom the diagnosis of subacute necrotizing encephalomyelopathy (SNE, Leigh's disease) was made, have been treated with large doses of thiamine hydrochloride, thiamine propyldisulfide (TPD) or thiamine tetrafurfuryldisulfide (TTFD). In 12, the diagnosis is secure and in 9 it is presumptive. The incidence and quality of remissi...
Article
Four cases of different forms of encephalopathy are reported. All patients had significant hyperpyruvicuria with or without changes in alanine concentration in the blood or urine. Proof of decarboxylase deficiency in one patient and biochemical similarity in all led to the deduction that their diseases might have a common defect in carbohydrate met...
Article
Daunomycin, used singly, was administered in several different dosage schedules to 21 children with metastatic or progressive soft tissue sarcoma. Objective tumor regressions occurred in 4 patients (two with embryonal rhabdomyosarcoma, one with leiomyosarcoma, and one with unclassified sarcoma). A mixed response was noted in one other child with le...
Article
Maple syrup urine disease (branched-chain ketoaciduria), a genetic disorder with a well-defined biochemical defect, was first described as a clinical syndrome by Menkes et al in 1954.¹ It is characterized by an accumulation of three keto acids corresponding to a partial breakdown of leucine, isoleucine, and valine which occur in excess in urine, bl...
Article
A patient with intermittent episodes of cerebellar ataxia has been described (Pediat. 43:1025, 1969). The child excreted large amounts of urinary pyruvic acid during an episode which was studied in detail. Fibrobalst tissue culture cells were assayed for pyruvic decarboxylase and found to contain a low concentration of this enzyme. further examinat...
Article
Two different regimens utilizing daunomycin, vincristine sulfate, and prednisone were administered in combination to treat 64 children with acute leukemia in relapse. All patients had received previous therapy with vincristine, prednisone, and/or daunomycin but none had received the 3 drugs in combination. Seven were known to be resistant to both v...

Questions

Question (1)
Question
Wernicke has occurred in hyperemesis with and without hyperalimentation and thiamine deficiency (TD) is the etiology of the hyperemesis. Since emotions are generated in the limbic system that is highly sensitive to TD, the emotional lability is due also to TD..PTSD is mitochondrial due also to TD. This patient needed an erythrocyte transketolase to show TD as cause rather than effect 

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Autism spectrum disorder (ASD) is a complex neurodevelopmental disorder characterized by pervasive deficits in social interaction, impairment in verbal and non-verbal communication, and stereotypical patterns of interests and activities.