Late developing supernumerary teeth in the mandible

A 2 5/12-year-old Chinese boy was investigated for refractory seizures and psychomotor regression. His birth history was unremarkable. Generalized seizures occurred at 2 weeks of age with hypocalcemia. They recurred at 7 months of age and have become aggravated since. During hospitalization, in addition to hypocalcemia and hypomagnesemia, he was found to have hypoparathyroidism, cardiomyopathy, and brain atrophy. Excessive renal loss of magnesium, general intestinal malabsorption, or inadequate dietary intake of magnesium were excluded. He was successfully treated with oral supplements of 19-25 mmole/day of magnesium. Over a few months, he made a dramatic progress in development. His hypoparathyroidism and cardiomyopathy gradually resolved. However, intermittent seizures and psychomotor retardation persisted up to his present age of 6 3/12 years. At 4 months of age his younger sister also developed seizures and was found to have isolated hypomagnesemia. This was corrected by oral magnesium and followed by resolution of the seizure. She has developed normally up to her present age of 1 10/12 years. Both patients are currently maintained on oral magnesium oxide.     

MELAS syndrome in a five year-old child: clinical, biological and genetic characteristics

BACKGROUND: MELAS syndrome is a rare mitochondrial cytopathy; its diagnosis can be difficult. CASE REPORT: A 6-month-old boy presented with febrile seizures, possibly due to viral meningitis. At 7 months, he developed myoclonia and "brain attacks" and, subsequently, myoclonical attacks, regression of psychomotor and mental acquisitions, and progressive visual loss. The ratio of lactatorachia/lactacidemia was increased. The molecular genetic analysis showed an heteroplasmic point mutation with A-to-G mutation at nucleotide 3243 of the mitochondrial tRNA(leu) (UUR) gene. He was the second child of a mother having frequent headaches. His great aunt, a sister of his maternal grandmother, was mentally retarded and had frequent epileptic seizures and hemiparesy since her childhood. CONCLUSION: Any unusual neurological symptom, particularly when combined with "illegitimate" symptoms, should lead to search for a mitochondrial cytopathy.     

Saul B. Sells (1913–1988).

Saul Sells was born in New York City on January 13, 1913. He graduated from Brooklyn College in 1933, after studying philosophy and psychology, and three years later earned a PhD degree from Columbia University. His dissertation work, supervised by Robert S. Woodworth, was on the atmosphere effect in syllogistic reasoning, an often-cited study. While a student at Columbia, he also worked closely with Edward L. Thorndike. Throughout his career, Saul stressed the significance of organism-environment interactions in understanding and predicting behavior; he also emphasized the need to study behavior in its natural setting. He devoted himself to bridging science and practice in psychology through three major and interrelated research domains. In addition to his 22 books and over 400 articles, Saul's breadth of professional interest and influence is reflected in his numerous awards and organizational affiliations (he was a fellow in 10 different APA divisions). He served on many editorial boards, but special dedication went into his founding efforts and over 20 years as Managing Editor for Multivariate Behavioral Research. On February 4, 1988, at age 75, Saul died of a heart attack while preparing to attend an early morning meeting on another new project. His work was his life and hobby, and he devoted himself to it fully. He and Helen had no children. A brother was his only surviving family member. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Symptomatic Helicobacter pylori infection: prospective study of epidemiological, diagnostic and therapeutic aspects in children in Tunisia]

CASE REPORT: The patient was a boy born in June, 1990. The proband's father had a history of nonspherocytic hemolytic anemia. The patient was anemic at birth (Hb 11.9 g/dl) and had a hemolytic attack on postnatal day 2. His hemolysis became well compensated, and his second hemolytic episode occurred at three years of age. CLINICAL AND LABORATORY FINDINGS: The patient's mental development had so far been normal and he has no neurological symptoms. His only clinical manifestation has been compensated hemolytic anemia with a hemoglobin concentration of about 11.0 g/dl and a reticulocyte count of 3-6%. He was positive on the Heinz body formation test, and target cells were seen on his peripheral blood smear. The osmotic fragility test yielded slightly increased value. Decreased reduced glutathione (GSH) was observed (4.4 mg/dlRBC) (normal range: 63.9 +/- 9.6), and he also had decreased glutathione synthetase (GS) activity of 0.03 U/gHb (0.38 +/- 0.08 U/gHb). A diagnosis of GS deficiency was made. Decreased glutathione S-transferase (GST) activity was also found (0.57 U/gHb) (normal range: 6.65 +/- 1.20). DISCUSSION: GS deficiency has been reported in about 30 families all over the world. This patient was the first Japanese patient with red cell GS deficiency.     

Kenneth E. Moyer (1919-2006).

Presents an obituary for Kenneth E. Moyer. Before becoming an internationally known expert in the field of aggressive behavior, Kenneth Evan (Keck) Moyer held jobs as an acrobat, a physical therapist, and a farmer. He served as a marine in World War II and was a consultant to the Norwegian government. The range of his life experiences amazed his friends, who wondered how he could have done all these things and still have acquired such a breadth of scholarly knowledge. Throughout his life, his scholarly pursuits had practical applications, and his practical endeavors had a scholarly basis. Keck died May 18, 2006, of an apparent heart attack. His son, Robert S. Moyer, a psychologist who recently retired from the faculty of Bates College, and his daughter, Cathy Noblick, a marriage and family counselor in New Jersey, survive him. Also surviving are five grandchildren and four great grandchildren. He was devoted to all of them and to children he supported through international programs. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Melvin R. Novick (1932-1986).

On May 20, 1986, Melvin Novick suffered a major heart attack and died before he reached the hospital. He and his wife were visiting at the time in Princeton, where he had been called on, along with several other professionals, as an expert consultant to lend advice to the staff and management of the Educational Testing Service (ETS) in reviewing matters of philosophy and methodology in examining test items for evidence of sex or ethnic discrimination. Mel Novick's contributions to psychology and measurement are well known and numerous. He was a scholar who insisted that theory must influence practice, and he committed himself to that end. Novick was frequently called on for his advice in measurement and in statistics generally and was known nationally and internationally as a person of exceptional competence in this field. He coauthored books on test theory, statistical methods for psychological research, and Bayesian statistics. His crowning achievement, however, was the production of the 1985 Standards for Educational and Psychological Testing. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Isolated biotin-resistant 3-methylcrotonyl-CoA carboxylase deficiency: long-term outcome in a case with neonatal onset

A patient with early-onset 3-methylcrotonyl coenzyme A carboxylase (MCC) deficiency showing a severe clinical course is described. Abnormal eye and head movements suggestive of seizures were noticed soon after birth. Tonic convulsions at the age of 10 weeks led to admission. Urinary organic acid analysis using gas chromatography-mass spectrometry at 3 months of age revealed elevated concentrations of 3-hydroxyisovaleric acid (3HIVA) and 3-methylcrotonylglycine but normal levels of lactate, 3-hydroxypropionate and methylcitrate suggesting isolated MCC deficiency. This was confirmed by enzyme assays in lymphocytes and cultured skin fibroblasts: MCC activity was virtually undetectable whereas activities of propionyl-CoA and pyruvate carboxylases were within the normal range. A low protein (0.8-1.5 g/kg/day) diet supplemented with a leucine-free amino acid mixture resulted in a marked decrease of 3HIVA excretion. L-Carnitine and biotin administration had no effect on the clinical condition or metabolite excretion. Supplementation with glycine resulted in only a temporary fall of 3HIVA excretion and was therefore discontinued. L-Carnitine therapy was reintroduced later because of secondary carnitine deficiency. Compliance with treatment was poor until the age of 27 months resulting in a severe episode with seizures and coma. The general clinical condition of the patient was always good but his psychomotor development was delayed and seizures were not continuously under good control due to poor therapy compliance. The boy is now 10.5 years old and attending a school for children with learning handicaps. Conclusion: Isolated MCC deficiency of early-onset is a rare condition exhibiting a more severe clinical course than the later-onset form described in most other cases. The prognostic value of 3 HIVA measurements in CSF and serum should be evaluated in future cases.     

Cerebral infarction associated with nephrotic syndrome in a young adult: a case report

We report a 19-year-old man who developed a cerebral infarction in the territory of the anterior choroidal artery and showed a hypercoagulable state and nephrotic syndrome after diarrhea and appetite loss. He had suffered from nephrotic syndrome from the age of three and had been treated for five years. MR-angiography showed an occlusion originating in the right internal carotid artery. The right anterior and middle cerebral arteries were imaged from the left internal carotid artery via the anterior communication artery. He showed symptoms of left hemiparesis, agnosia, loss of activity, anasarca and left hypacusis following his clinical course, but had recovered from all but left hemiparesis following medical treatments including steroid therapy. The histologic finding by a renal biopsy revealed focal glomerulosclerosis. In this case, we considered that when he was in a hypercoagulable state and had a second attack of nephrotic syndrome because of inflammation and dehydration due to diarrhea and appetite loss, his hypercoagulable state grew worse, and he then developed a cerebral infarction. When one see a patient with nephrotic syndrome, one should be attentive to the possibility of a complication of cerebral infarction.     

Robert Ward Leeper (1904-1986).

Robert Ward Leeper grew up near Braddock, Pennsylvania, a steel town where most of his classmates and friends were from the families of recent immigrants from many European countries. From childhood on, Leeper assumed that he was to contribute to the world's welfare. He chose his graduate school as carefully as he had chosen work experiences and entered Clark University in 1927. He received his MA degree in 1928 and his PhD in 1930. In 1930, at the depth of the Great Depression, Leeper was successful in finding a position at the University of Arkansas, where he spent the next three years. In 1933- 1934 he worked with Karl Lashley in Chicago on a National Research Council fellowship. In 1937, Leeper settled down at the University of Oregon, where he was to remain for the rest of his life. His contributions to psychology included ideas that were precursors of the cognitive learning theories that prevail today and a continuing attack on the concept of emotion as disorganized behavior. In addition, Leeper served as president of the Oregon Psychological Association, the Western Psychological Association, Division 1 of the American Psychological Association, and as a member of various committees and boards. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Leon J. Yarrow (1921–1982).

Presents an obituary for Leon J. Yarrow. Yarrow was born on September 30, 1921, in Shenandoah, Pennsylvania. His father died when Leon was very young, and he experienced some of the transitions in attachments and care that may well have influenced his life-long research interest in the importance of early experience for the developing child. At age 15 Leon entered the University of Pennsylvania, where he majored in psychology. In 1951 Leon accepted the first research position that was made available in the old U.S. Children's Bureau. He initiated a longitudinal investigation of adopted children that focused on the consequences of the infant's transition from foster care to the adoptive parents. As his findings became known, social work practice was modified to favor adoption at the earliest ages. Leon spent a major part of his career at the National Institutes of Health (NIH). His associates there were uniformly touched by his personal warmth, his unrelentingly high standards for developmental studies, and the conceptual elegance with which he grasped problems. Leon died July 28, 1982, of a heart attack, the day before he planned to embark on a summer vacation with his family. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Postoperative analgesia following total knee arthroplasty: a study comparing spinal anesthesia and combined sciatic femoral 3-in-1 block

We report on a 6-year-old Caucasian boy with direct insertion of genetic material from the short arm of chromosome 4 to the short arm of chromosome 2. He was referred for evaluation because of global developmental delay and seizure disorder. A karyotype performed at 4 1/2 months of age, by a laboratory elsewhere, reportedly showed a deletion of chromosome 4(p12). When we saw him, he had macrocephaly, hypotonia, psychomotor retardation, multiple minor congenital anomalies, and EEG abnormalities. Repeat chromosomes performed by our laboratory revealed that his karyotype was 46,XY,dir ins(2;4)(p24;p15.3p13). Fluorescence in situ hybridization (FISH) analysis, using chromosomes 2 and 4 painting probes confirmed that material from 4p has been translocated to 2p. Also, FISH analysis using the Wolf-Hirschhorn critical region probe revealed that both loci are intact. Parental chromosomes were normal. This complex rearrangement, though it appears balanced, probably might have resulted in either a structural loss of genetic material or functional loss of a gene action. Thus, his phenotype could be explained by this de novo insertion of chromosome 4 material into chromosome 2. There is no reported case of this specific chromosome rearrangement.     

Prenatal diagnosis of ring chromosome 6 in a fetus with hydrocephalus

A patient with ring chromosome 6/monosomy 6 mosaicism is presented. At 25 weeks' gestation, ultrasound examination demonstrated fetal hydrocephalus. Amniocentesis was performed. The fetal karyotype was 45,XY,-6/ 45,XY,-6,+f/46,XY,r(6)(p25q27). Delivery of this male infant was by Caesarean section at 37 weeks' gestation. The karyotype in peripheral blood lymphocytes was 46,XY,r(6)(p25q27) with no indications of mosaicism. The infant had hydrocephalus which required treatment with a ventriculoperitoneal shunt at 22 days of age. He had no other obvious serious congenital anomalies. By 17 months he had developed microcephaly, seizures, severe bilateral hearing loss, and global development delay. This patient provides information regarding phenotypic variability of ring chromosome 6 and also reinforces the importance of offering amniocentesis if fetal hydrocephalus is detected as an isolated anomaly.     

Oakley Stern Ray (1931-2007).

Presents an obituary for Oakley Stern Ray. Ray was a teacher--whether in the role of professor, chief, or colleague. Late in his life, he described himself as having had four "day jobs," although he could not have accomplished so much if he had limited himself to daylight hours. He so completely inhabited each role that the colleagues he knew in one capacity were often unaware the others existed. These four careers were Veterans Administration (VA) psychologist, professor, author, and executive. He always had at least two of these careers going at the same time, usually three. During the 1970s and 1980s, he was productive in all four simultaneously. Oakley's 76th birthday was February 6, 2007; he died of leukemia on February 7, 2007. He is survived by his wife Kathy Ray, his sons Steve, Christian, and Tom Ray, his daughter Deb Scanlon, his grandchildren--and thousands of students. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

George G. Thompson (1914–2008).

Presents an obituary for George G. Thompson (1914–2008). George G. Thompson died peacefully on July 12, 2008, in Worthington, Ohio, after a brief illness. He was 94 years old. Although he had retired almost 30 years before his death, he had a remarkable memory and keen analytical skills to the very end of his life. He was best known for his research and writing on developmental and educational psychology. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Obituary: Norman R. F. Maier (1900-1977).

Norman R. F. Maier was born in a small town in Michigan on November 27, 1900. He was educated in the Michigan public schools and received his BA from the University of Michigan in 1923. After a year of graduate work at the University of Berlin (1925-1926) he completed his PhD in 1928 at the University of Michigan. Following a year of teaching, Dr. Maier was for two years a National Research Council Fellow in Zoology at the University of Chicago, where he worked with Professor K. S. Lashley. In 1931 he joined the faculty of the University of Michigan, where he worked for the rest of his life, dying of a heart attack on September 24, 1977. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Quinn McNemar (1900–1986).

Quinn McNemar, Professor Emeritus of Psychology, Statistics, and Education at Stanford University, with his primary base in the Department of Psychology, died on July 3, 1986. Born on February 20, 1900, he was 86 at the time of his death. He had retired at the age of 65 after long years of service at Stanford and spent an additional five years as Professor of Psychology and Education at the University of Texas, Austin. He then returned to reside again in Palo Alto until his death. His widow, Olga, who had a PhD in psychology from Columbia University, was long associated with him in his professional work and survived him after their marriage of 55 years. His status as a psychologist was recognized by his election to the presidency of the American Psychological Association to serve in the year 1963-1964. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Specific activation of telomerase in pancreatic cancer tissue and preoperative diagnosis of pancreatic cancer by telomerase in pancreatic juice

OBJECTIVE AND IMPORTANCE: We describe the successful treatment of symptomatic giant arteriovenous malformations (AVMs) using staged volume radiosurgery followed by microsurgical resection. CLINICAL PRESENTATION: A 57-year-old man presented with Spetzler-Martin Grade 5 AVMs, with persistent headaches and seizures. He had previously undergone eight attempts at AVM embolization and a craniotomy for attempted AVM resection; he had suffered four episodes of brain hemorrhaging but had made a good neurological recovery. INTERVENTION: Because of the persistent symptoms of the patient and his history of hemorrhaging, a treatment plan based on staged radiosurgical treatments of different portions of the AVMs (three sessions, spaced 6 mo apart), followed by delayed microsurgical removal of the much-reduced residual AVMs (3 years later), was undertaken. The patient did not suffer any additional hemorrhaging episodes, his AVMs were completely removed, and he has made a good recovery. CONCLUSION: Staged volume radiosurgery followed by microsurgical resection of the residual AVMs represents a novel treatment strategy for the management of Grade 5 AVMs that might be untreatable by any single treatment method used alone.     

Russell Julian Bent (1929–2008).

Russell Julian Bent had a long career as an innovative administrative psychologist. He also worked as a chief clinical psychologist and later assumed an administrative role in academic health science centers. Following his career, Bent moved to Dayton, Ohio, where Wright State University was establishing its new School of Professional Psychology. Over the years, Bent served on its Midwest Board, later becoming president of the Midwest Board and also the Midwest Representative to the ABPP Board of Trustees (1992–1995). He served as president of the Board of Trustees from 1996 to 1997, during which time he participated in conceptualizing and carrying out ABPP’s reorganization from a regional to a specialty board structure. He served as the ABPP executive officer from 2000 to 2006. In these positions, he was instrumental in establishing systematic ABPP policies and procedures. When ABPP presented him with its Award for Distinguished Service and Contributions to the American Board of Professional Psychology in 2006, the award was renamed in his honor. Sadly Bent died after an extended illness on August 22, 2008, at his retirement home in Roswell, Georgia. He was 79 years of age. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

A 78-year-old man with young onset parkinsonism and sudden death

We report a right-handed 78-year-old man with early onset parkinsonism. The patient had an onset of micrographia at 23 years of the age in 1939. Seven years later he started to drag his right foot, and at 38 years of age, he walked with small steps with festination. Tremor was also present in his right hand. His daily life was independent as a otorhinolaryngologist. He visited our clinic on March 24, 1977 when he was mentally sound and showed mild parkinsonism consisting of masked face, stooped posture, small step gait, bradykinesia, and right side dominant rigidity and tremor. He showed good response to trihexyphenidyl and amantadine HCl. Two month later, he developed dyskinesia and some worsening of parkinsonism, and was admitted to our hospital for the first time. He was treated with 400 to 600 mg/day of levodopa/ carbidopa. He showed marked improvement, however, dyskinesia remained in his mouth. He was doing well until 77 years of age (June of 1993) when he developed hallucination and motor fluctuations. He was admitted again to our hospital on June 22, 1993. On admission, he was alert and appeared mentally sound. However, Hasegawa dementia scale was 18/30. Upward gaze was slightly restricted (3/5). Voice was somewhat small but no masking was noted. His posture was stooped and the gait was of small step. Dyskinesia was noted during walk. No rigidity nor tremor was noted. Deep tendon reflexes were lost but no sensory loss or motor weakness was noted. Routine laboratory studies were unremarkable. A cranial CT scan revealed only mild to moderate cortical atrophy. Motor and sensory conduction velocities were within normal limits, however, motor action potentials could not be obtained with stimulation to the right common peroneal nerve. He was treated with 600 mg/day of levodopa with carbidopa, 100 mg of amantadine HCl, 300 mg of Dops, and 25 mg of tiapride. He continued to show motor fluctuations, and was discharged on July 23, 1993. Since then his motor functions had become progressively worse with frequent falls, but he was still able to walk without support. On October 3 of 1994, he went to bed as usual. On the next morning, he was found dead in his bed at 9: 30. The patient was discussed in neurological CPC, and the chief discussant arrived at the conclusion that the patient had young-onset Parkinson's disease with Lewy bodies in the substantia nigra. Opinions were divided between Parkinson's disease and Lewy body negative young onset parkinsonism. Postmortem examination revealed obstruction of the trachea by aspirated foods, and the cause of death appeared to have been suffocation by the foods. Macroscopically, the external appearance of the brain was unremarkable except for slight frontal atrophy. The substantia nigra showed depigmentation in the lateral part, but the pigmentation of the medial part was well preserved. Upon histologic examination, the number of pigmented neurons in the dorsomedial part was well preserved. In the lateral part, pigmented neurons were well preserved in the dorsal area, however, in the ventral area, only non-pigmented neurons were seen; they appeared to be neurons in the pars reticulata. No gliosis was seen in any of the nigral areas. No Lewy bodies were seen in the remaining neurons. So-called immature neurons with rounded shape without neuromelanin could not be detected. The locus coeruleus neurons were well preserved. The putamen and the other basal ganglia structures were also intact. Slight myelin pallor was noted in the subcortical white matter, however, otherwise cerebral cortices were normal. The histology of this patient is unique in that only the ventrolateral part of the substantia nigra showed abnormal finding consisting of lack of pigmented neurons without gliosis. It is not clear whether the nigral change represents degeneration or a congenital "hypoplasia'. To our knowledge, such a unique pathology of the substantia nigra has not been reported in the literature. Our patient ma     

Combined liver-kidney transplantation in methylmalonic acidemia

A 13-year-old boy with non-B12-responsive methylmalonic acidemia (MMA) had chronic renal failure. Hemodialysis led to symptomatic and biochemical improvement. He subsequently received a combined liver-kidney transplant. After 16 months of follow-up he has a normal lifestyle and a marked reduction in plasma and urine methylmalonate.