Biomedical subjects
R A Brumback
Publications and source records attributed to R A Brumback.
Affective illness in children and adolescents: patterns of presentation in relation to pubertal maturation and family history.
Affective illness is now recognized as a common problem in all age groups, and the various patterns have been well documented in adults. The objective of this study was to evaluate the patterns of affective illness in children and determine changes with increasing age and family history. One hundred children/adolescents with affective illness (72 boys and 28 girls; age range 2-20 years; mean age 10 years), who were consecutively referred to the Pediatric Behavioral Neurology Program, Children's Medical Center at Dallas, were evaluated for the pattern and course of affective illness symptoms, family history, and pubertal stage. Seven patterns of affective illness were identified. In the 65 prepubertal children (Tanner stage 1), disorders with hypomanic/manic symptomatology were most common (47/65, 72%): mania (2/65, 3%), hypomania (8/65, 12%), cyclothymia (11/65, 17%), juvenile rapid-cycling bipolar disorder/ultradian cycling bipolar disorder (8/65, 12%), and dysthymia with bipolar features (18/65, 28%). In contrast, the 26 fully pubertal adolescents (Tanner stages 3-5) had a predominance of patterns with only depressive symptomatology (16/26, 61%): dysthymia (4/26, 15%) and depression (12/26, 46%), along with juvenile rapid-cycling bipolar disorder/ultradian cycling bipolar disorder (6/26, 23%). Affective illness, alcoholism, and drug abuse were prominent in the family histories, regardless of the child's pattern of symptoms. Family histories of character disorder and Briquet's syndrome were also common, but thought disorder, suicide, and homicide were infrequent. This study supports the clinical observation that the presentation of affective illness changes with age: manic features predominate in younger children, whereas depressive symptomatology is more evident with pubertal maturation.
Medullomyoblastoma: case report.
This 7-year-old boy presented with a 2-week history of headache, nausea, vomiting, anorexia, lethargy, and unsteadiness of gait. Brain magnetic resonance imaging (MRI) revealed a cystic mass within the vermis of the cerebellum. A suboccipital craniectomy was performed to remove a tumor that contained primitive neuroectodermal cells with florid skeletal muscle differentiation. Immunohistochemical studies and electron microscopy confirmed the presence of both a primitive neuroectodermal component and rhabdomyoblastic differentiation, consistent with the diagnosis of medullomyoblastoma. This exceedingly rare tumor of the cerebellar vermis of children is characterized by two components: primitive neuroectodermal tumor cells and skeletal muscle. Although the histogenesis remains uncertain, advances in immunohistochemistry and electron microscopy suggest the origin of this tumor from a multipotential stem cell precursor.
Authorship designation and the Journal of Child Neurology in 2002.
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Dementia: the University of Oklahoma autopsy experience.
The brain from 98 consecutive patients with the clinical diagnosis of dementia were examined at autopsy in a standardized fashion. Alzheimer's Disease was present in 79 of the cases, 76%, but represented the only diagnosis in 41%. Thus, almost 60% had another associated pathologic disorder. Cerebral amyloid angiopathy (CAA) represented the single largest subset, present in 25 cases. 40% were accompanied by either 1) small, microscopic infarcts or cortical scars, or 2) small collections of macrophages containing hemosiderin or small hemorrhages. CAA occurred with both atherosclerotic cortical infarcts and arteriolosclerotic subcortical pallor or lacunar infarcts. Alzheimer's Disease occurred with Diffuse Lewy Body (DLB) Disease in 13 cases. DLB Disease did not occur as a distinct entity, and thus may represent the second largest subset of Alzheimer's Disease. Both Alzheimer's Disease and DLB Disease accounted for dementia in Parkinson's Disease. Almost 25% of all cases had a disorder other than Alzheimer's Disease.
Weinberg's syndrome: a disorder of attention and behavior problems needing further research.
A subset of inattentive children have an underlying problem in sustaining wakefulness ("vigilance"). This disorder of vigilance, termed Weinberg's syndrome, is characterized by difficulty in maintaining wakefulness and alertness as evidenced by (among other symptoms) motor restlessness (fidgeting and moving about, yawning and stretching, talkativeness) and complaints of tiredness. During tasks requiring concentration (continuous mental activity) such as reading, children with Weinberg's syndrome will daydream, lose interest, complain of boredom, and become increasingly restless. Napping, while infrequent, usually is not refreshing. A distinct personality described by family members and friends as kind, affectionate, compassionate, or "angelic" also seems to characterize this condition. Weinberg's syndrome has a familial pattern suggesting autosomal-dominant inheritance. Additional neurophysiologic, pharmacotherapeutic, epidemiologic, and genetic studies will be necessary for a full understanding of Weinberg's syndrome.
Pediatric eighth cranial nerve schwannoma without evidence of neurofibromatosis.
Schwannomas of the eighth cranial nerve are rare in children. We report a 4 10/12 - year-old girl with no evidence of neurofibromatosis who presented with facial droop. Radiographic studies revealed a large cerebellopontine angle tumor. At surgery, the tumor was attached to the eighth cranial nerve and histologically was a schwannoma. This is the youngest reported case of unilateral eighth cranial nerve schwannoma in a patient without the stigmata of neurofibromatosis.
54-year-old man with progressive dementia: a clinicopathologic correlation conference from the University of Oklahoma College of Medicine.
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Alzheimer's disease as a defect of neuronal autotrophism: a hypothetical analogy with amateur radio operation.
Amyloid precursor protein appears to be a signaling protein that plays a role in neuronal autotrophism, indicating integrity of the nerve terminal and synapse. Analogous to amateur radio operation with a damaged antenna in which further attempts to improve signal propagation can result in damage to the radio equipment, abnormal signaling by the secreted amyloid precursor protein stimulates compensatory metabolic activity in the neuron that ultimately leads to paired helical filament formation (neurofibrillary tangles) and further neuronal dysfunction and death.
Citation and impact of the Journal of Child Neurology.
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Primary intracerebellar osteosarcoma arising within an epidermoid cyst.
The authors report a case of a primary extraskeletal osteosarcoma arising within an epidermoid cyst in the parenchyma of the cerebellum in a 64-year-old woman. On initial presentation, the tumor involved the midline cerebellum without attachment to the surrounding dura mater or calvarium. Complete medical and radiologic evaluation failed to reveal a primary skeletal or other extraskeletal osteosarcoma. To our knowledge, this is the first reported case of a primary extraskeletal osteosarcoma within the cerebellum. Osteosarcoma as a primary brain tumor is exceedingly rare, and only three cases (all occurring within the cerebral hemispheres) have been reported previously. The histogenesis of primary sarcomas of the brain is not evident. The associated finding of an epidermoid cyst suggests the tumor originated from a teratoma.
Child homicide in Oklahoma: a continuing public health problem.
Homicide is a leading manner of injury to cause death in children. To assess this phenomenon in Oklahoma, the demographic characteristics and causes of death of the victims of child homicide in Oklahoma have been reviewed. One hundred eleven consecutive cases of homicide in children less than age 13 years were reviewed and the demographic characteristics of the victims were analyzed. The majority of homicides occurred in Tulsa and Oklahoma Counties (55.8%). The ratio of male to female victims was approximately equal. The races of the victims were 66.6 percent White, 24.3 percent Black, 8.1 percent Native American and 0.9 percent Asian. The most common cause of death was head injury (45.9%). An unexpected finding was that in 23.4 percent of cases, an additional fatality occurred in the family due to family violence. This fatality involved either suicide of the perpetrator or homicide of a sibling. These findings indicate a continuing family violence problem in Oklahoma.
Autopsy-proven Alzheimer disease in a patient with dementia who retained musical skill in life.
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Attention deficit hyperactivity disorder: a disease or a symptom complex?
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Attention deficit hyperactivity disorder: a disease or a symptom complex?
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Medulloblastoma in a 7-year-old girl.
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Algorithms for the treatment of affective illness, primary disorder of vigilance, and task-dependent attention disorders (learning disabilities) in children and adolescents.
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Hypertrophic intrafusal muscle fibers in infantile spinal muscular atrophy.
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