Browse Items · Pediatric Palliative Care Library

Deep brain stimulation in children: experience and technical pearls

OBJECT: Deep brain stimulation (DBS) is an established technique for the treatment of several movement disorders in adults. However, the technical approach, complications, and results of DBS in children have not been well documented. METHODS: A…

Late onset globoid cell leukodystrophy (Krabbe's disease)--Swedish case with 15 years of follow-up.

We describe a male patient with late onset globoid cell leukodystrophy (GLD) (Krabbe's disease) still alive at 24 years of age, with a well preserved intellectual and communicative capacity, in contrast to visual failure and severe central pyramidal…

Diagnostic evaluation using whole-body technetium bone scan in children with cerebral palsy and pain.

BACKGROUND: Pain in noncommunicative children can be difficult to localize and diagnose. The purpose of this study is to report our experience using a 3-phase whole-body technetium bone scan as a screening tool in identifying the source of persistent…

Unilateral closed-lip schizencephaly and epilepsy: a comparison with cases of unilateral polymicrogyria

We compared the electroclinical features and evolution of patients with two different types of abnormal cortical organization: unilateral closed-lip schizencephaly (SCHZ) and unilateral polymicrogyria (PMG). Between February 1990 and June 2002, 51…

Gastric sensory and motor dysfunction in adolescents with functional dyspepsia

OBJECTIVES: Validated, noninvasive studies were used to compare sensation and motor function of the upper gastrointestinal tract in adolescents with functional dyspepsia (FD) and in control subjects. STUDY DESIGN: Fifteen adolescents with FD and 15…

Mitochondrial encephalomyopathy: variable clinical expression within a single kindred

The clinical manifestations of mitochondrial encephalomyopathy are described in four generations of a single kindred. The age of onset of major neurological disturbance varied from 3-70 years. In some patients, deafness was the only manifestation; in…

Tay-Sachs disease: Progression of changes on neuroimaging in four cases.

The neuroradiological findings in four patients with Tay-Sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted…

Modulation of cortical-limbic pathways in major depression: treatment-specific effects of cognitive behavior therapy

BACKGROUND: Functional imaging studies of major depressive disorder demonstrate response-specific regional changes following various modes of antidepressant treatment. OBJECTIVE: To examine changes associated with cognitive behavior therapy (CBT).…

Tags: 2004, Adult, Ambulatory Care, Antidepressive Agents, Archives Of General Psychiatry, Backlog, Bieling P, Blood Glucose/metabolism, Brain Mapping, Cerebral Cortex/physiopathology/radionuclide imaging, Cognitive Therapy, Comparative Study, Depressive Disorder, Emission-Computed, Energy Metabolism/physiology, Female, Fluorodeoxyglucose F18/diagnostic use, Follow-up Studies, Frontal Lobe/physiopathology/radionuclide imaging, Garson C, Goldapple K, Humans, Journal Article, Kennedy S, Lau M, Limbic System/physiopathology/radionuclide imaging, Major/physiopathology/radionuclide imaging/therapy, Male, Mayberg H, Middle Aged, Nerve Net/physiopathology/radionuclide imaging, Neural Pathways/physiopathology/radionuclide imaging, Non-U.S. Gov't, Outcome And Process Assessment (health Care), Paroxetine/therapeutic use, Personality Inventory, Prefrontal Cortex/physiopathology/radionuclide imaging, Research Support, Second-Generation/therapeutic use, Segal Z, Sensitivity and Specificity, Tomography

Neuroimaging-use trends in nonacute pediatric headache before and after clinical practice parameters

OBJECTIVES: The objective of this study was to determine trends in diagnostic neuroimaging-use rates in nonacute pediatric headache before and after publication of clinical practice guidelines. METHODS: Retrospective, cross-sectional analysis was…

Brain metabolism during short-term starvation in humans

During prolonged starvation, brain energy requirements are covered in part by the metabolism of ketone bodies. It is unknown whether short-term starvation of a few days' duration may lead to reduced brain glucose metabolism due to the change toward…

Joubert syndrome: long-term follow-up

Mitochondrial encephalomyopathy, lactic acidosis, stroke-like episodes (MELAS): clinical, radiological, pathological, and genetic observations

We reviewed 10 patients (5 males, 5 females) with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes. The age of symptom onset ranged from 3 months to 12 years. All had lactic acidosis, multiple stroke-like events with…

Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society

OBJECTIVE: The Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society develop practice parameters as strategies for patient management based on analysis of evidence. For this…

Neuronal ceroid-lipofuscinosis: A clinical and morphological study of 19 patients.

We report on clinical, electrophysiological, neuroradiological, and morphological data from 19 patients with different types (late infantile, juvenile, and adult) of neuronal ceroid-lipofuscinosis (NCL), observed in the last 10 years at the…

Postmortem computed tomography imaging in the investigation of nontraumatic death in infants and children

OBJECTIVE: To determine the accuracy of postmortem computed tomography (PMCT) for the assessment of causes in nontraumatic deaths in children. STUDY DESIGN: We enrolled cases of nontraumatic deaths of infants and children who underwent PMCT at a…

Tags: 2013, Autopsy, Backlog, Biomed Research International, Child, Death, Female, Humans, Ikeda S, Infant, Journal Article, Kaneko K, Kawasaki H, Kurokawa H, Male, Newborn, Noda Y, Ohashi A, Preschool, Tanigawa N, Tomography, Tsuji Shoji, X-Ray Computed, Yoshimura K

Neuroanatomy of holoprosencephaly as predictor of function: beyond the face predicting the brain

BACKGROUND: Despite advances in neuroimaging and molecular genetics of holoprosencephaly (HPE), the clinical spectrum of HPE has remained inadequately described. OBJECTIVE: To better characterize the clinical features of HPE and identify specific…

Percutaneous CT-guided cordotomy for the treatment of pediatric cancer pain

Percutaneous cordotomy using CT guidance has been shown to be a safe and effective means of reducing pain in adults with cancer in 2 large case series. Its effectiveness in pediatric patients, however, has not been reported. Here, the authors present…

Recurrent pain attacks in a 3-year-old patient with myoclonus epilepsy associated with ragged-red fibers (MERRF): a single-photon emission computed tomographic (SPECT) and electrophysiological study

We reported a 3-year-old girl with myoclonus epilepsy associated with ragged-red fibers (MERRF) who was afflicted with recurrent pain attacks and allodynia on the right side of the body. Although magnetic resonance imaging showed normal intensity in…

Diagnosis and management of MNGIE syndrome in children: case report and review of the literature

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) syndrome is a rare disorder that presents in childhood; however, marked delay in diagnosis is common. We report a case and review the literature describing the typical features that should…

Direct measurement of intracranial pressure at high altitude and correlation of ventricular size with acute mountain sickness: Brian Cummins' results from the 1985 Kishtwar expedition

OBJECTIVE AND IMPORTANCE: The "tight-fit" hypothesis and subsequent current understanding of acute mountain sickness (AMS) is that individuals with less compliant cerebrospinal fluid systems (smaller ventricles and cerebrospinal fluid spaces) have a…

Acute onset of X-linked adrenoleukodystrophy mimicking encephalitis

We report the case of a 6-year-old boy with X-linked adrenoleukodystrophy (ALD). In view of the acute onset of vomiting, fever, and coma, encephalitis was initially suspected. However, brain magnetic resonance imaging demonstrated a pattern of…

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