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Biomedical subjects

R G Curless

Publications and source records attributed to R G Curless.

At least 37 records · Page 2Linked to original sources

Subdural empyema in infant meningitis: diagnosis, therapy, and prognosis.

Collections of subdural fluid during the course of infant Hemophilus influenzae meningitis have been demonstrated in the past by routine subdural taps and, more recently, by computed tomography. While the fluid is usually a benign effusion, the clinical picture and CT pattern may suggest a subdural abscess (empyema) necessitating further subdural punctures. This is a review of nine infants with subdural empyema. As reported in the literature, a remarkably good prognosis is apparent following treatment with subdural taps and systemic antibiotics. Six of seven H. influenzae cases were ampicillin-resistant in this series.

Ampicillin↗

Cerebellar hemorrhage complicating methylmalonic and propionic acidemia.

Methylmalonic (MMA) and propionic (PPA) acidemias are inherited disorders characterized by a wide spectrum of clinical and biochemical findings. We report the neuropathologic findings in two young infants with MMA and PPA in which death probably resulted from cerebellar hemorrhage. When combined with a previous report of isovaleric acidemia complicated by diffuse subarachnoid and parenchymal cerebellar hemorrhage, it is apparent that cerebellar hemorrhage must be considered in any infant with a fulminating organic acidemia. Rapid neurologic deterioration despite an improvement in acidosis and hyperammonemia is an indication for computed tomography of brain. Surgical evacuation of the blood may improve the chance for survival in this catastrophic illness.

Acidosis↗

Paraldehyde therapy in childhood status epilepticus.

Intravenous (IV) diazepam or phenobarbital is generally accepted as the initial treatment of choice for status epilepticus in children. The risk of severe respiratory depression with either drug is a major problem, particularly in emergency centers that do not have appropriate equipment or personnel for rapid endotracheal intubation of infants. While some pediatric centers are not reluctant to recommend paraldehyde for secondary therapy in status epilepticus, most texts and publications recommend it only as a last resort because of reported complications. We investigated the benefits and complications from varied dosing regimens in 16 trials. The results indicated no significant complications in patients who did not receive an initial IV bolus. Even though treatment with phenobarbital or diazepam and phenytoin sodium had failed, 37% had a good therapeutic response.

Adolescent↗

Radionuclide cerebral perfusion scintigraphy in determination of brain death in children.

We evaluated a radionuclide scintigraphic technique for imaging relative cerebral perfusion in 18 children who had no evidence of cortical and brainstem function. Patients without scintigraphic evidence of cerebral perfusion all later met criteria for diagnosis of brain death. Patients who failed to satisfy brain-death criteria had persistent scintigraphic evidence of cerebral perfusion. Seven patients with normal scintigraphic studies were being treated with barbiturates and hypothermia at levels that attenuated or completely suppressed EEG activity. Four patients without scintigraphic evidence of cerebral perfusion had mean arterial pressures (MAP) higher than (54.8 +/- 7.6 torr) intracranial pressures (ICP) at the time of scintigraphic study, suggesting that ICP in excess of MAP is not the sole explanation for the absence of cerebral perfusion. Radionuclide cerebral perfusion scintigraphy (RCPS) is a rapid, portable, accurate test that appears to be useful in the diagnosis of brain death in the pediatric population.

Blood Pressure↗

Dominant chondrodysplasia punctata with neurologic symptoms.

There are few descriptions of major neurologic dysfunctions in either the recessive or the dominant form of chondrodysplasia punctata. In the dominant trait, often called Conradi-Hünermann disease, a normal life expectancy with normal neurologic development is the usual course for those who survive the first few weeks of life. We studied an affected infant with a severe spinal cord abnormality that was present at birth and has not been reported in either recessive or dominant chondrodysplasia punctata.

Chondrodysplasia Punctata↗

Neuronal ceroid lipofuscinosis. Diagnosis by semithin plastic-embedded sections of peripheral blood lymphocytes from a patient with a normal blood smear.

Neuronal ceroid lipofuscinosis is an important diagnostic consideration in a school-aged child with progressive blindness. Neurologic symptoms and signs may appear late, and the retinopathy is not specific. Ultrastructural abnormalities have been demonstrated in many body tissues, but skin has been claimed to be the most reliable. Light and electron microscopic examinations of WBCs have also been helpful in several instances. In the case reported, the skin biopsy specimen and light microscopy of a peripheral blood buffy coat failed to disclose diagnostic changes. Examination of semithin sections of plastic-embedded peripheral blood provided the answer.

Capillaries↗

Cranial computerized tomography in childhood leukemia.

The value of computerized tomography (CT) of the head in childhood leukemia is emphasized by a striking example. The response of the leukemic infiltrate to radiation suggests that an earlier (presymptomatic) scan might have led to major improvement in management. A routine CT scan may be warranted in children with a prolonged remission in search of a CNS reservoir for leukemia cells.

Brain Diseases↗

Neonatal intracranial abscess: two cases caused by Citrobacter and a literature review.

Intracranial abscesses are uncommon in the newborn. Observation of 2 infants and a review of the literature reveal that the offending organism was a Citrobacter in 12 of the 16 documented cases. In the Neonatal Meningitis Cooperative Study this organism was responsible for meningitis in only 4%. However, an analysis of all published cases of neonatal Citrobacter meningitis reveals a 42% incidence of intracranial abscess. Accordingly, early identification by computerized tomography is important in facilitating prompt surgical therapy.

Brain Abscess↗

Acute vestibular dysfunction in childhood. Central vs. peripheral.

Acute, vestibular symptoms in childhood are primarily caused by acute labyrinthitis (end organ dysfunction) or central nervous system diseases such as neoplasia involving the pons or cerebellar nuclei or infection manifested by pontine encephalitis. The evaluation, treatment, and prognosis of end organ versus central disease is so different that the ability to make a bedside, presumptive diagnosis provides an important advance. Both patients presented here had a central vestibulopathy. By paying particular attention to the character of the nystagmus and past pointing, the Romberg test, and the details of the vertiginous history, central disease was the presumptive diagnosis prior to invasive procedures. The neurophthalmological clues are reviewed to demonstrate their value in making more accurate diagnoses in children with acute vestibular dysfunction.

Adolescent↗

Histological patterns of muscle in infants with developmental brain abnormalities.

The histology of muscle obtained from 23 hypotonic infants without neuromuscular disease is presented. Long-term follow-up neurological examinations were carried out to determine the site of the lesion. 18 cases have strong evidence of cerebral dysfunction, 12 of whom have histologial abnormalities of fiber-type distribution and diameter. Of the remaining five patients without obvious cerebral abnormality, four also had histological changes, but individual circumstances partially explain the cause of the abnormal histological patterns in these four. No single histological abnormality could be identified, but the results indicate that histological abnormalities of muscle in hypotonic infants without clinical or microscopic evidence of neuromuscular disease may be useful in predicting a cerebral cause for the hypotonia.

Brain↗

Fingerprint body myopathy: a report of twins.

Ultramicroscopic changes of subsarcolemmal fingerprints in the muscle of children with infantile hypotonia and weakness may represent a specific congenital entity. Four children have been reported so far. The two children reported in the present paper are the first full siblings to be described and, in addition, are identical twins. Four of these six children also had mental retardation, which suggests that this disorder may carry with it a significant risk of central nervous system abnormality.

Child↗