Showing papers by "Donald L. Price published in 1971"
TL;DR: Four children in a family had a progressive neurologic disease characterized by diffuse lymphohistiocytic infiltrations of the central nervous system in association with multiple foci of parenchymatous destruction, but unlike those disorders, was largely confined to the CNS.
Abstract: Four children in a family had a progressive neurologic disease characterized by diffuse lymphohistiocytic infiltrations of the central nervous system (CNS) in association with multiple foci of parenchymatous destruction. The age range was 15 months to 12 years. The clinical features, although variable, comprised seizures, spastic weakness of limbs, and ataxia. The cerebrospinal fluid showed pleocytosis and increased protein. Histopathologically the disorder resembled what has been called familial hemophagocytic reticulosis, or familial erythrophagocytic lymphohistiocytosis, but unlike those disorders, was largely confined to the CNS. The cause and pathogenesis remain unknown. A genetically-determined atypical response to an undisclosed infection is possible.
41 citations
TL;DR: In 3 patients with diabetes mellitus and ketoacidosis and one with uremia from chronic renal disease, phycomycotic infection extended into the intracranial cavity from the paranasal sinuses, leading to death in all 4 cases.
21 citations
TL;DR: A postmortem examination on the brain of a patient with hypernatremia resulting from impaired thirst sensation in the presence of a normal ADH response could be ascribed to hydrocephalus, which they thought was most probably due to aqueductal stenosis.
Abstract: HYPERNATREMIA lesions has long of these patients, either decreaesd in patients with intracranial been recognized.1-6 In most the cause of hypernatremia is intake of water as a result of disturbed consciousness or excessive loss from the gastrointestinal tract, diaphoresis, the diuretic phase of acute tubular necrosis, diabetes insipidus, or excessive osmotic load either from dehydrating agents or tube-fed protein diets.**’-9 It has been suggested that hypernatremia in many patients with central nervous system lesions is a nonspecific phenomenon.8JOJ1 However, in a few patients, careful investigation has shown various combinations of impaired thirst sensation and antidiuretic hormone (ADH) secretion.12 Hays et al.13 reported the case of a 40-yearold patient in whom hypernatremia resulting from impaired thirst sensation in the presence of a normal ADH response could be ascribed to hydrocephalus, which they thought was most probably due to aqueductal stenosis. We recently have had the opportunity to carry out a postmortem examination on the brain of this patient and wish to report the further course and the neuropathological findings.
3 citations