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Biomedical subjects

A Mastri

Publications and source records attributed to A Mastri.

14 recordsLinked to original sources

Delayed traumatic midbrain syrinx. Clinical, pathologic, and electrophysiologic features.

Seven months following severe crushing closed-head trauma with initial excellent recovery, neurologic deficits referable to the right mesencephalon abruptly developed in a 28-year-old man. Computed tomography demonstrated a cystic midbrain lesion with apparent communication with the aqueduct (later confirmed at autopsy). Brain-stem auditory evoked potentials after the clinical deterioration showed depression of amplitude of wave V, compared with predeficit records, only when the ear contralateral to the brain-stem lesion was stimulated.

Adult↗

Maternal phenylketonuria-chronology of the detrimental effects on embryogenesis and fetal development: pathological report, survey, clinical application.

Maternal phenylketonuria (PKU) is likely to have detrimental effects on embryogenesis and fetal development. Manifestations in the offspring include spontaneous abortion, various congenital malformations, intrauterine growth retardation, and microcephaly. The time at which the metabolic abnormalities induce pathologic embryogenesis can be documented by knowing the time of the development of specifically damaged organ systems. This review reveals that, while the most recognized congenital malformations occur in the heart, the most common abnormality is growth inhibition occurring throughout pregnancy. The organ system most commonly affected by this growth inhibition is the brain, resulting in a high incidence of micrencephaly. It appears that maternal phenylketonuria interferes with appropriate fetal growth and that this effect occurs during the entire course of pregnancy and has no tissue specificity. This information can be both informative to pathologists and useful to clinicians.

Embryonic and Fetal Development↗

Fatal intracranial extension of spinal hemorrhage after lumbar puncture.

Lumbar puncture (LP) is sometimes performed in addition to computed tomography (CT) to exclude hemorrhage prior to anticoagulation for suspected cerebral ischemia. The LP may be complicated by spinal hemorrhage during subsequent anticoagulation. We report such a case with an eventually fatal outcome due to intracranial extension of blood. On balance, the use of LP in addition to CT in this setting appears to be unwise.

Aged↗

Dural venous sinus thrombosis in acute lymphoblastic leukemia.

Three children with acute lymphoblastic leukemia developed sagittal sinus thrombosis. One patient was in peripheral remission. One patient survived. In neither patient who died were the walls of the dural sinuses infiltrated with leukemic cells. Attention is drawn to this potentially treatable cause of central nervous system symptoms in childhood leukemia. Angiography is the diagnostic test of choice and can also demonstrate intracerebral hematoma and subdural hematoma, if present. Sinus thrombosis can occur either during exacerbation or remission of the basic leukemic process. The possibility that chemotherapeutic techniques predispose toward this complication is raised.

Adolescent↗

Acute multiple sclerosis with contrast-enhancing plaques.

In a case of clinically fulminant multiple sclerosis (MS), contrast-enhancing lesions were visible by computerized axial tomography (CT). The patient died less than three weeks after the study, making neuropathologic correlation possible. It appears that areas of contrast enhancement correlate in acute MS with early demyelinative lesions, and enhancement is related to breakdown of the blood-brain barrier. That such a disturbance is important in the pathogenesis of demyelination in MS has been suggested by other data as well. The characteristic CT findings of MS must therefore include enhancing lesions that reflect disease activity and that may be the only CT abnormality.

Acute Disease↗

Central hypoventilation syndrome: experience with bilateral phrenic nerve pacing in 3 neonates.

Successful long-term phrenic nerve pacing has been reported in adults with acquired central hypoventilation syndrome. This report summarizes our experience with phrenic nerve pacing in 3 infants with congenital central hypoventilation syndrome. The electrodes were implanted in the lower thoracic portion of each phrenic nerve. In all patients. bilateral simultaneous pacing was required to maintain an adequate arterial PO2, tidal volume, and minute ventilation during quiet sleep. Case 1 died of problems primarily related to the severe cor pulmonale that had been present before pacemaker insertion; at autopsy, the pacemaker system was intact and there were no significant phrenic nerve abnormalities. Case 2 later developed failure of awake ventilatory control and died because of extensive phrenic nerve damage incurred by 19 days of continuous pacing. Case 3 has received quiet sleep pacemaker support since September 1977 and has been able to maintain normal quiet sleep ventilation in this manner. Phrenic nerve pacing can be successful in infants as long as continuous pacing is not required. Bilateral simultaneous pacing appears to be an appropriate alternative to home-based intermittent positive-pressure breathing for long-term management of children with central hypoventilation syndrome.

Electric Stimulation↗

Posttraumatic cerebral hemispheric disconnection syndrome.

Gross and microscopic lesions of the corpus callosum and neighboring structures are common in severe closed head injury. This report is the first, to our knowledge, to confirm neuropathologically the occurrence of extensive traumatic destruction of the corpus callosum in a patient with left-sided apraxia and agraphia. It also demonstrates that large traumatic lesions of the corpus callosum may occur without prolonged posttraumatic coma, vegetative state, or death. In our patient, coexisting extracallosal hemispheric lesions may have modified the effects of callosal pathology. Cases of this type may be more common than generally appreciated, but since symptoms of hemispheric disconnection are not apparent in ordinary behavior, specific tests of callosal function must be employed if disconnection phenomena are to be detected in the posttraumatic period.

Aged↗

Leptomeningeal artery atherosclerosis visualized by angiography: clinical correlates.

Circumscribed atherosclerotic involvement of secondary and tertiary branches of major cerebral arteries is a common angiographic finding whose nature is rarely in question. However, widespread and severe changes are unusual, and radiologic interpretation may be more difficult. We recently cared for a patient whose angiogram demonstrated extensive involvement of leptomeningeal vessels and were prompted to review the clinical courses and autopsy findings of a number of other patients with similar angiographic findings. Our observations suggest that the radiological appearance of leptomeningeal artery atherosclerosis can be confused with that of an arteritis. Atherosclerosis of leptomeningeal arteries is strongly associated with a history of arterial hypertension and seems to parallel arterial lesions thought responsible for lacunar infarction and intraparenchymal hemorrhage.

Arachnoid↗