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Biomedical subjects

F J DiMario

Publications and source records attributed to F J DiMario.

51 records · Page 3Linked to original sources

Acute mental status changes in children with systemic cancer.

Acute changes in mental status (AMS) develop in children with cancer from a multitude of cancer- and treatment-related complications. To determine the incidence, etiology, and outcome of children with cancer who had AMS, the medical records of all children under 18 years of age with systemic cancer (excluding primary central nervous system tumors) who had AMS in our institution during the years 1981 through 1987 were reviewed. AMS developed in 89 of 815 children at risk (11%). The AMS was caused by seizures in 53 (60%), an encephalopathy in 24 (27%), and a stroke syndrome in 12 (13%). AMS occurred in 42 of 305 (14%) with leukemia, 16 of 139 (12%) with lymphoma, 14 of 136 (10%) with sarcoma, 10 of 104 (9%) with neuroblastoma, and 7 of 104 (5%) with other malignancies. Children with acute lymphocytic leukemia were more prone to having seizures (61%), while children with nonacute lymphocytic leukemia were almost equally likely to have encephalopathies, strokes, or seizures. Children with lymphoma were admitted for treatment most often with an encephalopathy (44%). Etiologies for AMS were evaluated vigorously, and one or more etiologies were identified in 80 of 89 (89%) patients. Dependent on the type of tumor, the anticancer treatment used and, timing during the course of illness AMS occurred, specific diagnoses were more likely. Neurologic morbidity and mortality were dependent on the cause of AMS. Children with seizures that were initially difficult to control were more likely to require long-term anticonvulsant therapy.

Acute Disease↗

Symmetrical thalamic degeneration with calcifications of infancy.

We describe the clinical and radiographic features of three premature infants with symmetric thalamic calcification recognized by computed tomographic scan on days 6, 12, and 49 of life and contrast our findings with those reported in the literature. These lesions follow prepartum or intrapartum hypoxiaischemia and are clinically distinguished by prominent bulbar dysfunction, featuring weak or absent cry, poor feeding, and facial weakness. Neonatal thalamic calcification in premature infants may serve as a radiological marker of an acute, short-lived hypoxic-ischemic event. The presence of brain-stem dysfunction, particularly of lower cranial nerves in association with thalamic calcifications, constitutes a distinctive clinical-radiological entity and usually portends a poor outcome. The presence of these calcifications implies that injury was sustained to diencephalic and brain-stem structures at least 2 to 4 weeks prior to their appearance on computed tomographic scan.

Atrophy↗

The cerebral palsy patient.

CP is not a disease but rather a descriptive term that identifies a heterogeneous group of children who manifest primarily chronic motor impairment. It should be appreciated that the large group of children identified under this heading have a broad range of associated handicaps. It is also important to recognize that there is an extremely wide range of variability in terms of the degree of impairment each child may exhibit. Therefore, appropriate management strategies and family counseling must be individualized. The importance of communicating with families cannot be overstressed. The management of children will require expertise in many fields. A multidisciplinary approach is therefore preferable for the comprehensive management of children with CP. If a specialized multidisciplinary clinic setting is not available, an "integrated effort coordinator," the primary physician, is essential to optimize care of the child with CP.

Cerebral Palsy↗

Multiple sclerosis presenting at 4 years of age: clinical and MRI correlations.

The authors present a patient with multiple sclerosis (MS) whose onset of illness was at 4 years of age. Three separate and distinct episodes of central nervous system (CNS) dysfunction associated with cerebrospinal fluid (CSF) pleocytosis, electroencephalographic (EEG) abnormalities, and extensive CNS white matter demyelination on both computerized tomography (CT) and magnetic resonance imaging (MRI) characterized her clinical course. Two episodes responded well to prednisone therapy.

Brain↗

Myasthenia gravis and primary squamous cell carcinoma of the thymus: a case report.

We detail a case of primary squamous cell carcinoma of the thymus in a patient with myasthenia gravis. The clinical course of the patient and the gross and histologic appearance of the thymus are discussed. Appropriate therapy for patients with these tumors includes resection of the tumor followed by radiation therapy and continued surveillance for recurrence. Review of the literature indicates less than 50% survival at 10 years and 22% recurrence rate at 5 years for patients with this tumor.

Carcinoma, Squamous Cell↗

The natural history of night terrors.

Night terrors are a sleep disorder, resulting from a partial arousal during slow-wave sleep. They usually occur within 2 hours of sleep onset and are characterized by agitation and unresponsiveness to external stimuli. Nineteen children (ten males, nine females) with onset of night terrors before age 7.5 years were studied by means of a questionnaire. Mean observation time (time from onset age to age at survey) was 8.5 years, but longer than 10 years in nine subjects. Seventy percent of the children had their initial frequency of night terrors as their peak frequency, with a tendency for shorter duration of the parasomnia in this group. Children with onset age less than 3.5 years may be expected to attain a peak frequency of at least one episode per week. Children with onset after 3.5 years, but before 7.5 years, may expect to attain a peak frequency of 1-2 episodes per month. There was a mean duration of 3.9 years, with a tendency for longer duration in children with positive family histories of sleep walking. Fifty percent stopped by age 8 years; 36 percent continued into adolescence. No common abnormal behavioral profile or psychopathology was found. Common precipitants of attacks were not identified.

Child↗

Lacunar infarction of the basal ganglia as a complication of hemolytic-uremic syndrome. MRI and clinical correlations.

Central nervous system (CNS) complications of hemolytic-uremic syndrome (HUS) commonly consist of alterations in mental status, seizures, and rarely hemiparesis. The authors report the clinical evolution of left hemiparesis and later choreo-athetoid movements in a patient who sustained a right lacunar infarction as a complication of HUS. The infarction is demonstrated on magnetic resonance imaging (MRI).

Athetosis↗

Transient oculomotor nerve paresis in congenital distal basilar artery aneurysm.

The clinical and pathologic findings of a 10-month-old girl with congenital heart disease who died after rupture of a congenital distal basilar artery aneurysm are reported. The patient developed transient minimal oculomotor nerve paresis 7 days prior to suffering a massive subarachnoid hemorrhage. The finding of transient third nerve dysfunction, particularly in the context of recurrent syncope, should prompt investigation for an intracranial arterial aneurysm.

Basilar Artery↗

Autonomic nervous system function in severe breath-holding spells.

This study attempted to determine noninvasively whether a dysregulation of autonomic nervous system reflexes exists in children with severe cyanotic breath-holding spells (BHS). This was a cross-sectional study performed in the neurophysiology laboratory at a tertiary care hospital. Patients, 18 months of age and older taken from a referral population of children with severe cyanotic BHS and normal controls, were studied. Fourteen children with cyanotic BHS and 12 controls were evaluated. Several noninvasive measures of parasympathetic and sympathetic reflex functions were assessed and statistically analyzed using analysis of variance and covariance, and Fisher's exact tests. The cyanotic BHS group had a significantly greater increase in pulse rate at 15 s of standing after rising from the supine position (P < .06), with a trend toward a concomitant higher mean arterial pressure (P < .09). After adjusting for age, sex, and mean supine systolic and diastolic blood pressures, breath-holders had a greater decrease in diastolic blood pressure (P < .02) without an increase in systolic blood pressure after standing from the supine position. Breath-holders also had significantly abnormal 30:15 R-R interval ratios compared with controls (P < .002). These results support the hypothesis that children with cyanotic BHS have underlying autonomic nervous system dysregulation. This dysregulation may contribute to the pathophysiology of severe BHS in these children.

Autonomic Nervous System↗