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Biomedical subjects

R Tien

Publications and source records attributed to R Tien.

14 recordsLinked to original sources

Successful therapy for trilateral retinoblastoma.

Trilateral retinoblastoma, the intracranial malignancy associated with bilateral retinoblastoma, is an uncommon and clinically aggressive malignancy with a uniformly fatal outcome. Three children with newly diagnosed trilateral retinoblastoma were treated with systemic (cyclophosphamide and vincristine) and intrathecal (methotrexate, hydrocortisone, and cytarabine) chemotherapy, as well as craniospinal irradiation (one patient) in addition to therapy of the eye lesions. All three patients have had partial or complete response of the pineal tumors to chemotherapy, with no active disease eight or more years, 33 or more months, and 12 or more months, respectively, after diagnosis of the lesions.

Antineoplastic Combined Chemotherapy Protocols

Hyponatremic encephalopathy: is central pontine myelinolysis a component?

PURPOSE: Severe hyponatremia is often associated with permanent brain damage. There has been substantial controversy about whether central pontine myelinolysis (CPM), a rare neurologic disorder of uncertain etiology, can complicate either hyponatremia or its therapy. This study was undertaken to determine how often hyponatremic patients with the clinical diagnosis of CPM actually have the disorder as an integral structural component of their encephalopathy. PATIENTS: Analyses were carried out in 20 patients who had severe symptomatic hyponatremia and a presumptive diagnosis of CPM, based on clinical and/or neuroradiologic findings. All had been referred for neuroradiology consultation. The mean age (+/- SD) was 47 +/- 14 years, the lowest serum sodium level was 104 +/- 8 mM, and 85% of the patients were female. The etiologies were diverse and included postoperative status, thiazide diuretics, polydipsia, infection, acute renal failure, chronic alcoholism with emesis, and beer potomania. METHODS: The original and subsequent films of 20 patients were reevaluated retrospectively by two neuroradiologists. The clinical course was also reevaluated, and in eight patients, the postmortem brain findings were reviewed. The diagnosis of CPM was made only on the basis of strict criteria relating to either (1) pathologic findings of CPM on postmortem examination; or (2) computed tomographic scan and/or magnetic resonance imaging findings diagnostic of CPM. RESULTS: No pontine lesions were present in 15 of 20 patients in whom the diagnosis of CPM had initially been made. All 15 had extrapontine demyelinating lesions but the pons was normal. Two others had only lateral pontine lesions, so that only three of 20 patients had definite CPM. All but one of the 20 hyponatremic patients had a definite hypoxic event prior to any therapy with intravenous sodium chloride. The involved brain areas included basal ganglia, thalamus, cortical gray matter, and periventricular white matter, areas often affected by hypoxia. Each of the three patients in whom unequivocal findings of CPM were present had long histories of chronic alcoholism and hepatic cirrhosis. CONCLUSIONS: These results suggest that: (1) Neither hyponatremic encephalopathy nor its therapy is commonly associated with CPM; (2) Patients with chronic alcoholism who also become hyponatremic can develop pontine demyelinating lesions; (3) Most patients with symptomatic hyponatremia who are diagnosed as having CPM in fact have diffuse cerebral demyelinating lesions with a normal pons; (4) The distribution of cerebral demyelinating lesions in patients with hyponatremic encephalopathy is compatible with hypoxic damage.

Adult

Amyloid of the facial nerve.

Although facial nerve paralysis has been reported in association with amyloidosis, histologic confirmation of facial nerve involvement with amyloid has not been previously demonstrated. A case of localized primary amyloidosis of the facial nerve is presented, and a new magnetic resonance technique for imaging the facial nerve is described.

Aged

Contrast-enhanced MR imaging of the facial nerve in 11 patients with Bell's palsy.

Contrast-enhanced MR images (at 1.5 T) were obtained in 11 patients with facial palsy. The group included five people with acute idiopathic facial (Bell's) palsy, three with chronic idiopathic facial palsy, and one each with acute facial palsy after local radiation therapy, acute facial palsy resulting from herpes zoster virus infection, and facial palsy caused by facial neuroma. Eight of the 11 patients demonstrated marked enhancement of the affected facial nerve from the labyrinthine portion through the descending canal. Three patients also demonstrated mild enhancement of the distal canalicular portion of the facial nerve, simulating small distal acoustic neuromas. No difference in the pattern of enhancement between the acute or chronic Bell's palsy patients was seen. Radiographic resolution appeared to lag behind clinical resolution. The facial neuroma appeared distinct from the other lesions as a focally enhancing mass. The enhancement pattern in the Bell's group correlated with the histopathologic features of Bell's palsy and is consistent with the viral hypothesis of the syndrome. Thin-section contrast-enhanced MR scans are recommended for individuals with atypical presentation of facial paralysis. In the proper clinical setting, contrast-enhanced MR imaging may permit a positive radiographic diagnosis of Bell's palsy, which has previously been a diagnosis of exclusion.

Adult

Circumferential perirenal urinoma mimicking nephromegaly on urography.

Four cases of circumferential perirenal urinoma in patients with cancer are described. Intravenous urography showed evidence of apparent renal enlargement. In 1 case, opacification of extravasated urine was dense enough to be seen on the urogram, and the diagnosis was confirmed by computed tomography (CT). In the other cases, sonography followed by CT diagnosed urinoma, after tumor invasion had been suggested because of the pseudonephromegaly. Ureteral obstruction was demonstrated in all cases, due to retroperitoneal adenopathy in 1 case and to pelvic tumor in the other 3. Sonography or CT is required for the diagnosis of circumferential perirenal urinoma mimicking nephromegaly urographically.

Adult

Thoracic gallium uptake in patients with lymphomatoid granulomatosis.

Lymphomatoid granulomatosis (LG) is a rare condition with histological similarities to Wegener's granulomatosis and malignant lymphoma. Characteristically there is an angiocentric, angiodestructive lymphoreticular cell infiltrate. The lungs are usually affected, and, less frequently, the skin, nervous system, kidney, and bowel are involved. The prognosis is poor and frank lymphoma develops, in some cases terminally. The usual radiological appearance of the lungs consists of bilateral nodular lower zone opacities. The authors report two patients (siblings) with LG, and their gallium scans are presented. In each case there was a significant accumulation of gallium in the lungs at times of clinically active disease. The limited role of gallium imaging in this disease is discussed.

Child

MR imaging of the brain in patients with diabetes insipidus.

Diabetes insipidus is a clinical syndrome characterized by the excretion of copious volumes of dilute urine combined with persistent intake of abnormally large quantities of fluid. There are two general forms of the disease, central (vasopressin deficient) and nephrogenic (vasopressin resistant). Diabetes insipidus of central origin most often results from lesions in the hypothalamic-neurohypophyseal axis. Twenty-six cases of central diabetes insipidus were evaluated with the use of high-field-strength MR imaging. A wide variety of precipitating conditions were found, including Langerhans cell histiocytosis, neoplasia, trauma, and infection. A thickened pituitary infundibulum was seen in most patients, and an absence of high intensity signal in the posterior pituitary lobe on T1-weighted images was seen in every case. Analysis of stalk morphology; associated brain findings; and correlation with the patient's age, sex, history, and radiographs of other body parts improved diagnostic specificity. When combined with clinical information, MR imaging is able to provide a specific diagnosis in almost all cases of central diabetes insipidus.

Adolescent

Contrast-enhanced MR imaging of the facial nerve in 11 patients with Bell's palsy.

Contrast-enhanced MR images (at 1.5 T) were obtained in 11 patients with facial palsy. The group included five people with acute idiopathic facial (Bell's) palsy, three with chronic idiopathic facial palsy, and one each with acute facial palsy after local radiation therapy, acute facial palsy resulting from herpes zoster virus infection, and facial palsy caused by facial neuroma. Eight of the 11 patients demonstrated marked enhancement of the affected facial nerve from the labyrinthine portion through the descending canal. Three patients also demonstrated mild enhancement of the distal canalicular portion of the facial nerve, simulating small distal acoustic neuromas. No difference in the pattern of enhancement between the acute or chronic Bell's palsy patients was seen. Radiographic resolution appeared to lag behind clinical resolution. The facial neuroma appeared distinct from the other lesions as a focally enhancing mass. The enhancement pattern in the Bell's group correlated with the histopathologic features of Bell's palsy and is consistent with the viral hypothesis of the syndrome. Thin-section contrast-enhanced MR scans are recommended for individuals with atypical presentation of facial paralysis. In the proper clinical setting, contrast-enhanced MR imaging may permit a positive radiographic diagnosis of Bell's palsy, which has previously been a diagnosis of exclusion.

Adult