Surgical Treatment of Intractable Seizures Due to Hypothalamic Hamartoma

Summary: A 6.5-year-old boy developed seizures at age 2.8 years consisting of episodes of unconsciousness and laughing attacks. By age 6 years, multiple seizure types, including generalized tonic-clonic (GTC), complex partial (CPS) and akinetic seizures, and drop attacks were occurring several times daily. EEG showed multifocal epileptic discharges. Antiepileptic drugs (AEDs) did not control the seizures. With progression of the epilepsy, cognitive deterioration developed. There were no manifestations of precocious puberty. Neuroimaging disclosed a suprasellar mass in continuity with the hypothalamus, and a diagnosis of hypothalamic hamartoma was made. After surgical resection of the hamartoma, the seizures were completely alleviated, and the epileptic EEG discharges disappeared. Improvement of mental function was also noted.     

The role of gallium 67 imaging in non-Hodgkin's lymphoma of the gastrointestinal tract

To evaluate the clinical usefulness of gallium 67 imaging in the detection of gastrointestinal (GI) non-Hodgkin's lymphoma (NHL) and in the assessment of the therapeutic effects, images were reviewed in 24 cases (25 lesions: stomach, 20; ileum, 2; and terminal ileum and/or cecum, 3) and were compared using barium studies and, in 16 cases, computerized tomography (CT). In all, 23 (92.0%) of the 25 lesions were detected by⁶⁷Ga citrate imaging, the barium studies detected all 25, and CT detected 15 of 16 lesions (93.8%). The two lesions not identified by imaging and the one not found by CT were the smallest of all. In 2 (8.7%) of the 23 lesions positively identified by⁶⁷Ga-citrate imaging, both CT and imaging revealed the extent of the tumor more accurately than did the barium studies. In all but one of the patients, a close correlation existed between the imaging results and the therapeutic effects. These data suggest that⁶⁷Ga imaging is useful in conjunction with CT and barium studies for the detection of GI NHL and for the assessment of both the spatial extent of disease and the therapeutic effects, although a lack of⁶⁷Ga uptake after therapy does not always indicate a good therapeutic effect.     

A 14-year-old with pulmonary hamartomatous lymphangiomyomatosis associated with bilateral pneumothoraces]

A 14-year-old girl was admitted because of cough, chest pain and hemosputum. Chest roentgenogram on admission showed a pneumothorax and a cavitary lesion with niveau formation in the right lung and cystic lesions in the bilateral lung fields. After bed rest and intravenous administration of antibiotics for two weeks, the right lung inflated well and the niveau formation disappeared, and the patient was discharged. One week later, she was readmitted with sudden-onset severe dyspnea, caused by bilateral pneumothoraces. Emergency tube thoracostomy and wedge resection of the bullous lesion was performed. Macroscopically, multiple small cystic changes were seen on the surface of the right lung. Histological examination revealed nodular proliferations of smooth muscle cells in the interstitium and vessel walls in the lung, which contained slit-like lymphatic channels. The diagnosis of pulmonary lymphangiomyomatosis was made. In this case, we could not measure receptors for estrogen and progesterone. Recently, hormonal therapy and oophorectomy have been reported as being useful. Tamoxifen (Norvadex) was therefore initiated, and the patient has remained well with slight dyspnea on exertion. There has been no recurrence of pneumothorax. Lymphangiomyomatosis is a rare disease of unknown etiology which occurs exclusively in women, mostly in those of reproductive age. We report a 14-year-old female patient with lymphangiomyomatosis associated with repeated pneumothorax, who had been under treatment for epilepsy. We believe this case to be of importance because of the long discussed relation between pulmonary lymphangiomyomatosis and tuberous sclerosis.     

Trends in antihypertensive drugs used for elderly patients]

The admission of antihypertensive medications in the elderly (65 y.o. or more, 269 pts) was compared to that in younger cases (less than 65 y.o., 348 pts). All were outpatients who visited our clinic in 1990. The number of patients on a single therapeutic regimen was almost equal in both age groups. Once-a-day regimens were more common in the young (56.9% vs 48.3%, p less than 0.05), and three times-a-day regimens were more common in the elderly (14.7% vs 23.1%, p less than 0.01). The choice of antihypertensive drugs in patients with single therapy or combined therapy in the young was beta blockers in 49.7%, Ca blockers in 39.4%, diuretics in 30.7% and ACE inhibitors in 17.8%, and those in the elderly were Ca blockers in 46.1%, diuretics in 44.2%, beta blockers in 33.8%, and ACE inhibitors in 16.4%. The patients were subdivided into three groups according to the time of the initial visit to the clinic; initial visit during 1969-1979 (phase 1), 1980-1984 (phase 2) and 1985-1990 (phase 3). In the young, choice of beta blockers and diuretics was most popular in phase 1. However, the choice of diuretics decreased in phase 2, and in phase 3 beta blockers were used in 50.4%, Ca blockers in 43.2%, ACE inhibitors in 22.3% and diuretics in only 17.3%. In the elderly, diuretics were most popular followed by Ca blockers and beta blockers in phase 1 and phase 2. In phase 3 Ca blockers were selected in 58.2% followed by both beta blockers and ACE inhibitors in 28.4% then diuretics in 23.9%.(ABSTRACT TRUNCATED AT 250 WORDS)     

Visibility of epidermoid tumors on steady-state free precession images.

PURPOSETo determine whether steady-state free precession sequences improve the MR visibility of epidermoid tumors in comparison with spin-echo images.METHODSPatients were four women and three men with epidermoid tumors in the subarachnoid spaces. MR was performed with a 1.5-T superconductive unit. For steady-state free precession imaging, three-dimensional Fourier transform fast imaging with steady-state free precession (FISP) images were used (20-40/7 [repetition time/echo time], flip angle of 25 degrees). The visualization and contrast-to-noise ratio were compared in FISP images and spin-echo images. In one case, the images of FISP and fast low-angle shot were obtained with variable repetition times and flip angles to evaluate the best pulse sequences for the visualization of epidermoid tumors.RESULTSThe contrast-to-noise ratios between tumors and cerebrospinal fluid ranged from 7.9 to 17.5 (average was 12.9) on FISP images. The average of contrast-to-noise ratios on T1, T2, and proton density-weighted spin-echo images were 1.6, 2.0, and 4.2, respectively. Three-dimensional Fourier transform FISP images best showed central nervous system and demonstrated epidermoid tumors excellently.CONCLUSIONSEpidermoid tumors in the subarachnoid spaces were better demonstrated on steady-state free precession (three-dimensional Fourier transform FISP) than on conventional spin-echo images.     

Germinoma originating in the basal ganglia and thalamus: MR and CT evaluation.

PURPOSETo describe MR and CT features of germinoma originating in the basal ganglia and thalamus and to discuss the roles of each modality for its diagnosis.METHODSMR and CT studies of six cases of germinomas, five of which were histologically proved, were retrospectively reviewed. T1-weighted, T2-weighted, and contrast-enhanced T1-weighted conventional spin-echo images, and unenhanced and contrast-enhanced CT images were evaluated.RESULTSTypically, the tumor consisted of an irregular solid area with contrast enhancement and various-size cysts. Cystic components were found in five cases and calcification in four. Intratumoral hemorrhage was noted in one. Ipsilateral cerebral hemiatrophy and brain stem hemiatrophy were noted in three cases each. MR was superior to CT in evaluating precise tumor extension, cystic components, and intratumoral hemorrhage, although in one case, extension of the tumor was better defined on CT in its early stage. Calcification was difficult to identify by MR alone. The solid components of the tumors generally showed slightly high density on CT, which seemed to be characteristic compared with nonspecific intensity pattern on MR.CONCLUSIONThe combination of CT and MR findings allows early detection and appropriate diagnosis of the mass in the basal ganglia and/or thalamus.     

An Autopsied Case of Interferon Encephalopathy

Abstract: A 78-year-old male with renal carcinoma was treated with a high dose infusion of interferon-alpha (IFN-alpha) for eight months. The patient had evidence of organlc brain syndrome such 88 : dysfunction of memory, slowing of behavior, and development of mental confusion that appeared eight months after the treatment. MRI at the time of mental confusion revealed difise white matter lesions. Neuropathologic findings were compatible to Binswanger's disease and Senile Dementia of Alzheimer Type (SDAT), Preexisting neurologic abnormalities including intracerebral arteriosclerosis and cerebral atrophy may increase susceptibility to unacceptably severe IFN neurotoxicity.     

Effects of intraaortic balloon position on renal artery blood flow.

Debate continues over what happens to renal blood flow when intraaortic balloons are adjacent to the renal arteries. Fourteen dogs were prepared by implanting instruments to measure heart rate; right atrial, pulmonary arterial, carotid arterial, and femoral arterial pressures; cardiac index; mixed venous oxygen saturation; urine output; and left and right renal blood flows. A 12-mL intraaortic balloon was inserted through the left (n = 9) or right (n = 5) femoral artery. The position of the balloon was randomized so that it was initially placed in either the control (thoracic) or renal position (at the level of the renal arteries). Intraaortic balloon pumping was performed for 4 hours in each position. In 8 dogs, at least one of the renal arteries had partial occlusion, 23% to 98% decrease in flow (mean decrease, 66%), while the intraaortic balloon was in the renal position. An intraaortic balloon in the renal position results in lower renal blood flow as well as a high risk (57%) of selective renal artery occlusion. Decreased renal blood flow is not apparent using conventional monitoring, as hemodynamics do not change.