Epilepsy or paroxysmal kinesigenic choreoathetosis?

A 71-year-old man was admitted due to brain infarction. An abnormal floating mass was found in the right atrium by echocardiography. The mass was assumed to be a thrombus. The patient was failed to be in danger of pulmonary embolism, so it was decided to remove surgically. The thrombus was removed under cardiopulmonary bypass including the atrial wall where it was attached, because it was difficult to rule out the possibility of cardiac tumor macroscopically. The tumor showed pathological findings of thrombus. Postoperative course was uneventful.     

Neurologic, MR imaging, and MR spectroscopic findings in eosinophilia myalgia syndrome

BACKGROUND AND PURPOSE: Eosinophilia myalgia syndrome (EMS), a multisystemic disease induced by exposure to L-tryptophan, may result in serious CNS abnormalities. The purpose of this study was to determine the pattern of neurologic characteristics, MR imaging abnormalities, and brain neurometabolites in EMS. METHODS: Sixteen patients with EMS and CNS abnormalities (CNS-EMS) and 12 control subjects underwent evaluation, including medical and neurologic examination, proton MR spectroscopy, and MR imaging. RESULTS: Neurologic findings that were increased in CNS-EMS included minor depression (100%), amnesia (88%), and intermittent confusion (38%), although fatigue (31%), motor disorders (31%), recurrent headache (19%), major depression (13%), and dementia (6%) also occurred, but at a lesser significance. Self-reported disability was markedly increased in CNS-EMS. MR imaging findings included subcortical focal lesions, focal lesions in deep white matter, cortical atrophy, ventricular dilatation, and diffuse and periventricular white matter abnormalities. MR spectroscopic findings established two distinct spectral patterns: 1) increased choline-containing compounds, decreased N-acetylaspartate, and increased lipid-macromolecules, consistent with inflammatory cerebrovascular disease; and 2) increased glutamine, decreased myo-inositol, and decreased choline, consistent with acute CNS injury or metabolic encephalopathy. CONCLUSION: Neurologic abnormalities, self-reported disability, brain lesions, and MR spectroscopic abnormalities are common in CNS-EMS. The pattern of cerebral lesions and neurometabolites is consistent with widespread inflammatory cerebrovascular disease. However, a subgroup of patients with CNS-EMS have neurometabolic changes consistent with a metabolic encephalopathy identical or similar to hepatic encephalopathy. The neurologic abnormalities in EMS and related hypereosinophilic syndromes should be interpreted cautiously, with the recognition that both cerebrovascular injury and secondary metabolic encephalopathies may be involved.     

Eleven cases of paroxysmal kinesigenic choreoathetosis; correlation with benign infantile convulsions

OBJECTIVE: To evaluate the role of the surgical access with regard to the generation of proinflammatory proteins in patients with aortoiliac occlusive disease. DESIGN: Non-random, prospective study of patients undergoing an aortobifemoral bypass procedure. MATERIALS: Twenty-six patients were divided into three groups. In the first group of eight patients, a transperitoneal median laparotomy was used. The second group comprised 10 patients in whom a laparoscopically assisted extraperitoneal minilaparotomy was performed and, in the third group, eight patients were operated on using a retroperitoneal access. METHODS: Biochemical analysis of acute phase proteins and the cytokines interleukin-6 (Il 6), interleukin-8 (Il 8) and tumour necrosis factor (TNF). RESULTS: Aortic cross-clamp and total operative time were significantly longer in the laparoscopic group. After 24 h Il-6 concentrations were significantly higher in the transperitoneal (p < 0.05) and the retroperitoneal group (p < 0.006). After 6 h there was a reduced Il-8 concentration in the laparoscopic group compared to patients with a standard retroperitoneal access. Neither TNF nor acute phase proteins showed any significant alterations. CONCLUSION: Laparoscopic-assisted vascular surgery allows for a smaller incision and reduces the surgical trauma, as it is reflected by interleukin levels.     

Proton MR spectroscopy of childhood adrenoleukodystrophy

PURPOSE: To determine the potential of proton MR spectroscopy to monitor patients with childhood-onset cerebral adrenoleukodystrophy (COCALD). METHODS: Single-voxel MR spectroscopy was performed in 16 children with COCALD (24 examinations) who had had no treatment and in 7 children (13 examinations) who had had bone marrow transplantation. RESULTS: In the untreated children with clinically active COCALD, the metabolite ratios N-acetyl-aspartate (NAA)/creatine (Cr) and NAA/choline (Ch) were decreased while Ch/Cr was increased. This trend agrees well with those reported by other researchers, although different experimental sequences and parameters were used in our study. Comparison of these ratios with those from a control group yielded significant differences in the occipital region. In the children who were clinically stable after bone marrow transplantation, the mean levels of the three ratios were between those of the control subjects and the patients with untreated COCALD: the differences in these ratios approached significance. In patients who had been monitored periodically, MR spectroscopy metabolite ratios correlated well with the dementia rating score, reflecting clinical status. CONCLUSION: There is good correlation between MR spectroscopy metabolite ratios and a patient's clinical status. MR spectroscopy appears to be a useful, noninvasive tool to monitor patients with adrenoleukodystrophy, and it increases the overall sensitivity of MR techniques in clinical applications.     

MR findings in hereditary spherocytosis

It has become clear that the proteolytic enzymes that depend upon a serine residue for their catalytic activity belong to many different families of proteins. We have attempted to group these families in "clans." A clan is defined as a group of families the members of which have a common ancestor. That is to say, they are homologous, although some of the similarities are in the "twilight zone" and the relationships cannot be proven by rigorous statistical methods. We believe we can recognize five, or perhaps six, clans of serine peptidases. In view of the separate evolutionary origins of the serine peptidases in different clans, it is not surprising to find that they may differ greatly and that indeed there are few generalizations that can be applied to all serine peptidases. In contrast, the members of a clan commonly have marked similarities.     

MR findings in pontocerebellar hypoplasia

We present four cases with combined hypoplasia of the cerebellum and the ventral pons-pontocerebellar hypoplasia (PCH). PCH represents an autosomal recessive neurodegenerative disorder with fetal onset. The disease is rare, with less than 20 cases having been reported. The main findings of PCH and the inclusion criteria for our cases can be summarised as progressive microcephaly from birth, pontocerebellar hypoplasia documented by MRI and marked chorea, which may change, later in childhood, to more dystonic patterns. The cerebral cortex becomes progressively atrophic. Motor and mental development are delayed, and epilepsy, mainly tonic-clonic seizures, is frequent. The MRI features in all of our cases were: (1) Hypoplastic cerebellum situated close to the tentorium. The hypoplastic cerebellum has a reduced number of folia, in contrast to the normal number of thin folia in simple cerebellar atrophy. (2) The cerebellar hemispheres are reduced to bean-like or wing-like structures. The cerebellar hemispheres appear to 'float' in the posterior fossa. (3) Markedly hypoplastic ventral pons. (4) Slight atrophy of the supratentorial gyral pattern. (5) Dilated cerebromedullary cistern and fourth ventricle. (6) Delayed myelination of the white matter. (7) No significant disorganisation of brain architecture and no severe corpus callosum defect.     

MR findings in oculocerebrorenal syndrome

Oculocerebrorenal syndrome is an X-linked recessive disorder characterized by congenital ocular abnormalities, mental retardation, renal disease, and metabolic bone disease. We report a case of oculocerebrorenal syndrome and, using T1-, proton density-, and T2-weighted imaging sequences, are able to characterize two distinct white matter abnormalities: one lesion is punctate and has signal characteristics that parallel that of cerebrospinal fluid; a second lesion, found in association with the first, consists of patchy white matter abnormalities that are hypointense on T1-weighted images but hyperintense on proton density- and T2-weighted images.     

Proton MR spectroscopy and neuropsychological testing in adrenoleukodystrophy

Melatonin, the chief hormone secreted by the pineal gland, has been previously shown to inhibit human breast cancer cell growth at the physiological concentration of 1 nM in vitro. In this study, using the estrogen receptor (ER)-positive human breast tumor cell line MCF-7, we have shown that 10 microM L-buthionine-[S,R]-sulfoximine (L-BSO), an inhibitor of gamma-glutamylcysteine synthetase (the rate-limiting enzyme in glutathione synthesis), blocks the oncostatic action of 1 nM melatonin over a 5-day incubation, indicating that glutathione is required for melatonin action. The result was repeated with ZR75-1 cells, suggesting that the glutathione requirement is a general phenomenon among ER+ breast cancer cells. Addition of exogenous glutathione (1 microM) to L-BSO-treated groups restored the melatonin response in both cell lines. Further demonstration of the importance of glutathione was shown using the ER- breast tumor cell line HS578T, which is normally unresponsive to melatonin. Growth in this cell line was inhibited in the presence of 1 microM ethacrynic acid (an inhibitor of glutathione S-transferase) plus 1 nM melatonin, and this effect was blocked with 10 microM L-BSO. We also observed a steady decrease of intracellular glutathione in MCF-7 cells over a 5-day incubation, suggesting that these cells metabolize glutathione differently than do normal cells.     

Proton MR spectroscopy and magnetization transfer ratio in multiple sclerosis: correlative findings of active versus irreversible plaque disease

PURPOSE: To characterize plaques of multiple sclerosis (MS) using both proton MR spectroscopy and magnetization transfer (MT) imaging. METHODS: The magnetization transfer ratio (MTR) was calculated from two series of three-dimensional gradient-recalled acquisition in the steady state (GRASS) images obtained with and without an MT saturation pulse. Proton spectra were acquired using the point-resolved spectroscopy (PRESS) sequence with a voxel size of 1.5 x 1.5 x 1.5 cm3. A total of 28 spectra were obtained in 13 patients who had clinically definitive MS. The spectra were analyzed together with the MTR. RESULTS: A positive relationship was found between the N-acetylaspartate (NAA)/creatine (Cr) ratio and the MTR in MS plaques, whereas no significant correlation was found between the metabolite ratios and the signal intensity on fast spin-echo T2-weighted MR images. CONCLUSION: Small changes in the MTR of MS plaques relative to the MTR of normal white matter may reflect inflammatory changes and edema, whereas larger changes in MTR correlate with decreased NAA/Cr ratio and therefore suggest demyelination and irreversible damage from chronic MS plaques.     

Proton MR spectroscopy in inflammatory and infectious brain disorders

This article reviews the proton MR spectroscopy literature regarding brain infarction and inflammatory diseases. We examine the salient findings reported for bacterial abscesses, intracranial tuberculomas, Creutzfeldt-Jakob disease, herpes simplex encephalitis and HIV. These processes demonstrate specific metabolic profiles which may be useful in differential diagnosis. The results reported in the literature support the view that MR spectroscopy can be employed in longitudinal studies to monitor the response to therapy and therefore may lead to individual optimized treatment effectiveness.     

Coccidioidal spondylitis: MR findings in 15 patients

Jem1p is a DnaJ-like protein of the yeast ER membrane, which is required for nuclear membrane fusion during mating. We have determined the position of translation initiation codon for the JEM1 gene. Translation of Jem1p starts from the second ATG codon of the previously assumed JEM1 open reading frame, leading to the synthesis of a precursor protein of 645 amino acids long. The translated Jem1p precursor contains an N-terminal hydrophobic sequence that functions as a signal sequence and is removed upon import into the ER lumen. Jem1p is peripherally associated with the ER membrane probably through protein-protein interactions.     

Kikuchi disease: CT and MR findings

Two cases of Kikuchi disease showed variable nodal enhancing features, including homogeneous enhancement and focal or extensive nodal necrosis on contrast-enhanced CT scans. At MR imaging, the area of central necrosis was isointense or hypointense on T1-weighted images and had a lower signal than nonnecrotic areas on T2-weighted images. The CT appearance of Kikuchi disease can be variable and can mimic not only lymphoma but various nodal diseases with nodal necrosis, including metastasis and tuberculosis.     

Stage IIIa endometrial carcinoma: MR findings

[PURPOSE]: An attempt was made to evaluate the ability of magnetic resonance (MR) imaging to diagnose stage IIIa endometrial carcinoma. [MATERIALS AND METHODS]: Thirty-three patients with endometrial carcinoma underwent MR imaging and surgery. Surgical staging was classified as I in 21 patients, II in 3 patients and III in 9 patients. The MR images of each patient were retrospectively reviewed by three radiologists. Only the clinical diagnosis of endometrial carcinoma was previously notified. Segmental disruption of the full thickness of the myometrium was considered serosal invasion. Intraperitoneal metastasis was diagnosed according to three criteria (intraperitoneal solid mass of isointensity compared with endometrial lesion, cystic mass excluding benign ovarian cysts, ascites). These evaluations were compared with the surgical findings and analyzed by the kappa statistic. [RESULTS]: The rates of sensitivity and positive predictive value (PPV) for serosal invasion were 33% and 6%, respectively. False positive evaluation frequently occurred when thickness of the intact myometrium was less than 5mm. The rates of sensitivity and PPV for intraperitoneal metastasis were 86% and 72%, respectively. The reason for false negative evaluation was small foci of intraperitoneal metastasis. Overall, sensitivity and PPV for stage IIIa were 86% and 69%, respectively. [CONCLUSION]: MRI was useful in detecting intraperitoneal metastasis of endometrial carcinoma with the exception of diagnosing serosal invasion. It is difficult to detect small foci of peritoneal metastasis. It is necessary to differentiate adnexal metastasis from benign adnexal masses.