MR imaging study of edema along the optic tract in patient with Rathke's cleft cyst]

We report an 80-year-old woman who was admitted to our hospital with symptoms due to diabetes insipidus. Magnetic resonance (MR) imaging demonstrated a sellar/suprasellar cystic lesion with marginal enhancement and the thick pituitary stalk. The MR imaging depicted edema spreading along the optic tract on fluid-attenuated inversion recovery (FLAIR) images. Upon neurological examination at the time of admission, there were no abnormal findings affecting the field of vision or visual acuity. In endocrinological examination, the basal plasma values of pituitary hormones were within normal range except for that of prolactin, which was 47.9 ng/ml. The preoperative diagnosis was craniopharyngioma, and the intrasellar mass was partially removed by the endoscopic transnasal transsphenoidal approach. Postoperative histopathological examination revealed Rathke's cleft cyst associated with squamous metaplasia. Lymphocytic infiltration was also confirmed in both the anterior and posterior pituitary lobe. The postoperative course was satisfactory. Edema spreading along the optic tract was reported as a characteristic MR imaging finding for diagnosis of craniopharyngiomas or optic nerve glioma. However, it is suggested that edema of the optic pathway seems to be caused not only by craniopharyngioma but also other suprasellar lesions. It was a rare case of secondary lymphocytic hypophysitis caused by Rathke's cleft with edema along the optic tract.     

Analysis of unknown cause subarachnoid hemorrhage with repeated negative angiogram]

Seven hundred and fifty five cases of acute non-traumatic subarachnoid hemorrhage were admitted to the department of neurosurgery of our hospital from July, 1995 to March, 2004. In 555 patients cerebral angiography was conducted but initial angiography was negative in 30 patients. Except 10 general condition poor patients, in 20 initial angiogram-negative patients were undergone repeated angiography. The cause of SAH could not be demonstrated in 13 cases. The SAH in perimesencephalic and non-perimesencephalic cisturns was seen in 7 and 6 cases, respectively. Occipital and/or neck pain on admission was statistically more common among patients with perimesencephalic SAH than those with non-perimesencephalic SAH (p = 0.029), and the prognosis of perimesencephalic SAH was good. We conclude that repeat angiography should not be recommended in patients with perimesencephalic SAH. Patients with non-perimesencephalic SAH had a higher rate of complication. In the non-perimesencephalic group, 3 patients developed hydrocephalus and 3 patients had vasospasm, which were found by repeated angiography. Therefore, repeated angiography is recommended for better clinical outcome by early detection and management of serious complications in this group of patients.     

A case of splenic vein occlusion caused by the intravenous tumor thrombus of nonfunctioning islet cell carcinoma

We report herein a case of successfully treated advanced, nonfunctioning islet cell carcinoma associated with left-sided portal hypertension. The splenic vein was obstructed by a huge intravenous tumor thrombus developing from the main pancreatic tumor. Direct invasion to adjacent organs such as the spleen, colon, left kidney and stomach was also observed, although liver metastasis was not present. Radical resection was carried out with removal of these five involved organs and the patient is alive without recurrence more than 5 years after surgery.     

An Application of Endoscopic Color Doppler Ultrasonography (ECDUS) in the Diagnosis of Hemodynamics of Gastric Varices,and the Therapeutic Effect of Endoscopic Therapy

Abstract: We studied 14 patients using endoscopic color Doppler ultrasonography (ECDUS) to evaluate the hemodynamics of gastric varices, and evaluated the endoscopic therapeutic effects on gastric varices in 8 patients. Three patients had F₃ type gastric varices and eleven had F₂. The ECDUS was performed with a PENTAX FG-32UA (7.5MHz, convex type) and a HITACHI EUB 565 was used as a display machine. The intramural blood flow in the gastric varices and inflows from the extra-gastric wall were clearly observed with the ECDUS in all 14 patients. The extramural blood flow (gastro or spleno-renal shunts) was detected in 9 of 14 patients. The velocity of the intramural flow in tumorous type varices (F₃) was higher than in the nodular or flat elevated type (Fa). Next, we evaluated the therapeutic effects on gastric varices of the ECDUS. The successful disappearance of intramural blood flow was observed in 6 of 8 patients who had this endoscopic therapy. In two of the 8 patients, there was not enough therapeutic effect on the intramural blood flow. The extramural blood flow, however, did not change before or after endoscopic therapy with the ECDUS. Therefore, we concluded that ECDUS is a very useful modality for the diagnosis of hemodynamics and to evaluate the therapeutic effects on gastric varices.     

Pyramidal tract degeneration in sporadic Creutzfeldt‐Jakob disease

We investigated the neuropathologic features of pyramidal tract degeneration in 27 cases of sporadic Creutzfeldt‐Jakob disease (sCJD). The present study included 13 cases of subacute spongiform encephalopathy (SSE), 13 cases of panencephalopathic‐type (PE‐type) sCJD and one case of thalamic‐type sCJD. Pyramidal tract degeneration was observed in PE‐type sCJD cases with disease duration of more than 13 months. Cases of SSE, PE‐type sCJD of relatively short duration and thalamic‐type sCJD showed no pyramidal tract degeneration. All cases with pyramidal tract degeneration showed severe cerebral neocortical involvement with widespread cerebral white matter degeneration. The loss of myelin and axons and macrophage infiltration and fibrillary gliosis seen in these pyramidal tract lesions is consistent with secondary tract degeneration due to cerebral neocortical involvement. Interestingly, some cases with pyramidal tract degeneration showed a distal‐dominant pathology that we believe indicates retrograde degeneration or a “dying‐back” phenomenon of the pyramidal tract. Our results indicate that pyramidal tract degeneration is a late pathologic event in sCJD. Investigation of pyramidal tract degeneration may help clarify the mechanisms underlying the development of sCJD white matter pathology, particularly that of PE‐type sCJD.     

Determination of ABO genotypes with DNA extracted from formalin-fixed,paraffin-embedded tissues

Summary The gene encoding the specific glycosyltransferases which catalyze the conversion of the H antigen to A or B antigens shows a slight but distinct variation in its allelic nucleotide sequence and can be divided into 6 genotypes when digested with specific restriction enzymes. We extracted DNA from formalin-fixed, paraffin-embedded tissues using SDS/proteinase K treatment followed by phenol/chloroform extraction. The sequence of nucleotides for the A, B and O genes was amplified by the polymerase chain reaction (PCR). DNA fragments of 128 by and 200 by could be amplified in the second round of PCR, using an aliquot of the first round PCR product as template. Degraded DNA from paraffin blocks stored for up to 10.7 years could be successfully typed. The ABO genotype was deduced from the digestion patterns with an appropriate combination of restriction enzymes and was compatible with the phenotype obtained from the blood sample.     

Low-dose combination therapy with temocapril and losartan reduces proteinuria in normotensive patients with immunoglobulin a nephropathy.

This study investigates the ability of low doses of angiotensin-converting-enzyme inhibitors, in combination with angiotensin II receptor blockers, to exert antiproteinuric effects in normotensive and proteinuric outpatients with immunoglobulin A (IgA) nephropathy confirmed by biopsy. We performed a prospective, randomized, 6-month study of the effects of temocapril 1 mg (n=10), losartan 12.5 mg (n=10), and both (n=11) on mild-to-moderate proteinuria 0.76+/-0.35 g/day (range, 0.4 to 1.6 g/day) and renal function. The study subjects comprised 31 normotensive and proteinuric outpatients with IgA nephropathy accompanied by normal, or mild-to-moderately reduced but stable renal function (glomerular filtration rate>50 ml/min) without steroid or immunosuppressive therapy. We prospectively evaluated blood pressure, proteinuria, renal function and biochemical parameters before and after 6 months of therapy. The combination therapy significantly reduced proteinuria (63.2%) compared with either temocapril or losartan alone (41.3% and 36.6%, respectively, p=0.04 and 0.01, respectively). Blood pressure was most decreased in the group that received combination therapy. The reduced proteinuria did not correlate with reduced systolic or diastolic blood pressure or mean arterial pressure in any of the groups. The glomerular filtration rate fell during the first 3 months of combined therapy, but became reversible after a further 3 months of therapy. The combination significantly decreased angiotensin II (p <0.01), and this decrease was greater than that by either drug alone. In conclusion, the effectiveness of the combined therapy may have been at least partly due to the greater inhibition of the action of angiotensin II in patients with IgA nephropathy. This strategy apparently reduced mild-to-moderate proteinuria in patients with normotensive IgA nephropathy.     

Prevalence of nonmotor symptoms in Parkinson's disease in an international setting; study using nonmotor symptoms questionnaire in 545 patients.

2006, there was, no single instrument (questionnaire or scale) for attempting a comprehensive assessment of the wide range of nonmotor symptoms (NMS) of Parkinson's disease (PD). The PD nonmotor group, a multidisciplinary group of experts including patient group representatives developed and validated the NMS screening questionnaire (NMSQuest) comprising 30 items. The NMSQuest is a self completed screening tool designed to draw attention to the presence of NMS. In this paper, we present the results gathered from 545 patients using the definitive version of the NMSQuest highlighting the prevalence of the wide range of NMS flagged in the NMSQuest from consecutive PD patients in an international setting.     

Role of the nucleus of the optic tract in monkeys in relation to optokinetic nystagmus

Nine monkeys were used in order to clarify the role of the nucleus of the optic tract (NOT) in the generation of optokinetic nystagmus (OKN). In 3 monkeys whose NOTs were almost totally damaged, optokinetic stimulus toward the lesioned side failed to generate either eye deviation or OKN and revealed only voluntary saccades, whereas that toward the side contralateral to the lesion generated normal gain of OKN. The phenomenon was identical in either monocular or binocular stimulation. In two of 3 monkeys whose NOTs were partially destroyed, optokinetic stimulus toward the lesioned side produced OKN, but the gain of OKN, but the gain of OKN was at maximum less than 60% in both binocular and monocular stimulation. In the remaining one monkey whose NOT was injured, but superficially, OKN showed normal gain in both directions. In 3 other monkeys whose NOTs were spared, this OKN asymmetry was not observed. Pursuit and saccadic eye movements were normal in all NOT-lesioned monkeys. Visually induced eye movements in the vertical axis were likewise normal. The present experiment suggests that the NOT in monkeys may be the first relay station in the horizontal optokinetic path and that in primates as in non-primates both crossed and uncrossed fibers reach motor centers for OKN via the NOT.