The Infundibular Recess Passes through the Entire Pituitary Stalk.

BACKGROUND AND PURPOSE: The infundibular recess (IR), commonly illustrated as a V-shaped hollow in the sagittal view, is recognized as a small extension of the third ventricle into the pituitary stalk. The precise morphology of the human IR is unknown. The present study sought to delineate the morphology of the IR using magnetic resonance imaging. MATERIALS AND METHODS: Subjects included 100 patients without acute cerebral infarcts, intracranial hemorrhage, intrasellar or suprasellar cysts, hydrocephalus, inflammatory disease, or brain tumors. Patients with symptoms of increased intracranial pressure, intracranial hypotension, or pituitary dysfunction were excluded. Thin-sliced, seamless T2-weighted sequences involving the optic chiasm, entire pituitary stalk, and pituitary gland were performed in axial and sagittal planes for each patient. The numbers of slices delineating the pituitary stalk and IR were recorded from the axial images and quantified as ratios. RESULTS: The pituitary stalk consistently appeared as a styloid- or cone-shaped structure with variable inclinations toward the third ventricle floor. The IR was delineated as a smoothly tapering, tubular extension of the third ventricle located in the central portion of the pituitary stalk. In 81 % of patients, the IR passed through the entire length of the pituitary stalk and reached the upper surface of the pituitary gland, which was identified in 40 % of the midsagittal images. CONCLUSIONS: The IR is a cerebrospinal fluid-filled canal passing through the center of the pituitary stalk and connects the third ventricle to the pituitary gland. It may function in conjunction with the pituitary gland.

Cranial arachnoid protrusions and contiguous diploic veins in CSF drainage.

BACKGROUND AND PURPOSE: Studies have suggested that arachnoid villi or granulations found in the walls of the cranial dural sinuses, olfactory mucosa, and cranial nerve sheaths function as outlets for intracranial CSF. However, their role as CSF outlets has not yet been verified. Here we show that arachnoid protrusions and contiguous diploic veins provide an alternative drainage route for intracranial CSF. MATERIALS AND METHODS: Four hundred patients with intact skull, dura mater, and dural sinuses underwent MR imaging to explore arachnoids protruding into the skull and diploic veins. Patients with symptoms of increased intracranial pressure or intracranial hypotension were excluded. For 15 patients undergoing craniotomy, both peripheral and diploic venous blood was collected. Albumin and the CSF-specific biomarkers were measured by enzyme-linked immunosorbent assay. RESULTS: With MR imaging, arachnoid protrusions into the skull and contiguous diploic veins were consistently identified throughout the cranium with their characteristic appearance depending on the cranial region. In addition, elevated amounts of prostaglandin D synthase and cystatin C were confirmed in diploic veins compared with peripheral venous blood. CONCLUSIONS: Diploic veins are distributed ubiquitously throughout the cranium. A portion of the intracranial CSF may be drained through arachnoid protrusions and contiguous diploic veins.

Magnetic resonance imaging appearance of primary spinal extradural Ewing's sarcoma: case report and literature review.

PURPOSE: Primary spinal extradural Ewing's sarcoma (PSEES) or primitive neuroectodermal tumor (PNET) is uncommon. The present study summarizes the magnetic resonance (MR) imaging appearance of PSEES. METHODS: Literature search from 1994 to 2012 with our representative case presentation. RESULTS: Twenty-one patients, 12 males and 9 females, aged 3 weeks to 44 years, were identified. The thoracic spine was most frequently affected, followed by the cervical, cervicothoracic, and thoracolumbar spine. Superior-inferior extension of lesions was three vertebral levels in 7, two in 7, five in 4, four in 1, one in 1 and unknown in 1. PSEESs appeared isointense in 9 cases, hypointense in 2, hyperintense in 1, and no description in 9 on T1-weighted imaging, while hyperintense in 6, hypointense in 3, heterogeneous in 1, and no description in 11 on T2-weighted imaging. Varying enhancement was noted in 13 cases (62 %), with no description of contrast study in the other 8 cases. Dumbbell-shaped configuration of PSEES was found in 5 cases, foraminal widening in 4, and erosions or scalloping of the adjacent vertebral bodies in 4. CONCLUSION: The MR imaging appearance of PSEESs is indistinguishable from other tumors. PSEES should be assumed as the differential diagnosis of spinal extradural tumors in pediatric, adolescent, and young adult patients, and prompt surgical exploration should be performed.

Solitary spinal extradural plasmacytoma: a case report and literature review.

Solitary spinal extradural plasmacytoma (SSEP) is a rare but distinct form of plasma cell disorder. The clinical picture and treatment of SSEP are reviewed using the seven previously reported cases. The three male and four female patients were aged 40-85 years. The location was cervical spine in one patient, cervicothoracic in one, thoracic in two, thoracolumbar in one, lumbar in one, and extensive involvement in one. Progressive paraparesis and sensory disturbance were the predominant symptoms. Neuroimaging showed a compressive extradural mass lesion in the dorsal spinal canal without findings of local bone destructive changes in all cases. Four of five patients who underwent decompressive surgical maneuver and tumor resection showed neurological improvement. Immunoglobulin (IgG) kappa subtype was the most predominant histological type, followed by IgD lambda and IgA kappa subtypes. SSEP should be included in the differential diagnosis of an extradural tumor located in the dorsal spinal canal without associated bony changes. Surgery may be effective for symptomatic relief.

Primary pineal melanocytic tumor. Case report.

A primary melanocytic lesion arising from the pineal gland is very rare. The authors report a case of primary pineal melanocytic tumor with dissemination to the right hippocampus in a 50-year-old woman who presented with memory disturbance. Magnetic resonance (MR) imaging revealed a mass that was hyperintense on T1-weighted and hypointense on T2-weighted MR images. The pineal tumor was removed subtotally via the occipital transtentorial approach, and the patient underwent whole-brain irradiation. Results of histological examination revealed that the tumor predominantly consisted of atypical cells with scanty melanin pigment and some necrotic foci. The strongly pigmented areas of the tumor contained well-differentiated cells similar to those of melanocytoma. An ultrastructural study demonstrated evidence of a mature type of melanosome. The patient died 11 months after surgery and radiotherapy (1.7 years after the onset of symptoms). The autopsy findings demonstrated tumor invasion into the parenchyma through the leptomeningeal space and the ventricular wall. The tumor was diagnosed as being malignant, and it was finally concluded that the atypical cells in the tumor were probably responsible. This pineal melanocytic tumor exhibited a wide spectrum of differentiation, ranging from highly malignant melanoma to well-differentiated melanocytoma, which may have contributed to the patient's relatively long survival period. The biological behavior and morphological characteristics of this tumor appear to be similar to those of other pineal parenchymal lesions.

Serum cystatin C may predict the prognostic stages of patients with IgA nephropathy prior to renal biopsy.

The relationship between the levels of serum cystatin C and the prognostic stages of IgA nephropathy was determined in a multicenter trial in Japan. The levels of serum cystatin C in patients with IgA nephropathy were measured using the Dade Behring N Latex Cystain C assay. In 1995, the Joint Committee of the Special Study Group on Progressive Glomerular Diseases, Ministry of Health and Welfare of Japan, and the Japanese Society of Nephropathy reported four prognostic stages. These are: good prognosis group (Group I), relatively good prognosis group (Group II), relatively poor prognosis group (Group III), and poor prognosis group (Group IV), for this disease. Three-hundred and six patients with IgA nephropathy and other glomerular diseases were examined. There were no significant changes in the levels of serum creatinine (Cr) or creatinine clearance (CCr) between Group I and Group II. The mean levels of serum cystatin C in Group II were significantly higher than those in Group I (P < 0.05). The mean levels of serum cystatin C in Group III or IV were significantly higher than those in Group I (P < 0.001, P < 0.005, respectively). These suggest that the measurement of serum cystatin C may predict the prognostic stages of patients with IgA nephropathy prior to renal biopsy.

Measurement of serum IgA and C3 may predict the diagnosis of patients with IgA nephropathy prior to renal biopsy.

The levels of serum IgA and C3 in patients with IgA nephropathy were determined using international standard serum (IFCC/CRM470) in a multicenter trial in Japan. The ratio of serum IgA to C3 (serum IgA/C3 ratio) without any information from renal biopsy was used for the diagnosis of IgA nephropathy. Three hundred and six patients with IgA nephropathy and other glomerular diseases, and 418 healthy adults were examined. The new diagnostic standardized criterion in patients with IgA nephropathy, obtained by nephelometric immune assay based on the international reference preparation CRM470, was 315 mg/dl. The serum IgA/C3 ratio was a more useful marker for distinguishing IgA nephropathy from non-IgA nephropathy together with serum IgA levels. This suggests that the measurement of serum IgA and C3 may predict the diagnosis of patients with IgA nephropathy prior to renal biopsy.

[A case of multiple metastatic brain tumors with repeated intracerebral hemorrhages].

We report a case of multiple metastatic brain tumors with repeated intracerebral hemorrhages. A 73-year-old man suffered from a cerebellar hemorrhage. Subsequent hemorrhages repeatedly occurred in the right temporal lobe, the 4th ventricle, the midbrain, and the septum pellucidum. Three months after admission, CT revealed enhanced masses with surrounding edema in the cerebellar vermis and midbrain, suggesting brain tumors. We eventually diagnosed these masses in an autopsy as metastatic brain tumors of lung adenocarcinoma. Intravascular embolization with tumor cells was a probable cause of the multiple repeated intracerebral hemorrhages.

Case of propylthiouracil-induced vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA); review of literature.

A 39-year-old Japanese woman had been receiving propylthiouracil for 5 years for hyperthyroidism when she developed myalgia, scleritis, proteinuria, fever, and inflammation of the nose. Examination of a renal biopsy specimen showed focal segmental necrotizing glomerulonephritis. Indirect immunofluorescent staining showed a highly positive perinuclear pattern of anti-neutrophil cytoplasmic antibody (ANCA) in her serum. Enzyme-linked immunosorbent assay (ELISA) of the ANCA showed positivity for anti-proteinase 3, anti-myeloperoxidase, anti-leukocyte elastase, and anti-lactoferrin, but anti-cathepsin G and anti-lysozyme were negative. Because ELISA showed the titer of anti-leukocyte elastase antibody to be markedly elevated, we challenged this data by performing dot blot analysis. The patient's serum reacted with the native form, but not with denatured leukocyte elastase. Propylthiouracil-induced vasculitis was suspected. Symptoms abated within 2 weeks and all values of ANCA were reduced after the drug was withdrawn. Vasculitis is a rare side-effect of propylthiouracil therapy. Recently it was reported in association with ANCA. We present the findings of this patient and compare them with those described in 19 published cases of propylthiouracil-induced vasculitis associated with ANCA.

Characterization of a rat liver Golgi sulphotransferase responsible for the 6-O-sulphation of N-acetylglucosamine residues in beta-linkage to mannose: role in assembly of sialyl-galactosyl-N-acetylglucosamine 6-sulphate sequence of N-linked oligosaccharides.

Rat liver Golgi membranes were found to contain an enzyme that can transfer sulphate from 3'-phosphoadenosine 5'-phosphosulphate (PAPS) to C-6 of the terminal GlcNAc in beta-linkage to mannose and has properties indicating that it is involved in the synthesis of the NeuAc alpha 2-3(6)Gal beta 1-4GlcNAc(6-SO4) sequences observed in the N-linked carbohydrate units of various glycoproteins. Assays performed with [35S]PAPS (Km 0.67 microM) and GlcNAc beta 1-6Man alpha 1-O-Me (GnMaMe) acceptor (Km 0.71 mM) indicated that the sulphotransferase had a pH optimum of approx. 7.0 and is markedly stimulated by Mn2+ ions (maximum approx. 15 mM) and Triton X-100 (0.05-0.1%). Hydrazine/nitrous acid/NaBH4 treatment of the 35S-labelled product yielded radiolabelled 2,5-anhydromannitol(6-SO4). The sulphated GnMaMc product of the GlcNAc-6-O-sulphotransferase could be galactosylated by a rat liver Golgi enzyme that was shown to have the same properties as the UDP-Gal:GlcNAc beta-1,4-galactosyltransferase from bovine milk. Competition studies performed with GlcNAc and GlcNAc-6-SO4 furthermore indicated that the same liver enzyme acted on both acceptors to produce Gal beta 1-4GlcNAc and Gal beta 1-4GlcNAc(6-SO4) with Km values of 1.04 and 1.68 mM respectively. Because the sulphated N-acetyl-lactosaminc could in turn serve as an acceptor for rat liver sialyltransferase, it seems that this enzyme, together with the Golgi galactosyltransferase and the GlcNAc-6-O-sulphotransferase, could act in concert in assembling the NeuAc alpha 2-3(6)Gal beta 1-4GlcNAc(6-SO4) branches of complex N-linked oligosaccharides.

[A clinicopathological study of 21 cases of primary central nervous system lymphoma].

A clinico-pathological study was carried out in 21 cases of primary central nervous system-non-Hodgkin's lymphoma (CNS-NHL). Their clinical profiles (age, prognosis, modalities of treatment) and findings of radio-imaging were analyzed. All specimens from surgery and/or autopsy were histologically classified according to the working formulation (WF) classification of the National Cancer Institute. Ontogeny of lymphoma cells was determined by immunohistochemical study in all cases and some cases were subjected to light (kappa, lambda) and heavy chain (IgG, IgA, IgM) analysis as well. Among 21 cases, 12 cases were located in the cerebral hemisphere, 7 in the thalamus-basal ganglia and 4 in the cerebellum. Radio-imaging study showed that 18 cases (86%) revealed isodensity mass lesions on plain CT, which were homogeneously enhanced by contrast medium. The pathological study showed that all cases were derived from B-cells. Five were classified as immunoblastic type (IBL), 9 as diffuse large type (DL), and the others were classified according to WF. 17 of 21 cases (81%) were sensitive to radiotherapy, and 15 of 19 cases (79%) responded to corticosteroid. A prognostic study revealed that patients with IBL had less hope than those with DL. From this result, it seems that WF classification is better than LSG classification for obtaining a prognosis in malignant lymphoma patients. The frequency of primary CNS-NHL has been increasing for the past several decades and will surpass that of any other brain tumors in the near future because of the explosive expansion of AIDS patients. Therefore, not only clinicopathological analysis but also biological study for CNS-NHL might be important.

[Effects of steroid therapy in IgA nephropathy].

In order to estimate the effects of corticosteroid therapy in IgA nephropathy cases with daily urinary protein excretion of 1.0 g/day or more. 26 patients (8 men and 18 women, aged 32.6 +/- 14.0 years old) were subjected to this study. The results obtained were as follows: Urinary protein excretion after 1 year from the beginning of steroid therapy (1.56 +/- 1.14 g/day) was significantly (p less than 0.05) lower than that at the beginning of the therapy (4.61 +/- 6.01 g/day). In serum creatinine levels, there was no statistically significant difference with them between at the beginning (1.15 +/- 0.48 mg/dl) of steroid therapy and at the time of 1 year after (1.05 +/- 0.34 mg/dl) the therapy. As for the outcome at the end of this study setting (mean follow-up duration: 3.7 +/- 2.6 years), complete remission was attained in 7 cases, improvement in 5 cases, unchanged condition in 11 cases, increased urinary protein excretion in 2 cases and aggravated renal function in 1 case. In clinical findings at the renal biopsy, duration of the disease (3.4 +/- 1.6 months) in complete remission cases before biopsy was significantly (p less than 0.01) shorter than that in unchanged cases (65.0 +/- 40.0 months). In histological findings, rate of global sclerosing glomeruli (2.6 +/- 4.6%) in complete remission cases was significantly (p less than 0.05) lower than that (24.6 +/- 23.1%) in unchanged cases. These results suggest that steroid therapy in IgA nephropathy with persistent proteinuria of 1.0 g/day or more is beneficial, especially in cases that are in early stage of the disease with lower rate of global sclerosing glomeruli.

[A case of familial lecithin: cholesterol acyltransferase deficiency].

Lecithin: cholesterol acyltransferase (LCAT) is an enzyme that catalyzes the esterifying reaction of cholesterol in plasma high density lipoprotein (HDL). Deficiency of LCAT is a rare hereditary disease characterized by several clinical symptoms such as proteinuria, corneal opacity, and anemia due to a shortened life span of erythrocytes. In this communication, we report a case of 40 year-old female patient of LCAT deficiency. She visited a hospital for work-up of proteinuria, corneal opacity and anemia. Activity of her serum LCAT was found to be extremely low, and characteristic changes in plasma lipids due to deficiency of LCAT was observed: those were marked decreases in HDL-cholesterol, degree of esterification in serum cholesterol, and apoprotein A-I, A-II, B and C-II levels. The diagnosis of LCAT deficiency was finally made. We studied about histopathological changes in the patient's kidney, and erythrocyte membrane lipid composition and fluidity. Histopathological findings in renal biopsy were follows: a) Light microscopy showed spherical deposits stained with periodic acid-Schiff in mesangial matrix and adjacent capillary loops, and hyaline deposits in arterioles, b) Electron microscopy showed vacuoles in mesangial matrix and along the glomerular basement membranes. In erythrocyte membrane lipids, increase of cholesterol to phospholipid molar ratio was evident, being accompanied by changes in phospholipid fractions: increase of phosphatidylcholine, and decreases of phosphatidylethanolamine, sphingomyelin and lysophosphatidylcholine. In phospholipid acyl chains, increase of C18:2 and decreased of C18:1 were evident in the patient. Erythrocyte membrane fluidity was found to be decreased in the patient in a measurement by pyrene, probably being related to the changes in membrane lipid composition.(ABSTRACT TRUNCATED AT 250 WORDS)

A case of HTLV-I-associated myelopathy with IgA nephropathy and pseudohypoparathyroidism type 1.

We report a case of HAM/TSP presenting with short stature, mental retardation, skin eruptions, uterine and ovarian hypogenesis and nephropathy. Skin erythema was noted since from the age of three years old and spasticity of lower extremities from elementary school age. Serum calcium level showed 4.1 mEq/l. Recombinant human PTH infusion resulted in no response of phosphate excretion. The persistent proteinuria prompted renal needle biopsy, which revealed IgA and C1q deposits in glomerular mesangium. A diagnosis of pseudohypoparathyroidism and IgA nephropathy was entertained. This patient with pseudohypoparathyroidism who has a deficient immune system was seized with the early onset of HAM/TSP and IgA nephropathy.

[A case of Fabry's disease detected by renal biopsy findings].

A 39 year-old man was found to have mild proteinuria by urinary examination since one year ago. He was for the first time diagnosed as having Fabry's disease by histopathological and electronmicroscopic findings of the renal biopsy specimens, which showed the presence of numerous vacuolated cells and electron dense bodies inside the cells. The level of WBC alpha-galactosidase was significantly lower than normal level. The pedigree of this patient showed a familial history of various types of renal disease. One of the patient's brothers also showed decreased level of WBC alpha-galactosidase, who has been treated by maintenance hemodialysis for 2 years. It is concluded that early diagnosis of this disease through renal biopsy and WBC alpha-galactosidase level is important to manage the future course of patients with Fabry's disease.

IgD multiple myeloma with renal involvement: case report.

IgD multiple myeloma is a unique type of multiple myeloma which is characterized by increased serum IgD and IgD type M-component in immunoelectrophoresis. It frequently shows renal involvement but it is a rare form of myeloma. The distinctive features of IgD myeloma are the dominance in males, high frequency in younger persons, and the uncertain appearance of M-component in serum electrophoresis. We experienced 3 cases of IgD multiple myeloma with renal failure which required hemodialysis before IgD myeloma was diagnosed. It is important to consider IgD myeloma when treating the patients with renal involvement of unknown origin.

Lupus nephritis with adult T cell leukemia.

A 64-year-old Japanese male patient with lupus nephritis associated with adult T cell leukemia (ATL) is described. Percutaneous renal biopsy demonstrated findings consistent with membranous lupus nephritis. To our knowledge, this is the first case of lupus nephritis complicated by ATL, suggesting that human T cell leukemia virus type I may be correlated not only to outbreaks of ATL but also to lupus nephritis/systemic lupus erythematosus.

[Investigation on antibodies to human T cell leukemia virus type I in patients with primary glomerulonephritis].

We investigated on antibodies to human T cell leukemia virus type I (anti HTLV-I) in 101 patients with primary glomerulonephritis in Kagoshima prefecture, an endemic area of HTLV-I in Japan. The positive rate in patients with primary glomerulonephritis was 17.8%, which was not significantly higher than in healthy residents 4741 persons of 11.9% in the same area. Classified by renal biopsy findings, the positive rate in patients with membranous nephropathy was 21.4% (9/42), minimal change nephrotic syndrome 14.3% (2/14), IgA nephropathy 15.4% (2/26), and others 15.8% (3/19) respectively, which were not significantly differed from that in healthy residents. Positivity of aged patients (60 years or over) in membranous nephropathy on the contrary was significantly higher than that in healthy residents (p less than 0.01). Further study is required to clarify the relationship between HTLV-I infection and glomerulonephritis.

Metabolic alterations in fiber layers of the CA 1 region of the gerbil hippocampus following short-term ischemia: high-energy phosphates, glucose-related metabolites, and amino acids.

The CA 1 neurons of the gerbil hippocampus die at 4 days following 5 min of bilateral ischemia. The fiber and somal layers of the CA 1 region of the gerbil hippocampus were analyzed for high-energy phosphates, glucose-related metabolites, and amino acids from 0.75 hr to 4 days of postischemia. The results were compared to those from two layers of the CA 3 region and the cerebral cortex. The metabolite changes in the fiber layers of the CA 1 region were qualitatively similar to those in the somal layer. The energy status of the tissues taken from the CA 1 region was never compromised for up to 2 days of recirculation, after which the ATP and P-creatine in the somal layer decreased to 43 and 56% of the control, respectively, whereas the average decreases in the CA 1 fiber layers were only 71 and 88% of the control, respectively. Thus, the high-energy phosphate response of the neuronal elements in the fiber layers was temporally similar to that found in the somal layer of the CA 1 region. The biphasic increases in glycogen, glucose, glucose-6-phosphate, and high-energy phosphates to values greater than the control indicated that the metabolic restoration following transient ischemia is a dynamic process which persists for up to 2 days of recirculation, even in resistant tissues. A similar pattern of delayed changes was observed in glutamate, gamma-aminobutyric acid (GABA), and glutamine, but the change in each amino acid appeared to be independent of the others despite their close metabolic relationship. The delayed decreases in GABA would favor a loss of inhibition to the CA 1 neurons and may be related to the phenomenon of delayed neuronal death.