Generation of a nude mouse tumor model for in vivo replication of human cytomegalovirus

PURPOSE: Our purpose was to report the patterns of injury observed in five patients who suffered brain damage consequent to neonatal hypoglycemia. METHODS: The imaging studies and clinical records of five patients with brain damage caused by neonatal hypoglycemia were reviewed retrospectively. Patterns of injury were compared with those described in the literature and those seen in neonatal hypoxic-ischemic injury. RESULTS: Diffuse cortical and subcortical white matter damage was seen, with the parietal and occipital lobes affected most severely. Globus pallidus injury was present in one patient who had the most severe cortical injury. CONCLUSION: We found a specific pattern of injury that correlates well with the sparse pathologic and imaging reports on neonatal hypoglycemia. We speculate that the patterns of damage are the result of regional hypoperfusion and excitatory toxicity with cell-type-specific injury.     

Correlation between cerebral visual impairment and magnetic resonance imaging in children with neonatal encephalopathy

To identify brain lesions most often associated with cerebral visual i mpairment (CVI) after neonatal encephalopathy and to evaluate the prognostic value of MRI for visual outcome, the authors reviewed visual assessments and brain MRI of 80 infants and young children with documented perinatal hypoxic-ischaemic and/or haemorrhagic insults. MRIs were classified according to the severity of lesions at the optic radiations and at the visual cortex; visual acuity was tested with the acuity card procedure. Among the 48 children found to have a CVI, 42 had moderate to severe lesions of the optic radiations and 19 had lesions of the visual cortex. In both cases visual acuity was statically correlated with MRI grading, but the damage at the optic radiations was the better predictor. Early detection of abnormal MR findings in the visual cortex and especially, at the optic radiations may indicate which infants with neonatal encephalopthy should receive longitudinal visual assessment and specific rehabilitation and educational management.     

ACR presidential address: Rheum for more. American College of Rheumatology

Regional brain injury in three neonates with hypoglycemic encephalopathy are presented using serial 99mTc-hexamethyl propyleneamine oxime (HMPAO) SPECT and, for comparison, MRI. During the acute stage, both 99mTc-HMPAO SPECT and MRI reveal abnormalities in the posterior cerebrum. Technetium-99m-HMPAO SPECT reveals further areas of insult, for example the frontal lobes. The degree of hypoperfusion correlates with the clinical severity of hypoglycemia during the neonatal period and subsequent neurological sequelae. Follow-up with HMPAO SPECT several months after insult demonstrates persistent hypoperfusion in some areas, mainly in the occipital and posterior parietal regions. MRI can depict morphological changes with superior resolution. Because morphological change generally follows slowly after functional change, MRI is less sensitive than HMPAO SPECT in detecting and predicting the extent of hypoglycemic cerebral injury during the acute phase. HMPAO SPECT during the acute stage is a valuable tool for evaluating the extent and severity of brain injury in neonates with hypoglycemic encephalopathy.     

Unilateral neonatal cystic disease of the kidney as first manifestation of tuberous sclerosis]

Traumatic optic neuropathy (TON) causes blindness of varied severity and occurs infrequently as a complication of closed head injury. A case is presented of TON that occurred in a patient who suffered complete T4 paraplegia from a motorcycle accident but in whom no severe head injury took place. In this case, high-dose intravenous methylprednisolone was begun for the spinal cord injury and repeated 24 hours later for the TON. Vision improved from near total blindness to 20/400 in the left eye (OS) and 20/130 in the right eye (OD). Two weeks later, however, the patient's vision suddenly worsened. Magnetic resonance imaging (MRI) using fat suppression confirmed a lesion along the optic nerve consistent with TON. A third course of methylprednisolone again led to improved vision. The steroids were then tapered orally over 2 weeks and the patient had no further relapses. Moderate to severely impaired vision of 20/ 400 OS and 20/130 OD continues to interfere with the patient's function and spinal cord rehabilitation program. It was concluded that a steroid taper was important in maintaining initial visual gains in this case. Awareness of TON and careful attention to the patient's clinical course can minimize deficit and maximize functional outcomes.     

The apparent diffusion coefficient of water in gray and white matter of the infant brain

PURPOSE: The purpose was to obtain normal values of the apparent diffusion coefficient (ADC) in the infant brain and to compare ADC maps with T1- and T2-weighted images. METHOD: Diffusion was measured in nine infants with an ECG-gated SE sequence compensated for first-order motion. One axial slice at the basal ganglia level was investigated with the diffusion-encoding gradients in the slice-selection direction. RESULTS: On ADC maps, the corpus callosum and the optic radiations appeared dark before the onset of myelination, and the crus posterior of the internal capsule could be visualized before it appeared on T1- or T2-weighted images. In gray and white matter, the mean ADC ranged from 0.95 x 10(-9) to 1.76 x 10(-9) m2/s. In the frontal and occipital white matter, in the genu corporis callosi, and in the lentiform nucleus, the ADC decreased with increasing age. The cortex/white matter ratio of the ADC increased with age and approached 1 at the age of 30 weeks. CONCLUSION: ADC maps add information to the T1 and T2 images about the size and course of unmyelinated as well as myelinated tracts in the immature brain.     

Fulminant angiopathy caused by allogenic transplantation of bone marrow with cerebral involvement: clinico-pathologic correlation

We present a 41 woman who underwent an allogeneic bone marrow transplantation as a treatment for a multiple myeloma. After presenting graft-versus-host disease, hyperbilirubinemia, cyclosporine toxic levels, renal failure and peripheral schistocytosis, she developed complex partial seizures and bilateral hypodensity lesions in occipital lobes. Fulminant microangiopathy was diagnosed; though promptly treated with immunoglobulins and fenitoin, she died in few days. Necropsy showed diffuse thrombotic disease and a haematoma in left occipital lobe. We review the types of thrombotic microangiopathy described up to now, and the different pathogenic theories related to them. According to our reviewing of the literature, this is the first case of fulminant thrombotic microangiopathy ever described in Spanish language.     

Cortical blindess as symptom of pre-eclampsia

Neurological complications during preeclampsia are not infrequent; the appearance of total blindness is rare, however. We report the case of a 32-year-old primipara with no outstanding history, who presented total loss of vision with no accompanying neurological symptoms during week 37 of the pregnancy. Effective early management of blood pressure to levels at which self-regulation of cerebral perfusion could be established allowed total recovery of vision 8 hours after admission to the intensive care recovery ward. Cranial computerized axial tomography (CAT) showed bilateral occipital subcortical edema that resolved after 8 days. The patient was released 13 days after admission with no sequelae.     

A self-report of sensory deficit after posterior right brain infarction.

Presents the case report of infarction in the right occipital pole, during which a 62-yr-old, right-handed man experienced (1) an acute period of transient but severe sensory deficit of his left arm, (2) permanent left hemianopia and transient total cortical blindness, (3) 2-dimensional visuospatial positional deficit (simultanagnosia) and place agnosia, and (4) environmental orientation deficit. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Superficial siderosis appeared in a case of suprasellar embryonal carcinoma]

A case of superficial siderosis that appeared in a case of suprasellar embryonal carcinoma is reported. A 24-year-old man presented polydipsia and vertigo. MRI revealed a suprasellar tumor. The tumor contained high intense spots on T1-weighted images, suggesting intratumoral hemorrhage. He underwent a surgery, which proved it as embryonal carcinoma pathologically. Cerebrospinal fluid was xanthochromic at surgery, suggesting terminal hemorrhage. After surgery, he received a total dose of 56 Gy of irradiation. Tumor decreased in size and symptoms improved. However, he presented occipital headache 7 months after surgery. MRI showed disseminated tumors in the subarachnoid spaces. He received irradiation for the whole spine and adjuvant chemotherapy. During treatment, MRI demonstrated low signals on the surface of the brain stem, suggesting the superficial siderosis. The lesions spread to the surface of the cerebellum and tentorium cerebelli. Superficial siderosis is characterized by the deposition of hemosiderin in the leptomeninges, cranial nerves and spinal cord. The etiology of the hemosiderin deposition is thought to be chronic or recurrent bleeding into subarachnoid space. Experimentally, similar lesions have been produced in the animals following intrathecal injection of blood or hemolysed red cells. In the literature, MRI demonstrated a rim of marked hypo-intensity on T2-weighted images, consistent with hemosiderin deposits, on the surface of cerebellum and brain stem. Gradient-echo sequences have been more sensitive than T2-weighted images of spin echo sequences. In the present case, the superficial siderosis seems to be due to chronic tumoral hemorrhage. This phenomenon could be related to chemotherapy using CDDP and etoposide.     

Nitric oxide production by pig endothelial cells in response to human-derived injury

BACKGROUND: Metamorphopsia is a visual illusion that distorts the size, shape, or inclination of objects. Reversal of vision metamorphopsia (RVM) is a rare transient form of metamorphopsia described as an upside-down, 180 degrees rotation of the visual field in the coronal plane. The pathophysiological characteristics of RVM remain unclear. DESIGN: Patients with RVM had a complete neurologic examination during or shortly after an episode of metamorphopsia, with particular emphasis on gaze disorders, visual fields, visually guided hand movements, and perceptual or cognitive deficits. Workup included imaging studies, visual field examinations, and brainstem auditory and visual evoked response. SETTING: Department of Neurology, Hadassah University Hospital, Hebrew University-Hadassah Medical School, Jerusalem, Israel. PATIENTS: Six consecutive patients were evaluated from 1991 to 1996. RESULTS: Five patients had parieto-occipital brain insult sparing the primary visual cortex, and 3 also had evidence of a concomitant brainstem or cerebellar syndrome. One patient had pure brainstem syndrome underlying the RVM. Three patients had complete RVM as well as oblique RVM of less than 180 degrees. CONCLUSIONS: These cases imply a possible anatomical localization of the central integrator of visual extrapersonal orientation. Our observations suggest that a separate central mechanism of visual orientation might exist in each cerebral hemisphere and that occipital and parietal lesions that spare the optic radiations may account for the oblique and complete RVM. We postulate that failure to perceive space in an allocentric coordinate frame, particularly in the coronal roll plane, is potentially the critical event underlying RVM.     

Adrenoleukodystrophy with disease of the eye and optic nerve

Adrenoleukodystrophy is an X-chromosome-linked recessive disease characterized by primary atrophy of the adrenal glands with or without Addison's disease and low plasma cortisol levels, and a degeneration of white matter of the central nervous system with blindness. In suspected cases of adrenoleukodystrophy an impaired rise in plasma cortisol levels after adrenocorticotrophin stimulation may be diagnostic. With the electron microscope, pathognomonic intracytoplasmic lamellar inclusions have been seen in adrenal cortical cells, peripheral nerve Schwann's cells, testicular interstitial cells, and in macrophages of the brain. Adrenoleukodystrophy appears to be a genetically determined lipid storage disease with an error in membrane sterol metabolism. A 10-year-old boy with adrenoleukodystrophy had visual loss, a prominent early symptom. The ocular abnormality consisted of a disproportionate loss of nerve fibers from the macular region. No intracytoplasmic lamellar inclusions were identified in cells representing macrophages within the optic nerve. They contained myelin debris suggestive of end-stage disease.     

Experimental approaches for studies of particulate matter: the case for stronger consideration of dosimetric and exposure issues

We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded to intravenous immunoglobulin and pulsed methylprednisolone. This case illustrates that life-threatening haemorrhage can occur in patients with Fechtner syndrome. Although there was no effective treatment reported in the literature, high dose steroid and immunoglobulin seemed to be useful in our patient. Our patient also had nephritic-nephrotic syndrome with renal insufficiency, which is unusual in adolescent female patients.     

A quantitative study of the EMI values obtained for normal brain cerebral infarction and certain tumours

A quantitative study has been made of the EMI numbers of normal brain, cerebral infarction and certain tumours. The scans were recorded on magnetic tape and analysed using a minicomuter linked to a graphic display unit. This system not only yielded 16 grey scales compared with the ten currently available, but was programmed to allow selected regions of the scans to be outlined. From these regions the computer calculated the area, the mean EMI number and its standard deviation. It was found that in 15 normal brain scans, the EMI values obtained for normal frontal and temporal lobes were similar, but that the values for the basal ganglia and occipital lobes were significantly different from the first two regions and from each other. Ten cases of cerebral infarction and 30 cases of cerebral tumour were analysed, and it was shown that analysing representative areas was more informative than surveying the whole lesion. Whilst only half of the scans of brain tumours had a significantly altered EMI number compared with that of normal brain, enhancement of tumour density with sodium iothalamate revealed a consistent and significant elevation of the EMI number for all tumours. In particular, the value for enhanced meningiomas was almost double and malignant tumours more than a third larger than normal brain. It was not possible to differentiate quantitatively between astrocytomas and metastases.     

Heterogeneity of pituitary lactotrophs: immunocytochemical identification of functional subtypes

Phospholipase A2 (PLA2) has been demonstrated to play an important role in the reperfusion injury of the kidney, gut, brain, heart and pancreas. This study was carried out to clarify whether PLA2 was involved in the ischemia-reperfusion injury of the liver. Rats were anesthetized and underwent laparotomy. They were allocated into one of 4 groups, i.e., the groups of renal ischemia (group RI), renal control (group RC), hepatic ischemia (group HI), and hepatic control (group HC). In group RI, the left renal pedicle was occluded for 1 hr, and the left kidney was removed after 1-hr reperfusion. In group HI, the portal and the hepatic artery supplying the left and middle lobes were clamped for 1 hr, followed by reperfusion. After predetermined periods of reperfusion up to 24 hr, the ischemic lobes were removed, homogenized and centrifuged. PLA2 activities in the mitochondrial fraction and the cytosolic fraction were measured with 14C-phosphatidylcholine (PC) and 14C-phosphatidylethanolamine (PE) as exogenous substrates. PLA2 activities of the both fractions in the kidney were significantly enhanced after 1-hr ischemia followed by 1-hr reperfusion. However, there was no enhancement of PLA2 activity of the either fraction in the group HI compared with the group HC. The results indicate that PLA2 is activated in the kidney but not in the liver during ischemia-reperfusion.     

Congruous quadrantanopia and optic radiation lesion

Homonymous visual field defects are rare in patients with MS and are usually caused by large lesions in the white matter of the posterior optic radiations and visual cortex. The postulate of Holmes and others, early this century, that optic radiation lesions could produce quadrantic defects has recently been questioned. We report the full recovery of a patient with probable MS who presented with congruous quadrantanopia from a small white matter lesion in the contralateral trigone area.     

Synchronous multifocal development of invasive transitional cell carcinoma of the urinary tract in a patient with renal failure receiving long-term hemodialysis: a case report

We report a case of transitional cell carcinoma in a patient with chronic renal failure receiving hemodialysis for 22 years. A 55-year-old man was admitted to our hospital. Under diagnosis of invasive bladder cancer and left renal pelvic tumor, removal of the whole urinary tract, e.g., bilateral nephroureterectomy and total cystourethrectomy was performed. Transitional cell carcinoma was found in bilateral renal pelvis, left ureter, bladder and prostate in the resected specimen. Thirteen months after the operation, multiple lung metastases and pathologic bone fracture of the 4th lumber vertebra were found. Chemotherapy (3 courses of modified CISCA, consisting of cisplatin, adriamycin and cyclophosphamide) was performed, but the died of systemic metastases of cancer and bleeding due to perforation of multiple gastric ulcers.     

Postoperative hyperperfusion in dural arteriovenous fistula associated with venous ischemia: case report

BACKGROUND: It is well known that carotid endarterectomy and extracranial-intracranial arterial bypass sometimes cause postoperative hyperperfusion, and vasoparalysis attributable to long-standing ischemia has been suggested as the cause. It is also well known that dural arteriovenous fistula (AVF) sometimes causes cerebral ischemia attributable to venous hypertension. However, there are few reports regarding the postoperative changes of regional cerebral blood flow (rCBF). METHODS: We report a case of dural AVF of the left transverse/sigmoid sinuses, occurring in a 64-year-old man. Intraoperative transvenous embolization combined with transarterial embolization was performed, and the rCBF was measured pre- and postoperatively using 99mTc-hexamethyl-propylene amine oxime and single-photon emission computed tomography (SPECT). RESULTS: Preoperative SPECT disclosed a marked rCBF reduction in the left temporal, parietal, and occipital lobes. Complete obliteration of the AVF was attained after the intraoperative transvenous embolization, without any neurological deterioration. However, postoperative SPECT demonstrated temporary hyperperfusion in these regions. CONCLUSIONS: Sudden resolution of venous ischemia can lead to postoperative hyperperfusion, and pre- and post-treatment rCBF studies are important to prevent complications related to hyperperfusion.     

Psychiatrist and nonphysician mental health provider staffing levels in health maintenance organizations

OBJECTIVE: To describe a patient with a paraneoplastic cerebellar syndrome and optic neuritis with circulating anti-CV2 antibodies and clinical improvement after excision of a small cell lung carcinoma. DESIGN: Report of a case. SETTING: A 62-year-old man simultaneously developed a severe cerebellar syndrome and a bilateral optic neuritis predominantly in the left eye (visual acuity, 20/25 in the right eye; < 20/400 in the left eye; and bilateral swelling of the optic discs). MAIN OUTCOME AND RESULTS: Anti-CV2 antibodies, recently described as associated with paraneoplastic neurological syndrome, were detected in the patient's serum sample. These antibodies were demonstrated to react with the cytoplasm of a subpopulation of oligodendrocytes in the white matter of rat brain in the cerebellum, brainstem, spinal cord, and optic chiasm. The patient was found to have a small cell lung carcinoma, which was removed. After excision of the tumor, the cerebellar syndrome improved dramatically and the papilledema disappeared despite aftereffects of the optic neuritis. CONCLUSIONS: These findings were consistent with the diagnosis of a paraneoplastic neurological syndrome, although both optic neuritis and remission of the cerebellar syndrome are uncommon patterns of paraneoplastic syndromes. CV2 antigen expression by the oligodendrocytes of the cerebellum, brainstem, spinal cord, and optic chiasm correlated with the clinical syndrome observed in our patient. However, the precise pathophysiological role of anti-CV2 antibodies is still unknown.     

In vitro antiphagocytic effect of Melia azedarach leaf extracts on mouse peritoneal exudate cells

The first reported case of cerebellar metastasis from primary clear cell adenocarcinoma of the fallopian tube is presented. Initially diagnosed as stage Ia, the patient underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy and omentectomy, followed by cisplatin-based chemotherapy and whole pelvic irradiation. Thirty six months later multiple pulmonary metastases were detected that did not respond to chemotherapy. Later the patient presented with cerebellar metastasis. She received whole brain radiotherapy and steroids. The brain lesion partially responded and the patient's neurologic symptoms improved. Throughout there was no evidence of local recurrence. This case suggests that with the prolonged survival achieved by aggressive treatment occult brain metastases might become apparent.     

A case of progressive multifocal leukoencephalopathy with methionine uptake demonstrated by PET]

We report here a 55-year-old man with progressive multifocal leukoencephalopathy (PML) associated with chronic adult T cell leukemia (ATL). Neurological examination revealed mild dementia, right homonymous hemianopsia and visual agnosia. Serologically anti-HTLV-I antibody was positive. Peripheral blood analysis showed ATL cells up to 23% in white blood cells. Because he did not have symptoms or signs directly related to ATL, it was considered that he had chronic ATL. T2-weighted cranial MRI demonstrated multiple hyperintensity lesions confined to the white matter from the bilateral occipital to parietal lobes, without enhancement after gadolinium administration or mass effect. We performed stereotactic biopsy of the left occipitoparietal white matter. Histological examination of the biopsied specimens showed demyelinated lesions, containing foamy macrophages and bizarre astrocytes. Oligodendrocytes contained nuclear inclusions which reacted with an antibody against the JC virus (JCV) antigen. These findings were consistent with those of PML. The genomic analysis of JCV from the biopsied brain revealed deletions in the regulatory region. We investigated cerebral blood flow, glucose and amino acid metabolism in this patient using positron emission tomography, and obtained the following three characteristic findings in the lesions: 1) luxury perfusion state, 2) decreased fluorodeoxyglucose (FDG) uptake, and 3) increased methionine (Met) uptake. These findings resembled those of low grade tumors.