Lipid composition of outer leaflet of chloroplast outer envelope determines topology of OEP7.

OEP7, a 6.7-kDa outer envelope protein of spinach chloroplasts inserts into the outer envelope of the organelle independent of a classical cleavable targeting signal. The insertion of OEP7 was studied to describe the determinants for association with, integration into, and orientation of the protein in the outer envelope of chloroplasts. The insertion of OEP7 into the membrane is independent of outer membrane channel proteins and can be reconstituted with the use of protein-free liposomes. In situ, the binding of OEP7 to the membrane surface is not driven by electrostatic interaction because reduction of phosphatidylglycerol or phosphatidylinositol did not reduce the association with the liposomes. The positively charged amino acids flanking the transmembrane domain at the C terminus are essential to retain the native N(in)-C(out) orientation during insertion into chloroplasts. OEP7 inserts with reversed orientation into liposomes containing the average lipid composition of the outer envelopes. The native like N(in)-C(out) orientation is achieved by reduction of the phoshpatidylglycerol concentration mimicking the composition of the outer leaflet of the outer envelope of chloroplasts. We conclude that the unique lipid composition of the outer leaflet due to lipid asymmetry of the outer envelope is essential for the correct topology of OEP7.

Intrathecal 131I-labeled antitenascin monoclonal antibody 81C6 treatment of patients with leptomeningeal neoplasms or primary brain tumor resection cavities with subarachnoid communication: phase I trial results.

We aimed to determine the maximum tolerated dose (MTD) of 131I-labeled 81C6 in patients with leptomeningeal neoplasms or brain tumor resection cavities with subarachnoid communication and to identify any objective responses. 81C6 is a murine IgG monoclonal antibody that reacts with tenascin in gliomas/carcinomas but does not react with normal adult brain. 131I-labeled 81C6 delivers intrathecal (IT) radiation to these neoplasms. This study was a Phase I trial in which patients were treated with a single IT dose of 131I-labeled 81C6. Cohorts of three to six patients were treated with escalating doses of 131I (starting dose, 40 mCi; 20 mCi escalations) on 10 mg 81C6. MTD is defined as the highest dose resulting in serious toxicity in no more than two of six patients. Serious toxicity is defined as grade III/IV nonhematological toxicity or major hematological toxicity. We treated 31 patients (8 pediatric and 23 adult). Eighteen had glioblastoma multiforme. Patients were treated with 131I doses from 40 to 100 mCi. Hematological toxicity was dose limiting and correlated with the administered 131I dose. No grade III/IV nonhematological toxicities were encountered. A partial response occurred in 1 patient and disease stabilization occurred in 13 (42%) of 31 patients. Twelve patients are alive (median follow-up, > 320 days); five are progression free >409 days median posttreatment. The MTD of a single IT administration of 131I-labeled 81C6 in adults is 80 mCi 131I-labeled 81C6. The MTD in pediatric patients was not reached at 131I doses up to 40 mCi normalized for body surface area.

Magnetic resonance imaging of AIDS-related polyradiculopathy.

AIDS-related polyradiculopathy is a syndrome associated with cytomegalovirus infection. We report two cases of AIDS-related polyradiculopathy in which spinal T1-weighted MRI with gadolinium-DTPA showed enhancement of the pial lining of the conus medullaris, cauda equina, and lumbar nerve roots. Both patients clinically improved with ganciclovir treatment.

Neuro-Behçet's disease: factors hampering proper diagnosis.

We reviewed the clinical course of nine patients with neuro-Behçet's disease to assess difficulties in making this diagnosis. Factors delaying proper diagnosis included lack of accurate history and physical examination, lack of recognition of an underlying systemic syndrome and its relationship to the neurologic symptoms, presence of intermittently normal CSF studies, and use of noncontrasted neuroimaging techniques.

Hyponatremic encephalopathy: is central pontine myelinolysis a component?

PURPOSE: Severe hyponatremia is often associated with permanent brain damage. There has been substantial controversy about whether central pontine myelinolysis (CPM), a rare neurologic disorder of uncertain etiology, can complicate either hyponatremia or its therapy. This study was undertaken to determine how often hyponatremic patients with the clinical diagnosis of CPM actually have the disorder as an integral structural component of their encephalopathy. PATIENTS: Analyses were carried out in 20 patients who had severe symptomatic hyponatremia and a presumptive diagnosis of CPM, based on clinical and/or neuroradiologic findings. All had been referred for neuroradiology consultation. The mean age (+/- SD) was 47 +/- 14 years, the lowest serum sodium level was 104 +/- 8 mM, and 85% of the patients were female. The etiologies were diverse and included postoperative status, thiazide diuretics, polydipsia, infection, acute renal failure, chronic alcoholism with emesis, and beer potomania. METHODS: The original and subsequent films of 20 patients were reevaluated retrospectively by two neuroradiologists. The clinical course was also reevaluated, and in eight patients, the postmortem brain findings were reviewed. The diagnosis of CPM was made only on the basis of strict criteria relating to either (1) pathologic findings of CPM on postmortem examination; or (2) computed tomographic scan and/or magnetic resonance imaging findings diagnostic of CPM. RESULTS: No pontine lesions were present in 15 of 20 patients in whom the diagnosis of CPM had initially been made. All 15 had extrapontine demyelinating lesions but the pons was normal. Two others had only lateral pontine lesions, so that only three of 20 patients had definite CPM. All but one of the 20 hyponatremic patients had a definite hypoxic event prior to any therapy with intravenous sodium chloride. The involved brain areas included basal ganglia, thalamus, cortical gray matter, and periventricular white matter, areas often affected by hypoxia. Each of the three patients in whom unequivocal findings of CPM were present had long histories of chronic alcoholism and hepatic cirrhosis. CONCLUSIONS: These results suggest that: (1) Neither hyponatremic encephalopathy nor its therapy is commonly associated with CPM; (2) Patients with chronic alcoholism who also become hyponatremic can develop pontine demyelinating lesions; (3) Most patients with symptomatic hyponatremia who are diagnosed as having CPM in fact have diffuse cerebral demyelinating lesions with a normal pons; (4) The distribution of cerebral demyelinating lesions in patients with hyponatremic encephalopathy is compatible with hypoxic damage.

Imaging in acquired immune deficiency syndrome dementia complex (AIDS dementia complex): a review.

1. Since 1986, the AIDS dementia complex has been recognized increasingly as a significant sequela of HIV infection. Though the pathogenesis remains poorly characterized and the diagnosis is one of exclusion, research utilizing various imaging techniques has resulted in greater understanding of the progressive character of ADC. 2. CT and MR studies have revealed general atrophy, atrophy of the basal ganglia, and white matter lesions that appear to increase in severity with progression of HIV infection. 3. CT and MR are relatively insensitive to the presence of clinical dementia, neurological signs, or positive findings on neuropsychological tests. In addition, they show poor correlation with histopathologic findings. 4. PET, SPECT, and MRS show the greatest sensitivity to changes wrought by primary HIV infection of the CNS and thus the greatest potential for early diagnosis of ADC. 5. PET, SPECT, and MRS show involvement of the basal ganglia, bloodflow abnormalities, and metabolite disturbances in HIV-seropositive subjects without findings on examination or neuropsychological tests.

Successful therapy for trilateral retinoblastoma.

Trilateral retinoblastoma, the intracranial malignancy associated with bilateral retinoblastoma, is an uncommon and clinically aggressive malignancy with a uniformly fatal outcome. Three children with newly diagnosed trilateral retinoblastoma were treated with systemic (cyclophosphamide and vincristine) and intrathecal (methotrexate, hydrocortisone, and cytarabine) chemotherapy, as well as craniospinal irradiation (one patient) in addition to therapy of the eye lesions. All three patients have had partial or complete response of the pineal tumors to chemotherapy, with no active disease eight or more years, 33 or more months, and 12 or more months, respectively, after diagnosis of the lesions.

MR imaging of the brain in patients with diabetes insipidus.

Diabetes insipidus is a clinical syndrome characterized by the excretion of copious volumes of dilute urine combined with persistent intake of abnormally large quantities of fluid. There are two general forms of the disease, central (vasopressin deficient) and nephrogenic (vasopressin resistant). Diabetes insipidus of central origin most often results from lesions in the hypothalamic-neurohypophyseal axis. Twenty-six cases of central diabetes insipidus were evaluated with the use of high-field-strength MR imaging. A wide variety of precipitating conditions were found, including Langerhans cell histiocytosis, neoplasia, trauma, and infection. A thickened pituitary infundibulum was seen in most patients, and an absence of high intensity signal in the posterior pituitary lobe on T1-weighted images was seen in every case. Analysis of stalk morphology; associated brain findings; and correlation with the patient's age, sex, history, and radiographs of other body parts improved diagnostic specificity. When combined with clinical information, MR imaging is able to provide a specific diagnosis in almost all cases of central diabetes insipidus.

Ethmoid mucocele in an infant with a benign fibroosseous lesion.

We present a case of ethmoid mucocele occurring in a two-month-old child, and associated (presumably causally) with a benign fibroosseous lesion of the ethmoid bone. These two conditions may be more frequently associated than previously recognized; MR greatly facilitates their evaluation.

MRA delineation of the vertebral-basilar system in patients with hemifacial spasm and trigeminal neuralgia.

Detailed depiction of the vertebral-basilar system is often obscured by other vascular structures on the MR angiogram. A special MR angiography technique that can better delineate the branches of the vertebral-basilar system has been designed and has proved particularly useful in the identification of tortuous vascular branches when they come in contact with the seventh or fifth cranial nerves.

Contrast-enhanced MR imaging of the facial nerve in 11 patients with Bell's palsy.

Contrast-enhanced MR images (at 1.5 T) were obtained in 11 patients with facial palsy. The group included five people with acute idiopathic facial (Bell's) palsy, three with chronic idiopathic facial palsy, and one each with acute facial palsy after local radiation therapy, acute facial palsy resulting from herpes zoster virus infection, and facial palsy caused by facial neuroma. Eight of the 11 patients demonstrated marked enhancement of the affected facial nerve from the labyrinthine portion through the descending canal. Three patients also demonstrated mild enhancement of the distal canalicular portion of the facial nerve, simulating small distal acoustic neuromas. No difference in the pattern of enhancement between the acute or chronic Bell's palsy patients was seen. Radiographic resolution appeared to lag behind clinical resolution. The facial neuroma appeared distinct from the other lesions as a focally enhancing mass. The enhancement pattern in the Bell's group correlated with the histopathologic features of Bell's palsy and is consistent with the viral hypothesis of the syndrome. Thin-section contrast-enhanced MR scans are recommended for individuals with atypical presentation of facial paralysis. In the proper clinical setting, contrast-enhanced MR imaging may permit a positive radiographic diagnosis of Bell's palsy, which has previously been a diagnosis of exclusion.

Gadolinium enhancement of the leptomeninges caused by hydrocephalus: a potential mimic of leptomeningeal metastasis.

A patient had severe hydrocephalus and diffuse leptomeningeal enhancement on MR which mimicked leptomeningeal spread of a primary brain tumor. The leptomeningeal enhancement resolved completely after decompression of the hydrocephalus. Data suggest that the leptomeningeal enhancement is caused by vascular stasis induced by the hydrocephalus.

Neuroimaging findings in rare amebic infections of the central nervous system.

The imaging findings in a case of panamebic meningoencephalitis and in a case of granulomatous amebic encephalitis, two rare infections of the central nervous system caused by amebae, are presented and the world literature is reviewed. The brain CT findings in panamebic meningoencephalitis are nonspecific; our case showed diffuse edema. In the case of granulomatous amebic encephalitis, there was evidence of large arterial occlusions and MR demonstration of spinal cord infarctions.

Detection of cerebrospinal fluid metastasis: CT myelography or MR?

PURPOSE: To determine the sensitivity of contrast MR versus myelography followed by CT in the detection of cerebrospinal fluid metastases in children with primary central nervous system tumors. METHODS: Thirty-three patients who had primary central nervous system malignancies had spinal MR with gadolinium within 2 weeks of a myelogram followed by CT. MR technique included T1-weighted image sequences of the entire spine with and without gadolinium. CT scans were routinely performed at T-12 to L-2, L-4 to S-1, and foramen magnum to C-2. All studies were reviewed blindly; the number, character, and location of all metastases was recorded and the results of the two studies compared. Cerebrospinal fluid cytologic findings were recorded for each patient, and compared with the results of the imaging studies. RESULTS: Seven of the 33 patients had metastases detected; metastases were seen on both MR and myelography followed by CT. However, MR showed 24 lesions and myelography followed by CT showed only 15. When a lesion was seen on both MR and myelography followed by CT, the MR was usually more convincing. Superficial lesions seen on MR sometimes would be missed on myelography followed by CT. Both MR and myelography followed by CT were quite sensitive in the detection of small lesions (2 to 3 mm) when present on spinal nerve roots. Whereas MR showed multiple lesions not seen on myelography followed by CT, CT failed to show any metastases not seen on MR. Imaging studies showed metastases in 3 patients who had normal cytologic findings. CONCLUSIONS: MR shows significantly more cerebrospinal fluid metastases than myelography followed by CT.

Intra- and paraorbital lesions: value of fat-suppression MR imaging with paramagnetic contrast enhancement.

The orbital area of 18 individuals was examined by using a combination of fat-suppression contrast-enhanced MR imaging to determine whether contrast between fat and surrounding tissues could be improved over that obtained with conventional fat-suppression techniques alone. We used a hybrid technique combining two independent methods of fat suppression. Subjects consisted of 16 patients and two normal volunteers. Fifteen individuals received gadopentetate dimeglumine, and conventional T1-weighted, T2-weighted, and fat-suppression T1-weighted images were obtained. The fat-suppressed T1-weighted images obtained after contrast administration provided more information than did the conventional MR images. Intraorbital and paraorbital lesions could be distinguished easily from intraorbital fat that had been suppressed. Cases of chorioretinitis and optic neuritis could be confidently diagnosed only by this technique. Cases of optic nerve meningioma and mixed conal lesions also were better appreciated. Because of sharp contrast between tissue planes, this technique was helpful for detecting any intraorbital invasion from paraorbital lesions. Fat-suppression MR imaging with paramagnetic contrast enhancement can significantly improve the delineation of both normal and abnormal structures and better define lesional margins in the orbit, where large amounts of fat are present. Our results support earlier findings, and we suggest that postcontrast fat-suppressed T1-weighted imaging be used instead of conventional T1-weighted postcontrast imaging in evaluating orbital and paraorbital lesions.

Improved detection and delineation of head and neck lesions with fat suppression spin-echo MR imaging.

To compare conventional and fat suppression MR imaging in their ability to detect head and neck lesions, we prospectively studied 17 patients with head and neck tumors and one normal volunteer. Five patients had benign tumors (one mixed cell tumor, one hemangioma, one lipoma, and two plexiform neurofibromas), 10 had malignant tumors (six squamous cell carcinomas, two minor salivary gland carcinomas, one lymphoma, and one malignant fibrous histiocytoma), and two had nonspecific lymphadenopathy. All subjects were studied with standard spin-echo T1- and T2-weighted images (T2-weighted imaging was done with and without fat suppression technique). In addition, T1-weighted images with contrast enhancement and fat suppression were obtained in nine patients. A four-point grading system was used for comparison of the conventional and fat suppression images. Grades ranged from 0 (unsatisfactory, the lesion cannot be seen) to 3 (excellent, the lesion and its margins can be seen clearly with sharp contrast from surrounding normal tissue). We found that postcontrast fat suppression T1-weighted images and fat suppression T2-weighted images were most useful; these sequences obtained an average score close to grade 3 (2.77 and 2.85, respectively). On the other hand, the conventional T2-weighted images had an average score of about 2 (1.82) and the conventional T1-weighted image had a score of about 1 (1.33). Fat suppression T2-weighted sequences generally were superior in cases of lymphadenopathies. Postcontrast T1-weighted images were most useful in a case of plexiform neurofibroma, owing to their fibrous component and lower proton density.(ABSTRACT TRUNCATED AT 250 WORDS)

Frontoethmoidal giant cell reparative granuloma.

We present a case of a giant cell reparative granuloma of the frontoethmoidal region that had a large intracranial extraaxial component and was studied with MR. Although rare, giant cell reparative granuloma can be suggested in the correct clinical setting and when MR features suggest a fibrous lesion.

Cerebral amyloid angiopathy presenting as a brain mass.

The MR images of a patient with cerebral amyloid angiopathy (a localized vascular deposition of amyloid without evidence of systemic amyloidosis) showed an extensive right temporoparietal lobe mass with frontal lobe extension that was slightly hypointense on T1-weighted images and heterogeneously hyperintense on T2-weighted images. No contrast enhancement was identified on MR imaging.

Thickened pituitary stalk on MR images in patients with diabetes insipidus and Langerhans cell histiocytosis.

The MR images of four female patients with acute onset of central diabetes insipidus and pathologically confirmed Langerhans cell histiocytosis were evaluated retrospectively for evidence of lesions in the hypothalamic-pituitary axis. The examinations were conducted on a 1.5-T MR system with thin-section sagittal and coronal T1-weighted (short TR/short TE) and T2-weighted (long TR/long TE) images. Three patients underwent T1-weighted MR after IV administration of gadopentetate dimeglumine. Compared with 20 normal subjects who were evaluated with the same MR protocol, three of the four patients had a symmetrically thickened pituitary stalk that demonstrated homogeneous signal enhancement following contrast administration. The high signal intensity of the posterior lobe, which was seen in normal subjects on T1-weighted sagittal images, was absent in all four patients. Two patients had associated abnormalities on either chest films or imaging studies of the temporal bone and two patients had isolated CNS Langerhans cell histiocytosis. The combination of a thickened pituitary stalk and absent posterior pituitary hyperintensity, while nonspecific for Langerhans cell histiocytosis, should nevertheless prompt further studies, such as chest films, bone scanning, or temporal bone CT, to attempt to narrow the differential diagnosis. Gadopentetate dimeglumine, in particular, may be a useful adjunct in the MR examination of the patient with diabetes insipidus.

Loculated intracranial leptomeningeal metastases: CT and MR characteristics.

Studies of twenty-five patients with loculated leptomeningeal tumor metastases diagnosed by CT and/or MR were analyzed retrospectively. Medulloblastoma was the most frequent primary tumor (8/25, 32%). Four subgroups of loculated patterns were identified. Type A included mass(es) limited to the subarachnoid space without obvious direct parenchymal infiltration; this pattern occurred in 12 patients, of whom five had associated diffuse pattern. Type B was characterized by mass(es) still predominantly in the subarachnoid space but with minor transpinal parenchymal infiltration; this pattern was found in five patients. Type C comprised subarachnoid mass(es) with marked transpinal extension mimicking parenchymal lesion; this pattern was observed in three patients. Type D consisted of subarachnoid mass(es) growing along the perineural CSF space; this pattern was noted in two patients. Additionally, two patients presented with combined A and C patterns, and one patient had a combined B and C pattern. More than half the patients (14/25, 56%) presented with a single lesion. The most frequent locations were the suprasellar cistern, ventricular walls, and lateral recesses of the fourth ventricle, Gd-DTPA-enhanced T1-weighted MR images appeared best for demonstrating the site and extent of disease. Recognition of the loculated patterns of leptomeningeal metastases, which are less common than the diffuse pattern, is important to radiologists and clinicians for correct diagnosis and proper management of patients with this disease.