Abnormal Leptomeningeal Enhancement Research Articles

Radio-pathologic correlation: no pial angioma-subarachnoid varicose network drainage pathway in Sturge-Weber syndrome.

The traditional imaging findings reported in Sturge-Weber syndrome (SWS) include endpoints of cortical injury-cortical atrophy and cortical calcifications-but also what has been termed a "leptomeningeal angiomatosis," the latter recognized and reported as a leptomeningeal enhancement on magnetic resonance imaging (MRI). The objective of this study is to demonstrate through neuropathological correlation that the "leptomeningeal angiomatosis" in patients with Sturge-Weber syndrome (SWS), represents a re-opened primitive venous network in the subarachnoid space that likely acts as an alternative venous drainage pathway, seen separately to abnormal pial enhancement. Retrospective review of MR imaging and surgical pathology of patients that underwent surgery for epilepsy at a tertiary, children's hospital. A pediatric radiologist with more than 20years of experience reviewed the MR imaging. Surgically resected brain specimens that had been sectioned and fixed in 10% paraformaldehyde for histologic processing, following processing and paraffin embedding, were cut into 5-µm unstained slides which were subsequently stained with hematoxylin and eosin (H&E). Slides were re-examined by a board-certified pediatric neuropathologist, and histologic features specifically relating to cerebral surface and vascularity were documented for correlation with MR imaging of the resected region performed prior to resection. Five patients were reviewed (3 boys and 2 girls; the median age at the onset of seizures was 12months (IQR, 7 to 45months); the median age at surgery was 33months (IQR, 23.5 to 56.5months)). Surgical procedures included the following: 4, hemispherotomy (right: 2, left: 2) and 1, hemispherectomy (right). A subarachnoid space varicose network was present on both MRI and histology in 4 patients. Calcifications were seen on both MRI and histology in 3 patients. Abnormal leptomeningeal enhancement was present in 5 patients and seen separately from the subarachnoid vascular network in 4 patients. Histopathology confirmed the MRI findings of a subarachnoid space varicose network seen separately from leptomeningeal enhancement and presumed to represent an alternative venous drainage pathway to compensate for maldevelopment of cortical veins, the primary abnormality in SWS. No pial-based angioma was identified.

A Case of Primary Intracranial Spindle Cell Carcinoma with DICER-1 Mutation Presenting as Abnormal Leptomeningeal Enhancement in an Immunocompetent Patient (P6-5.028)

A Case of Primary Intracranial Spindle Cell Carcinoma with DICER-1 Mutation Presenting as Abnormal Leptomeningeal Enhancement in an Immunocompetent Patient (P6-5.028)

Contrast Enhanced Fluid-Attenuated Inversion Recovery Versus Contrast Enhanced T1-Spin Echo Magnetic Resonance Imaging in the Evaluation of Infectious Meningitis

Background Infectious meningitis is an infection of the subarachnoid space causing inflammation of the leptomeninges. Early diagnosis is crucial to initiate appropriate therapy, avoid complications, and improve prognosis. Cerebrospinal fluid analysis is important in the diagnosis and is done by performing lumbar puncture with some limitations and complications. MRI plays a role in the diagnosis by depicting abnormal leptomeningeal enhancement. Aim of the study To evaluate the efficiency of contrast-enhanced fluid-attenuated inversion recovery compared with contrast-enhanced T1 spin echo in the diagnosis of infectious meningitis. Patients and Methods A prospective analytical study of diagnostic tests was conducted. Brain MRI was done using a 3-Tesla system. Gadolinium was used as contrast material. The final diagnosis of meningitis was made based on cerebrospinal fluid analysis. Validity parameters, predictive values, and diagnostic accuracy for both sequences were calculated and compared to each other. Quantitative and qualitative analysis was performed. Results Fifty-six patients were involved (31 males and 25 females) in this study, and they ranged from 15 to 68 years in age. Cerebrospinal fluid analysis was positive in 41 patients and negative in 15. Contrast enhanced–fluid attenuated inversion recovery has higher sensitivity, specificity, positive predictive value, negative predictive value, and accuracy compared with contrast enhanced–T1 spin echo. The mean net meningeal enhancement is significantly higher in contrast enhanced–fluid attenuated inversion recovery. Substantial interobserver agreement between the two sequences was observed. Conclusion Contrast enhanced–fluid attenuated inversion recovery is superior to contrast enhanced–T1 spin echo in the diagnosis of infectious meningitis. .

INNV-01. THE CASE OF LEPTOMENINGEAL CARCINOMATOSIS RELATED HYDROCEPHALUS MANAGED WITH SYSTEMIC AND INTRATHECAL CHEMOTHERAPY

Abstract INTRODUCTION Leptomeningeal carcinomatosis (LC) is an end-stage sequela of metastatic cancer that commonly leads to severe neurological symptoms due to hydrocephalus and commonly treated with surgery or radiation. As these procedures are invasive and have associated risks, chemotherapy could be a great alternative. We report a case of a hydrocephalus related to LC in a patient with Stage IV breast cancer that resolved after the administration of HD-MTX and intrathecal cytarabine. CASE A 64-year-old right-handed woman with Stage IV ER positive, PR positive, HER2 negative invasive lobular carcinoma of the left breast on chemotherapy with palbociclib and alpelisib who presented with mild occipital headaches, diplopia and imbalance. Imaging revealed an extensive abnormal leptomeningeal enhancement withing the posterior fossa compatible with leptomeningeal carcinomatosis. CSF cytology was consistent with metastatic adenocarcinoma. Liquid biopsy detected circulating tumor cells. During the admission for the cycle 1 of systemic chemotherapy with HD-MTX and intrathecal cytarabine, neurological status deteriorated as she became stuporous due to the newly developed moderate obstructive and communicating hydrocephalus. Surgical innervation was considered but given her response to serial Ommaya taps and dexamethasone, we proceeded with observation. Symptomatic improvement was noted within few days and confirmed on imaging showing the resolution of previously seen hydrocephalus. DISCUSSION This case highlights the role of non-invasive combination of systemic and intrathecal chemotherapy in patients with LC-related hydrocephalus aimed to preserve patient’s quality of life. No relevant disclosures.

Imaging of Neurologic Disease in Hospitalized Patients with COVID-19: An Italian Multicenter Retrospective Observational Study.

Of 725 consecutive hospitalized patients with coronavirus disease 2019, 108 (15%) had acute neurologic symptoms necessitating neurologic imaging.

Primary leptomeningeal B-cell lymphoma.

A 47-year-old man presented with a 6-week history of left facial paraesthesia, diplopia and progressive ataxia with altered sensation in both feet. Contrast magnetic resonance imaging (MRI) of brain and spinal cord demonstrated abnormal thickening and leptomeningeal enhancement of all visible cranial nerves, most striking in cranial nerves III (top left) and V (top right). Further leptomeningeal enhancement was evident in the anterior pons, brain stem and cauda equina but no parenchymal brain or spinal cord lesions were present. Whole body positron emission tomography showed no evidence of disease elsewhere. Cerebrospinal fluid (CSF) examination showed total protein 5.25 g/l [normal range (NR) 0.1–0.5], glucose <0.3 mmol/l (NR 2.5–4.5) and elevated white cell count at 165/mm3 (NR < 10), comprising 80% lymphoid cells and 20% neutrophils. A CSF cytospin demonstrated a population of large, pleomorphic cells with prominent nucleoli on a background of small, mature T cells (bottom left). The large cells were strongly positive for CD20 and CD79a (bottom right) but negative for CD10 and terminal deoxynucleotidyl transferase. A diagnosis of primary leptomeningeal B-cell lymphoma was made. The patient commenced MATRIX (methotrexate, cytarabine, rituximab and thiotepa) immunochemotherapy with rapid improvement in symptomatology and resolution of the cranial nerve thickening on MRI. He completed 4 cycles of MATRIX followed by a thiotepa-conditioned autologous stem cell transplant and remains in complete remission 14 months after presentation.

To Compare Diagnostic Ability of Contrast-Enhanced Three-Dimensional T1-SPACE with Three-Dimensional Fluid-Attenuated Inversion Recovery and Three-Dimensional T1-Magnetization Prepared Rapid Gradient Echo Magnetic Resonance Sequences in Patients of Meningitis.

Aims:The aim of this study is to compare postcontrast three-dimensional (3D) T1-Sampling perfection with application-optimized contrasts by using different flip angle evolutions, 3D fluid-attenuated inversion recovery (FLAIR), and 3D T1-magnetization prepared rapid gradient echo (MPRAGE) sequences in patients of meningitis.Settings and Design:A hospital-based cross-sectional study done in the Department of Radiodiagnosis, IGMC Shimla for a period of 1 year from June 1, 2016, to May 30, 2017.Subjects and Methods:A total of 30 patients suspected of meningitis underwent magnetic resonance imaging evaluation with postcontrast 3D T1-MPRAGE, 3D T1-SPACE, and 3D FLAIR sequences. The abnormal leptomeningeal enhancement was noted by two radiologists together on these sequences and scores were given to the abnormal leptomeningeal enhancement.Statistical Analysis Used:The sensitivity of 3D T1-SPACE, 3D T1-MPRAGE, and 3D FLAIR was calculated and compared. The level of agreement between these sequences was assessed by kappa coefficient. P < 0.05 was taken as statistically significant.Results:3D T1-SPACE shows superiority in meningeal enhancement along basal cisterns, Sylvian fissure and along cerebral convexities. It is also found to be better in delineating parenchymal lesions. 3D FLAIR failed to demonstrate enhancement along cerebral convexities however found to be better than 3D T1-MPRAGE in delineating enhancement along basal cisterns and Sylvian fissures. 3D T1-MPRAGE has shown subtle enhancement in basal cisterns, Sylvian fissure and along cerebral convexities. 3D T1-SPACE, 3D FLAIR, and 3D T1-MPRAGE has sensitivity of 91.67%, 66.67%, and 54.17%, respectively.Conclusion:Postcontrast 3D T1-SPACE sequence is an excellent tool than postcontrast 3D T1-MPRAGE and 3D FLAIR sequences in the evaluation of meningeal enhancement and depiction of additional lesions in brain parenchyma.

Contrast-enhanced fluid-attenuated inversion recovery magnetic resonance imaging was useful in detecting inflammation of the meninges in neuropsychiatric systemic lupus erythematosus: a case report

A 49-year-old woman was admitted to our hospital with recurrent episodes of paresthesia attacks evolving in 5 to 15 minutes from the left hand to the left leg through the left trunk. Neurological examination revealed cortical sensory disturbance in her left hand. Although contrast-enhanced T1-weighted MRI findings were unremarkable, contrast-enhanced fluid-attenuated inversion recovery (FLAIR) MRI revealed abnormal leptomeningeal enhancement over the sulcus of the parietal lobe, including the sulcus around the postcentral gyrus. Because we assumed the cause of the recurrent sensory attack to be meningeal inflammation around the primary somatosensory cortex, we treated this patient by increasing the dose of prednisolone. The increase in prednisolone dose completely resolved the symptom, as well as the abnormal leptomeningeal enhancement on contrast-enhanced FLAIR MRI. In patients with suspected meningeal inflammation, contrast-enhanced FLAIR MRI, which is reported to be more sensitive than contrast-enhanced T1-weighted MRI in detecting subtle abnormal leptomeningeal enhancement, should be the modality of choice.

Krukenberg Tumor with Leptomeningeal Carcinomatosis Misdiagnosed as Acoustic Neuroma

Objective: Leptomeningeal carcinomatosis (LMS) is defined as the diffuse infiltration of the leptomeninges and subarachnoid space by malignant cells metastasizing from systemic cancer. Patients usually have a known underlying malignancy, but primary presentation can be with symptoms of meningeal involvement. We describe a rare case of Krukenberg tumor with LMS initially presented as bilateral progressive sensorineural hearing loss, which was misdiagnosed as acoustic neuroma. Patient: A 50 year old man with a history of cervical cancer was referred to the Ear Nose and Throat (ENT) clinic because of progressive hearing loss, tinnitus, dizziness and blurred vision for 5 months. Results: A CT scan of the abdomen and pelvis showed a right adnexal mass, suspicious for an ovarian neoplasm. The patient underwent surgical resection of her pelvic mass. Surgical pathology revealed poorly differentiated adenocarcinoma with scattered signet ring cells, favorable with metastatic gastric adenocarcinoma. Magnetic resonance imaging revealed abnormal leptomeningeal enhancement. A lumbar puncture was performed, the CSF analysis showed elevated protein and positive for signet malignant cells. Conclusion: Our case highlights the importance of the development of new diagnostic tools and treatment regimens for LMC.

NI-80 * CASE REPORT: TREATMENT RESPONSE IN LEPTOMENINGEAL CARCINOMATOSIS FROM GLIOMATOSIS CEREBRI TREATED WITH BEVACIZUMAB (AVASTIN)

Gliomatosis cerebri (GC) is a diffuse glioma, usually astrocytic with remarkable infiltration in at least 3 cerebral lobes considered a Grade III tumor according to WHO classification. Leptomeningeal gliomatosis(LG) occurs in 17% of patients with GC, although reported as microscopic dissemination in up to 70% of post-mortem studies amongst all gliomas. GC is a very rare and aggressive glioma, while, leptomeningeal metastasis is a severe complication of malignancy, both presenting with very poor prognosis. There is limited data on standard of care for these patients. Bevacizumab is an anti-angiogenic drug approved for treatment of malignant gliomas. Studies have shown prolongation of progression-free survival and improvement of quality of life. There are a few reports of Avastin being used for GC or LG. We report a case of a 52 year-old male presenting with 3 weeks of nausea and vomiting, preceded by 12 months of mild cognitive decline. MRI studies revealed extensive areas abnormal Flair signal and diffuse leptomeningeal enhancement seen throughout the brain and spinal cord. Diagnosis of GC with leptomeningeal dissemination was confirmed with brain biopsy. The patient completed radiotherapy and received Temozolomide, in addition to 6 intrathecal cytarabine injections. A repeat MRI showed progression of disease and therapy was switched to Avastin. We monitored his progress with serial MRI's, which revealed marked decrease in size and enhancement of previous lesions, consistent with a response to Avastin. The patient remained clinically stable during this interval. A literature review of patients already on Avastin for systemic malignancies revealed some case reports of patients progressing to LG. A decrease in enhancement of lesions was also observed in these patients. However, there are few reported cases of leptomeningeal carcinomatosis secondary to GC. These MRI changes may be clinically relevant and additional studies are required to further investigate a correlation.

A 20-Year-Old Woman With Headache and Transient Numbness

A 20-year-old woman with a history of migraine presented with 4 days of progressive ptosis, discoordination, paresthesias in her hands and feet progressing to her forearms and shins, and diplopia after an upper respiratory tract illness the prior week. Laboratory evaluation at this time was notable for a positive immunoglobulin (Ig)G Lyme antibody (negative IgM), antinuclear antibody (ANA) 1:80 in a speckled pattern, and a positive GD1b antibody. Lumbar puncture (LP) showed white blood cells 30/mL (87% lymphs), red blood cells 3/mL, protein 68 mg/dL, glucose 49 mg/dL, and a negative Gram stain and bacterial culture. Brain magnetic resonance imaging (MRI) was normal. She was diagnosed with Miller-Fisher variant Guillian-Barre Syndrome and treated with a 5-day course of intravenous Ig, after which her symptoms markedly improved. She was evaluated in the clinic 3 times over the course of the next 4 months, and was found to have a normal neurologic examination except for absent reflexes at each clinic visit, and was otherwise asymptomatic. She was then lost to follow-up. Two-and-a-half years later, she again sought medical attention after a transient sensory disturbance which she described as numbness that began in the roof of her mouth with progression over the course of 10 minutes to involve her entire face and left arm. The numbness lasted 1 hour and then fully resolved. She had experienced a minor headache 2 hours prior that was alleviated by caffeine. Neurologic examination at this time was normal, but a brain MRI showed abnormal leptomeningeal enhancement (Figure 1), and a subsequent LP showed a lymphocytic pleocytosis with a markedly elevated protein and low glucose (Table 1). She underwent an extensive evaluation but no cause for the leptomeningeal enhancement on MRI or the abnormal cerebrospinal fluid (CSF) profile was identified. Two months later, she began to develop progressively worsening vertigo, headache, nausea, and diplopia. The MRI showed new enhancing lesions in the brain and enhancement of the leptomeninges along the entire length of the spinal cord (Figure 1). Evaluation of the spinal fluid at this time again showed CSF pleocytosis and hypoglycorrhacia with an opening pressure of 14 cm water (Table 1), but a cause for these abnormal radiologic and CSF findings could not be identified. Neurosurgical evaluation deemed a biopsy of the affected area would subject the patient to an unacceptably high risk of serious permanent neurologic deficit, and therefore biopsy was deferred. She was placed on empiric treatment for tuberculous meningitis with rifampin, isoniazid, pyridoxine, ethambutol, and a steroid taper. Her symptoms improved rapidly and she was asymptomatic over the course of the next 2 months, with an interval LP demonstrating improvement in the CSF profile (Table 1), and brain MRI showing near resolution of her leptomeningeal enhancement. Unfortunately, after completing her steroid taper she developed nausea, vomiting, and a headache that was worse when lying in the recumbent position. The MRI showed worsening hydrocephalus with return of contrast enhancement of the basilar leptomeninges, as well as the presence of fluid in the maxillary sinus with mucosal thickening. A right inferior nasal turbinate biopsy was unrevealing. Lumbar puncture again showed a CSF lymphocytic pleocytosis with elevated protein and low glucose (Table 1). She was restarted on dexamethasone. One month later, she had a generalized tonic-clonic seizure that was witnessed by her boyfriend. The MRI showed worsening hydrocephalus, and a LP revealed an elevated opening pressure of 40 cm water. A ventriculoperitoneal (VP) shunt was placed at this time to

Central nervous system histoplasmosis in an immunocompetent patient

Histoplasma capsulatum is endemic in parts of Africa, Asia, the United States, and Latin America. Most infections are benign and self-limiting, but disseminated disease can occur in immunocompromised patients, particularly in the setting of HIV infections [3]. Central nervous system (CNS) involvement is found in 5–10% of cases of disseminated histoplasmosis [6], but isolated CNS histoplasmosis is rare. Here we present an unusual case of recurrent granulomatous disease of the CNS and epiglottis in an immunocompetent adult due to histoplasmosis. A 46-year-old Ugandan woman, who came to England in her teens, presented in 2001 with progressive headaches, vomiting, meningism and confusion. Recent travel history was limited to North American and European cities. Chest X-ray and MRI of the brain were normal. Cerebrospinal fluid (CSF) examination revealed 350 lymphocytes, 1.19 g/l protein and 2.3 mmol/l glucose (6 mmol/l plasma), and negative culture. She was treated for probable tuberculous meningitis with dexamethasone and anti-tuberculous therapy for 12 months. Her condition improved significantly although repeat MRI of the brain in 2002 showed abnormal leptomeningeal enhancement around the pons and occipital sulci. She developed hydrocephalus in 2003, necessitating insertion of a ventriculo-peritoneal shunt. She remained well until 2004 when she developed dysphonia. Laryngoscopy detected an epiglottic mass with non-caseating granulomata on biopsy. No mycobacteria were seen. Two subsequent biopsies confirmed the findings. Whole body fluorodeoxyglucose PET in 2005 revealed increased laryngeal uptake. She received courses of high dose oral steroids for probable epiglottic sarcoidosis with partial clinical response. She presented to us in December 2006 with worsening dysphonia, spastic paraparesis, sensory deficit to the T8 level and urinary retention. Blood tests including inflammatory markers, autoantibodies, angiotensin converting enzyme and HIV serology were normal or negative. MRI showed diffuse meningeal enhancement around the brainstem and entire spinal cord but no intramedullary lesions. CSF examination showed normal opening pressure, 44 mononuclear cells, 2.5 mmol/l glucose (8.1 mmol/l plasma), 3.44 g/l protein, and intrathecal synthesis of oligoclonal bands. She was treated empirically with high dose steroids and anti-tuberculous therapy (rifampicin, isoniazid, pyrazinamide and ofloxacin). Her condition continued to decline with ascending weakness and sensory deficit (T1 level). MRI in January 2007 showed more extensive meningeal enhancement and intramedullary nodules of enhancement in the cervical cord and medulla (Fig. 1). CSF examination showed 180 polymorphs, 0.2 mmol/l glucose (6.6 mmol/l plasma) and 12.3 g/l protein. CSF culture and PCR for mycobacteria were negative. Serum brucella and histoplasma antibodies and aspergillus precipitins were negative. Laryngeal biopsy showed non-caseating granulomata with no organisms. Protionamide was added for possible resistant tuberculosis. Her condition did not improve and intravenous liposomal amphotericin B was initiated to cover granulomatous fungal infection. Y. F. Tai (&) D. M. Kullmann R. S. Howard N. P. Hirsch T. Revesz S. M. Leary National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK e-mail: yen.tai@imperial.ac.uk

An unusual central nervous system involvement in rheumatoid arthritis: combination of pachymeningitis and cerebral vasculitis

Severe primary central nervous system (CNS) involvement such as vasculitis and pachymeningitis can rarely occur in rheumatoid arthritis (RA) even in the absence of systemic disease activation. The authors illustrate a female patient with well-controlled RA who presented with headaches, encephalopathy, seizures and relapsing focal neurological deficits. Primary rheumatoid cerebral vasculitis and pachymeningitis were diagnosed based on suggestive brain magnetic resonance (MR) imaging, MR angiography, cerebrospinal fluid analysis and cerebral angiography. MR showed abnormal leptomeningeal enhancement and hyperintense FLAIR signal in the cortical subarachnoid spaces consistent with pachymeningitis. Cerebral angiography findings were consistent with vasculitis. Aggressive treatment resulted in significant clinicoradiological resolution. Cerebral vasculitis is a rare but certain manifestation of RA. This complication can be diagnosed in the presence of suggestive angiographic and CSF findings. The condition may be steroid resistant, and needs to be treated more aggressively.

Magnetic resonance imaging of eosinophilic meningoencephalitis caused by Angiostrongylus cantonensis following eating freshwater snails

Angiostrongyliasis cantonensis is a worldwide-existing parasitic disease. However, the relevant reports on its radiological appearances are limited. In this study, we investigated magnetic resonance imaging (MRI) features of eosinophilic meningoencephalitis in a group of consecutive patients caused by human infection with Angiostrongylus cantonensis after eating freshwater snails. We performed brain MR imaging on 74 patients with angiostrongyliasis cantonensis. The scanner was a 0.5T unit. For each patient MR pulse sequences of SE T1-weighted image (T1WI) and FSE T2-weighted image (T2WI) were used. After intravenous administration of gadolinium chelate (Gd-DTPA) repeated T1-weighted images were obtained. MRI features of the lesions in the brain and meninges were analyzed and recorded after observing initial and follow-up MR images. The classification of the types of angiostrongyliasis cantonensis infection was done on the basis of locations of the disorders. Forty-one (55%) normal and 33 (45%) abnormal MRI appearances in the brain were found. According to locations of the disorders, the types of angiostrongyliasis cantonensis infection were determined as follows: seventeen cases of type meningitis, three of type myeloencephalitis, one of type neuritis and twelve of mixed type (eight of type ventriculitis and five of type pneumonitis were among them). In type meningitis, abnormal leptomeningeal enhancement was visualized. In type myeloencephalitis, lesions in the brain parenchyma may have iso- or slightly low signal intensity on T1WI and high signal intensity on T2WI. Enhanced nodules in various shapes were shown on gadolinium-enhanced T1WI, a few lesions appeared as crescent enhancements and some lesions did not reveal abnormal enhancement. Other than brain lesions, an enhanced nodule was seen in the cervical spinal cord in one patient. In type ventriculitis, brain ventricular enlargement was demonstrated. In type neuritis, a nodule and abnormal enhancement in the right optic nerve was revealed. In type pneumonitis, patchy ground-glass opacity and consolidative lesions at the periphery of the lungs were seen. Follow-up results indicated that most lesions in the brain could resolve in 2 to 8 weeks. Angiostrongyliasis cantonensis presented as both single type and mixed type. Nodular enhancing lesions in the brain and/or linear enhancement in the leptomeninges were the main findings, while crescent enhancement would be the characteristic sign of the disease on gadolinium-enhanced T1WI. Focal edematous changes without contrast enhancement in the brain could be seen on MRI in some cases.

Cerebral Perfusion Abnormalities in Children With Sturge-Weber Syndrome Shown by Dynamic Contrast Bolus Magnetic Resonance Perfusion Imaging

Sturge-Weber syndrome is characterized by leptomeningeal angiomatosis and a facial naevus that is usually unilateral. Magnetic resonance imaging is the cornerstone of confirming the disease and judging the extent of the abnormalities. It has been shown, however, that brain perfusion abnormalities on nuclear medicine imaging often are more extensive than the abnormal leptomeningeal enhancement on magnetic resonance. In this article, we assess the utility of magnetic resonance perfusion in demonstrating perfusion abnormalities in pediatric cases of Sturge-Weber syndrome. Magnetic resonance perfusion studies were performed on 7 consecutive children who presented to our department with clinically suspected Sturge-Weber syndrome. The extent of time to peak abnormality on dynamic gadolinium bolus magnetic resonance perfusion imaging was compared with the extent of leptomeningeal enhancement and the presence of venous abnormalities. Good magnetic resonance perfusion data were obtained in all 7 cases. Perfusion abnormalities were closely anatomically related to meningeal enhancement on postcontrast T1-weighted imaging. However, perfusion abnormalities were found consistently in the vicinity of developmental venous anomalies that were present in 4 of 7 cases. In 1 child, there was a perfusion deficit in the cerebellar lobe contralateral to the leptomeningeal angiomatosis, consistent with crossed cerebellar diaschisis. Magnetic resonance perfusion is a sensitive indicator of perfusion abnormalities in Sturge-Weber syndrome and can be performed easily at the same time as the diagnostic scan. Magnetic resonance perfusion imaging therefore is useful in the assessment of this disease. This approach has the extra advantage of correlating the perfusion abnormalities with the high-resolution imaging that is provided from magnetic resonance imaging.

An Unusual Presentation of Rheumatoid Meningitis

Central nervous system involvement in rheumatoid arthritis can rarely occur in the absence of systemic disease. Rheumatoid meningitis has not been reported to present as spells of neurologic dysfunction. The authors describe a woman with a history of well-controlled rheumatoid arthritis who presented with headaches and spells of focal neurological dysfunction. Brain magnetic resonance imaging, brain biopsy, and temporal artery biopsy were required to make the diagnosis of rheumatoid meningitis with arteritis. Neuroimaging revealed abnormal leptomeningeal enhancement. Necrotizing granulomatous inflammation was seen on meningeal and brain biopsy. A temporal artery biopsy showed evidence of arteritis without giant cells. The possibility of central nervous system involvement by rheumatoid arthritis should be considered in patients with a history of rheumatoid arthritis even in the absence of systemic symptoms. Making the diagnosis may require meningeal and brain biopsy. The condition may be steroid responsive.

Experimental bacterial meningitis in rats: Demonstration of hydrocephalus and meningeal enhancement by magnetic resonance imaging

We investigated whether magnetic resonance imaging (MRI) is able to detect intracranial manifestations of advanced bacterial meningitis in rats. Meningitis was induced in nine animals by injecting 150 µl 107 colony forming units per ml of Streptococcus pneumoniae into the cisterna magna. MRI was performed 24 h (n = 5) and 48 h (n= 4) after infection. Controls included (I) animals that were injected intracisternally with 150 µl phosphate-buffered saline or (II) animals without puncture of the cisterna magna. T2-weighted and T1-weighted MR images before and after administration of 0.3 mmol kg-1 of gadolinium-DTPA were obtained. Hydrocephalus was found in 7 of 9 infected animals, but not in the control group. Abnormal leptomeningeal enhancement was found in all infected animals, but in none of the controls. The animals imaged after 48 h showed a more pronounced hydrocephalus and a more intense leptomeningeal enhancement than animals imaged after 24 h. Even in small animals such as rats, MRI can be used to detect the presence of bacterial meningitis and its associated complications. MRI may be a useful noninvasive method for monitoring the possible effect of adjunctive therapeutic strategies in experimental studies of meningitis. [Neurol Res 2002; 24: 307-310]

High-dose gadolinium-enhanced MRI for diagnosis of meningeal metastases.

We compared high-dose (0.3 mmol/kg) and standard-dose (0.1 mmol/kg) gadolinium-enhanced MRI for diagnosis of meningeal metastases in 12 patients with suspected meningeal metastases. They were imaged with both standard-dose and high-dose gadolinium. All patients with abnormal meningeal enhancement underwent at least one lumber puncture for cerebrospinal fluid (CSF) cytology, while patients with normal meningeal enhancement were followed clinically. All patients with negative CSF cytology also were followed clinically. A single observer reviewed all the images, with specific attention to the enhancement pattern of the meninges. Abnormal leptomeningeal enhancement was present in three cases, and abnormal pachymeningeal enhancement in three other patients. All of these patients had abnormal CSF analyses. In two of the three cases of abnormal leptomeningeal enhancement the disease was more evident on high-dose than on standard-dose imaging; in one case the abnormal enhancement was visible only on high-dose imaging. In one of the three cases with abnormal pachymeningeal enhancement, the disease was evident prospectively only with high-dose imaging.

The spectrum of MRI findings in CNS cryptococcosis in AIDS

We retrospectively reviewed the cranial MRI appearances of 25 patients with AIDS and microbiologically proven central nervous system (CNS) cryptococcosis. Four patients had a normal scan. Ten patients had dilated perivascular Virchow-Robin spaces that were hyperintense on T2-weighted images. Nine of these patients developed progressive cryptococcomas, eight in the basal ganglia and one in the cerebral white matter. The cryptococcomas displayed high signal on T2-weighted and intermediate to low signal on T1-weighted images. None enhanced after dimeglumine gadopentetate. No abnormal dural or leptomeningeal enhancement was detected in any patient. One patient developed an acquired arachnoid cyst during treatment of CNS cryptococcosis which was thought to represent a focal collection of organisms and mucoid material within the subarachnoid space. In addition either cerebral atrophy and/or background white matter hyperintensity on T2-weighted images was present in 19/25 patients. In two patients the neuropathological findings at autopsy correlated well with the imaging abnormalities. In conclusion, this spectrum of MRI appearances in CNS cryptococcosis reflects the pathological mechanism of invasion by the fungus, but a normal scan or one with features of CNS HIV infection such as atrophy or white matter hyperintensity does not exclude the diagnosis.

Diffuse alveolar haemorrhage associated with progressive systemic sclerosis.

A 41 year old man with an eight year history of progressive systemic sclerosis developed severe diffuse alveolar haemorrhage and died. The importance of diffuse alveolar haemorrhage as a rare but potentially serious complication of connective tissue disease should not be overlooked.