Parenchymal Forms Research Articles

Diffuse Leptomeningeal Glioneuronal Tumors: A Case Series of Five Patients with Parenchymal Forms and an Analysis of the Diagnostic Challenges, Treatment Options and Outcomes.

Diffuse leptomeningeal glioneuronal tumors (DL-GNT) are rare glioneuronal neoplasms with oligodendroglioma-like cells. These tumors can present as a dominant intracranial mass or as a solitary spinal cord mass without leptomeningeal involvement. In this study, we aimed to determine the magnetic resonance imaging and histopathological features, treatment modalities, and clinical outcomes of the parenchymal forms of DL-GNTs. This is a retrospective three-center case series study of 5 patients with a confirmed parenchymal form of DLGTs, out of which 4 patients were adults. Brain and spinal cord MR imaging were performed in all patients at either 1.5 or 3T. The patients' age ranged from 5 years to 50 years with a mean age of 27.6 years at presentation. Four of the tumors were located in the frontal lobe, and one in the tectum. They were usually solid-cystic enhancing tumors as the other mixed neuronal-glial tumors. All of the tumors had an extension to the superficial surface of a cerebral hemisphere. One had systemic bone metastases. The clinical signs and symptoms of the parenchymal form varied based on the location of the mass, in contrast to the leptomeningeal form associated with hydrocephalus. In one case, the tumor's initial grade was defined as intermediate. The initial histopathology of the two cases was low-grade and no upgrade occurred in the follow-up period. In two cases, although the tumors were low grade initially, they progressed to an anaplastic form in the follow-up period. The parenchymal form of DL-GNTs is common in adults. Extension to the superficial surface of a cerebral hemisphere is a distinctive imaging feature. Systemic osseous metastasis may occur. Due to the presence of common histopathological features, including the biphasic composition of glial and neuronal cell elements and oligodendroglioma-like cells, a proposed classification approach might be more beneficial for the histopathological and imaging description, and management of the glioneuronal tumors with oligodendroglioma-like features.

Cysticercotic fourth ventricle ependymitis

Neurocysticercosis is endemic in many regions of the world. Extra parenchymal forms of neurocysticercosis are infrequent but may be associated with hydrocephalus. We report the case and show a typical MRI of fourth ventricle ependymitis caused by cysticercosis. Keywords: ependymitis; cysticercosis.

Cysticercotic fourth ventricle ependymitis

Neurocysticercosis is endemic in many regions of the world. Extra parenchymal forms of neurocysticercosis are infrequent but may be associated with hydrocephalus. We report the case and show a typical MRI of fourth ventricle ependymitis caused by cysticercosis. Keywords: Ependymitis; cysticercosis.

L’hamartochondrome : cause inhabituelle d’obstacle endobronchique chez des patients fumeurs

Endobronchial hamartochondroma is a rare benign tumor which differs from the parenchymal form in its symptomatology and also by its treatment which should be as conservative as possible. The endobronchial location is exceptional. Here we present the cases of two patients with endobronchial hamartochondroma associated with clinical manifestation, chest pain and repeated pulmonary infections, respectively. The diagnosis was made after performing a CT-scan, a PET-SCAN and histological analysis. After discussion in a multidisciplinary staff meeting, conservative treatment was chosen in both cases.

Cerebrospinal Fluid Changes in Experimental Model of Extraparenchymal Neurocysticercosis: preliminary results

Introduction: Neurocysticercosis (NCC) is the most common parasitic disease affecting the CNS and is considered a public health problem in many countries around the world, including Brazil. The cerebrospinal fluid (CSF) changes provoked by the disorder in humans are well defined in literature and were first described in 1940 by Lange. These modifications include pleocytosis, hyperproteinorrachia and eosinophilia. The majority of the experimental models of NCC published are focused on the parenchymal form of the disease. Few models studied the extraparenchymal form or analyzed the CSF parameters. Objective: To analyze the temporal modifications in the CSF of rabbits after the experimental induction of extraparenchymal NCC. Method: We included 14 male rabbits in this study. Each animal underwent a suboccipital injection of 50 cysts of T. crassiceps into the cisterna magna. Just before the inoculation, CSF samples were collected (sham). The animals were observed for 6 months, with monthly suboccipital punctures for CSF sample collection. Cellularity and protein concentration changes were analyzed throughout the observation period. Results: The initial cell counting average was only 1 (±0.88) cell/μL, raising to 1138.9 (±624.1) cells/μL in the first month and then dropping to 17.2 (±14.4) cells/μL in the sixth month. The differential analysis proved that, after the inoculation, lymphocytes were the most common cells in the CSF (85.1%, ±14.6). A similar pattern was identified with the proteinorrachia: an initial average of 25.8 (±8.3) mg/dL, increasing to 66.5 (±22.5) mg/dL one month later and then gradually decreasing to 40.2 (±15.6) mg/dL in the last month. Conclusion: It was possible to identify a substantial increase in cellularity and protein concentration in the CSF after the induction of NCC in rabbits, followed by a gradual fall over the remaining months. These variations are comparable to the findings in humans. The authors are currently giving continuity to this study, and stronger data will be released in future publications.

Detection of Taenia solium DNA in the Urine of Neurocysticercosis Patients.

Neurocysticercosis (NCC), caused by Taenia solium larvae that reside in the central nervous system, results in serious public health and medical issues in many regions of the world. Current diagnosis of NCC is complex requiring both serology and costly neuroimaging of parasitic cysts in the brain. This diagnostic pipeline can be problematic in resource-constrained settings. There is an unmet need for a highly sensitive and clinically informative diagnostic test to complement the present diagnostic approaches. Here, we report that T. solium-derived cell-free DNA is readily detectable in the urine of patients with the subarachnoid and parenchymal forms of NCC, and discuss the potential utility of this approach in enhancing and refining T. solium diagnostics.

MORPHOLOGICAL FEATURES OF INFLAMMATORY AND REACTIVE-DYSTROPHIC LESIONS OF THE MINOR SALIVARY GLANDS OF PATIENTS WITH DISEASES OF THE GASTROINTESTINAL TRACT

The authors conducted a histological study of biopsy a small salivary gland (SSG) in 12 patients with various diseases of the gastrointes-tinal tract. It was found that in SSG there were signs of chronic sialadenitis and sialadenosis, the latter being primarily expressed in parenchymal form. Sialadenosis and chronic sialadenitis had signs of dysfunction of sialocits, sclerosis and lipomatosis of the stroma and/or parenchyma, often with dilation of the ducts, lymphoid and lymphomacrophagal infiltration of the stroma and/or parenchyma. Noted that there was a pronounced of the plethora of microcirculatory vessels with edema of the stroma.

The sociodemographic characteristics and risk factors for tuberculosis morbidity between two decades at the beginning of the 21st century at the north of Kosovo, Serbia

Background/Aim. Tuberculosis (TB) is a major cause of mortality and morbidity worldwide, affecting different countries disproportionally. Effective diagnosis and treatment of TB saved an estimated 43 million lives between 2000 and 2014. The aim of our study was to show sociodemographic influences, risk factors for the morbidity and clinical characteristics of tuberculosis among residents of northern Kosovo, Serbia, between two decades at the beginning of the 21st century. Methods. A prospective study was conducted at the Department for Pulmonology of the Clinical-health Centre, Kosovska Mitrovica, included all tuberculosis patients treated during two three-year periods, between 2000?2002 and 2012?2014. In total, 134 patients with tuberculosis were treated, 91 in the first observed period and 43 in the second period. Results. In both observed periods, male patients suffered from tuberculosis more frequently. In the first observed period, younger and middle age (18?49) persons suffered from tuberculosis more frequently (p = 0.014). In relation to the place of residence, in the first reporting period there were more patients from urban areas, while in the second, there were significantly more patients from rural areas (p = 0.008). In the second observed period, TB was significantly more associated with chronic obstructive pulmonary disease (p = 0.001) and comorbidities with TB were significantly more frequent (p = 0.006). During the 2000?2002 period, there were more severe clinical forms, with severe radiological changes, bilateral parenchymal and cavernous forms (p = 0.08). Mild unilateral parenchymal lesions were more common in the last 3 years (p = 0.02). Conclusion. Social determinants, older age and comorbidities are the most important risk factors for the persistent number of patients, and therefore this target group needs attention during active approach in TB screening.

Magnetic resonance imaging spectrum of intracranial tubercular lesions: one disease, many faces.

Tuberculosis is a devastating disease and has shown resurgence in recent years with the advent of acquired immunodeficiency syndrome. Central nervous system involvement is the most devastating form of the disease, comprising 10% of all tuberculosis cases. The causative organism, Mycobacterium tuberculosis, incites a granulomatous inflammatory response in the brain, the effects of which can be appreciated on magnetic resonance imaging (MRI), which can thus be used for diagnosis of the same. Neurotuberculosis can present in various patterns, which can be identified on MRI. The meningeal forms include leptomeningitis and pachymeningitis. Parenchymal forms of neurotuberculosis include tuberculoma in its various stages, tubercular cerebritis and abscess, tubercular rhombencephalitis, and tubercular encephalopathy. Each pattern has characteristic MRI appearances and differential diagnoses on imaging. Complications of neurotuberculosis, usually of tubercular meningitis, include hydrocephalus, vasculitis, and infarcts as well as cranial nerve palsies. Various MRI sequences besides the conventional ones can provide additional insight into the disease, help in quantifying the disease load, and help in differentiation of neurotuberculosis from conditions with similar imaging appearances and presentations. These can enable accurate and timely diagnosis by the radiologist and early institution of treatment in order to reduce the likelihood of permanent neurological sequelae.

AN ISCHEMIC STROKE CAUSED BY MENINGOVASCULAR SYPHILIS

Fourteen percent of all ischemic strokes occur in young patients (< 45 years). One of the causes of ischemic stroke in young patients is the isolated cerebral arteritis related to a neuroinfection. The author describes a clinical case of ischemic stroke in a young patient with neurosyphilis. Diagnosis of syphilis was confirmed by laboratory investigations; liquor tests and clinical data allowed to put a diagnosis of meningovascular syphilis. Timely diagnostics and treatment of syphilitic infection are helpful in avoiding progression of neurosyphilis, including its parenchymal forms.

Bacterial, Fungal, and Parasitic Infections of the Central Nervous System: Radiologic-Pathologic Correlation and Historical Perspectives.

Despite remarkable progress in prevention and treatment, infectious diseases affecting the central nervous system remain an important source of morbidity and mortality, particularly in less-developed countries and in immunocompromised persons. Bacterial, fungal, and parasitic pathogens are derived from living organisms and affect the brain, spinal cord, or meninges. Infections due to these pathogens are associated with a variety of neuroimaging patterns that can be appreciated at magnetic resonance imaging in most cases. Bacterial infections, most often due to Streptococcus, Haemophilus, and Neisseria species, cause significant meningitis, whereas the less common cerebritis and subsequent abscess formation have well-documented progression, with increasingly prominent altered signal intensity and corresponding contrast enhancement. Atypical bacterial infections are characterized by the development of a granulomatous response, classically seen in tuberculosis, in which the tuberculoma is the most common parenchymal form of the disease; spirochetal and rickettsial diseases are less common. Fungal infections predominate in immunocompromised hosts and are caused by yeasts, molds, and dimorphic fungi. Cryptococcal meningitis is the most common fungal infection, whereas candidiasis is the most common nosocomial infection. Mucormycosis and aspergillosis are characterized by angioinvasiveness and are associated with high morbidity and mortality among immunocompromised patients. In terms of potential exposure in the worldwide population, parasitic infections, including neurocysticercosis, toxoplasmosis, echinococcosis, malaria, and schistosomiasis, are the greatest threat. Rare amebic infections are noteworthy for their extreme virulence and high mortality. The objective of this article is to highlight the characteristic neuroimaging manifestations of bacterial, fungal, and parasitic diseases, with emphasis on radiologic-pathologic correlation and historical perspectives.

Radiological findings in Behçet disease.

Between 5 and 30% of patients with Behçet's disease will present neurological signs during the course of their illness. In order to evaluate the radiological signs on neuro-behçet disease, we studied consecutive patients in whom the diagnosis of this disease was retained, and who referred from January 2004 to December 2011 to the neurology and internal medicine departments in Mohammed VI universitary hospital in Marrakesh. Using 1.5T magnetic resonance imaging (MRI) with axial and coronal T2- weighted, axial Fluid Attenuated Inversion Recovery (FLAIR), pre- and post-contrast axial, coronal and sagittal T1-weighted sequences. The final number of patients in whom the diagnosis criteria of behçet disease were fulfilled and in whom an MRI was performed was 68 cases. Among these patients, 52 had parenchymal form of neuro-behçet with abnormalities in the MRI, 12 had vascular form and 4 patients had normal MRI. The brainstem, cerebral white matter, basal ganglia, internal capsule, thalamus and spinal cord were involved in forty four, thirty one, thirty, twenty nine, seventeen and four patients, respectively. The cerebral peduncle was the brainstem structure mainly involved with thirty cases followed by the pons with, twenty cases. Midbrain involvement interested forty patients. Brainstem atrophy was seen in eighteen cases. Finally, control MRI were obtained in four cases only, and showed changes in lesions size and shape.

Neuro-Behçet Disease and Autoinflammatory Disorders

Misregulation of innate immunity leads to autoinflammation. Behçet disease is an autoinflammatory condition involving recurrent attacks of inflammation in skin, eyes, joints, and even the nervous system. The etiology may involve vascular inflammation. Central nervous system involvement in neuro-Behçet disease (NBD) comes in the form of parenchymal NBD or nonparenchymal NBD. The parenchymal form has a predilection for the brainstem, diencephalon and cerebral hemispheres, and represents a meningoencephalitis thought to be related to small vessel vasculitis. Cerebral venous sinus thrombosis, arising from a vasculitic process of large veins, comprises the majority of vascular NBD cases. The rarer monogenetic autoinflammatory syndromes are characterized by periodic fever, and typically present in the pediatric population. Neurologic involvement in these syndromes typically presents in the form of an aseptic meningitis. Treatment of autoinflammatory disorders involves immune modulation with corticosteroids, disease-modifying antirheumatic medications, and increasingly antibodies targeting cytokines like tumor necrosis factor α and interleukin 1.

Diffuse parenchymal form of malakoplakia in renal transplant recipient: a case report

Malakoplakia is an unusual chronic inflammatory disease related to prior urinary tract infection. It is characterized by the presence of macrophages with foamy cytoplasm exhibiting larger PAS positive inclusions that stain for calcium and iron. Malakoplakia affects renal allograft and is associated with severe morbidity. Herein, the authors report a new case of renal graft malakoplakia in a 23-year-old female patient. The patient received a living-related donor renal transplantation with a high immunological risk. Plasmapheresis and intravenous immunoglobulin (i.v. Ig) treatment, pre- and post-transplant, and induction with rabbit anti-thymocyte globulins were used due to presence of donor specific antibodies and positive B cross match by flow cytometry. The patient had an early urinary tract infection with a good outcome. On Day 36 post-transplant (PO), the patient returned to the clinic with fever, graft pain and acute renal dysfunction leading to hemodialysis. Escherichia coli (E. coli) was present in the blood and urine culture. At the time, the renal biopsy revealed numerous sheets of macrophages with foamy, eosinophilic cytoplasm showing several PAS positive granules and large inclusions that stained strongly with hematoxylin, calcium (von Kossa method) and iron (Prussian blue). The patient was diagnosed with malakoplakia related to a kidney transplant. Despite prolonged treatment with antibiotics, determined by a susceptibility test, the patient did not recover renal function and remained on dialysis.

Treatment of neurocysticercosis: is it beneficial?

Evaluation of: Carpio A, Kelvin EA, Bagiella E et al.; Neurocysticercosis Group TE. The effects of albendazole treatment on neurocysticercosis: a randomized controlled trial. J. Neurol. Neurosurg. Psychiatry DOI: 10.1136/jnnp.2008.144899 (2008) (Epub ahead of print).Neurocysticercosis is the most common parasitic disease of the CNS. Intracranial neurocysticercosis is classified into cerebral parenchymal and extraparenchymal forms (subarachnoidal and intraventricular). Current medical therapy for parenchymal and extraparenchymal neurocysticercosis invovles the use of anticysticercal drugs. Albendazole and praziquantel are two commonly used drugs. Most experts prefer to always use oral or parenteral corticosteroids along with anticysticercal drugs. The role of anticysticercal drugs in the treatment of parenchymal neurocysticercosis has always been a subject of intense controversy. Whether therapy with anticysticercal drugs results in improved seizure-related prognosis remains a major concern. A recent meta-analysis evaluating six controlled trials suggests that treatment with anticysticercal drugs results in better resolution of colloidal and vesicular cysticerci; in patients taking anticysticercal drugs, there was a lower risk of seizure reccurence in patients with colloidal cysticerci and a significant reduction in the rate of generalized seizures in patients with vesicular cysticerci. However, the findings of the study by Carpio et al. have revived the controversy regarding this treatment. These authors noted that the effect of a single dose of albendazole is, at best, only modest. Only a minority of patients with parenchymal neurocysticercosis benefitted and became cyst free and there was a lack of effectiveness toward the associated seizure disorders. No response was seen in patients with degenerative parenchymal or extraparenchymal forms of neurocysticercosis. Carpio et al. suggest that there is a need for further studies that employ different approaches, such as the use of high initial doses of albendazole, the use of two anticysticercal drugs or repeated prolonged courses of albendazole.

Dificultades diagnósticas y terapéuticas en la neurocisticercosis: Presentacion de 6 casos y revisión de la literatura

The incidence of neurocysticercosis (NCC) is increasing currently in developed countries due to the migration movements from endemic countries. Due to NCC polymorphism, treatment would be individualized in each case. Countries not used to this disease have to deal with. To set up diagnostic and therapeutic guidelines in all sorts of NCC and choose the correct treatment would be challenging. To perform a descriptive and retrospective analysis of six cases of NCC seen in the Neurosurgery Department of the Hospital Clinic de Barcelona from 1992 to 2000 (both included). We have performed a revision of the literature about diagnostic and therapeutic methods. Definitive or probable diagnosis of NCC is based on clinical, imaging, immunological, and epidemiological criteria. In patients with inactive disease only symptomatic treatment is indicated. In active parenchymal forms there are not consensus if antiparasitic treatment is indicated. However, in extraparenchymal active disease aggressive treatment with antiparasitic agents and steroids is recommended. In cases of intracranial hypertension, neurological deficits or hydrocephalus surgery is the treatment of choice.

Treatment of neurosyphilis

In 2001, the incidence of primary and secondary syphilis increased in the United States for the first time in a decade. Increasing rates of early syphilis among men who have sex with men have been reported in many American cities, with similar outbreaks noted in Canada and Europe. In San Francisco, the increase has been particularly sharp and accompanied by an increase in the incidence of neurosyphilis. Early neurosyphilis develops within weeks to years of primary infection and primarily involves the meninges. Syndromes include syphilitic meningitis (often accompanied by cranial neuropathies), meningovascular syphilis (with associated ischemic stroke), or asymptomatic neurosyphilis. Late neurosyphilis occurs years to decades after exposure as cerebral or spinal gummatous disease or the classic parenchymal forms affecting the brain (general paresis or syphilitic encephalitis) or spinal cord and nerve roots (tabes dorsalis). Treponema pallidum, the causative agent, cannot be cultured in vitro, and microscopic techniques are laborious. Thus, diagnosis depends on serologic tests and cerebrospinal fluid (CSF) examination. The suboptimal sensitivity and specificity of these tests complicate diagnosis, particularly among patients coinfected with HIV. CSF examination should be performed to evaluate for neurosyphilis in all patients with positive serum syphilis serology and neurologic, ophthalmic, or tertiary disease, or in those who have failed therapy, and in HIV-infected patients with late latent syphilis or syphilis of unknown duration. Intravenous penicillin G is the recommended treatment for all forms of neurosyphilis and for syphilitic eye disease. An outpatient alternative, if adherence can be assured, is intramuscular benzathine penicillin with oral probenecid. Newer drugs that penetrate CSF, such as ceftriaxone or azithromycin, have not yet been adequately tested for neurosyphilis. Syphilis facilitates transmission of HIV (and vice versa), and thus all patients diagnosed with syphilis should be offered HIV testing.

Metabolic lung disease: imaging and histopathologic findings

Metabolic lung disease includes pulmonary alveolar proteinosis (PAP), pulmonary amyloidosis, metastatic pulmonary calcification, dendritic pulmonary ossification, pulmonary alveolar microlithiasis, and storage diseases. In pulmonary alveolar proteinosis, CT demonstrates air-space consolidation with thickened interlobular septa, producing the so-called “crazy paving” appearance. Pulmonary amyloidosis can appear as parenchymal nodules (nodular parenchymal form), diffuse interstitial deposit (diffuse interstitial form), or submucosal deposits in the airways (tracheobronchial form). Metastatic pulmonary calcification may appear on high-resolution CT as numerous 3- to 10-mm diameter calcified nodules or, more commonly as fluffy and poorly defined nodular opacities. In pulmonary microlithiasis, high-resolution CT demonstrates diffuse punctuate micronodules showing slight perilobular predominance resulting in apparent calcification of interlobular septa. Niemann–Pick disease appears as ground-glass attenuation in the upper lung zone and thickening of the interlobular septa in the lower lung zone. Radiologic study including high-resolution CT will be helpful for the diagnosis and follow-up of these diseases.

Neurocysticercose de diagnostic tardif : à propos de deux cas

Introduction. – Neurocysticercosis is the most frequently encountered cerebral parasitic infection worldwide. It is due to infection of the central nervous system by Taenia solium larval form. According to the location of the cysts, parenchymal and extra-parenchymal forms may be identified, with different clinical expressions. Exegesis. – We report two cases of neurocysticercosis, one with typical parenchymal involvement and the second with extra-parenchymal involvement revealed by increased intra-cranial pressure. In both cases, the diagnosis was established over 10 years after the onset of symptoms. Conclusion. – Neurocysticercosis is very frequent in non-Islamic developing countries, and its incidence is increasing in industrialized nations in relation to tourism and immigration from highly endemic areas. Symptoms usually appear several years after infection and this accounts for the frequent delays before the diagnosis is established.

Interrelationship between radiologic findings and prognosis of epilepsy in children with neurocysticercosis.

Epileptic manifestations of Neurocysticercosis (NC) appear to depend on number and localization of the cysts. The objective of this study was to investigate the relationship between CT findings, number of parasites and the evolutive stage of the cysts, and the prognosis of epilepsy in children with NC. We studied 28 patients with the parenchymal form of NC, considering: epilepsy duration; seizure frequency before and after AED treatment; seizure control; number of AED and recurrence after AED withdrawal. Clinical information was crossed with the number of lesions and disease activity in univariate comparison. The analysis of the clinical data in relation to the number of lesions and disease activity showed no statistical difference among the variables (p>0.05). We conclude that the course of epilepsy due to NC in childhood cannot be based exclusively on the number or stage of the parasites.