Extraparenchymal Forms Research Articles

Concurrent Extraparenchymal and Spinal Neurocysticercosis in an Immunocompetent Patient Treated for Obstructive Hydrocephalus and Spinal Cord Compression

Introduction: Neurocysticercosis (NCC) is a condition caused by infection of the central nervous system (CNS) by Taenia solium, or pork tapeworm, larvae. Despite commonly infecting the CNS, it may be easily misdiagnosed for more common pathologies, such as arachnoid cysts or tumors. Though most frequently seen in endemic areas, immigration patterns are altering its traditional distribution. Extraparenchymal neurocysticercosis (including ventricular, spinal, and subarachnoid types) carries a poorer prognosis Objective: We present a case of NCC presenting in a young immunocompetent patient which was initially thought to be an arachnoid cyst due to non-pathognomonic imaging results and symptoms. This case illustrates a challenging NCC diagnosis. Case Report: A 24-year-old male without known past medical history presented with 2 years of chronic headache, 2 months of blurry vision in the left eye, and 1 week of progressive nausea and vomiting found to have aqueductal stenosis with obstructive hydrocephalus and bilateral 1.5 cm, peripherally-enhancing cystic lesions of the cerebellopontine angle with associated mass effect, initially described as arachnoid cysts, as well as a left sided foramen magnum cyst. He had an external ventricular drain placed and later underwent ventriculoperitoneal shunt (VPS) insertion. He presented 10 days later with sepsis symptoms and the initial workup was concerning for tuberculosis-associated meningitis and he underwent shunt externalization and re-internalization after clinical improvement, though he was lost to follow up. Two months later he presented with acute urinary retention and right sided weakness. He had spinal cord compression of the cervicothoracic and thoracolumbar spine due to cystic lesion and underwent right C7-T1 and T10-T11 hemilaminotomies for cyst resection, with pathology consistent with NCC. Conclusion: Neurocysticercosis (NCC) is a complex disease which is largely overlooked and is increasing in prevalence in non-endemic areas such as the United States. NCC has various symptoms and presentations depending on location and progression of larval development, which may mimic more common pathologies. The clinical manifestations depend on the parasite load and the host’s immune response regarding their location in CNS and their evolutionary stage. There are two types of NCC, the parenchymal and the extraparenchymal form. The extraparenchymal racemose NCC has the worst prognosis among all forms of NCC with mortality ranging between 30% and 80%. This reinforces the need for a broad differential diagnosis in these scenarios, as well as the importance of close Infectious Disease follow up for patients diagnosed with concurrent cranial and spinal NCC.

Standardizing an Experimental Murine Model of Extraparenchymal Neurocysticercosis That Immunologically Resembles Human Infection.

Neurocysticercosis (NCC) is endemic in non-developed regions of the world. Two forms of NCC have been described, for which neurological morbidity depends on the location of the lesion, which can be either within the cerebral parenchyma or in extraparenchymal spaces. The extraparenchymal form (EXP-NCC) is considered the most severe form of NCC. EXP-NCC often requires several cycles of cysticidal treatment and the concomitant use of glucocorticoids to prevent increased inflammation, which could lead to intracranial hypertension and, in rare cases, to death. Thus, the improvement of EXP-NCC treatment is greatly needed. An experimental murine model of EXP-NCC, as an adequate model to evaluate new therapeutic approaches, and the parameters that support it are described. EXP-NCC was established by injecting 30 Taenia crassiceps cysticerci, which are less than 0.5 mm in diameter, into the cisterna magna of male and female Wistar rats. Cyst implantation and infection progression were monitored by detecting the HP10 antigen and anti-cysticercal antibodies in the serum and cerebral spinal fluid (CSF) of infected rats and by magnetic resonance imaging. Higher HP10 levels were observed in CSF than in the sera, as in the case of human EXP-NCC. Low cell recruitment levels were observed surrounding established cysticerci in histological analysis, with a modest increase in GFAP and Iba1 expression in the parenchyma of female animals. Low cellularity in CSF and low levels of C-reactive protein are consistent with a weak inflammatory response to this infection. After 150 days of infection, EXP-NCC is accompanied by reduced levels of mononuclear cell proliferation, resembling the human disease. EXP-NCC does not affect the behavior or general status of the rats. This model will allow the evaluation of new approaches to control neuroinflammation and immunomodulatory treatments to restore and improve the specific anti-cysticercal immunity in EXP-NCC.

Neuroimaging Findings in Racemose Neurocysticercosis: Case Description and Literature Review

Neurocysticercosis (NCC), the most common central nervous system (CNS) parasitic infection among the immunocompetent population can imitate every clinical feature of brain-diseases accurately, drawing attention away from the real culprit and delaying the proper treatment. There are two types of NCC, the parenchymal and the extraparenchymal form. The extraparenchymal NCC include the ventricular cysticercosis, the subarachnoid cysts including giant cysts or racemose cysticercosis with chronic meningitis, the spinal (intra- or extramedullary) cysticercosis and the ophthalmic cysticercosis. It is estimated that about 30% of epilepsy cases in endemic countries are due to NCC and especially the racemose NCC is more aggressive and associated with higher mortality rates. There is a significant heterogeneity in clinical phenotypes, regarding the racemose NCC, which depends on the parasite load and evolutionary stage in association with its location in CNS and the host’s immune response. Crucial for the management of the racemose NCC is the early recognition of the symptoms and the swift initiation of antiparasitic therapy with anti-inflammatory agents in combination with the shunt-insertion in cases of obstructive hydrocephalus. In view of the former considerations we conducted a narrative literature review on racemose NCC and described the diagnostic challenges of a relevant case that we had evaluated in our Department of Neurology.

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.

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.

Behçet's Syndrome and Nervous System Involvement.

Although Behçet's syndrome (BS) is classified as a rare disease in European countries and the USA, its neurologic involvement "neuro-Behçet's syndrome (NBS)" is commonly included in the differential diagnosis of many inflammatory and vascular central nervous system (CNS) disorders. Clinical and neuroimaging findings support two major forms of NBS: parenchymal NBS (p-NBS) and an extra-parenchymal form that presents with cerebral venous sinus thrombosis (CVST). The present review summarizes recent findings on the etiopathogenesis and clinico-radiological features of this disorder as well as its treatment options. Recent laboratory and clinical findings include the discovery of antibodies to human and mouse neuro-fibrils showing cross-reactivity with Streptococcus spp. and Mycobacterium tuberculosis heat shock proteins (HSP) in NBS. Diagnostically, two distinct magnetic resonance imaging (MRI) patterns of spinal cord involvement have been observed: (a) "Bagel sign" and (b) "motor neuron" patterns. Therapeutic studies have shown that infliximab effectively prevents further relapses and stabilizes the symptoms of patients experiencing ongoing clinical relapses while being treated with single or multiple immunosuppressant drugs. Primary neurological involvement referred to as NBS. The majority of patients with NBS present with parenchymal involvement that commonly affects the brain stem-diencephalic region. Headache, dysarthria, ataxia, and hemiparesis are the main clinical features of NBS.

Neurocisticercosis racemosa subaracnoidea gigante y ventricular: a propósito de un caso

Neurocysticercosis is the most frequent parasitic disease of the central nervous system. It is caused by the larvae of Taenia solium, which can affect different anatomical sites. In Spain there is an increasing prevalence mainly due to immigration from endemic areas. The extraparenchymal forms are less common, but more serious because they usually develop complications. Neuroimaging plays a major role in the diagnosis and follow-up of this disease, supported by serology and a compatible clinical and epidemiological context. First-line treatments are cysticidal drugs such as albendazole and praziquantel, usually coadministered with corticosteroids, and in some cases surgery is indicated. We here report a case of neurocysticercosis with simultaneous intraventricular and giant racemose subarachnoid involvement.

Diagnóstico y manejo de la neurocisticercosis en el Perú

Neurocysticercosis (NCC) is the most common parasitic disease of the central nervous system and is caused by larvae of the tapeworn Taenia solium. NCC is endemic in almost all developing countries. It presents as intraparenchymal forms associated with seizures or as extraparenchymal forms associated with intracranial hypertension. The clinical and epidemiological suspicion are important but the diagnosis is made primarily by images and confirmed by serology. Computed tomography (CT) and magnetic resonance imaging tests are used. Inmunodiagnosis by Western Blot, which is currently perform in the Instituto Nacional de Ciencias Neurológicas in serum and cerebrospinal fluid serves as confirmatory test. Treatment involves symptomatic measures (control of seizures or intracranial hypertension) and anticysticercal medications (albendazole and praziquantel). Anticysticercal treatment should be used under hospital conditions because of secondary effects.

Diagnóstico y manejo de la neurocisticercosis en el Perú

Neurocysticercosis (NCC) is the most common parasitic disease of the central nervous system and is caused by larvae of the tapeworm Taenia solium. NCC is endemic in almost all developing countries. It presents as intraparenchymal forms associated with seizures or as extraparenchymal forms associated with intracranial hypertension. The clinical and epidemiological suspicion are important but the diagnosis is made primarily by images and confirmed by serology. Computed tomography (CT) and magnetic resonance imaging tests are used. Immunodiagnosis by Western Blot, which is currently perform in the Instituto Nacional de Ciencias Neurológicas in serum and cerebrospinal fluid serves as confirmatory test. Treatment involves symptomatic measures (control of seizures or intracranial hypertension) and anticysticercal medications (albendazole and praziquantel). Anticysticercal treatment should be used under hospital conditions because of secondary effects.

Chronic Myelopathy Associated to Intramedullary Cysticercosis

Case report. Discuss an isolated intramedullary neurocysticercosis (NCC) case in an adult patient with chronic progressive onset myelopathic symptomatology with clinical, radiologic, and pathologic correlation. NCC is the most common parasitic infection in the central nervous system. Spinal NCC belongs to the group of extraparenchymal forms of NCC and it is considered an extremely rare form characterized by a distinct clinical entity due to mass effect on the spinal cord. Description of a 62-year-old male patient case who presented with chronic but progressive low cord myelopathy who underwent radiologic investigation through magnetic resonance imaging depicting a thoracic intramedullary cystic lesion at level T11. Surgical excision of the intramedullary lesion was preformed and pathologic study confirmed a cysticercus. There was complete resolution of the neurologic symptoms and follow-up monitoring was unremarkable. Although intramedullary is a rare NCC location, it should be considered in the differential diagnosis in high-risk populations especially when cord compression and myelopathy symptoms are present. Magnetic resonance imaging remains the investigative and follow-up modality of choice, and promptly lesion recognition is fundamental for surgical planning and to improve the patient outcome.

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.

Neurocysticercosis Masquerading as Eclampsia

Neurocysticercosis Masquerading as Eclampsia

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.

Surgical Treatment of Cysticercosis of the Central Nervous System

Summary: Cysticercosis is the most frequent parasitosis of the central nervous system. Because anticysticercal drugs do not prevent complications such as hydrocephalus, many patients with cysticercosis require surgical intervention. A review of the pathophysiology and surgical treatment of this disease is presented. Free spinal cysts may need surgical removal, generally with transient good results. Cysts compressing cranial nerves or the brainstem may be removed, generally with good results. Frequently patients with cysticercosis need surgery to control increased intracranial pressure. Based on the pathophysiological mechanisms of intracranial hypertension, different surgical approaches may be indicated. Giant cysts are removed with good results. Patients with the pseudotumoral form of cyst whose clinical treatment has failed generally have poor outcomes when subjected to decompressive craniectomies. Removal of cysts and/or ventriculoperitoneal shunting are performed in patients with hydrocephalus. Removal of free ventricular cysts generally produces a good outcome. Patients with adherent cysts need ventriculoperitoneal shunting posteriorly. Patients with inflammatory hydrocephalus require ventriculoperitoneal shunting. Despite many complications, ventriculoperitoneal shunting is effective to control increased intracranial pressure. Generally, patients with intraparenchymal forms of cysticercosis (normal size or giant cysts) have a better prognosis than do patients with the extraparenchymal form (cisternal, ventricular, and spinal), except for cases of free intraventricular cysts. We present our recent policy for management of patients with this disease.