1/9. guillain-barre syndrome: perspectives with infants and children.An acute flaccid paraparesis or ascending quadriparesis in an infant or child constitutes a very important pediatric neurology emergency. The guillain-barre syndrome (GBS) is the most frequent cause. This is primarily an autoimmune, post-infectious, demyelinating, peripheral nervous system process. A small percentage of children develop a primary axonal process not unlike that identified more commonly in china. Because of the potential for acute respiratory compromise, any child suspected of having GBS needs immediate hospitalization. The major considerations in differential diagnosis include transverse myelitis, toxic neuropathies, tick paralysis, infantile botulism, myasthenia gravis, and dermatomyositis. On occasion, some younger children present with an acute severe pain syndrome that may mask as a pseudo-encephalopathy. Another clinical variant is the Miller-Fisher syndrome characterized by ataxia, ophthalmoparesis, and areflexia. This is associated with a high frequency of the anti-GQ-1-b antibodies. Although most children with GBS have a relatively benign clinical course, some become very ill and require intubation with intensive care monitoring. Immunomodulating treatment should be used for any child who loses the ability to walk. To date, no well-controlled study has been completed analyzing the relative merits of the two most commonly used therapies, namely plasmapheresis or intravenously administered immunoglobulin.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/9. Cervical intramedullar schistosomiasis as a rare cause of acute tetraparesis.INTRODUCTION: The trematode infection schistosomiasis affects at least 200 million people in endemic areas. Granulomas cause the typical manifestations of urogenital, intestinal and hepatolienal schistosomiasis. Involvement of other organs especially the central nervous system (CNS) is uncommon. CASE REPORT: We describe a 40-year old male with a history of repeated contact with schistosome contaminated water. After having suffered from flu-like symptoms with fever and arthralgias, he first presented with a polyradiculopathy of unknown origin. Then 4 weeks later an acute tetraparesis occurred. Spinal magnetic resonance imaging (MRI) revealed a spinal stenosis and query medullary hyperintensities at C6-C8 without contrast-enhancement. Serologic testing was positive for schistosomiasis. The intraoperative appearance at decompressive laminectomy revealed a myelitic form of schistosomiasis. Under therapy with praziquantel, initially high dose cortisone and intensive physiotherapy, symptoms slowly improved over months. On follow-up 1 year later, the patient presented with a spastic distally marked tetraparesis and sensory impairment from C6 downwards. CONCLUSION: Cervical intramedullar schistosomiasis is a rare cause of acute tetra- or para-paresis in patients, who have had contact with schistosomes. early diagnosis is essential because of the excellent prognosis with specific therapy.- - - - - - - - - - ranking = 4.2313940899683keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/9. Longitudinal myelitis associated with systemic lupus erythematosus: clinical features and magnetic resonance imaging of six cases.Myelopathy is a rare central nervous system (CNS) complication associated with systemic lupus erythematosus (SLE). Acute transverse myelitis (ATM) is the most frequent form of SLE-related myelopathy. magnetic resonance imaging (MRI) typically shows increased signal intensity in T2-weighted images and cord swelling. In the present paper, we describe six cases of SLE-related myelopathy with multiple increased signals in the T2-weighted images involving continuous levels of the cervical and thoracic spinal cord, a distinctive feature recently named 'longitudinal myelitis'. The clinical and laboratory findings are similar to those presented by ATM patients, including paraparesis, sensory level and sphincter disturbances. Four patients had positive antiphospholipid antibodies (aPL) suggesting that this could be a characteristic of longitudinal myelitis. Treatment in all cases included high doses of corticosteroids and immunosuppressive agents (intravenous (i.v.) cyclophosphamide). Anticoagulation therapy was given to one patient and two others received low doses of aspirin. The outcome was mainly unfavorable with slow improvement in only one case, no improvement in two and relapse of the myelopathy in the remaining three. In conclusion, longitudinal myelitis is an unusual form of SLE-related myelopathy, it might be associated with aPL and it has a poor prognosis.- - - - - - - - - - ranking = 4.2313940899683keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/9. Report of a Turkish child with Sjoren-Larsson syndrome associated with peripheral nerve involvement.Sjoren-Larsson syndrome is a rare hereditary neurocutaneous disorder characterized by ichthyosis, spastic di- or tetra-plegia, and mild to moderate mental retardation. In this article, we present a nine-year-old girl with the classical features of the syndrome associated with peripheral nerve involvement because of its rare presentation. To the best of our knowledge, only three cases of Sjoren-Larsson syndrome with peripheral nerve involvement have been previously reported in the literature. We assume that Sjoren-Larsson syndrome involves extensive disorders of the ectodermal tissues, including the peripheral nerves as well as the skin and the central nervous system.- - - - - - - - - - ranking = 4.2313940899683keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/9. The importance of suspecting superficial siderosis of the central nervous system in clinical practice.Once the central nervous system surface is greatly encrusted with haemosiderin, even removing the source of bleeding will have little effect on the progression of clinical deterioration. Superficial siderosis of the central nervous system is rare and insidious, but magnetic resonance imaging has turned a previously late, mainly autoptical diagnosis into an easy, specific, in vivo, and possibly early one. Avoiding long diagnostic delay will be very important in those cases susceptible of causal treatment.- - - - - - - - - - ranking = 25.38836453981keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/9. Delayed central nervous system superficial siderosis following brachial plexus avulsion injury. Report of three cases.Chronic subarachnoid hemorrhage may cause deposition of hemosiderin on the leptomeninges and subpial layers of the neuraxis, leading to superficial siderosis (SS). The symptoms and signs of SS are progressive and fatal. Exploration of potential sites responsible for intrathecal bleeding and subsequent hemosiderin deposition may prevent disease progression. A source of hemorrhage including dural pathological entities, tumors, and vascular lesions has been previously identified in as many as 50% of patients with SS. In this report, the authors present three patients in whom central nervous system SS developed decades after brachial plexus avulsion injury. They believe that the traumatic dural diverticula in these cases may be a potential source of bleeding. A better understanding of the pathophysiology of SS is important to develop more suitable therapies.- - - - - - - - - - ranking = 21.156970449841keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/9. A new syndrome of long-term idiopathic, severe CD4 lymphocytopenia: isolated paraparesis and conjunctival ischemic microangiopathy.An extraordinary case report of an adult patient followed-up for a decade with an extremely severe idiopathic CD4 T-lymphocytopenia (as expressed by an absolute CD4 count of 8-25 cells/microL), associated with an isolated paraparesis and a conjunctival ischemic microangiopathy is described, and discussed on the grounds of the available literature. Despite such a severe and prolonged immunodeficiency, no opportunistic disease occurred, in a observation period longer than ever reported to date. The neurological disorder was diagnosed concurrently with idiopathic CD4 lymphocyte depletion, while the ocular complication occurred two years later, but remained stable thereafter. Both disorders remained stable during the subsequent eight years. Despite extensive and repeated instrumental and laboratory workout, only very limited immunological abnormalities were detected (besides the extremely low CD4 lymphocyte count), and no apparent explaination was found for the disabling paraparesis syndrome. Idiopathic CD4 lymphocytopenia, whose pathogenesis deserves careful investigation, has been associated with a very broad spectrum of signs and symptoms, ranging from negligible or no disturbances, to severe lymphoproliferative disorders, different opportunistic infections, and other focal diseases, including neurological pathologies. However, the association of a long-lasting profound peripheral CD4 lymphocyte depletion in absence of any opportunistic infection or neoplasm, and isolated paraparesis and conjunctival microangiopathy, represents an absolutely unique finding, especially due to the apparently stable course of the above-mentioned syndrome.- - - - - - - - - - ranking = 0.10016393685166keywords = neoplasm (Clic here for more details about this article) |
8/9. Lumbar intramedullary spinal schistosomiasis presenting as progressive paraparesis: case report.OBJECTIVE AND IMPORTANCE: blood fluke infection or schistosomiasis affects up to 300 million people worldwide. Although infection of the hepatic and urogenital systems commonly occurs, central nervous system involvement is rare. When presenting in the spinal cord, schistosomiasis can be difficult to diagnose because it can present as mass lesion or transverse myelitis. We describe a patient with lumbar intramedullary spinal schistosomiasis who presented to a tertiary medical center in the united states and discuss the diagnosis and treatment of this rare disease. CLINICAL PRESENTATION: A 25-year-old Brazilian man presented with progressive, subacute, bilateral lower extremity motor and sensory deficits and disturbances in bladder function. magnetic resonance imaging revealed a diffusely enhancing mass expanding the region of the conus medullaris. INTERVENTION: Laboratory investigation disclosed serum and cerebrospinal fluid eosinophilia. Ultimately, cerebrospinal fluid serology demonstrated the presence of schistosoma mansoni. The patient was treated with praziquantel and steroids and made a marked recovery. CONCLUSION: The clinical and radiographic manifestations of spinal schistosomiasis can mimic those of intra-axial spinal tumors and transverse myelitis. To avoid unnecessary surgery or delay in treatment, the clinician must have knowledge of this type of presentation. The increasing volume of international travel and high prevalence of the disease worldwide increases the possibility that the practicing neurosurgeon in the united states may encounter this rare but treatable disease.- - - - - - - - - - ranking = 4.2313940899683keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/9. Spinal fibrous hamartoma of infancy: case report.OBJECTIVE AND IMPORTANCE: Fibrous hamartoma of infancy (FHI) in the spinal cord is very rare. It is characterized histopathologically by three elements: fibrous, adipose, and myxoid mesenchymal tissues. CLINICAL PRESENTATION: A 10-month-old boy presented with paraparesis. magnetic resonance imaging showed a large intradural mass that occupied the space between the T10 and L4 levels. INTERVENTION: We performed partial removal of the mass. Histopathological examination of the lesion revealed that it was composed of collagen bundles and fibrous tissue interspersed with adipose tissue, mesenchymal tissue, and glial tissue, and it was diagnosed as FHI. CONCLUSION: FHI in the central nervous system, especially in the spine, is very rare, and the presence of glial tissue admixed with particular components of FHI is quite exceptional.- - - - - - - - - - ranking = 4.2313940899683keywords = central nervous system, nervous system (Clic here for more details about this article) |