1/4. infection-related atlantoaxial subluxation in two adults: Grisel syndrome or not?Grisel's syndrome involves the subluxation of the atlanto-axial joint from inflammatory ligamantous laxity following an infectious process in the head or neck. It is a rare disease usually affecting children, but infrequent adult cases do occur. Today, due to the widened use of antibiotics and availability of MR imaging, some cases presenting with neck pain, resulting from the infectious inflammation of C1 and C2 but without pronounced subluxation, can be a challenging problem for most neurosurgeons. Several theories have been proposed to explain the pathogenesis of inflammatory subluxation. The primary treatment of Grisel's syndrome is medical. The underlying infectious organism must be isolated and appropriate antibiotics must be prescribed. The subluxation is reduced in holter or skeletal traction. This paper reports two cases of infection-related atlanto-axial subluxation in two adults. The literature on this subject is briefly reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/4. Disseminated infection with prototheca zopfii after unrelated stem cell transplantation for leukemia.Disseminated infection with prototheca zopfii is a rare disease in immunosuppressed patients. We here report the first case of lethal infection with P. zopfii following unrelated stem cell transplantation for leukemia. Breakthrough protothecosis occurred during long-term administration of voriconazole in the case of pulmonary aspergillosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/4. Grisel's syndrome. Cervical spine clinical, pathologic, and neurologic manifestations.Grisel's syndrome involves the subluxation of the atlantoaxial joint from inflammatory ligamentous laxity following an infectious process. Even though it was first described in 1830, it is a rare disease usually affecting children, but infrequent adult cases do occur. patients generally seek treatment for progressive unrelenting throat and neck pain followed by torticollis and subluxation. Neurologic complications occur in approximately 15% of cases and can range from radiculopathy to myelopathy and even death. Principles of management include bacteriologic cure and correction of bony deformity and neurologic protection. The authors present two adult patients with Grisel's syndrome. The first illustrates the permanent spinal deformity that can occur if the disease remains unrecognized for a prolonged period of time. The second case demonstrates a delayed neurologic complication in an adult who had Grisel's syndrome in childhood. It is hoped that these two examples, together with a detailed discussion of the literature, will inform physicians of an unusual but important condition to be considered in the differential diagnosis of any patient complaining of neck pain.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/4. Acute necrotizing encephalopathy of childhood (infantile bilateral thalamic necrosis): two non-Japanese cases.This report describes two Spanish cases of acute necrotizing encephalopathy of childhood, a rare disease first described in japan by Mizuguchi and colleagues. Similar cases have been reported from taiwan and other countries of the far east. Two cases have been reported from the UK and one from the USA. The disease affects young children and is characterized by acute encephalopathy after a viral illness, with seizures and decreased levels of consciousness. The hallmark of the disease is diffuse and symmetrical CNS lesions of both thalami, internal capsules, upper brainstem tegmentum, and cerebral white matter. The aetiology is unknown but an infectious or parainfectious process seems likely. Because of the predominant involvement of the thalami, we propose the term 'infantile bilateral thalamic necrosis', a more specific term and one which distinguishes the entity from other basal ganglia diseases in childhood.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |