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1/46. arachnoid cyst of the middle fossa with paradoxical changes of the bony structures.

    Two patients with an arachnoid cyst of the middle fossa showed paradoxical changes of the adjoining bony structures of the skull. There was a diminution of the middle fossa and hyperplasia of the sphenoid sinus (pneumosinus dilatans) as well as a marked bulging of the squamous part of the temporal bone. In one case in which scinticisternography was performed, communication between the cyst and the subarachnoidal space was proven as well as an extremely slow cerebrospinal fluid circulation in the cyst. The pathogenesis of the cyst is discussed, based upon the structural changes of the skull, the angiographic findings and the locally disturbed cerebrospinal fluid circulation. The primary disturbance seems to be a temporal lobe agenesis.
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2/46. E.N.T. manifestations of Von Recklinghausen's disease.

    Von Recklinghausen's Disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade.
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keywords = bone
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3/46. Fibro-osseous lesions of the central nervous system: report of four cases and literature review.

    Fibro-osseous lesions, also reported as calcifying pseudoneoplasms of the neural axis, are uncommon lesions of the CNS. We report four additional cases: two extraaxial and two intraaxial, in patients ages 33, 47, 49, and 59 years at presentation. Fibro-osseous lesions involving the CNS demonstrate variable proportions of fibrous stroma, bone, palisading spindle to epithelioid to multinucleated cells in association with a highly distinctive, perhaps pathognomonic, chondromyxoid-like matrix often distributed in a nodular pattern. This histopathologically distinctive lesion can be seen in many regions of the neuraxis, often with a dural association, and most commonly along the vertebral column. It appears to be a slow-growing lesion and, with wide excision, the prognosis is excellent. The etiology remains unclear, but the preponderance of data favors a reactive rather than neoplastic process. If this putative pseudotumor is not recognized histopathologically, a neoplastic or infectious differential might result in inappropriate investigations and potentially harmful therapies.
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4/46. cyclosporine A-associated fatal central nervous system angiopathy in a bone marrow transplant recipient: an autopsy case.

    We report here the case of a 32-year-old woman who suffered from a unique angiopathy in the central nervous system (CNS). She died of multiple infarcts in the brain stem and cerebellum during treatment with cyclosporine A after bone marrow transplantation for refractory anemia with excess of blasts. The autopsy findings showed segmental narrowing of the basilar artery, in which circumferential dissection of the internal elastic lamina had occurred. The distal portion of the basilar artery was obstructed by upward dislocation of the dissected intima. Similar angiopathy was also observed at multiple sites along the basilar artery branches. These findings suggest endothelial damage, including vasoconstriction and dissection of the CNS arteries.
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5/46. New imaging findings in a patient with central nervous system dysfunction after bone marrow transplantation.

    central nervous system disorders are an important complication of bone marrow transplantation (BMT). We have recently performed cerebral angiography to examine central nervous system dysfunction in a 22-year-old woman with acute lymphoblastic leukaemia who had undergone BMT. Angiography demonstrated multiple stenoses and occlusions in the peripheral branches of the anterior and middle cerebral arteries, a pattern similar to that seen in vasculitis. She was thought to most likely have cytomegalovirus (CMV) vasculitis, but other forms of vasculitis, such as angiitis-like-syndrome-associated graft-versus-host disease could not be excluded. This case suggests that CMV vasculitis may cause central nervous system dysfunction after BMT and that imaging studies may provide useful information about central nervous system disorders in these patients.
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keywords = bone
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6/46. central nervous system complications in patients with diffuse histiocytic and undifferentiated lymphoma: leukemia revisited.

    Fifteen of 52 patients (29%) with diffuse histiocytic and undifferentiated pleomorphic lymphoma developed central nervous system (CNS) complications, primarily leptomeningeal lymphoma. Lumbar puncture with cerebrospinal fluid cytology was the most useful test for diagnosis, and for following the response to therapy. Leptomeningitis developed during all stages of the patients' clinical course: at time of diagnosis, during progression of systemic disease, and most importantly as the initial site of relapse within 7 mo of attaining a complete clinical remission. patients with bone marrow involvement are at high risk for the development of leptomeningeal lymphoma. Pathologic findings suggest that entry into the leptomeninges involves extension from the medullary bone marrow cavity along perforating vessels through dura into the arachnoid space. The leptomeningeal lymphoma has been successfully controlled in all patients receiving intensive central nervous system therapy consisting of a combination of intrathecal drug administration and radiotherapy. The high frequency of this syndrome and the success in its control suggest that a controlled trial of prophylactic CNS therapy be instituted in patients with these histologic types of non-Hodgkin's lymphomas.
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keywords = bone
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7/46. Langerhans cell histiocytosis: central nervous system involvement treated successfully with 2-chlorodeoxyadenosine.

    A case of disseminated Langerhans cell histiocytosis with resistant central nervous system (CNS) disease in an adolescent is described. The child presented with visible cranial lesions, emesis, headaches, and short-term memory loss. Diagnostic evaluation revealed multiple osseous lesions in the cranium, ribs, vertebral bodies, and pelvis. The clinical course with complications and response to each therapy are sequentially reviewed. Remission, as evidenced clinically and by magnetic resonance imaging, was ultimately accomplished with 2-chlorodeoxyadenosine (2-CDA). The full course of 2-CDA was not tolerated due to bone marrow suppression. CNS histiocytosis is known to be resistant to therapy. Earlier introduction of 2-CDA for CNS disease might offer more successful treatment with less toxicity than seen in patient.
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keywords = bone
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8/46. Central and peripheral nervous system complications following allogeneic bone marrow transplantation.

    Although graft vs. host disease (GvHD) is a frequent complication of allogeneic bone marrow transplantation (BMT), involvement of the central and peripheral nervous systems (CNS and PNS, respectively) has not been demonstrated conclusively. Here, we report of a patient who, following allogeneic BMT for lymphoblastic T-cell lymphoma, suffered a syndrome characterized by self-remitting cerebellar and pyramidal signs associated with a progressive involvement of the peripheral nervous system (PNS). Clinical course and laboratory findings correlated with relapses of systemic GvDH, thus suggesting the possibility that involvement of CNS and PNS may be sustained by a similar pathogenic mechanism.
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ranking = 5
keywords = bone
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9/46. scoliosis in a patient with alexander disease.

    alexander disease is a rare, degenerative disorder of the central nervous system. It is characterized clinically by spasticity, seizures, dementia, loss of developmental milestones, and macrocephaly. Here we describe a 13-year-old boy with alexander disease and severe scoliosis. The patient initially presented at 9 months of age, with profound mental retardation and a history of seizures. When he was 7 years old, a pediatrician had diagnosed alexander disease (hypotonia, macrocephaly, and progressive low-density white matter predominantly in the frontal region on computed tomography examination). From the age of 10, thoracolumbar scoliosis had gradually become severe. Because treatment using a corrective brace would have produced major problems because of the patient's mental retardation, the scoliosis was successfully treated surgically, by careful posterior spinal fusion with instrumentation, and an autologous iliac crest bone graft. A 64 degrees curve was corrected to 18 degrees (72% correction). scoliosis with alexander disease is considered to be very rare because patients with the disease seldom survive long enough to develop spinal deformities.
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keywords = bone
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10/46. Spontaneous activity in premature infants affects bone strength.

    OBJECTIVE: Determination of bone strength of lower extremities in very low birth weight (VLBW) premature infants with central nervous system pathology resulting in reduced unilateral spontaneous leg movements. STUDY DESIGN: Quantitative ultrasound (QUS) measurements of speed of sound (SOS) of the tibiae of both legs in three VLBW premature infants with brain insult and unilateral reduced spontaneous activity. Results were compared to QUS measurements of both legs in healthy premature infants. Measurements were performed by the same investigator who was blinded to the clinical course of the participants. RESULTS: Reduced spontaneous activity of one leg due to brain pathology resulted in decreased tibial SOS in the affected side. There was no difference in bone SOS between the legs of the healthy controls. CONCLUSION: Spontaneous movements (mainly antigravity flexion and extension) are important for bone structure and mineralization in VLBW premature infants. QUS may become an important diagnostic modality for the evaluation, treatment, and follow-up of bone strength and osteopenia in this unique population.
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ranking = 8
keywords = bone
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