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1/107. 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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2/107. central nervous system sarcoidosis--diagnosis and management.

    A series of 68 patients with neurosarcoidosis is reported, with particular emphasis on clinical aspects, diagnosis and treatment. A classification system based on clinical diagnostic probability is proposed, consisting of probable and definite disease, the latter being dependent on finding sarcoid granulomas on nervous system histology, which was obtained in 12 patients (18%). The role of investigations, including magnetic resonance imaging (MRI), chest radiography, Kveim skin test, gallium 67 isotope scanning and cerebrospinal fluid (CSF) studies, is considered. Sixty-two percent of patients presented with nervous system disease, most commonly affecting the optic nerve and chiasm. Other common presentations included cranial nerve palsies, spinal cord and brainstem manifestations. Investigations yielding most diagnostic information included the kveim test (41/48, 85% positive), raised CSF protein and/or cells (50/62, 81%) and gallium 67 scan (14/31, 45%). Eleven out of 29 patients (38%) patients showed meningeal enhancement on MRI scanning and 43% of scans demonstrated multiple white-matter lesions. Mean follow-up for the group was 4.6 years. Forty-seven patients were seen for > 18 months, and over half of these patients progressed despite corticosteroid and other immunosuppressive therapies. The benefit of a large patient database prospectively studied, with extended follow-up is discussed in order to learn more about prognosis and advance therapy in neurosarcoidosis.
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3/107. Primary sjogren's syndrome with severe central nervous system disease.

    OBJECTIVE: central nervous system (CNS) involvement in primary sjogren's syndrome (pSS) is controversial with regard to frequency, significance, and etiology. methods: We describe a young woman with pSS and severe CNS disease and review the literature on the pathophysiology, clinical significance, symptoms, diagnostic examinations, and treatment of CNS disease with concomitant pSS (CNS-SS). RESULTS: Our patient with pSS had a 5-month history of benign lymphadenopathy and myositis, after which she developed severe CNS disease, vasculitic lesions on her hands, and a neurogenic bladder attributable to spinal cord involvement. The diagnosis was based on the clinical picture and the results of a brain magnetic resonance imaging (MRI) scan, electroencephalography (EEG), and cerebrospinal fluid (CSF) analysis. The disease did not respond to corticosteroids, but the administration of cyclophosphamide resulted in recovery. In the literature, the incidence of CNS-SS varies widely, from rare to incidence rates of 20% to 25%. The clinical picture is diverse, ranging from mild cognitive symptoms to fatal cerebrovascular accidents. The pathophysiology of CNS-SS is unclear, specific diagnostic methods are not available, and diagnosis is based on the clinical picture and a combination of examinations. MRI is the most sensitive test and cerebral angiography the most specific. CSF reflects involvement of the leptomeninges, and EEG is nonspecific. There are no controlled studies of the treatment of CNS-SS. Regimens for vasculitis are commonly used. CONCLUSIONS: CNS-SS is uncommonly recognized and difficult to diagnose. Increasingly accurate and available diagnostic examinations will yield more information about the association of CNS disease with pSS.
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4/107. Meningeal leukemia in the blastic phase of chronic granulocytic leukemia.

    One hundred one patients were treated for Ph' positive chronic granulocytic leukemia (CGL) in the blastic phase. In seven of these (6.9 per cent), meningeal leukemia developed. Of the 99 patients who died of their disease, a complete remission was achieved in 12 with a median survival of 12 months (three to 28 months). Incomplete responders had a median survival of only 2.5 months (one to 14 months). In five of the 12 complete responders (42 per cent), but in only two of the incomplete responders (2.3 per cent), meningeal leukemia developed. The principal neurologic signs were cranial nerve palsies and papilledema. All patients had pleocytosis with myeloblasts in the cerebrospinal fluid. As in patients with acute leukemia and diffuse histiocytic lymphoma, increased survival of patients in whom hematologic remission from the blastic phase of CGL is achieved may allow sufficient time for the development of meningeal leukemia. Intrathecal methotrexate is extremely successful in treating this complication. cerebrospinal fluid pleocytosis was eradicated in all seven of our patients, and neurologic symptoms and signs were completely eliminated in five patients. No evidence of meningeal leukemia was found in three of the five patients in whom an autopsy was performed.
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5/107. cerebrospinal fluid pleocytosis in acute lymphoblastic leukemia without central nervous system relapse: a report of three cases.

    Three patients with acute lymphoblastic leukemia (ALL) developed mononuclear cells in the cerebrospinal fluid (CSF) after a flu-like history during maintenance treatment. None of the patients showed evidence of central nervous system (CNS) involvement by either clinical or laboratory follow-up. Although the presence of > 5 mononuclear cells/microl in the CSF is important, it may not necessarily indicate CNS disease. Clinical findings, history and cell morphology must be evaluated before deciding on further treatment.
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6/107. 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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7/107. Thoracic spine arachnoid ossification with and without cord cavitation. Report of three cases.

    Thoracic spine arachnoid ossification is a relatively rare disease that affects mainly women and causes sensory, motor, and sphinctal symptoms associated with inferior limb pain. Based on three cases, the authors comment on pathogenic and surgery-related aspects of the disease. The patient in Case 1 was followed over the course of 23 years. Spinal cavitation is highlighted in Case 2, and yellow, gross, half-ring ossification is described in Case 3. calcium deposits usually occur in the middle and lower thoracic spine where the majority of trabeculated arachnoid cells are located. Operative treatment does not interrupt the ossification process, which continues over time, causing progressive deterioration in the patient. Spinal cavitation can occur due to spinal cord tethering, stretching, and central cord edema formation, accompanied by cerebrospinal fluid blockage and pulse pressure changes. The results of surgical intervention are poor, offering short-term recovery with later deterioration. Multiple pathogenic factors are involved in this clinical syndrome including metabolic changes.
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8/107. Cerebrospinal trypanosomiasis masquerading as pulmonary infectious disease in a 1-year-old boy.

    A 1-year-old boy with cerebrospinal trypanosomiasis presented with severe respiratory symptoms, hepatosplenomegaly and no neurological signs of trypanosomiasis. Agitation and high fever on the 2nd day in hospital prompted a lumbar puncture and trypanosomes were recovered from the cerebrospinal fluid.
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9/107. central nervous system superficial siderosis, headache, and epilepsy.

    Almost 95 cases of superficial siderosis of the central nervous system have been reported in the literature. These patients showed a clinical syndrome characterized by ataxia, deafness, pyramidal system involvement, and mental deterioration with xanthochromic cerebrospinal fluid and neuroradiological findings of hemosiderin deposits. About 30% of the patients had headache as an accompanying symptom. In the present case report, we describe a 33-year-old man with the typical clinical features of superficial siderosis, who complained, since aged 8, of a severe recurrent frontal headache often associated with loss of consciousness occurring after at least 2 hours of pain. The MRI and CSF findings were consistent with subarachnoid bleeding. In our patient, headache due to meningeal irritation by subarachnoid blood induced seizures as a probable reflex of extreme pain. carbamazepine and nimodipine prophylaxis dramatically reduced the frequency of headaches and seizures.
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10/107. The application of the polymerase chain reaction of cerebrospinal fluid in the clinical management of AIDS-related CNS disorders.

    In AIDS patients central nervous system (CNS) illness may be caused by hiv disease itself or by opportunistic agents, resulting in serious morbidity such as behavioral and motor disturbances, meningitis or encephalitis, among other disorders. early diagnosis can allow specific treatment (e.g., antimicrobial treatment) that may prevent, ameliorate, or slow the catastrophic sequelae of infection, as well as reduce the need for expensive diagnostic procedures. Conventional microbiology techniques have proven inadequate for the diagnosis of most AIDS-related CNS diseases. However, the development in the past decade of the application of polymerase chain reaction (PCR) to clinical specimens has facilitated the early diagnosis of a number of infectious diseases in these patients. The technique permits the amplification of target nucleic acids such that common laboratory methods may then be used for diagnosis. The application of PCR to cerebrospinal fluid for early diagnosis of AIDS-related neurologic complications has been an impressive example of the application of PCR and may form the basis of new algorithms for diagnosis and possibly the evaluation of treatment protocols.
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