1/200. Steroid-responsive multifocal demyelinating neuropathy with central involvement.We describe 2 patients with associated central and peripheral demyelination. Electrophysiological studies revealed a demyelinating polyneuropathy with sensory and motor conduction blocks. Visual evoked potentials were abnormal. Motor evoked potentials showed abnormal central conduction time in 1 patient. magnetic resonance imaging revealed regions of abnormal high signal in the spinal cord and brain; sural nerve biopsy disclosed a demyelinating neuropathy. Both patients showed clinical and electrophysiological improvement after steroid therapy.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/200. 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.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
3/200. 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.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
4/200. 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.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
5/200. Superficial siderosis of the central nervous system and anticoagulant therapy: a case report.Superficial siderosis of the central nervous system is a rare condition characterized by deposition of haemosiderin in the leptomeninges and in the subpial layers of the brain and spinal cord. With the widespread use of magnetic resonance imaging, an increasing number of cases of superficial siderosis are being discovered, secondary forms being more frequent than idiopathic ones. We report a 78-year-old man in oral anticoagulant therapy, who presented neurosensory hearing loss, gait ataxia and spastic paraparesis. magnetic resonance imaging suggested the diagnosis of superficial siderosis of the central nervous system, without an evident bleeding source.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
6/200. 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.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
7/200. 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.- - - - - - - - - - ranking = 5keywords = spinal (Clic here for more details about this article) |
8/200. neurology. 4: multiple sclerosis.multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system (CNS) and a common cause of disability in young adults; it is most likely an autoimmune disease. Typically, MS initially follows a relapsing-remitting course, but most patients eventually develop secondary progressive MS, where there is progressive deterioration without relapses or remissions; in some patients, MS has a primary progressive course. The diagnosis of MS requires evidence of CNS lesions disseminated in time and place, as well as the exclusion of other likely causes of these lesions; the clinical history, neurological examination and investigations, such as magnetic resonance imaging of the brain and spinal cord, all have key roles in the diagnosis. education and counselling of the patient and family members are essential for good patient management. Moderate to severe attacks of MS are best treated with intravenous infusions of high-dose methylprednisolone. Interferon beta reduces the frequency of attacks and the progression of disability in relapsing-remitting MS. Symptomatic therapy is important in the management of spasticity, pain, urinary problems and the other symptoms or complications of MS.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
9/200. Severe lightning pain after subarachnoid block in a patient with neuropathic pain of central origin: which drug is best to treat the pain?OBJECTIVE: There have been many reports that spinal anesthesia induces severe lightning pain in the lower limbs of patients with phantom limb pain, tabes dorsalis, or causalgia. We report on a patient with neuropathic pain of central origin who showed newly developed severe lightning pain after therapeutic subarachnoid block (SAB). We performed SAB 16 times in this patient, and he complained of severe pain each time. We investigated which drug was best for treating such induced pain by administering various drugs to the patient. SETTING: The patient was hospitalized for treatment and observation. PATIENT: The patient was a 48-year-old man with neuropathic pain secondary to an incomplete spinal cord injury at the cervical segment. INTERVENTIONS: Various drugs were administered for relieving the newly developed severe pain, and the effectiveness of these agents was compared. RESULTS AND CONCLUSIONS: Intravenous thiopental, fentanyl, butorphanol, ketamine, midazolam, droperidol, and sevoflurane-oxygen anesthesia were quite effective. Intramuscular butorphanol was not effective. Intravenous physiologic saline and atropine sulfate as a placebo, intrathecal morphine hydrochloride, intravenous mexiletine, and lidocaine were ineffective. Intravenous thiopental (approximately 1 mg/kg) was thought to obtain the best pain relief because it stopped the pain quickly, the dose needed was subanesthetic, and there was no adverse effect.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
10/200. 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.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
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