Recurrent posterior fossa haemangioblastomas
J. Julow1, K. Bálint2, P. Gortvai3, E. Pásztor2, F. Slowik2
MAY 20, 1994
Clinical Neuroscience - 1994;47(05-06)
J. Julow1, K. Bálint2, P. Gortvai3, E. Pásztor2, F. Slowik2
MAY 20, 1994
Clinical Neuroscience - 1994;47(05-06)
We presented reviews of various aspects of posterior fossa haemangioblastomas operated on at the Hungarian National Institute for Neurosurgery and at St. John's Hospital. (2, 3, 4) The present work examines some problems concerning recurrence of these tumours.
Clinical Neuroscience
In opposite of the benign biological behaviour of the posterior fossa epidermoids the operation of these tumours a great challenge for the surgeon both theoretical and surgical point of view. We analysed our 14 operated cases clinico pathologically in this retrospective study.
Clinical Neuroscience
This paper is a shortened version of an invited lecture given at the Karolinska Institute, Stockholm, on 17th September, 1993, illustrated with 120 slides.
Clinical Neuroscience
Brain stem gliomas are rare, predominantly pediatric tumours. Histologically, they are comparable to adult supratentorial astrocytomas. Most of the pediatric brain stem tumours were classified as low-grade astrocytoma (WHO II), anaplastic astrocytoma (WHO III) or glioblastoma multiforme (GBM, WHO IV). Survival of patients with malignant brain stem gliomas as WHO grade III and IV rarely exceeds more than two years. Recently developed molecular genetic techniques gave new insights in tumour biology. Oncogenes and tumour suppressor genes are genetic alterations which can cause tumorous transformation and furthermore malignant progression. Molecular genetic studies of malignant brain stem gliomas have rarely been investigated. Therefore, we set out to study 12 such tumours clinically and 2 by molecular biological methods.
Clinical Neuroscience
Long lasting intracranial hypertension is considered to be a major pathogenic factor of syringomyelia in patients with a Chiari malformation or posterior fossa tumor.
Clinical Neuroscience
Cerebral autoregulation was investigated in the cerebral and cerebellar cortex, and subcortical gray matter (caudate nucleus) of the rat by means of Laser-Doppler flowmetry. As the vascular architecture of the basal ganglia, the cerebral cortex and the cerebellar cortex have substantial geometrical, onto genetical and pathological differences (3), we tested the working hypothesis that autoregulation of the blood supply to these areas may also be different. Laser-Doppler flowmetry has an ideal time resolution, and it enables analysis of flow-pressure curves (1, 2). The dependency of autoregulation on the rate of change in systemic blood pressure (SABP) in all three regions were confirmed. Control of CBF was significantly different in the subcortical gray matter and the neocortex. Interestingly, no autoregulatory capacity of the cerebellar vasculature was found.
Clinical Neuroscience
Background: This overview provides a summary of the applications of transcranial Doppler (TCD) in ischemic stroke. Results: A fast-track neurovascular ultrasound protocol has been developed for detecting occlusion or stenosis. The technique is more reliable in the carotid area than in the posterior circulation. By monitoring the pulsatility index the increased intracranial pressure can be diagnosed. TIBI score was developed for grading residual flow. TCD has been shown to accurately predict complete or any recanalization. Regarding recanalization, TCD has a sensitivity of 92%, a specificity of 88%, a positive predictive value of 96%, a negative predictive value of 78% and an overall accuracy of 91%, respectively. Sonothrombolysis seemed to be a promising application but randomized controlled trials have shown that it does not improve clinical outcome. TCD examination can detect microembolic signals (MES) which are associated with an increased risk of stroke. Microemboli were detected in symptomatic and asymptomatic carotid artery stenosis and during carotid endarterectomy. The number of microemboli can be decreased by antithrombotic therapy. Contrast enchanced examination and Valsalva maneuver with continuous TCD monitoring can accurately screen for right-to-left shunt.
Clinical Neuroscience
[Multiple sclerosis (MS) is typically a disease of young adults. Childhood MS can be defined in patients under 18 years of age, although some authors set the limit under the age of 16 formerly known as “early-onset multiple sclerosis” or “juvenile multiple sclerosis”, seen in 3-5% of all MS patients. Nowadays, owing to ever-evolving, better diagnostic tools and well-traced, strictly defined diagnostic criteria, childhood MS is showing an increasing incidence worldwide (0.05-2.85/100 000). MS is characterized by recurrent episodes of the central nervous system with demyelination separated in space and time. In childhood almost exclusively the relapsing-remitting (RR) type of MS occurs. Based on experience in adults, the goal in the pediatric population is also the early diagnosis, to initiate adequate DMT as soon as possible and to achieve symptom relief and good quality of life. Based on efficacy and safety studies in the adult population, interferon β-1a and glatiramer acetate were first approved by the FDA and EMA for the treatment of childhood MS also. The increased relapse rate and rapid progression of childhood MS and unfavorable therapeutic response to nearly 45% of the first DMT necessitated the testing of more effective and second-line drugs in the population under 18 years of age (PARADIGMS, CONNECT). Although natalizumab was reported to be effective and well-tolerated in highly active RRMS in childhood, evidence based studies were not yet available when our patients’ treatment started. In this article, we report on the successful treatment of three active RRMS patients with individually authorized off-label use of natalizumab.]
Clinical Neuroscience
Interferon-beta (IFN-β) 1a and glatiramer acetate (GA) are first-line therapies for multiple sclerosis (MS) with immunomodulatory effects. We present a patient who developed lymphopenia and tuberculous lymphadenitis under treatment with these agents. The female patient who at present 65 year old is followed at our MS outpatient clinics had received GA (20 mg/day, subcutaneous injection) and later IFN-β 1a (44 µg, thrice weekly, subcutaneous injection). During the course of her treatment, she developed mild to severe lymphopenia. A follow up thoracic spinal MRI (when lymphocyte count was 800/µl) showed multiple enlarged lymph nodes in the posterior mediastinum incidentally. Further investigation revealed tuberculous lymphadenitis. She received anti-tuberculosis (TB) treatment for nine months and her condition resolved. Although immunomodulatory treatments are considered safe with regard to opportunistic infections, and lymphopenia under these treatments are generally accepted as mild and asymptomatic, our experience was different with this patient. Further studies on the management of patients with lymphopenia and assessment of the risk of TB under immunomodulatory agents are needed.
Clinical Neuroscience
[A case of a 61-year-old male patient suffered chronic renal failure and dialysed for 23 years with destructive cervical spondylarthropathy is presented. The patient presented with sudden onset of cervical pain radiating into his shoulders without neurological deficits. CT and MRI of the cervical and thoracic spine revealed severe destructive changes and compressive fractures of C6 and C7 vertebrae which caused the narrowing of the nerve root canals at these levels. A 360-degree fixation was performed to treat the unstable fracture and the patient’s pain (C6 and C7 corpectomy, autolog bone graft replacement of the two vertebral bodies, anterior plate fixation and posterior instrumentation with screws and rods). Postoperatively the patient had no significant pain, no neurological deficit and he was able to manage independent life himself. During the immediate follow-up CT of the neck showed the satisfactory position of the bone graft and the metal implantations. The 6 months follow-up CT revealed the anterior migration of the two screws from the Th1 vertebral body and 2 mm ventral elevation of the caudal end of the plate from the anterior surface of the Th1 vertebral body. The 1-year follow-up could not be performed because the patient died due to cardio-pulmonary insufficiency. This is the second Hungarian report of a chronic dialysis related severe spondylarthropathy which may cause pathologic fractures of the vertebral bodies. The typical radiological and histological findings are discussed. This disease affect patients’ quality of life and the conservative treatment alone seems to be ineffective in most cases. Based on the literature and personal experiences, the authors suggest 360-degree fixation of the spine to provide sufficient stability for the vertebrae of ”bad bone quality”, and early mobilisation of the patient can be achieved.]
Clinical Neuroscience
Pisa syndrome is a movement problem defined by tonic, sustained lateral flexion with a slight posterior rotation of the trunk. It seems to be a side effect of antipsychotic medicine in most cases. The clinical duration of Pisa syndrome can be acute, chronic, or recurrent. As far as we know, no reports are available in the literature on the chronic form of Pisa syndrome caused by low-dose amisulpride. A case of refractory tardive dystonia form of Pisa syndrome during treatment with stable low-dose amisulpride is presented in this report. Long-term, low-dosage amisulpride therapy may induce tardive dystonia even in patients with no other risk factors for dystonia.
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