1/135. Pontine cryptococcoma in a nonimmunocompromised individual: MRI characteristics.The case of a pontine cryptococcoma in a nonimmunocompromised, previously healthy 16-year-old boy is presented. The patient had slowly progressive brainstem signs with right cranial nerves V, VII, and VIII palsies, and contralateral corticospinal and spinothalamic deficits. Magnetic resonance images (MRI) revealed, within the right pons, a 1-cm diameter round mass lesion, hypointense on T1-weighted images, hyperintense on T2-weighted images, and with rim enhancement after infusion of gadopentetate dimeglumine. This is the only report of the MRI findings in an isolated pontine cryptococcoma in an immunocompetent patient. Early recognition of this specific MRI pattern is essential, because complete recovery can be achieved with prompt antifungal treatment.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/135. Congenital facial neuropathy in oculoauriculovertebral dysplasia-hemifacial microsomia (Goldenhar-Gorlin syndrome).Four patients with clinical features of Goldenhar-Gorlin syndrome who showed facial paralysis on clinical examination are presented. The fourth case died following surgery for cleft lip. autopsy revealed hypoplasia of the right facial nerve in its intracranial segment, with small right facial nucleus in the brain stem. Nosological aspects of the Goldenhar-Gorlin syndrome are discussed. Peripheral facial paralysis, as a part of this syndrome, is reviewed in the light of clinical and pathological findings and in its relationship to cardiac anomalies. It is suggested that Goldenhar-Gorlin syndrome is a part of a so-called cardiofacial syndrome.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
3/135. Genetic factors in human sleep disorders with special reference to Norrie disease, prader-willi syndrome and Moebius syndrome.Sleep-wake problems are common in specific inborn errors of metabolism and structure of the central nervous system. Psychological factors, behavioural difficulties, metabolic disturbances, and widespread rather than focal damage to the nervous system are present in many of these diseases and all influence the sleep-wake cycle. However, a number of conditions cause relatively focal damage to the neuroanatomical substrate of sleeping and waking. These include fatal familial insomnia, with involvement of the prion protein gene on chromosome 20, Norrie disease, the prader-willi syndrome and the Moebius syndrome. The last three important conditions, although rare, are considered in detail in this review. They result in sensory deprivation, hypothalamic and mid-brain damage, and involve the X-chromosome, chromosome 15, and chromosome 13, respectively. These conditions cause a wide variety of sleep disturbance, including parasomnias, daytime sleepiness, and a condition like cataplexy. The place of the relevant gene products in normal sleep regulation needs further exploration.- - - - - - - - - - ranking = 3.5613817190232keywords = central nervous system, brain, nervous system (Clic here for more details about this article) |
4/135. Mobius sequence, hypogenitalism, cerebral, and skeletal malformations in two brothers.Two brothers born to a healthy, consanguineous Spanish couple have a syndrome of Mobius sequence with involvement of cranial nerves V, VI, VII, IX, and XII, central nervous system malformations; characteristic face with creased earlobes, short philthrum, and a short, arched upper lip, skeletal anomalies with short sternum and delayed bone maturation, hypogenitalism, and profound mental retardation. We suggest that this is a new multiple congenital anomalies condition and mental retardation (MCA/MR) syndrome with autosomic recessive inheritance.- - - - - - - - - - ranking = 1.8592358064152keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/135. facial nerve grafts: from cerebellopontine angle and beyond.OBJECTIVES: To review the outcomes from facial nerve cable grafting in a variety of pathologic conditions and to determine the possible causes of poor facial function after facial nerve grafting. STUDY DESIGN: A retrospective review of patients undergoing facial nerve grafts through a neurotologic procedure. SETTING: This study was performed at a university-based tertiary referral center. patients: All 27 patients undergoing a neurotologic procedure that required a facial nerve graft from 1982 to 1997 were reviewed. Fourteen patients had grafts from the facial nerve stump at the brainstem. INTERVENTIONS: All patients had facial nerve function determined by the senior neurotologist and through use of historical data and photographs. MAIN OUTCOME MEASURE: A facial nerve repair recovery score was developed for facial nerve transection and repair. The House-Brackmann scale was found to be inappropriate for transected and repaired nerves. RESULTS: Facial function was found in 23/25 (92%) patients with at least 8 months follow-up. Statistical analysis revealed no significant correlation between graft length and recovery score. patients who had the nerve grafted to a site distal to the meatal foramen had a mean outcome that was nearly one grade level better than those with an anastomosis proximal to the meatal foramen (i.e., at the brainstem), but this did not reach statistical significance. CONCLUSIONS: facial nerve grafting is an effective way of restoring facial motor function. A new facial recovery grading scale is proposed for repaired or grafted facial nerves.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
6/135. Acute cerebellar ataxia of a patient with SLE.We described a 28-year-old woman with systemic lupus erythematosus (SLE) presented with digestive tract, skin and renal symptoms and afterwards developed acute cerebellar ataxia, a paresis of the right inferior rectus muscle, left abducens paralysis and left facial palsy which seemed to be consistent with a brainstem lesion visible on magnetic resonance imaging (MRI). This lesion disappeared within 9 days of corticosteroid treatment. It is suggested that this lesion is focal edema induced by acute changes in the blood brain barrier secondary to a vasculopathy. Other causes, including local infarction, are unlikely.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
7/135. Current surgical results of retrosigmoid approach in extralarge vestibular schwannomas.BACKGROUND: Vestibular schwannomas (VS) are common tumors that can be cured; they are reported to comprise 6 approximately 8% of all intracranial tumors. The primary objective in the surgery of extralarge VS is total removal of the tumor mass while preserving the facial nerve. In extralarge tumors, complete excision of the tumor carries a significant risk of injuring the facial nerve and adjacent vital brain structures. The authors are reporting the techniques and results of operation on extralarge VS. methods: The material consisted of 30 patients during the last 6 years with surgically treated VS that had a maximal extrameatal diameter exceeding 4 cm. Suboccipital craniotomy and tumor removal was performed with patients in the lateral position. Results and complications of the surgical technique will be reviewed. RESULTS: Average age of patients was 45.2 years; there was a slight female predominance (1.5:1). Size of the mass ranged from 41 to 70 mm; all were removed by the retrosigmoid transmeatal approach. Peritumoral edema on MRI was seen in 50% (15/30). Total removal was achieved in 73.3% (22/30) with no significant relationship to peritumoral edema. In the cases of total removal, the facial nerve preservation rate was 86.4% (19/22). There was no mortality. Surgical complications were hemorrhage and CSF leakage in 1 case (3%) and 8 cases (26.7%), respectively, but in most of these cases, conservative treatment was adequate. In patients in whom anatomic preservation of the facial nerve was achieved, facial nerve function improved progressively within a year. In all cases except for one with gait disturbance, a good outcome was achieved.CONCLUSIONS: Our surgical techniques, including the prediction of facial nerve displacement, not using retractors, and replacement of bone, contributed to good surgical results in a series of extralarge VS.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
8/135. radiosurgery for bilateral neurinomas associated with neurofibromatosis type 2.OBJECTIVE: The clinical course of bilateral acoustic tumors associated with neurofibromatosis (NF2) is generally troublesome, and no definite treatment strategy has been established. Follow-up results of bilateral acoustic tumors after radiosurgery are reported herein. methods: The current indications for radiosurgery are 1) a growing tumor less than 30 mm in mean diameter, 2) the ipsilateral ear has no serviceable hearing, and 3) there is risk of brain stem compression or brain stem dysfunction. Twenty cases of bilateral acoustic tumors were treated with the gamma knife, including 7 males and 13 females. The mean age was 38.2 years and the mean tumor size 24.4 mm. The tumors were treated with mean maximum and marginal doses of 26.8 Gy and 13.0 Gy, respectively. Among them, 12 patients had profound hearing loss in the ipsilateral (treated) ear, but the other 8 had serviceable hearing.RESULTS: Tumors treated with radiosurgery showed central necrosis in 60% of the cases at 6 months and in 70% at 9 months after radiosurgery. Thereafter, the tumors often demonstrated slow regression. The rate of tumor shrinkage was 20% at 12 months, 35% at 24 months, and almost 60% at 36 months. At the last follow-up (mean 33.6 months), the tumors demonstrated shrinkage in 50% and tumor control in 100%. The contralateral tumors were stable in 12 (60%) and enlarged in 8 (40%). Preservation of serviceable hearing ipsilaterally was obtained in 33.3%. Deterioration of ipsilateral facial nerve function, either in the natural course or as a complication, occurred in 10%. CONCLUSIONS: Because of good tumor control and tumor shrinkage as well as an acceptable complication rate, radiosurgery should be incorporated in the treatment strategy for bilateral acoustic tumors associated with NF2.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
9/135. Facial palsy in cerebral venous thrombosis : transcranial stimulation and pathophysiological considerations.BACKGROUND: Cranial nerve palsy in cerebral sinovenous thrombosis (CVT) is rare, its pathophysiology remains unclear, and data from electrophysiological examinations in such patients are missing. CASE DESCRIPTION: We report the case of a 17-year-old woman with familial protein s deficiency who was admitted with extensive multiple CVT. Two weeks after onset of symptoms, she developed isolated right peripheral facial palsy, and MR venography showed segmental occlusion of the ipsilateral transverse sinus. Complete recovery of facial palsy occurred concomitant with recanalization of the transverse sinus. Facial neurography, including transcranial magnetic stimulation of the facial nerve and related motor cortex, ruled out a coincidental idiopathic palsy and revealed conduction block proximal to the facial canal. CONCLUSIONS: Facial palsy in our patient was caused by transient neurapraxia in the intracranial segment of the nerve. We suggest that elevated venous transmural pressure in the nerve's satellite vein, which belongs to the affected drainage territory of the transverse sinus, might have caused venous blood-brain barrier dysfunction in the intrinsic vascular system of the nerve, with leakage of fluids and ions into the endoneurial space and thus an increase in interstitial resistance.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
10/135. magnetic resonance imaging findings in a patient with bilateral facial paralysis due to malignant lymphoma.In malignant lymphomas, especially non-Hodgkin's lymphomas, invasion to the central nervous system (CNS) often occurs. A patient is reported here with bilateral facial paralysis due to invasion of a malignant lymphoma to the CNS. Contrast magnetic resonance imaging (MRI) revealed swelling of the entire length internal auditory meatus and enhancement in the entire length of the facial nerve. transcranial magnetic stimulation and MRI revealed that later facial nerve injury was present before the onset of paralysis due to a malignant lymphoma. Therefore treatment should be performed with consideration given to the possible invasion of the tumor to the CNS.- - - - - - - - - - ranking = 1.8592358064152keywords = central nervous system, nervous system (Clic here for more details about this article) |
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