1/131. Progressive ponto-bulbar palsy with deafness. A clinico-pathological study.Progressive ponto-bulbar palsy with deafness is a rare disease. It seems to be an abiotrophic process with autosomal recessive inheritance in most instances. Only one autopsy case had been briefly described (Lelong et al., 1941). The clinical features and the pathological findings of a new case are reported. The structures primarily involved are the grey matter of the brain stem and the spinal cord, including to some extent the optic tracts and most of the fiber tracts in the brain stem with exception of the pyramidal tracts.- - - - - - - - - - ranking = 1keywords = palsy (Clic here for more details about this article) |
2/131. Epidermoid cysts of the pineal region.Localization of epidermoid cysts to the pineal region is rare. The 7-year-old boy now reported presented with an 18-month history of progressive ataxia. CT and MRI scans demonstrated a 2.5x2.5 cm cyst at the pineal region with obstructive hydrocephalus. At surgery via an occipital transtentorial approach, a characteristic "pearly tumour" was encountered, and complete resection was achieved. We present the management of this child with pineal region epidermoid cyst and review 11 cases reported in the literature since 1968. In all, 8 of the 12 patients were males. The age at the time of diagnosis ranged from 7 years to 69 years. Parinaud's syndrome and hydrocephalus are the most common presenting findings. All but 1 patient underwent direct surgical resection; 1 had stereotactic decompression. Surgical treatment brought about complete resolution of the presenting symptoms and signs in 10 of the 12 cases. One patient had persistent upgaze palsy. One patient died from progression of the pineal region mass. This patient presented with hemiparesis, which is a marker of clinical aggressiveness. The authors advocate direct surgical attack as opposed to stereotactic diagnosis and aspiration to: (1) obtain maximal resection and thereby limit the potential for recurrence and delayed complications of the cyst; (2) possibly avoid shunt placement in patients who present with hydrocephalus; and (3) decrease the likelihood of sampling error.- - - - - - - - - - ranking = 0.2keywords = palsy (Clic here for more details about this article) |
3/131. Histiocytic lesion mimicking intrinsic brainstem neoplasm. Case report.This 10-year-old girl presented with a 1-month history of progressive bulbar palsy and a solitary enhancing mass originating within the floor of the fourth ventricle. Results of initial imaging studies and presentation were suggestive of neoplasia. Subtotal resection was performed and pathological examination revealed the mass to be a histiocytic lesion, with no evidence of a glioma. The patient had no other stigmata of histiocytosis and was treated with steroid medications, resulting in prolonged resolution of the lesion. This case demonstrates that for discrete brainstem lesions the differential diagnosis includes entities other than glioma for which treatment is available. biopsy sampling should be considered when technically feasible.- - - - - - - - - - ranking = 0.2keywords = palsy (Clic here for more details about this article) |
4/131. Etiology and surgical management of horizontal pontine gaze palsy with ipsilateral esotropia.INTRODUCTION: An esotropia ipsilateral to a horizontal pontine gaze palsy has been infrequently reported. We discuss the etiology and review the surgical management of this ocular motility defect. methods: Four patients with radiographically documented dorsal pontine lesions and persistent horizontal gaze palsy with esotropia ipsilateral to the gaze palsy were treated. In each patient, the esotropia was present in attempted primary gaze, producing symptomatic diplopia. An anomalous face turn was required to attain single binocular vision. RESULTS: All 4 patients underwent surgical correction to alleviate the anomalous head position and diplopia. Bilateral, asymmetric surgery was required to achieve a long-term successful result. Single binocular vision in the primary position with elimination or marked improvement of the compensatory head posture was initially achieved in all 4 patients. One patient, who had not undergone asymmetric strabismus surgery to reconcile incomitance produced by the esotropia, rapidly developed a residual esotropia. CONCLUSIONS: Ophthalmologists should recognize that concurrent esotropia may occur in patients with horizontal pontine gaze palsy. Single binocular vision in the primary position, an expanded binocular visual field, and alleviation of a compensatory head position are achievable with strabismus surgery.- - - - - - - - - - ranking = 1.6keywords = palsy (Clic here for more details about this article) |
5/131. Foix-Chavany-Marie (anterior operculum) syndrome in childhood: a reappraisal of Worster-Drought syndrome.Foix-Chavany-Marie syndrome (FCMS) is a distinct clinical picture of suprabulbar (pseudobulbar) palsy due to bilateral anterior opercular lesions. Symptoms include anarthria/severe dysarthria and loss of voluntary muscular functions of the face and tongue, and problems with mastication and swallowing with preservation of reflex and autonomic functions. FCMS may be congenital or acquired as well as persistent or intermittent. The aetiology is heterogeneous; vascular events in adulthood, nearly exclusively affecting adults who experience multiple subsequent strokes; CNS infections; bilateral dysgenesis of the perisylvian region; and epileptic disorders. Of the six cases reported here, three children had FCMS as the result of meningoencephalitis, two children had FCMS due to a congenital bilateral perisylvian syndrome, and one child had intermittent FCMS due to an atypical benign partial epilepsy with partial status epilepticus. The congenital dysgenetic type of FCMS and its functional epileptogenic variant share clinical and EEG features suggesting a common pathogenesis. Consequently, an increased vulnerability of the perisylvian region to adverse events in utero is discussed. In honour of Worster-Drought, who described the clinical entity in children 40 years ago, the term Worster-Drought syndrome is proposed for this unique disorder in children.- - - - - - - - - - ranking = 0.2keywords = palsy (Clic here for more details about this article) |
6/131. Neuroborreliosis and isolated trochlear palsy.We report here for the first time a child with isolated trochlear palsy and neuroborreliosis. IgG and IgM antibodies against borrelia burgdorferi were highly positive in serum and cerebrospinal fluid respectively. The symptoms resolved completely after initiation of antibiotic treatment with ceftriaxone.- - - - - - - - - - ranking = 1keywords = palsy (Clic here for more details about this article) |
7/131. Painful ophthalmoplegia: report of a case with cerebral involvement and psychiatric complications.The diagnosis of tolosa-hunt syndrome should be suspected in the presence of recurrent "painful ophthalmoplegia." The most useful tests are the rapid (within 48 hours) response to steroids and positive findings on orbital venography. It should be emphasized that Tolosa-Hunt's syndrome may not be a "pure syndrome." Perhaps it is only an occasional presentation of another rather poorly understood syndrome, that of "recurrent cranial neuropathies." The present patient had at least three episodes of painful ophthalmoplegia prior to this hospitalization. During the last hospitalization, he presented with painful ophthalmoplegia, showed a rapid response to steroids, had narrowing of the carotid artery on arteriogram and an abnormal orbital venogram. However, during his hospitalization he developed involvement of cranial nerves II, III, V, VI and VII, papilledema, pyramidal tract signs and severe psychiatric disturbances, all of which remitted. This, coupled with the abnormal pneumoencephalogram and electroencephalogram and organicity on psychological testing, suggests cerebral involvement in our case.- - - - - - - - - - ranking = 140.89200156686keywords = ophthalmoplegia (Clic here for more details about this article) |
8/131. tuberculoma of the cavernous sinus.A case of tuberculoma of the cavernous sinus is described and 4 other previously reported cases are reviewed. The condition is very rare, and predominantly affects Asians. Periorbital pain, ptosis and ophthalmoplegia are the most common presenting features. Clinical and radiological findings are not sufficiently sensitive to differentiate it from other diseases affecting the cavernous sinus. Treatment is primarily medical, with antituberculous drugs, and the outcome is good. Surgery has a limited role in the management of this condition, mainly for establishing the diagnosis.- - - - - - - - - - ranking = 20.127428795266keywords = ophthalmoplegia (Clic here for more details about this article) |
9/131. Dejerine's syndrome or Spiller's syndrome?We report the difference existing between two clinical syndromes: Spiller's syndrome is caused by a complete involvement of the medial hemimedulla, while Dejerine's syndrome is determined by lesions restricted to the anterior portion of the medial hemimedulla and is characterized by hypoglossal nerve palsy and contralateral hemiparesis.- - - - - - - - - - ranking = 0.2keywords = palsy (Clic here for more details about this article) |
10/131. Detection of a pineoblastoma with large central cyst in a young child.THE PATIENT: We present the very rare case of a pineoblastoma with large central cyst in a 7-year-old boy who presented with a short history of gradually worsening headache and upward gaze palsy.IMAGING INVESTIGATIONS: On CT and MRI studies, it was seen as a peripherally calcified, irregularly shaped mass with heterogeneous low signal intensity on T1-weighted images and ringed enhancement after gadolinium administration; there was homogeneous high signal intensity on T1-weighted images.DISCUSSION: We discuss differential diagnosis for several types of cystic tumors in the pineal region, including pineoblastomas.- - - - - - - - - - ranking = 0.2keywords = palsy (Clic here for more details about this article) |
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