1/411. 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 = spinal (Clic here for more details about this article) |
2/411. Cerebral heterotopia of the temporofacial region. Case report.The authors report a case of cerebral heterotopia in the right temporofacial region of a neonate. The lesion presented as a massive cystic swelling containing cerebrospinal fluid. It produced calvarial and facial bone deformities. After the mass was excised, histological examination revealed glial tissue and choroid plexus.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
3/411. Successful use of alternate waste nitrogen agents and hemodialysis in a patient with hyperammonemic coma after heart-lung transplantation.BACKGROUND: Lethal hyperammonemic coma has been reported in 2 adults after lung transplantation. It was associated with a massive elevation of brain glutamine levels, while plasma glutamine levels were normal or only slightly elevated. In liver tissue, glutamine synthetase activity was markedly reduced, and the histologic findings resembled those of reye syndrome. The adequacy of therapy commonly used for inherited disorders of the urea cycle has not been adequately evaluated in patients with this form of secondary hyperammonemia. OBJECTIVE: To determine whether hemodialysis, in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy, would be efficacious in a patient with hyperammonemic coma after solid-organ transplantation. DESIGN: Case report. SETTING: A children's hospital. PATIENT: A 41-year-old woman with congenital heart disease developed a hyperammonemic coma with brain edema 19 days after undergoing a combined heart and lung transplantation. methods: Ammonium was measured in plasma. amino acids were quantitated in plasma and cerebrospinal fluid by column chromatography. The effectiveness of therapy was assessed by measuring plasma ammonium levels and intracranial pressure and performing sequential neurological examinations. RESULTS: The patient had the anomalous combination of increased cerebrospinal fluid and decreased plasma glutamine levels. To our knowledge, she is the first patient with this complication after solid-organ transplantation to survive after combined therapy with sodium phenylacetate, sodium benzoate, arginine hydrochloride, and hemodialysis. Complications of the acute coma included focal motor seizures, which were controlled with carbamazepine, and difficulty with short-term memory. CONCLUSIONS: The aggressive use of hemodialysis in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy may allow survival in patients after solid-organ transplantation. An acute acquired derangement in extra-central nervous system glutamine metabolism may play a role in the production of hyperammonemia in this illness that resembles reye syndrome, and, as in other hyperammonemic disorders, the duration and degree of elevation of brain glutamine levels may be the important determining factors in responsiveness to therapy.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
4/411. Changing characteristics of a colloid cyst of the third ventricle.We describe extremely unusual radiological characteristics in a colloid cyst of the third ventricle, where the cyst became dense and its size decreased following a cerebrospinal fluid diversion procedure. Such a course of events has been reported in colloid cysts only twice in the literature.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
5/411. 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 = spinal (Clic here for more details about this article) |
6/411. Glossopharyngeal neuralgia referred from a pontine lesion.Paroxysmal pain in the form of glossopharyngeal neuralgia is less frequent and less well understood than that of trigeminal neuralgia. Diagnostic confusion can arise especially when both conditions occur in the one patient. We report a patient with a 20-year history of left-sided glossopharyngeal neuralgia with trigger zones in both the trigeminal and glossopharyngeal dermatomal distributions. magnetic resonance imaging revealed a single T2-weighted hyperintense signal in the left pons with no other abnormality. It is postulated that ephaptic transmission between central pain fibers and the trigeminal or glossopharyngeal fibers, which both enter the spinal trigeminal tract, resulted, respectively, in conventional and "referred" glossopharyngeal neuralgia.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
7/411. A distinct difference in clinical expression of two siblings with Aicardi-Goutieres syndrome.Two sibs with an encephalopathy, including intracerebral calcification and a white matter disease, are reported. In the younger sister, the cerebrospinal fluid showed chronic pleocytosis and clinically she strictly fits to the diagnosis of Aicardi-Goutieres syndrome. Both sisters were affected by a spastic tetraplegia, truncal hypotonia and dystonic posturing, but the clinical course and the neuroradiological findings were milder in the older sister and she showed no cerebrospinal fluid pleocytosis. The present cases and recent reports of intrafamilial variability of Aicardi-Goutieres syndrome may raise interesting aspects as to the limits and criteria of this syndrome.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
8/411. movement disorders following nonfunctional neurosurgery.OBJECT: knowledge is scarce about movement disorders that follow neurosurgical operations other than functional stereotactic surgery. The cases of 14 patients who suffered from movement disorders secondary to craniocerebral or spinal surgery are analyzed. None of these patients was initially treated by any of the authors. methods: Twelve patients underwent surgery for cerebral diseases. Nine of these patients harbored tumors and three patients had neurovascular disorders. Two patients underwent spinal surgery for cervicothoracic ependymoma or for multiple cervical disc herniations. Twelve of the 14 patients had immediate postoperative side effects such as hemiparesis, ataxia, and somnolence. In all but two patients, movement disorders became manifest only after a delay. Dystonic movement disorders developed in eight patients, unilateral tremors in three patients, unilateral facial myokymia in one patient, and hemichorea-hemiballism in two patients. The mean delay of onset for tremor was 5 weeks and that for dystonic movement disorders was 5.5 months. movement disorders were transient in three patients; however, they were persistent in 11 patients at a mean follow-up period of 5 years. These movement disorders caused marked persistent disability in four patients. Lesions of the contralateral striatum were identified in patients with dystonic syndromes and lesions of the dentatothalamic outflow in patients with tremors. In three patients who had postoperative basal ganglia lesions after partial removal of astrocytomas, tumor regrowth was later documented. Medical treatment in patients with persistent movement disorders rendered only limited benefit. Two patients improved with botulin injections. In one patient postoperative hemidystonia was alleviated by contralateral thalamotomy. CONCLUSIONS: Dystonic syndromes and tremors are the most common movement disorders that occur after craniocerebral and spinal surgery. Postoperative movement disorders can lead to various degrees of functional disability. The pathoanatomical correlations are similar to those described in other patients with secondary movement disorders.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
9/411. blindness from bad bones.Progressive visual loss is the most common neurologic finding in osteopetrosis. Several mechanisms may explain this phenomenon, including compression of the optic nerves caused by bony overgrowth of the optic canals and retinal degeneration. We report a child with osteopetrosis and progressive visual loss, even though patent optic canals were demonstrated by computed tomography and digital holography. This patient's visual loss was caused by increased intracranial pressure secondary, to obstruction of cerebral venous outflow at the jugular foramen. This case points to the importance of a full evaluation of the skull base foramina in the diagnostic workup of visual loss in patients with osteopetrosis.- - - - - - - - - - ranking = 0.042635217691566keywords = canal (Clic here for more details about this article) |
10/411. Expanding lacunae causing triventricular hydrocephalus. Report of two cases.Two patients are reported in whom the presence of triventricular hydrocephalus and aqueductal obstruction or stenosis due to multiple expanding lacunae in the mesencephalothalamic region possibly corresponds to abnormally dilated perivascular spaces. Placement of a ventriculoperitoneal cerebrospinal fluid (CSF) shunt in one patient and the performance of a third ventricle cisternotomy in the other reversed the hydrocephalic syndrome, but did not modify the complex neuroophthalmological disturbance and rubral tremor presumably related to the compressive effects of the lacunae on adjacent parenchyma. In one patient the number and size of the lacunae were increased 4 years after CSF shunt placement. A review of the literature revealed two cases in which magnetic resonance imaging demonstrated a similar, poorly understood pathological condition.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
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