1/45. Corticobasal ganglionic degeneration with Balint's syndrome.Corticobasal ganglionic degeneration (CBGD) is a neurodegenerative dementia characterized by asymmetric parkinsonism, ideomotor apraxia, myoclonus, dystonia, and the alien hand syndrome. This report describes a patient with CBGD who developed Balint's syndrome with simultanagnosia, oculomotor apraxia, and optic ataxia.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/45. Is cochlear implantation possible after acoustic tumor removal?HYPOTHESIS: This study aimed to assess the possibility of hearing restoration after acoustic tumor removal. BACKGROUND: Hearing restoration surgery may be indicated after surgery of bilateral acoustic tumors or surgery of acoustic tumor in the only hearing ear. The choice is between cochlear implantation and brainstem implantation. methods: From the temporal bone collection at the House Ear Institute, los angeles, the author histologically examined eight temporal bones from seven patients who had undergone acoustic tumor removal during their lifetime. Special emphasis was put on examining the patency of the cochlear turns and on survival of the spiral ganglion cells and cochlear nerve. RESULTS: This study showed that after translabyrinthine acoustic tumor removal, there is progressive osteoneogenesis of the cochlea associated with almost complete degeneration of the spiral ganglion cells and cochlear nerve. Similar findings were noticed after middle fossa removal of acoustic tumor with unsuccessful hearing preservation. CONCLUSIONS: The histologic changes described in the cochlea and cochlear nerve represent the effects of ischemia resulting from inadvertent cutting of the blood supply during acoustic tumor removal. It is possible to do cochlear implantation after acoustic tumor removal provided that the result of promontory electrical stimulation test is positive (the cochlear nerve is intact) and that implantation is done at the time of acoustic tumor removal or shortly thereafter, before cochlear ossification is complete.- - - - - - - - - - ranking = 0.4keywords = ganglion (Clic here for more details about this article) |
3/45. temporal bone histopathologic and genetic studies in Mohr-Tranebjaerg syndrome (DFN-1).OBJECTIVE: To describe the temporal bone histopathologic and genetic abnormalities in a case of Mohr-Tranebjaerg syndrome. BACKGROUND: Mohr-Tranebjaezrg syndrome (DFN-1) is an X-linked, recessive, syndromic hearing loss, characterized by postlingual sensorineural hearing loss with onset in childhood, followed in adult life by progressive dystonia, spasticity, dysphagia, and optic atrophy. The syndrome is caused by mutations in the DDP (deafness/dystonia peptide) gene, which are thought to result in mitochondrial dysfunction with subsequent neurodegeneration. The temporal bone pathologic changes in this syndrome have not been reported. methods: hearing loss developed in the patient at age 4, blindness at age 48, and dystonia at age 57. Genetic studies on peripheral blood showed a l51delT mutation in his DDP gene. He died at age 66. The right temporal bone was subjected to light microscopy and polymerase chain reaction-based analysis of the DDP gene sequence. RESULTS: There was near complete loss of spiral ganglion cells with loss of nearly all peripheral and central processes. Only 1,765 spiral ganglion cells remained (8.5% of mean normal for age). The organ of corti (including hair cells), stria vascularis, and spiral ligament were preserved. There was also a severe loss of Scarpa's ganglion cells with preservation of vestibular hair cells. The population of geniculate and trigeminal ganglion cells appeared normal. sequence analysis from temporal bone dna showed the 15ldelT DDP gene mutation. CONCLUSION: Sensorineural hearing loss in Mohr-Tranebjaerg syndrome is the result of a postnatal, progressive, severe auditory neuropathy.- - - - - - - - - - ranking = 0.8keywords = ganglion (Clic here for more details about this article) |
4/45. Lewy body-free nigral degeneration--a case report.A 70-year-old Japanese woman developed progressive, dopa-responsive parkinsonism consisting of akinesia, resting tremor, rigidity, and postural instability. Neuropathological examination revealed a marked loss of nigral neurons, but no lewy bodies (LBs) were observed. lewy bodies were also absent from their usual site, with the exception of a small number seen in the dorsal motor nucleus of the vagus nerve (DVN) and sympathetic ganglion. We propose that our case and several similar reported cases represent Lewy body-free nigral degeneration.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
5/45. Histopathological observations of presbycusis.temporal bone histopathology of 17 aged patients who had spontaneous and gradually progressive bilateral sensorineural hearing losses associated with aging was studied. Six cases in the present material showed the gradually sloping audiometric curve; nine cases, abrupt high tone hearing loss; and two cases, the flat audiometric curve. The most prominent histopathological change in the inner ear was a decrease in the population of the spiral ganglion cells. However, diffuse senile atrophy was also often seen in the organ of corti and the stria vascularis. A positive correlation between the degree of artheriosclerosis and the degree of sensorineural degeneration in the cochlea was not obtained in the present cases. Also, the correlation was not found to be consistent between the type of the audiometric curve and the localization of lesions in the sensory, the neural or the vascular elements in the cochlea. Our observations show that a certain type of audiometric curve does not necessarily indicate a lesion in a specific cochlear element.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
6/45. Cortico-basal ganglionic degeneration. A case report.The case of a Brazilian patient with cortico-basal ganglionic degeneration (CBGD) is presented. Since three years ago, a 71-year old male displays asymmetric ideomotor apraxia, gait apraxia, cortical sensory impairment, myoclonus, limp dystonia and rigidity. His mental status is spared. There is neither consanguinity nor similar cases in his family. The differential diagnosis of CBGD is discussed. A brief review of the literature is made stressing the clinical and pathological features of CBGD. This disease is poorly known and probably underdiagnosed. Its diagnosis can be safely made based on clinical grounds.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
7/45. Neurophysiological diagnosis of acquired sensory ganglionopathies.We examined 29 patients with chronic progressive ganglionopathy of different etiology. Neurophysiological abnormalities were dominated by a widespread decrease in sensory nerve action potential amplitudes, which involved both upper and lower limb nerves, even in patients with asymmetrical or patchy clinical presentation. This impairment of sensory nerve conduction, reflecting a nonlenght-dependent pattern of peripheral axon degeneration, should be considered the hallmark of ganglionopathies. The evidence of central sensory pathway impairment, which allows to localize the pathology to the dorsal root ganglion neurons, could be better confirmed by cervical magnetic resonance imaging, which showed a diffuse hyperintensity in the posterior columns in all patients, than by somatosensory evoked potentials, which were undetectable in most of the patients. Few patients showed an impairment of individual motor nerves, which appeared more severe in paraneoplastic associated ganglionopathies. Neurophysiological abnormalities did not appear significantly changed at the 4-year follow-up. We emphasize that distinct abnormalities allow to differentiate ganglionopathies from axonal sensory neuropathies on routine neurophysiological examination.- - - - - - - - - - ranking = 1.8keywords = ganglion (Clic here for more details about this article) |
8/45. Cortical basal ganglionic degeneration.In this case study, we describe the symptoms, neuropsychological testing, and brain pathology of a retired mason's assistant with cortical basal ganglionic degeneration (CBGD). CBGD is an extremely rare neurodegenerative disease that is categorized under both Parkinsonian syndromes and frontal lobe dementias. It affects men and women nearly equally, and the age of onset is usually in the sixth decade of life. CBGD is characterized by Parkinson's-like motor symptoms and by deficits of movement and cognition, indicating focal brain pathology. Neuronal cell loss is ultimately responsible for the neurological symptoms.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
9/45. Autonomic failure as the initial presentation of parkinson disease and dementia with lewy bodies.The authors report the clinical and postmortem neuropathologic findings of two patients, one with parkinson disease (PD) and one with dementia with lewy bodies (DLB), both of whom initially sought treatment for isolated autonomic failure. These cases suggest that neurodegeneration in PD and DLB may begin outside the CNS in autonomic postganglionic neurons, a finding with potential diagnostic and therapeutic implications.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
10/45. Case report: autonomic postganglionic denervation--sural nerve and saphenous vein biopsy.The authors describe a 31-year-old woman of British Isle ancestry who developed a syndrome resembling familial dysautonomia in her early teenage years. Predominant manifestations included achalasia, severe orthostatic hypotension, and abnormal sweating. The study included resting and stimulated fractional catecholamines, which were almost nonexistent in both situations, and urinary catecholamines, demonstrating an increase in dopamine degradation products. immunohistochemistry of saphenous vein was negative for dopamine beta-hydroxylase (DBH), serotonin (5-HT) and several vasoactive neuropeptides. The only neuropeptide detected at levels thought to be physiologically relevant was calcitonin gene-related peptide (CGRP), a vasodilator. This was in contrast to control veins, all of which had DBH and neuropeptide y immunoreactive fibers but few CGRP fibers. Also in contrast to controls, electron microscopy of the saphenous vein indicated a close to total absence of terminals with norepinephrine containing vesicles. sural nerve biopsy showed, on electron microscopy, a considerable reduction in the number of myelinated fibers, while unmyelinated fibers appeared to be in the normal range. The authors suggest, from the above findings, that the autonomic fibers were undergoing some form of distal axonal degeneration. Their findings differ from most biopsies performed in dysautonomic children, and they believe their patient has a different neurologic entity.- - - - - - - - - - ranking = 0.8keywords = ganglion (Clic here for more details about this article) |
| | Next -> |