1/11. Radiofrequency lesions of the dorsal root ganglion in the treatment of hip flexor spasm: a report of two cases.The current popular model of spasticity is that the abnormalities are primarily due to increased sensitivity of the reflex are at the segmental level of the spinal cord. neurosurgical procedures, such as open selective dorsal rhizotomy for the reduction of spasticity, have been based on this assumption. We describe two patients with hip flexor spasm of different origin treated with radiofrequency lesions of the dorsal root ganglion.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/11. Autosomal recessive spastic ataxia of Charlevoix-Saguenay.Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) was originally found among the inhabitants of the Charlevoix-Saguenay region of quebec, canada. This disease is characterized by early-onset ataxia, spasticity, peripheral neuropathy, finger and foot deformities, and hypermyelination of the retinal nerve fibers. The mentality of the patients is usually intact. The principal neuropathology comprises atrophy of the upper vermis and the loss of purkinje cells in the cerebellum. Although the lateral corticospinal tracts are degenerated, the precentral gyrus, dentate nucleus, and inferior olivary nucleus are intact. Recently, the gene responsible for ARSACS was determined to encode the sacsin protein in the quebec patients. In 2004, we first reported a Japanese family with a SACS mutation. So far, we have identified the SACS mutations in a total of five Japanese families with ARSACS and analyzed the clinical features of eight patients. Interestingly, we found some atypical clinical features in the Japanese patients: a slightly later onset than that of the quebec patients, an absence of myelinated retinal fibers, intellectual impairment, and a lack of spasticity. To date, there have been descriptions of non-quebec patients with SACS mutations in japan, italy, tunisia, and turkey. Hereafter, as more SACS mutations are identified, the clinical spectrum of the "sacsinopathies" could expand.- - - - - - - - - - ranking = 0.059320316638605keywords = nucleus (Clic here for more details about this article) |
3/11. Hereditary spastic dystonia: a new mitochondrial encephalopathy? Putaminal necrosis as a diagnostic sign.A large kindred, in which either Leber's hereditary optic atrophy, or a hereditary spastic dystonia, or a combination of both manifested over many generations was restudied after the first report on it in 1964. NMR scans revealed bilateral, and, in two patients with hemidystonia, unilateral necrosis with shrinkage of the putamen, in one case associated with total disappearance of the head of the caudate nucleus. Except for age-appropriate cortical atrophy in one instance, no other changes were observed in the brain, brainstem, and cerebellum. The putaminal necrosis appears as typical "striatal slits" on the NMR scans. It is argued that this rare disease, since the princeps description in 1964 only reported in england (1986) and the U.S.A (1986), is most likely a singular type of mitochondrial encephalopathy: it is associated with Leber's optic atrophy, and the NMR changes observed have been signalled in other mitochondrial encephalomyelopathies, such as Leigh's disease and MELAS.- - - - - - - - - - ranking = 0.029660158319303keywords = nucleus (Clic here for more details about this article) |
4/11. Hereditary sensory neuropathy with spastic paraplegia.Five cases of spastic paraplegia with a progressive symmetrical sensory neuropathy producing ulceration and osteomyelitis of the hands and feet are reported. The pathology in one patient, who died of secondary amyloidosis, was similar to that found by Denny-Brown in hereditary sensory radicular neuropathy with severe loss of posterior root ganglion cells and loss of myelinated fibres in both peripheral nerves and posterior columns of the spinal cord. A sural nerve biopsy in another case showed a striking loss of both myelinated and unmyelinated fibres, with some evidence of degeneration and regeneration. The inheritance is probably by an autosomal recessive gene. The prognosis in the more severe form of the disorder is poor.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
5/11. pathology in brainstem regions of individuals with primary dystonia.Examination of brains from four individuals with the clinical diagnosis of primary dystonia revealed histopathologic abnormalities in two cases. A 29-year-old man with a 15-year history of dystonia musculorum deformans (DMD) had numerous neurofibrillary tangles (NFT) and mild neuronal loss within the locus ceruleus; occasional NFT were also recognized in the substantia nigra pars compacta, pedunculopontine nucleus, and dorsal raphe nucleus. A 68-year-old man with a 35-year history of meige syndrome had moderate-to-severe neuronal loss in several brainstem nuclei, including the substantia nigra pars compacta, locus ceruleus, raphe nuclei, and pedunculopontine nucleus. Infrequent NFT were also noted in substantia nigra. An examination of these and other brain regions in a 10-year-old boy with a 6-year history of DMD and a 50-year-old woman with a 3-year history of spasmodic torticollis did not disclose similar abnormalities.- - - - - - - - - - ranking = 0.088980474957908keywords = nucleus (Clic here for more details about this article) |
6/11. Complete hemidiaphragmatic paralysis in a patient with multiple sclerosis.We present a case history of a patient with definite multiple sclerosis who developed an abrupt onset of unilateral diaphragmatic paralysis, minor increase in lower extremity spasticity and complaint of marked neck stiffness. Her vital capacity during this episode was 600 mL and she was in impending respiratory failure. The diaphragmatic paralysis was demonstrated by radiographic plain films and fluoroscopy. phrenic nerve stimulation was performed during fluoroscopy and the evoked motor response from the diaphragm recorded. There was a normal amplitude diaphragmatic twitch observed with an evoked motor response latency of 1 ms and amplitude of 300 microV. After high dose intravenous steroids, her neck stiffness and spasticity improved, her vital capacity improved to 1500 mL and her diaphragm regained its normal position and movement confirmed by followup radiographic plain films and fluoroscopy. We postulate the presence of a demyelinating plaque in the brainstem fibers descending to the phrenic nucleus as the etiology of the diaphragmatic paralysis. We are unaware of any other case reports of unilateral "upper motor neuron" phrenic nerve paralysis secondary to multiple sclerosis.- - - - - - - - - - ranking = 0.029660158319303keywords = nucleus (Clic here for more details about this article) |
7/11. Prenatal symmetrical thalamic degeneration with flexion spasticity at birth.The unusual occurrence of spasticity at birth with symmetrical thalamic damage was found in a male infant delivered at 36 weeks' gestation following an episode of traumatically induced premature labor at 32 weeks. The infant was found to be spastic in flexion with increased stretch reflexes, depressed primitive reflexes, and moderate flexion contractures. Computerized tomographic scans showed bilateral nonenhancing thalamic densities. Neuropathologically, the lateral thalamic nuclei and the red nucleus showed neuron loss, astrocytosis, and, as confirmed by electron microprobe analysis, calcified neurons. The striatum was uninvolved. These findings closely resemble those reported as "symmetrical thalamic degeneration in infancy" and are strongly reminiscent of the pattern of thalamic involvement frequently seen in status marmoratus. It would appear that there is a period during perinatal life in which the lateral thalamus can be rendered vulnerable to hypoxic-ischemic injury, and that the thirty-second week of gestation must be included within this period.- - - - - - - - - - ranking = 0.029660158319303keywords = nucleus (Clic here for more details about this article) |
8/11. Autosomal recessive spino-olivo-cerebellar degeneration without ataxia.Five adult siblings from a sibship of ten suffering from an external ophthalmoplegia with a spastic paraplegia are reported. In addition, optic nerve atrophy was present in three of the patients and dementia in two; extrapyramidal signs and cerebellar ataxia were found only in one patient. Contrary to earlier studies of patients with comparable neurological signs the pattern of inheritance was autosomal recessive. Neuropathological investigation of the index case, who had never shown ataxia, nevertheless showed demyelination of the spinocerebellar and the olivocerebellar pathways, and also a severe loss of purkinje cells, of cells in Clarke's column and in the inferior olives. The dentate nucleus was severely gliotic but showed no cell loss. Earlier neuropathological investigations of this disorder, but with an autosomal dominant heredity, were incomplete. It is concluded that the five siblings of this family have a unique autosomal recessive disorder, which should be considered a distinct entity.- - - - - - - - - - ranking = 0.029660158319303keywords = nucleus (Clic here for more details about this article) |
9/11. Chronic self-stimulation of the dentate nucleus for the relief of spasticity.It has been assumed but not yet proved that cerebellar cortical stimulation activates the purkinje cells, with subsequent inhibition of the deep cerebellar nuclei. However, the relatively crude, widespread excitation induced by several surface electrode arrays and the parameters of stimulation currently used, may produce other effects than selective activation of only one specific cellular type which, furthermore, seems to be rarely present in these particular patients, as demonstrated by biopsy studies prior to electrode placement. The dentate nucleus was chronically implanted with a stimulating system in a patient with spasticity due to cerebral palsy. Chronic self-stimulation induced a significant improvement in motor function, with relief of spasticity and improvement in speech, posture, balance and gait. Electrophysiological studies demonstrated a decrease in the amplitude of V1 and V2 responses and in the H/M and T/M ratios, an increase in the silent period, and marked effects in the H reflex recovery curve, as well as diminished contralateral cortical somato-sensory evoked potentials. This result seems to indicate that the clinical effects of cerebellar cortical stimulation are not due to prosthetically induced inhibition of the dentate nucleus.- - - - - - - - - - ranking = 0.17796094991582keywords = nucleus (Clic here for more details about this article) |
10/11. Primary lateral sclerosis: a case report.The syndrome of primary lateral sclerosis (PLS) has been reported clinically on many occasions. Pathologic confirmation in the modern era, however, has generally been lacking. In a recently reported case of PLS, the disorder was complicated by a pontine infarct. We describe a 65-year-old woman whose illness began with spastic dysarthria, which gradually worsened to the point that 18 months later she could barely utter a sound. Meanwhile, dysphagia, brisk reflexes, and a pseudobulbar affect had developed. Three years after onset she had a spastic contractured right-sided hemiplegia and walked with short shuffling steps. The spasticity slowly progressed, and she died of aspiration pneumonia 3.5 years after the onset of dysarthria. Neuropathologic examination showed bilateral atrophy of the precentral gyri, which microscopically showed a paucity of Betz cells. There was loss of myelin throughout the corticospinal system, yet the anterior-horn cells of the spinal cord and hypoglossal nuclei were well preserved. Intracytoplasmic eosinophilic inclusion bodies, of unknown cause and significance, were observed in occasional motor neurons, one in the hypoglossal nucleus and two in spinal cord anterior horns. Clinically and pathologically, this case meets the criteria for PLS.- - - - - - - - - - ranking = 0.029660158319303keywords = nucleus (Clic here for more details about this article) |
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