1/229. SSPE following neonatal measles infection.The authors report a case of subacute sclerosing panencephalitis in a child who had measles during the neonatal period. At 3 years, 6 months of age, over a period of a few weeks, the patient lost the ability to sit unaided as a result of progressive truncal ataxia, without apparent cognitive changes, simulating acute cerebellar ataxia. His symptoms improved in 1 month, and he was able to walk again with support, but mental alteration and periodic mild head nodding on awakening followed. His illness was diagnosed as subacute sclerosing panencephalitis on the basis of the elevated titers of measles antibodies in the cerebrospinal fluid. measles infection before 1 year of age is a risk factor of subacute sclerosing panencephalitis, but reports about patients with neonatal measles infection are rare. Immaturity of the brain at the time of measles infection may not only be a risk factor but may also influence the clinical course of the disease.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/229. Clinical, pathologic, and neurochemical studies of an unusual case of neuronal storage disease with lamellar cytoplasmic inclusions: a new genetic disorder?A child of first-cousin Puerto Rican parents had global developmental delay, failure to thrive, and hypotonia since early infancy. At 1 1/2 years of age, she developed clinical and electrophysiologic evidence of progressive motor and sensory neuropathy. At 2 1/2 years, she developed visual impairment and optic atrophy followed by gradual involvement of the 7th, 9th, 10th, and 12th cranial nerves. Uncontrollable myoclonic seizures began at 4 years and she died at 6 years of age. Motor nerve conduction velocities were initially normal and later became markedly slowed. Sensory distal latency responses were absent. Lysosomal enzyme activities in leukocytes and fibroblasts were normal. sural nerve and two muscle biopsies showed only nondiagnostic abnormalities. Electron microscopy of lymphocytes, skin, and fibroblasts showed cytoplasmic inclusions. light microscopy of frontal cortex biopsy showed neuronal storage material staining positively with Luxol fast blue, and electron microscopy showed cytoplasmic membranous bodies in neurons, suggesting an accumulation of a ganglioside. At autopsy, all organs were small but otherwise normal and without abnormal storage cells in the liver, spleen, or bone marrow. Anterior spinal nerve roots showed loss of large myelinated axons. The brain was small and atrophic; cortical neurons showed widespread accumulation of storage material, most marked in the pyramidal cell layer of the hippocampus. Subcortical white matter was gliotic with loss of axons and myelin sheaths. In cortical gray matter there was a 35% elevation of total gangliosides, with a 16-fold increase in GM3, a three- to four-fold increase in GM2 gangliosides, and a 15-fold elevation of lactosyl ceramide. GM3 sialidase activity was normal in gray matter at 3.1 nmols/mg protein per hour and lactosyl ceraminidase I and II activities were 70% to 80% of normal. In white matter, total myelin was reduced by 50% but its composition was normal. Phospholipid distribution and sphingomyelin content were normal in gray matter, white matter, and in the liver. These biochemical findings were interpreted as nonspecific abnormalities. The nature of the neuronal storage substance remains to be determined.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
3/229. An ACTH-producing pituitary carcinoma developing Cushing's disease.An autopsy case of an ACTH-producing pituitary carcinoma in a 59-year-old man who developed Cushing's disease is reported. The surgically removed pituitary tumor was diagnosed as chromophobe adenoma, however, pulmonary metastases appeared 2 years after the operation. autopsy revealed a residual pituitary tumor in the sella turcica with systemic metastases to the lungs, liver, pulmonary lymph nodes, hypothalamus, dura mater, and the subarachnoid space of the midbrain and spinal cord. immunohistochemistry revealed ACTH positivity in the tumor cells. Further immunohistochemical study showed positive high expression of Ki-67 in the tumor removed at surgery as well as in the autopsied tumor. Ki-67 labeling index provided valuable information about the invasive and proliferative potential compared to noninvasive benign pituitary adenoma.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
4/229. 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) |
5/229. Chronic progressive hematomyelia: case reports and review of the literature.BACKGROUND: Hematomyelia usually has an acute onset and rapid progression, which results in a poor prognosis. However, there have been a few cases in which the clinical symptoms have progressed chronically, with a good prognosis. These two different clinical courses should be analyzed separately. The differential diagnosis of spinal tumor and other chronic progressive diseases due to the similarity of the clinical courses is also important. CASE DESCRIPTION: Two cases of hematomyelia with slowly progressive symptoms are reported. Unlike the acute onset and rapid progression or recurrent episodic deterioration usually seen in hematomyelia, the symptoms of chronic hematomyelia progressed over months and resulted in a better clinical prognosis than the acute course. The cases of "chronic progressive hematomyelia" from the literature are briefly summarized. CONCLUSIONS: Chronic progressive hematomyelia should be considered as a different clinical entity from the acute version because of its slowly progressive clinical course and good outcome. magnetic resonance imaging is the procedure of choice to exclude spinal tumors or other slowly progressive intraspinal diseases.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
6/229. Laminar and arch fractures with dural tear and nerve root entrapment in patients operated upon for thoracic and lumbar spine injuries.OBJECTIVE: To determine the neurological outcome in patients with laminar fractures associated with dural tears and nerve root entrapment, operated upon for thoracic and lumbar spine injuries. PATIENT population: Out of 103 patients operated upon consecutively for thoracic and lumbar spine injuries during the period 1990 to 1994 inclusive, 24 (23.3%) patients had laminar fractures out of whom 3 (2.9%) had an associated dural tear and an other 17 (16.5% or 70.8% of the total patients with laminar fractures) had an associated dural tear and nerve root entrapment. RESULTS: Twelve (70.5%) patients had injury at the thoraculumbar junction, 13 (76.5%) had Magerl's type A3 or above, 10 (58.8%) had a kyphotic angle deformity greater than 5 degrees. Seven (41.1%) had their spinal canal's sagittal diameter reduced by at least 50% and two had dislocations. Nine (52.9%) had initial neurological deficits. Four (50%) out of 8 patients with no initial neurological deficits (Frankel E) worsened to Frankel D. However, one patient among the 3 with initial Frankel A improved to Frankel C while both patients with initial Frankel C usefully improved to final Frankel grades D and E respectively. Two of the four patients with initial Frankel D improved to Frankel E, the other 2 remaining unchanged. All in all five patients neurological status improved, 4 worsened and 8 remained unchanged after neurosurgical treatment. CONCLUSIONS: Vertical laminar fractures with dural tears and nerve root entrapment represent a special group of thoracic and lumbar spine injuries that carry a poor prognosis. However, special operative precautions lead to significant improvement in some of them although a majority remain unchanged or even worsened.- - - - - - - - - - ranking = 1.8021400601756keywords = spinal, spinal canal, canal (Clic here for more details about this article) |
7/229. Spinal deformity after selective dorsal rhizotomy in patients with cerebral palsy.Selective dorsal rhizotomy is used widely as a means of treating spasticity associated with cerebral palsy. Little is known regarding the effect of the procedure on the development or progression of spinal deformity. The authors reviewed six patients with progressive deformity after rhizotomy. Prerhizotomy and postrhizotomy records of physical examinations and radiographs were reviewed retrospectively in an attempt to identify risk factors for development of and/or rapid progression of, spinal deformity. Detailed preoperative and postoperative evaluation of spinal alignment should be undertaken, particularly in those patients who may be at risk of rapidly progressive deformity.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
8/229. 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 = 0.092618371836432keywords = canal (Clic here for more details about this article) |
9/229. Erythropoietic protoporphyria with fatal liver failure.A 33-year-old woman with a history of photosensitivity, persistent abdominal pain, and liver dysfunction was admitted to our department because of abdominal pain and progression of liver dysfunction. On admission, levels of protoporphyrin and coproporphyrin within erythrocytes were markedly increased. Autofluorescent erythrocytes were also detected, leading to a diagnosis of erythropoietic protoporphyria. A liver biopsy specimen revealed cirrhosis with dark brown granules filling hepatocytes, bile canaliculi, and bile ductules. Transfusion of washed erythrocytes, hemodialysis, and administration of cholestyramine and beta-carotene transiently improved levels of porphyrins and liver function. The patient died of rupture of esophageal varices followed by multiple organ failure. However, the treatments were believed to have extended survival.- - - - - - - - - - ranking = 0.046309185918216keywords = canal (Clic here for more details about this article) |
10/229. Subretinal lesions in subacute sclerosing panencephalitis.subacute sclerosing panencephalitis (SSPE) is a rare progressive neurologic disorder. A 9-year-old boy was seen who had progressive neurocognitive decline, myoclonic jerking of the extremities, and an abnormal result of an electroencephalogram (EEG). Ophthalmoscopic examination revealed multifocal subretinal lesions. The diagnosis of SSPE was made on the basis of the clinical examination and elevated serum and spinal fluid measles titer. We describe subretinal lesions in a patient with SSPE.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
| | Next -> |