1/11. Familial infantile bilateral striatal necrosis: clinical features and response to biotin treatment.BACKGROUND: Infantile bilateral striatal necrosis (IBSN) encompasses several syndromes of bilateral symmetric, spongy degeneration of the caudate nucleus, putamen, and globus pallidus. The familial form of IBSN is rare, and inheritance is either autosomal recessive or maternal. METHOD: The authors describe an Israeli Bedouin kindred in which 15 children born to consanguineous parents were affected with familial IBSN. They evaluated the clinical and radiologic evolution of the disease in 11 patients and the cerebral pathologic findings in one patient. Three of the children were treated with oral biotin 100 mg/day. RESULTS: Inheritance was apparently autosomal recessive. The untreated children had a similar clinical picture including developmental arrest beginning at the age of 7 to 15 months, choreoathetosis, and dysphagia. Pendular nystagmus appeared at a late stage. MRI, performed at various stages of the disease, showed severe basal ganglia atrophy. Postmortem study in one patient showed severe atrophy of the lenticular nuclei with gliosis and loss of neurons. biotin, 100 mg/day, administered to the proband over a period of 15 months, may have slowed progression. In two other children treatment was initiated earlier and appeared to arrest or improve disease. CONCLUSIONS: Familial infantile bilateral striatal necrosis was inherited as an autosomal recessive trait. Clinical features included developmental arrest, dysphagia, and choreoathetosis. Imaging and pathology showed atrophy and degeneration of the basal ganglia. Oral biotin may have benefited three children.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
2/11. Glutaric aciduria type I: value of diffusion-weighted magnetic resonance imaging for diagnosing acute striatal necrosis.Glutaric aciduria type I is a rare disorder of organic acid metabolism caused by deficiency of glutaryl-coa dehydrogenase. We report the cranial computed tomography (CT) and magnetic resonance (MR) imaging findings in a 5-month-old girl with this disorder who presented with an acute dystonic syndrome. CT findings demonstrated only subtle loss of attenuation in the basal ganglia, MR spectroscopy was normal, and conventional MR images showed increased T2-signal limited to the putamina. diffusion-weighted MR imaging demonstrated more extensive disease than was apparent either on CT or on the conventional MR images, including bilateral involvement of the putamina, globus pallidus, and caudate nuclei, consistent with acute necrosis of the corpus striatum and lentiform nuclei.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
3/11. Bilateral striatal necrosis in hemolytic-uremic syndrome.A 41-year old resident of a nursing home presented with bloody diarrhea, and subsequently developed hemolytic-uremic syndrome. E. coli serotype O157:H7 was isolated from the stool culture. At autopsy she was found to have bilateral symmetrical striatal necrosis involving mainly the putamen and lateral globus pallidus. The main microscopic findings consisted of coagulative necrosis, endothelial damage and microthrombosis. Scattered microscopic lesions of similar appearances were noted in the parietal cortex, external capsule and fornix. This case is of particular interest because of the rarity of bilateral striatal necrosis in hemolytic-uremic syndrome and the recent experimental data which implicate E. coli endotoxin in the pathogenesis of cerebral lesions in this syndrome.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
4/11. Symmetrical necrosis of the basal ganglia in methylmalonic acidaemia.In a patient with methylmalonic acidaemia (MMAA), persistent neurological symptoms were observed in addition to the acute episodes of metabolic dysequilibrium. CT scan and magnetic resonance imaging revealed bilateral symmetrical necrosis of the globus pallidus. Different episodes of metabolic decompensation, one with severe acidosis, had occurred. Persistent neurological symptoms in patients with MMAA who are appropriately treated suggest irreversible brain damage which appears to occur preferentially at the level of the basal ganglia.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
5/11. Infantile bilateral striatal necrosis. Clinicopathological classification.Two cases of infantile bilateral striatal necrosis (IBSN) were studied retrospectively, and the literature was reviewed. The two children had presented with progressive neurologic signs of involuntary movements or muscle hypertonia from infancy. Initial computed tomography scans showed mild atrophy of the caudate nuclei or basal ganglia, and the neuropathologic examination revealed diffuse neuronal loss with some patchy preservation and marked astrogliosis in the striatum and globus pallidus. The 27 reported cases of IBSN were divided into three groups with characteristic clinical and pathologic features: early, acute onset (four cases); early, gradual onset (16 cases); and late onset (seven cases). Although metabolic changes in the developing corpus striatum may be important in the pathogenesis of IBSN, the origin is uncertain.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
6/11. Major depression and carbon monoxide-induced parkinsonism: diagnosis, computerized axial tomography, and response to L-dopa.A case of major depressive disorder complicated by carbon monoxide (CO)-induced Parkinson's syndrome is reported. Computerized axial tomography (CAT) revealed bilateral globus pallidus necrosis. Clinical, CAT, and neuropathological findings in other cases of CO encephalopathy with and without parkinsonism are reviewed. The utility of CAT in the diagnostic workup and in following clinical course is discussed, as are the difficulties of making a diagnosis of an antecedent primary psychiatric disorder in the presence of neurological and psychiatric sequelae of CO intoxication. There was no clinical response to a tricyclic antidepressant, but both the mood and movement disorders responded fully to L-dopa. The implications of these findings with regard to the central neurochemical pathophysiology in this patient and in major depressive disorder in general are discussed.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
7/11. Acute basal ganglia infarction in propionic acidemia.An 8-year-old girl with propionic acidemia had acute and rapidly fatal symmetric necrosis of the caudate, globus pallidus, and putamen. Clinical presentation was with acute aphasia, generalized hypotonia, and muscle weakness. There was no evidence of metabolic decompensation, and analysis of the organic acids of the urine indicated good metabolic control. Organic acids in the cerebrospinal fluid were unremarkable. These observations indicate that the pathophysiology of "metabolic stroke" is more complicated than previously thought.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
8/11. Infantile bilateral striatal necrosis: chronic and acute manifestations in a single case.We report a girl who exhibited a slowly progressive extrapyramidal disorder with onset in early infancy. CT examinations demonstrated progressive atrophy of the cerebrum. At the age of 6 years, acute exacerbation occurred with impairment of consciousness and autonomic functions. During the acute episode, CT revealed lesions in the bilateral striatum and globus pallidus.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
9/11. Hemorrhagic necrosis and vascular injury in carbon monoxide poisoning: MR demonstration.MR imaging of a patient 3-years status post-carbon monoxide (CO) poisoning revealed areas of abnormal signal bilaterally in the region of the globus pallidus that had shorter T1 characteristics and longer T2 characteristics than cerebrospinal fluid, probably representing methemoglobin, and that is surrounded by a rim of decreased signal on T2-weighted images, felt to represent hemosiderin. This case demonstrates characteristic findings on MR imaging of CO poisoning, as well as observations that suggest prior focal hemorrhage. Typical findings, neuropathology, and the role of vascular injury and prognosis are discussed.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
10/11. Symmetrical necrosis of globus pallidus with severe gait disturbance in a patient with myelodysplastic syndrome given allogeneic marrow transplantation.A 21-year-old Caucasian man received an allogeneic marrow transplant (BMT) from his HLA-identical brother because of myelodysplastic syndrome. He remained red blood cell (RBC) transfusion dependent with persistent antibodies against the donor's RBC. Six months following BMT the patient suddenly developed a severe akinetic syndrome with gait disturbance and frequent falls and bilateral symmetrical lesions in basal ganglia. Concomitantly, micrococcus species septicemia from an infected Hickman catheter developed. Despite antimicrobial therapy and withdrawal of cyclosporin A, neurologic abnormalities persisted and were unresponsive to various therapies. Ischemic damage due to a vascular event during severe infection could be the most probable reason for the lesions seen in our patient, although infectious or toxic complications cannot be ruled out.- - - - - - - - - - ranking = 4keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
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