1/20. Putaminal necrosis presenting with hemidystonia.A 7-year-old female presented with putaminal necrosis associated with hemidystonia. Cranial magnetic resonance imaging revealed bilateral putaminal lesions appearing as hypointense signals on T(1)-weighted images and hyperintense signals on T(2)-weighted images. After a differential diagnosis of basal ganglial degeneration was made, putaminal necrosis was diagnosed. Low doses of levodopa (0.5 mg/kg daily) were administered, but her clinical signs worsened. Positron emission tomography scanning with [(18)F]-6-fluoro-L-dopa revealed asymmetric uptake and right-sided dominant decreases of [(18)F]-6-fluoro-L-dopa uptake of the putamen. On the basis of these findings, standard doses of levodopa (10 mg/kg daily) were administered, and her clinical signs improved. These results suggest that hemidystonia is associated with a disturbance of the dopamine system.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
2/20. Infantile bilateral striatal necrosis following measles.A previously healthy 4-year-old boy presented with typical measles and demonstrated lesions confined to basal ganglia. The clinical symptoms were an abrupt onset, impaired consciousness and mutism, extrapyramidal signs and severe neurovegetative dysfunction. No modification of the cerebrospinal fluid was observed; laboratory tests were all normal with the exception of a positive serologic test for measles. Cranial magnetic resonance imaging showed abnormal signals in the striatum, affecting the putamen and the caudate nuclei bilaterally. Neurologic improvement occurred within 2 months, with regression of lesions on cranial imaging, suggesting that edema played an important role in the initial stage of the disease.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
3/20. August 2000: Two cases with necrosis and hemorrhage in the putamen and white matter.The August COM: Acute methanol poisoning is an uncommon, but well-recognized, cause of central nervous system injury. We present two autopsy cases showing the classic neuropathologic injuries in acute methanol poisoning: putamen and white matter necrosis and hemorrhage. In Case 1, putamen hemorrhages were striking; white matter pathology predominated in Case 2. The precise mechanism of methanol toxicity is unclear. Direct toxicity of metabolites, particularly formic acid, as well as ischemic injury and acidosis likely play a role. methanol is readily available in many commercial products, and may be ingested accidentally or intentionally.- - - - - - - - - - ranking = 6keywords = putamen (Clic here for more details about this article) |
4/20. brain involvement in haemolytic-uraemic syndrome: MRI features of coagulative necrosis.We describe radiological demonstration of brain involvement in haemolytic-uraemic syndrome (HUS) in two siblings with a very different clinical course. While the brother presented with a mild, reversible encephalopathy, his sister developed high-signal lesions in the cortex, putamen and caudate nucleus on T1-weighted images, seen as dense areas on CT. biopsy revealed coagulative necrosis due to microthrombosis without haemorrhage, calcification or infection. These findings suggest a possible prognostic role for MRI in cases of encephalopathy due to HUS.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
5/20. dysarthria, progressive parkinsonian features and symmetric necrosis of putamen in a family with painful lipomas (Dercum disease variant).We describe painful subcutaneous lipomatosis in four members of a two-generation family. Lipomas appeared in adulthood, were circumscribed, painful on touch and mainly localized to the trunk and proximal parts of the extremities. The disorder was associated with dysarthria, visual pursuit defect and progressive dystonia. MRI showed bilateral increasing cystic lesions in the basal parts of the putamen. No other abnormalities were detected. The lesions corresponded well with the clinical presentation in the patients. Investigation for mitochondrial disease with muscle biopsy and mitochondrial dna gave normal results. No consistent biochemical changes were found. The disorder in this family was considered to differ from MERRF with lipomatous lesions and multiple symmetric lipomatosis but compatible with a Dercum disease variant.- - - - - - - - - - ranking = 5keywords = putamen (Clic here for more details about this article) |
6/20. 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 = putamen (Clic here for more details about this article) |
7/20. Infantile acute encephalopathy with combined symmetrical hypodensities in the thalami and the putamen on computed tomography.An eight-month-old boy with clinical features of acute encephalopathy with symmetrical low-density areas in the thalami and the putamen on computed tomography is presented. These particular computed tomography features suggest potential aetiology common to acute encephalopathy with low-density areas in the thalami and infantile bilateral striatal necrosis with an acute onset. The therapeutic consideration of these conditions is also discussed.- - - - - - - - - - ranking = 5keywords = putamen (Clic here for more details about this article) |
8/20. MRI findings of hypoxic cortical laminar necrosis in a child with hemolytic anemia crisis.We present magnetic resonance imaging findings of a 5-year-old girl who had a rapidly installing hemolytic anemia crisis induced by trimethoprim-sulfomethoxazole, resulting in cerebral anoxia leading to permanent damage. magnetic resonance imaging revealed cortical laminar necrosis in arterial border zones in both cerebral hemispheres, ischemic changes in subcortical white matter of left cerebral hemisphere, and in the left putamen. Although cortical laminar necrosis is a classic entity in adulthood related to conditions of energy depletions, there are few reports available in children. A wide review of the literature is also presented.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
9/20. 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 = putamen (Clic here for more details about this article) |
10/20. Bilateral striatal necrosis, dystonia and optic atrophy in two siblings.Two siblings developed a neurological disorder in the first decade characterised by generalised dystonia, hypokinesia, and subacute visual loss. CT and serial MRI examinations showed bilateral lesions of the striatum, mainly in the putamen. The classification of these patients is discussed in relation to infantile bilateral striatal necrosis (IBSN), Leigh's disease, and Leber's optic neuropathy. The literature shows a clinical and aetiopathogenetic overlap between these syndromes. In our cases parental consanguinity and the involvement of a single generation suggest a new clinical condition with autosomal recessive transmission.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
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