1/78. Spatz-Lindenberg disease: a rare cause of vascular dementia.BACKGROUND: Isolated cerebral thromboangiitis obliterans (Spatz-Lindenberg disease) is not well recognized as a cause of vascular dementia. CASE DESCRIPTION: A 58-year-old woman presented with dementia and pyramidal signs. neuroimaging showed multiple areas of white matter change. brain biopsy showed intimal thickening of the walls of leptomeningeal and intraparenchymal arteries, almost to complete occlusion, with an intact internal elastic lamina and media and without inflammation or infiltration. The cortex showed only moderate gliosis. CONCLUSIONS: Spatz-Lindenberg disease should be considered in the differential diagnosis of vascular dementia. Additional studies of its pathogenesis are required to determine appropriate treatment.- - - - - - - - - - ranking = 1keywords = white matter, matter, white (Clic here for more details about this article) |
2/78. FK506-induced intractable leukoencephalopathy following allogeneic bone marrow transplantation.FK506-related leukoencephalopathy has been reported to be reversible and readily treated by discontinuation or reduction of FK506. We describe two pediatric cases of FK506-related leukoencephalopathy following allogeneic bone marrow transplantation, which could not be readily controlled. These cases show that FK506-related leukoencephalopathy is not always reversible, and patients may develop epilepsy. bone marrow transplantation (2000) 25, 331-334.- - - - - - - - - - ranking = 690.62852359354keywords = leukoencephalopathy (Clic here for more details about this article) |
3/78. Deletion (9) (p13.1 p21.1).We report on a 22-month-old girl with minor facial anomalies, global developmental delay, growth retardation, seizures, and leukoencephalopathy. Initial clinical assessment suggested the diagnosis of williams syndrome. Results of fluorescence in situ hybridization testing for elastin were normal. However, chromosome analysis showed a 46,XX,del(9)(p13.1p21.1) karyotype in peripheral lymphocytes. Parental chromosomes were normal, indicating a de novo deletion. This patient's manifestations are compared with those of two other cases with overlapping deletions of the proximal short arm of chromosome 9.- - - - - - - - - - ranking = 98.66121765622keywords = leukoencephalopathy (Clic here for more details about this article) |
4/78. Transient ischemic attack in heavy cannabis smokers--how 'safe' is it?Drugs are lately considered high-risk factors for cerebrovascular disease. Three male patients (mean age 24.6 years) who were heavy cannabis smokers presented with transient ischemic attacks (TIA) shortly after cannabis abuse. The complete examination of all 3 consisted of: EEG, brain CT scan, brain MRI, cerebral vessel angiography (digital subtraction and magnetic resonance angiography); also a full cerebrospinal fluid, urine and blood analysis (immunological, biochemical and hormonal tests were included). urine was further examined for drug metabolites. An extensive cardiological investigation was carried out. Small vessel leukoencephalopathy was revealed by the brain CT and MRI. EEG recordings of the first patient showed paroxysmal sharp waves with left hemispheric dominance. The other 2 patients had diffuse delta and theta activity in their EEG tracings. The urine analysis was positive for cannabis metabolites. There were no other abnormal findings in the rest of the meticulous and thorough study of all 3 patients, which leads to the conclusion that cannabis was the only risk factor responsible for the observed TIA, contradictory to other studies, which support that cannabis is a 'safe' drug. More research is required in order for this issue to be completely elucidated.- - - - - - - - - - ranking = 98.66121765622keywords = leukoencephalopathy (Clic here for more details about this article) |
5/78. Exceptionally long absence status: multifactorial etiology, drug interactions and complications.To our knowledge, petit mal status lasting longer than 2-3 days has been documented only once [1]. We report a 66 year-old man with well-documented, idiopathic generalized epilepsy who developed petit mal status lasting 6 weeks. Valproate levels remained low because of interactions with phenytoin. When phenytoin was discontinued, valproate levels increased, and he progressively improved. Chronic ischemic changes in the white matter may have been an additional factor in the causation and, most likely, in the duration of his status. Exceptionally long status and stupor increase the risk of medical complications. Valproate remains the medication of choice for the treatment of petit mal status. Despite the extraordinary course in this patient, complete recovery took place, confirming the benign nature of even such a prolonged episode.- - - - - - - - - - ranking = 1keywords = white matter, matter, white (Clic here for more details about this article) |
6/78. Evidence for hypomethylation in two children with acute lymphoblastic leukemia and leukoencephalopathy.BACKGROUND: The prognosis of patients with acute lymphoblastic leukemia (ALL) in childhood has improved with intensive chemotherapy. In particular, central nervous system (CNS) leukemia has been well controlled by the presymptomatic administration of intrathecal methotrexate (MTX), high dose systemic MTX, and irradiation. However, the prolonged intrathecal administration and/or the administration of high doses of systemic MTX, especially when combined with irradiation, can lead to leukoencephalopathy (LE), a serious CNS complication of such prophylaxis. Because the mechanisms by which MTX causes this complication have not been elucidated, the authors investigated the transmethylation status of the cerebrospinal fluid (CSF) in two children with ALL and LE to investigate the pathophysiology of that disorder. methods: The levels of s-adenosylmethionine (SAM) and s-adenosylhomocysteine (SAH) were measured in the CSF of 2 children with ALL and LE, 7 children with ALL only who were undergoing presymptomatic administration of MTX, and 18 reference children in whom diagnostic lumbar puncture was indicated for other reasons. A sensitive, high performance liquid chromatography (HPLC) method was used with fluorescence detection. RESULTS: The concentrations of SAM in the CSF were lower in the patients with ALL during treatment with MTX compared with the reference children. The SAM levels in the 2 patients with both ALL and LE were slightly lower than the levels in the 7 patients with ALL only. The SAH concentrations in the CSF were higher in the patients with ALL and LE compared with the patients with ALL only and the reference children. The mean concentration of SAH in the CSF was similar in the reference children to that found in the 7 patients with ALL only. The SAM-to-SAH ratios were lower in the 2 patients with ALL and LE and in the 7 patients with ALL only compared with the reference children. The ratios in the patients with ALL and LE were still lower than in those with ALL only, thus providing supporting evidence of hypomethylation in the 2 patients with ALL and LE. CONCLUSIONS: The data suggest that the treatment of children with ALL using MTX causes subclinical hypomethylation and that progressive hypomethylation in the CNS, as evidenced in the 2 patients with ALL and LE, may be responsible for the demyelination in the LE induced by MTX.- - - - - - - - - - ranking = 493.31032659747keywords = leukoencephalopathy, central nervous system, nervous system, childhood (Clic here for more details about this article) |
7/78. Visual electrophysiological responses in subjects with cerebral autosomal arteriopathy with subcortical infarcts and leukoencephalopathy (cadasil).OBJECTIVES: To evaluate visual electrophysiological responses in subjects with cerebral autosomal arteriopathy with subcortical infarcts and leukoencephalopathy (cadasil). methods: Three subjects (one male and two females, mean age 55.3 /-2.9 years) belonging to an Italian family already diagnosed with cadasil through clinicopathological and genetic studies and 14 control subjects (6 males and 8 females, mean age 52.7 /-3.6 years) were enrolled in the study. Flash electroretinogram (ERG), oscillatory potentials (OPs) and simultaneous recordings of pattern electroretinogram (PERG) and visual evoked potentials (VEPs) were assessed in all 3 subjects with cadasil and age-matched controls. RESULTS: Subjects with cadasil showed: reduced ERG, OP and PERG (N35-P50, P50-N95) amplitudes with respect to our normal limits; delayed PERG (N35, P50) and VEP (P100) implicit times when compared with our normal limits; and VEP (N75-P100) amplitudes and retinocortical times within our normal limits. CONCLUSIONS: Subjects with cadasil present a dysfunction in the outer, middle and innermost retinal layers when the index of neural conduction in the postretinal visual pathways is normal. The delay in visual cortical responses observed in subjects with cadasil may be ascribable to retinal impairment with a possible functional sparing of the postretinal visual structures.- - - - - - - - - - ranking = 493.3060882811keywords = leukoencephalopathy (Clic here for more details about this article) |
8/78. siblings with cystic leukoencephalopathy and megalencephaly.Cystic leukoencephalopathy with megalencephaly is a newly described entity with mild clinical involvement. patients suffer from developmental problems and seizures in childhood. Progression is gradual into adulthood. Typical magnetic resonance imaging findings include subcortical cysts and diffuse leukoencephalopathy. The etiology is unknown with possibly autosomal-recessive inheritance. We present two pairs of siblings with this disease and emphasize the characteristic and variable patterns even within the same family.- - - - - - - - - - ranking = 591.96758504324keywords = leukoencephalopathy, childhood (Clic here for more details about this article) |
9/78. depression in late life, cognitive decline and white matter pathology in two clinico-pathologically investigated cases.case reports: We report two cases of late life depression who became progressively more resistant to treatment, developed cognitive impairment, and began to exhibit neurological abnormalities and evidence of vascular disease. A discussion of the clinical features of the cases is accompanied by reports of neuropathology and neuroimaging findings. Extensive white matter lesions were present on computed tomography in both patients, and basal ganglia infarcts were seen in one. Neuropathology revealed evidence of cerebral atrophy, demyelination and white matter lesions in addition to cerebrovascular and generalised vascular disease. Neither patient exhibited Alzheimer pathology outwith the norm for their age. We believe this to be the first report of neuropathological findings in depression with white matter changes. literature review: The pathological basis of white matter lesions and their relationship to depression, its age of onset and clinical features is addressed in relation to the cases described. Pathological investigation of white matter lesions has not previously been carried out in depression and hypotheses regarding their nature in this illness are based on extrapolation from research in a variety of other disorders. The association of depression with vascular risk factors is considered, as is the relationship between depression and cognitive deficits. There is a need for further investigation in this area.- - - - - - - - - - ranking = 9keywords = white matter, matter, white (Clic here for more details about this article) |
10/78. Gabapentin for treatment of behavioral and psychological symptoms of dementia.OBJECTIVE: To report the use of gabapentin in the treatment of behavioral and psychological symptoms of dementia (BPSD) and to review the available literature relating to the use of gabapentin in this population. CASE SUMMARY: A 62-year-old white man was admitted to the hospital due to a worsening state of confusion, anxiety, depressed mood, insomnia, and verbal and physical aggressiveness toward his wife. He had a past medical history significant for vascular dementia. He had been intolerant of or had failed to respond to numerous antidepressants, benzodiazepines, and neuroleptics. The addition of gabapentin to the patient's medication regimen resulted in reduced agitation, sexual inappropriateness, and lability. He was discharged to his home on a dose of gabapentin 300 mg three times daily. DATA SOURCES: A medline search (1966-August 2000) was performed to identify case reports and clinical trials discussing the efficacy of gabapentin in the treatment of BPSD. DISCUSSION: Gabapentin, like other anticonvulsants, has been used with success in several psychiatric illnesses. Available literature indicates that the drug may have some efficacy in the treatment of BPSD. It has a favorable adverse effect profile in the elderly, which makes it an attractive altemative to standard therapies, including benzodiazepines and neuroleptics. Optimal dosing remains unclear. CONCLUSIONS: This case suggests that gabapentin is a reasonable alternative therapy for patients whose behavioral symptoms do not respond to conventional agents.- - - - - - - - - - ranking = 0.010851212895006keywords = white (Clic here for more details about this article) |
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