Cases reported "Dementia, Vascular"

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1/128. 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. ( info)

2/128. Dural arteriovenous malformation manifesting as dementia due to ischemia in bilateral thalami. A case report.

    BACKGROUND: Although dementia is one of the curable manifestations of a dural arteriovenous malformation (AVM), the pathophysiology remains unclear. CASE DESCRIPTION: We describe an elderly patient who had an AVM in the tentorium and manifested signs of dementia from ischemia, predominantly in the bilateral thalami. Intravascular embolization of the dural AVM resulted in amelioration of signs of dementia, and this improvement was consistent with that of neuroradiological and hemodynamic conditions in the thalami. CONCLUSION: The coincidental improvement of CBF in the thalami and signs of dementia after embolization of the AV shunt supports the concept of primary participation of the thalami in the pathophysiology of dementia of vascular origin in our patient. ( info)

3/128. The cognitive syndrome of vascular dementia: implications for clinical trials.

    dementia is common among patients with cerebrovascular disease, particularly in a setting of one or more clinically evident strokes. Prior cohort and case studies have suggested that the cognitive syndrome of vascular dementia is characterized by predominant executive dysfunction, in contrast to the deficits in memory and language function that are typical of patients with alzheimer disease. The course of cognitive decline may also differ between those dementia subtypes, with many, but not all, patients with vascular dementia exhibiting a stepwise course of decline caused by recurrent stroke and most patients with alzheimer disease exhibiting a gradually progressive course of decline. The findings of prior studies of the cognitive syndrome of vascular dementia must be interpreted with caution, however, because of (1) possible inaccuracies in the determination of the dementia subtype and the loss of precision that might result from pooling heterogeneous subgroups of patients with vascular dementia, (2) difficulties inherent in identifying a pattern of strengths and weaknesses in patients who are required to have memory impairment and other deficits to meet operationalized criteria for dementia, and (3) the use of limited test batteries whose psychometric properties are incompletely understood. Specific questions that should be addressed by future studies are discussed. ( info)

4/128. 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. ( info)

5/128. 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. ( info)

6/128. 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. ( info)

7/128. 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. ( info)

8/128. 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. ( info)

9/128. 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. ( info)

10/128. 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. ( info)
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