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Birklein et al. pursued the possibility of neurogenic inflammation in CRPS by studying calcitonin gene-related peptide (CGRP) in venous blood from limbs affected with CRPS. They found higher levels affected with CRPS. They found higher levels on the affected side; levels were higher in limbs with nerve lesions and hyperhidrosis and decreased with therapy.
see page 2179
The accompanying editorial by Bennett notes conceptual and methodologic limitations of the Birklein et al. report, including the study of relatively acutely injured controls vs the study of chronic CRPS patients, as well as some of the paradoxes of proposing that calcitonin gene-related peptide as being a predictor of CRPS.
see page 2161
ANT-1 in autosomal dominant progressive external ophthalmoplegia
Napoli et al. identified a new adenine nucleotide translocator-1 gene mutation in a Greek adPEO family. ANT1 gene should be screened in adPEO patients with population background different from Italians.
see page 2295
CPEO and exercise intolerance due to a mitochondrial microdeletion
Raffelsberger et al. demonstrate the association of a single nucleotide deletion in the mitochondrial tRNATyr with CPEO and exercise intolerance, emphasizing the importance of screening the mitochondrial genome for small-scale rearrangements.
see page 2298
Molecular diagnosis in chronic progressive external ophthalmoplegia (CPEO)
In the editorial accompanying the Napoli et al. and Raffelsberger et al. articles, Hirano and DiMauro review the dramatic progress in molecular diagnosis of CPEO, tabulating the more than 20 causes including recent discoveries of ANT1, Twinkle, and POLG. They point out the wide variety of clinical featuressuch as exercise intolerance or psychiatric presentation characteristic in some of the disorders.
see page 2163
Cerebellar ataxia associated with heteroallelic ceruloplasmin gene mutation
Miyajima et al. describe three patients who had cerebellar ataxia with hypoceruloplasminemia. They were heterozygous for a nonsense mutation of the ceruloplasmin gene. Marked loss of Purkinje cells and iron deposition in the cerebellum were found.
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see page 2205
Response to first AED predicts 2-year outcome in childhood TLE
"Failure of first AED trial accurately predicted refractory TLE at two years after onset."
Dlugos et al. report that the results of the first AED trial predict 2-year outcome with 93% accuracy in a cohort of 120 children with TLE. Once the first AED trial was considered, other factors did not add to the accuracy of outcome prediction.
see page 2259
An "atraumatic" needle reduces the risk of post-LP headaches
Strupp et al. demonstrated the benefit of using the "atraumatic" Sprotte needle in diagnostic LP: 24.4% of patients in the "traumatic" needle group developed post-LP headaches vs only 12.2% of the "atraumatic" needle group.
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see page 2310
Localization of Claudes syndrome
The precise localization of Claudes syndrome is undefined. Reviewing MRI findings of six patients, Seo et al. localized the syndrome to the superior cerebellar peduncle.
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see page 2304
Hippocampal volume loss in healthy women
Cohen et al. followed 25 healthy women in their 6th decade of life for 2 years and found a significant association between the presence of a single APOE < 4 allele and accelerated hippocampal volume loss.
see page 2223
Engagement in leisure activities may reduce the risk of dementia
Scarmeas et al. report that in a community sample of 1772 healthy elderly, engaging in more leisure activities (intellectual, social, and physical) was associated with lower risk of developing incident dementia. Leisure activities may provide a cognitive reserve that delays the onset of clinical manifestations of the disease.
see page 2236
Rippling muscle disease: A novel caveolinopathy
Vorgerd et al. report a sporadic patient with rippling muscle disease. They found a de novo missense mutation in the caveolin-3 gene associated with reduced caveolin-3 and
-dystroglycan, but normal nNOS expression in a muscle biopsy.
see page 2273
Fever prophylaxis in acute ischemic stroke
In their placebo-controlled pilot study, Koennecke and Leistner demonstrated that fever can be prevented effectively and safely by prophylactic administration of acetaminophen in patients with acute ischemic stroke.
see page 2301
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