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Neurology 2001;57:2155-2156
© 2001 American Academy of Neurology

December 26 Highlights

Complex regional pain syndrome (CRPS): Neuropeptides and information

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 features—such 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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Atrophy of the cerebellum

 
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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Atraumatic LP needle— small dural defect

 
see page 2310

Localization of Claude’s syndrome

The precise localization of Claude’s syndrome is undefined. Reviewing MRI findings of six patients, Seo et al. localized the syndrome to the superior cerebellar peduncle.



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Claude’s syndrome

 
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 {alpha}-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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