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Goizet et al. describe a patient with progressive bulbospinal amyotrophy as part of the disease called Triple A (adrenal insufficiency, achalasia, and alacrima) syndrome, also know as Allgrove’s syndrome. The disorder is caused by mutations in the gene for ALADIN, which is proposed to play a role in regulating the function of peroxisomes.
see page 962
The accompanying editorial by Richard Orrell and Adrian Clark (who was a member of one of the teams that discovered the gene for Triple A syndrome) reviews this autosomal recessive disorder, together with the recently reported genetic mutations in another recessive condition, which includes ALS and primary lateral sclerosis presentations.
see page 847
Treatment of ciguatera fish-poisoning
Ciguatera poisoning is the most frequent nonbacterial food poisoning worldwide. The Schnorf et al. randomized, controlled study found that mannitol, the standard treatment of ciguatera poisoning, is no more effective than normal saline in improving neurologic symptoms and signs at 24 hours.
see page 873
Is the prevalence of migraine changing?
A large epidemiologic study by Lipton et al. showed that migraine prevalence has not changed in the past decade. However, patients have sought medical consultation at more than twice the previous rate. Migraine patients were also taking more prescription medications.
see page 885
Familial aggregation of psychosis in Alzheimer disease (AD)
Sweet et al. found that the risk for an individual developing psychotic symptoms during the course of AD was increased when a sibling with AD had also manifested psychotic symptoms. The demonstration of a familial risk for psychosis in AD suggests an underlying genetic basis.
see page 907
Sleep apnea following acute stroke
Iranzo et al. performed polysomnography the first night after acute cerebral infarction in 50 patients. Sleep apnea was found in 62% of the subjects and was associated with stroke onset during sleep and with early neurologic worsening, but had no effect on prognosis in terms of infarct size or functional outcome at 6 months.
see page 911
Sleep deprivation for laboratory diagnosis of sleepwalking
Joncas et al. examined the value of sleep deprivation as a diagnostic tool for adult sleepwalking. Sleep deprivation resulted in a significant increase in the frequency and complexity of somnambulistic events recorded in the sleep laboratory.
see page 936
The mystery of the Doctor’s son
The syndrome of infantile spasms (West syndrome) was first reported by Dr. West in his own son. This Historical Neurology by Eling et al. considers West’s 1841 Lancet report and the history of West and his son.
see page 953
Flying foxes, cycad neurotoxins and ALS-PDC in Guam
In a Medical Hypothesis, Cox and Sacks propose that villagers who ate flying foxes unwittingly ingested large amounts of cycad neurotoxins, leading to the observed 100-fold increase in incidence of the ALS-Parkinson dementia complex in Guam. There, flying foxes actively forage on cycad seeds. If the flying foxes sequester cycad neurotoxins or their metabolites, the prominence of flying fox flesh in the Chamarro diet may account for ALS-PDC being the leading cause of adult death in some villages.
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see page 956
Screening for the Huntington’s disease (HD)–like 2 mutation
The majority of patients with a HD phenotype have CAG expansion of the huntingtin gene on chromosome 4. Stevanin et al. report that the CAG/CTG repeat expansion in the gene encoding junctophilin-3 accounts for 
0.2% of the patients with HD and appears restricted to patients of African ancestry. Further genetic heterogeneity of HD is expected.
see page 965
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