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December Highlights

Dementia

Frontotemporal lobar degeneration is the third most common cause of cortical dementia following AD and Lewy body disease. Neary et al. (p. 1546) present the outcome of a consensus conference to establish diagnostic criteria for frontotemporal lobar dementia. Mueller et al. (p. 1555) report on longitudinal changes in MR brain volumes in healthy elderly subjects. Their findings confirm that atrophy increases with age, but they find that after age 65 there are minimal and fairly constant changes in brain volume over time. In the healthy elderly, brain atrophy does not progress more rapidly as people grow older.

Neuromuscular disorders

Flanigan et al. (p. 1634) draw attention to an ischemic myopathy in renal failure caused by medial calcification of small- to medium-sized arteries (calciphylaxis). The clinical picture resembles dermatomyositis. Recognition of this entity and early treatment of the underlying metabolic disturbance may improve the outcome. Articles by Marra et al. (p. 1678) and Kieburtz et al. (p. 1682) discuss the recognition and treatment of distal sensory neuropathy in HIV-infected individuals. Reliable recognition of this syndrome by non-neurologists, who provide the bulk of care, is important in initiating anti-HIV therapy. Amitriptyline, the standard symptomatic treatment of pain in other types of neuropathy, was found to be no better than placebo. Good et al. (p. 1735) report on initial open-label observations of cyclophosphamide therapy in chronic inflammatory demyelinating polyneuropathy. They suggest that their observation of benefit warrants further controlled investigations.

Ataxias and movement disorders

Moseley et al. (p. 1666) report on the incidence of specific genetic defects in families with ataxia syndromes. Pathogenic trinucleotide repeat expansions were identified in the majority of dominant kindreds and in some patients without a family history or in apparently recessive kindreds. The observations underscore the importance of identifying genetic mutations with the potential dominant inheritance pattern. Schöls (p. 1603) reports on the prevalence of restless legs syndrome in individuals with spinocerebellar ataxia type 3. The author suggests that restless legs may be a characteristic symptom of the dominantly inherited ataxias. In a small, randomized, blinded trial for restless legs syndrome, Earley et al. (p. 1599) found that the dopamine agonist pergolide was significantly better than placebo in improving clinical symptoms and sleep efficiency. Ziv et al. (p. 1583) report that parkinsonian patients have enhanced muscle fatigue during a continuous motor performance task. They suggest that this muscle fatigue is an integral symptom of PD and is related to dopamine deficiency rather than a muscle abnormality. Uitti et al. (p. 1755) investigated the efficacy of levodopa therapy before and after pallidotomy and found that the magnitude and time course of the response to levodopa is generally unaffected by surgery.

Cerebrovascular disease

Using National Hospital Discharge Survey data, Lanska and Kryscio (p. 1622) assessed the rates of stroke and intracranial venous thrombosis during pregnancy and puerperium. Stroke was associated with hypertension. The overall incidence of intracranial venous thrombosis was associated with advanced maternal age and was lower than that found in earlier studies. This study has an advantage over earlier epidemiologic surveys as it includes neuroradiologic data. In a Brief Communication, Evers et al. (p. 1709) report TIAs during trumpet playing in a young musician with a patent foramen ovale. Operative closure of the defect led to symptom resolution.

Multiple sclerosis

Khan and Dhib-Jalbut (p. 1698) found that MS patients treated with interferon beta frequently developed neutralizing antibodies, and that these antibodies frequently cross-reacted between the 1a and 1b formulations of interferon beta. This observation calls into question the clinical utility of switching patients to the alternate interferon beta preparation when they develop neutralizing antibodies.

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Search for Related Content