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Neurology 2000;54:1017
© 2000 American Academy of Neurology

March 14 Highlights

Movement disorders

Rivaud-Péchoux et al. (p. 1029) assessed eye movements longitudinally in patients with corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). Distinctive abnormalities separated the two disorders: PSP—decreased saccade velocity; CBD—preserved velocity but increased saccade latency. The accompanying editorial by Leigh and Riley (p. 1018) places eye movement abnormalities in PSP and CBD into the context of other syndromes with parkinsonian features and emphasizes the clinical testing of patients.

{blacklozenge} The limited availability of human embryonic tissue and ethical concerns have prompted a search for alternative fetal tissue sources for treatment of PD. Schumacher et al. (p. 1042) report that embryonic porcine mesencephalon transplantation into 12 PD patients was well tolerated and produced significant clinical benefit.

{blacklozenge} Schmand et al. (p. 1058) studied cognition and behavior in 35 PD patients who were treated with unilateral pallidotomy. The study was controlled by dividing the patients into two groups: 19 who were promptly pallidotomized and 6 who were reassessed after 6 months before proceeding with pallidotomy. There was no difference between pallidotomized and control patients except for decreased verbal fluency in left side–treated patients.

{blacklozenge} Rothdach et al. (p. 1064) studied the prevalence of restless legs syndrome (RLS) in 369 patients 65 to 83 years of age. Overall prevalence was 9.8% (women 13.9%; men 6.1%). RLS patients had more depression than controls.

Inclusion body myositis

Inclusion body myositis (IBM) is the commonest muscle disease presenting after age 50. The etiology of IBM is not known and anti-inflammatory agents and other treatments are of no benefit. Banwell and A.G. Engel (p. 1033) report that there is enhanced expression of the heat shock protein {alpha}B-crystallin in all abnormal fibers in IBM muscle biopsies. Importantly, the heat shock protein was also overexpressed in a larger number of normal-appearing fibers. The accompanying editorial by Karpati and Hohlfeld (p. 1020) notes that their observation suggests the hypothesis that the heat shock protein could be a compensatory mechanism protecting cells from an as-yet undefined stress. The work adds evidence that muscle inflammation in IBM is a late sequel to a primary muscle abnormality.

Nerve growth factor for HIV neuropathy

McArthur et al. (p. 1080) report a randomized controlled trial of nerve growth factor in 270 patients with HIV-associated sensory neuropathy. Both neuropathic pain and pin sensitivity were benefited. Injection site pain was frequent and unblinding in 39% of the patients. Epidermal nerve fibers (studied by punch biopsies) did not change.

HIV encephalopathy in infants

Tardieu et al. (p. 1089) compared the occurrence and characteristics of HIV encephalopathy in infants with that in children and adults using large cohorts of patients: 3,364 mother-child pairs. Azidovudine (AZT) treatment protocol but not highly active antiretroviral therapy was part of the study. Infant encephalopathy differs from that of older children and adults; it was associated with decreased intrauterine brain growth and occurred despite AZT treatment, better immunocompetence, and low CSF HIV-1 RNA load.

Dementia and stroke

Desmond et al. (p. 1124) studied 453 patients (mean age 72 years) 3 months after ischemic stroke. Dementia was identified in 119 (26%), vascular dementia in the majority (57%), and AD in 39%. A number of determinants of dementia were identified.

Neurofibromatosis 2: Genetic basis of a mild phenotype

Sainio et al. (p. 1132) report a large family with neurofibromatosis whose bilateral vestibular nerve schwannomas were of late onset and slow growth. The specific merlin gene abnormality permitted expression of variant merlin that probably has some tumor suppressor activity.

Multiple sclerosis

Two multicenter study groups (p. 1145) report the clinical and MRI outcomes of a controlled trial of the lymphocytotoxic purine analogue cladribine. Cladribine was well tolerated but did not produce clinical benefit. It did significantly decrease the presence, number, and volume of contrast-enhanced T1 brain lesions.

Narcolepsy

The US Modafinil in Narcolepsy Multicenter Study Group (p. 1166) report a 9-week controlled trial in 271 patients. Significant improvement was seen in both objective measures (the Multiple Sleep Latency Test and Maintenance of Wakefulness Test) as well as subjective patient symptoms. Nighttime sleep was not adversely affected.

Reducing the pain of EMG needle insertion

Pohl et al.’s (p. 1201) randomized controlled study showed that pain could be reduced by finger slapping adjacent to insertion of the EMG needle. This Korean nurse-originated strategy for needle insertion was without risk.





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