MS disease activity in RESTORE: A randomized 24-week natalizumab treatment interruption study
The RESTORE trial investigated the effects of stopping natalizumab treatment in patients with MS. MRI disease activity recurred starting at 12 weeks while relapses were reported as early as 4 weeks. Results suggest that planned natalizumab interruption is unlikely to be a useful strategy to reduce natalizumab risks.
EEG-fMRI in myoclonic astatic epilepsy (Doose syndrome)
Simultaneous EEG-fMRI recordings were performed in 13 children with myoclonic astatic epilepsy. Individual generalized spike and wave discharges associated with blood oxygenation level–dependent signal changes were analyzed in every patient. The results demonstrated that somatosensory cortex, putamen, and cerebellum are involved in the generation of myoclonic seizures.
See p. 1508
From editorialists Zempel & Mano: “EEG-fMRI may be particularly helpful diagnostically by providing a biomarker for distinguishing subtypes of these often overlapping generalized epilepsy syndromes.”
See p. 1486
Modifiable cardiovascular risk factors and axial motor impairments in Parkinson disease
The authors reported that elevated Framingham risk scores were associated with a more rapid rate of axial motor progression in patients with Parkinson disease. Early modification of cardiovascular risk factors, including hypertension, deserves further study as a novel disease-modifying strategy in Parkinson disease.
Progressive encephalomyelitis with rigidity and myoclonus: A new variant with DPPX antibodies
This case series described the clinical, paraclinical, and serologic features of 3 patients with progressive encephalomyelitis with rigidity and myoclonus. Hallmark features were striking hyperekplexia, cerebellar ataxia, trunk stiffness, inflammatory CSF, and slowly progressive course, as well as response to immunotherapy, indicating that testing for antibodies to DPPX (dipeptidyl peptidase-like protein 6) should be considered in these patients.
See p. 1521
Clinical trial of an inhibitor of RAGE-Aβ interactions in Alzheimer disease
This trial tested doses of the study drug, an inhibitor of the receptor for advanced glycation end products (RAGE), vs placebo in patients with Alzheimer disease over 18 months. The higher dose was stopped due to falls and confusion and the lower dose was discontinued later as a result of a futility analysis. Post-futility analyses were ambiguous; however, the lower dose had benefits on the Alzheimer's Disease Assessment Scale-cognitive.
See p. 1536
Dementia and dependence: Do modifiable risk factors delay disability?
The authors followed 4,922 individuals without activities of daily living (ADL) limitations but with varying degrees of cognitive impairment to test predictors of onset of ADL limitations and whether those predictors varied by level of cognitive impairment. Smoking, not drinking, and low income increased the risk of incident ADL limitations in those with cognitive impairment.
See p. 1543
SPECIAL ARTICLE
Systematic review: Efficacy and safety of medical marijuana in selected neurologic disorders
The authors performed a systematic review of medical marijuana to address treatment of symptoms of multiple sclerosis, epilepsy, and movement disorders. Cannabinoids should be studied as other drugs are, to determine their efficacy, and when evidence is available, should be prescribed as other drugs are.
See p. 1556
NB: “Mystery Case: Pendular see-saw nystagmus as a delayed complication of traumatic brain injury,” see p. e147. To check out other Resident & Fellow Mystery Cases, point your browser to Neurology.org and click on the link to the Resident & Fellow Section.
- © 2014 American Academy of Neurology
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