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This Month in Archives of Neurology
August 2008

This Month in Archives of Neurology

Arch Neurol. 2008;65(8):1013-1014. doi:10.1001/archneur.65.8.1013

Clot Removal Devices in Acute Ischemic Stroke

Stead and colleaguesArticle provide a very insightful and clinically useful review of the value of percutaneous mechanical embolectomy for acute ischemic stroke. Their extensive review of the literature indicates that percutaneous mechanical embolectomy for acute ischemic stroke is feasible and provides a therapeutic option for patients presenting after the intravenous tissue plasminogen activator window.

γ-Secretase Inhibitor as Therapy in Alzheimer Disease

Fleisher et alArticle study the safety, tolerability, and amyloid β response to a γ-secretase inhibitor (LY450139) in Alzheimer disease. The clinical trial provides promising results, as LY450139 was generally well tolerated at doses of up to 140 mg taken daily for 14 weeks, with several findings indicating the need for close clinical monitoring in future studies. Decreases in plasma amyloid β concentrations were consistent with inhibition of γ-secretase. Clearly, a larger study group, neuropsychological testing, magnetic resonance imaging evaluations, and a longitudinal follow-up are required to determine its clinical effectiveness to alter the course of the disease in a meaningful manner. This study offers important new data for planning future studies.

Thrombolysis in Patients With Marked Clinical Fluctuation in Neurologic Status Due to Cerebral Ischemia

The clinical course of patients with acute stroke who presented with marked fluctuations in neurologic status and who received thrombolytic therapy was evaluated by Ozdemir and colleaguesArticle. They report that all patients had a favorable neurologic and functional outcome at 3 months after thrombolysis. Thus, thrombolysis may indeed benefit patients with fluctuating symptoms and signs due to cerebral ischemia.

High-Dose Cyclophosphamide Reduces Disease Activity in Aggressive Multiple Sclerosis

Krishnan et alArticle explored the safety and effectiveness of high-dose cyclophosphamide (HiCy) without bone marrow transplantation in patients with aggressive multiple sclerosis (MS). They conclude that HiCy is safe and well tolerated in patients with MS. Patients experienced a pronounced reduction in disease activity and disability after HiCy. Their data support the view that immunoablative cyclophosphamide treatment for aggressive MS is worthy of further study and may be an alternative to bone marrow transplantation.

Brain Morphology in African American, Caribbean Hispanic, and White Individuals

Brickman et alArticle find that increased age and vascular disease, particularly in African Americans, are associated with increased brain atrophy and white matter hyperintensity burden. African American and Hispanic participants have larger relative brain volumes and more white matter hyperintensities than white individuals.

Duration and Severity of Diabetes Are Associated With Mild Cognitive Impairment

In this article, mild cognitive impairment (MCI) was associated with onset of diabetes before age 65, diabetes duration longer than 10 years, treatment with insulin, and presence of complications after adjustment for age, sex, and education. The frequency of diabetes was similar in subjects with and without MCI. Roberts and colleaguesArticle conclude that these findings suggest an association between earlier onset, longer duration, and greater severity of diabetes and MCI.

Odds ratios and 95% confidence intervals (logarithmic scale) for the association of mild cognitive impairment with type of treatment for diabetes mellitus (DM) (no treatment, oral hypoglycemic agent, or insulin with or without oral hypoglycemic agent) compared with subjects without DM (odds ratio, 1).

Odds ratios and 95% confidence intervals (logarithmic scale) for the association of mild cognitive impairment with type of treatment for diabetes mellitus (DM) (no treatment, oral hypoglycemic agent, or insulin with or without oral hypoglycemic agent) compared with subjects without DM (odds ratio, 1).

Incidental Lewy Body Disease Reflects Preclinical Parkinson Disease

DelleDonne et alArticle have determined if diminished striatal dopaminergic innervation and nigral cell loss are present in incidental Lewy body disease (iLBD) if it is a forerunner of Parkinson disease (PD). They find that for iLBD cases, decreased striatal dopaminergic immunoreactivity was documented for both tyrosine hydroxylase and vesicular monoamine transporter 2, compared with subjects without iLBD. These reductions were even greater in patients with PD. These results indicate that iLBD has nigrostriatal pathology that is intermediate between patients with and without PD. They conclude that iLBD probably represents presymptomatic PD rather than nonspecific, age-related α-synuclein pathology.

Embryonic Myosin Heavy-Chain Mutations Cause Distal Arthrogryposis

Tajsharghi et alArticle identified patients from 3 families with novel myosin heavy chain (MYH3) mutations. Recently, mutations in MYH3 were demonstrated to be associated with congenital joint contractures corresponding to distal arthrogryposis (DA) syndromes. Their results show that DA associated with MYH3 mutations is secondary to myosin myopathy and that postnatal muscle manifestations are variable.

Staging Dementia With Clinical Dementia Rating Scale Sum of Boxes Scores

O'Bryant et alArticle report that the Clinical Dementia Rating (CDR) Sum of Boxes scores compare well with the CDR Global score for dementia staging. Owing to the increased range of values, the Sum of Boxes score offers several advantages over the Global score, including increased utility in tracking changes within and between stages of dementia severity.

Motor Function and Human Immunodeficiency Virus–Associated Cognitive Impairment in a Highly Active Antiretroviral Therapy–Era Cohort

Robinson-Papp and colleaguesArticle find that motor, affective, and behavioral abnormalities predict cognitive impairment in human immunodeficiency virus (HIV)–positive patients in this highly active antiretroviral therapy–era cohort. The HIV-dementia motor scale reported here may be useful in the assignment of HIV-associated neurocognitive impairment in populations with HIV where normative data or neuropsychological test design is not optimal.

β-Site Amyloid Precursor Protein–Cleaving Enzyme 1 Activity in Alzheimer Disease

Zetterberg et alArticle find that elevated β-site amyloid precursor protein–cleaving enzyme 1 activity in cerebrospinal fluid may contribute to the amyloidogenic process in sporadic Alzheimer disease and is associated with the intensity of axonal degeneration.

Hepatocerebral Form of Mitochondrial DNA Depletion Syndrome: Novel MPV17 Gene Mutations

Spinazzola et alArticle describe the clinical, morphological, and genetic findings in 3 children from 2 unrelated families with MPV17- related mitochondrial DNA depletion. Their data confirm that MPV17 mutations are associated with a 2-stage syndrome. The first symptoms are metabolic and are related to rapidly progressive hepatic failure; this stage is then followed by neurological features affecting both central and peripheral systems.