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This Month in Archives of Neurology
Arch Neurol. 1998;55:903-904.
What to Include in Inclusion Body Myopathies
Askanas and Engel (SEE ARTICLE) provide a state-of-the art, elegant review of inclusion body myositis and hereditary inclusion body myopathies. Clinical, neuropathologic, and molecular details are clearly reviewed, with a view toward understanding these disorders from the perspective of oxidative stress and aging.
Elderly Brain Tumors
The clinical presentation of malignant brain tumors in the elderly differs from that in younger patients. Confusion, aphasia, or memory losses are more common in patients older than 65 years. Important diagnostic details are provided by Lowry et al (SEE ARTICLE) , and an expert perspective on this subject is included in an editorial by Fathallah-Shaykh and McIntire (SEE ARTICLE) .
1H-MRS and Amyotrophic Lateral Sclerosis
Block et al (SEE ARTICLE) studied patients with amyotrophic lateral sclerosis using proton magnetic resonance spectroscopy (1H-MRS) and found changes in brain ratios of N-acetylaspartate-choline, N-acetylaspartate-phosphocreatine, and choline-phosphocreatine compared with measurements in controls. These observations indicate both neuronal dysfunction and plasma membrane alteration and are new and important noninvasive findings to monitor the course of disease and future therapy. An insightful and focused editorial by Elliott (SEE ARTICLE) places this important contribution into clinical and neurobiological perspective.
The Highs and Lows of CSF in Alzheimer Disease
Galasko and colleagues (SEE ARTICLE) examined cerebrospinal fluid (CSF) from patients with Alzheimer disease and found significantly lower levels of amyloid protein at amino acid 42 (A 42) and significantly higher titers of tau compared with that in controls. These findings provide insight into the biological characteristics of the disease and are useful diagnostic criteria in establishing the diagnosis of this disease, especially in diagnostically difficult cases.
Eye Movement Testing in Ataxia
Specific eye movement abnormalities have been analyzed by Wessel et al (SEE ARTICLE) in a variety of patients with sporadic or inherited ataxia. Specific diagnostic statements can be made since these patients have been identified as having definite genotypic mutations and the specificities of the eye movement abnormalities are clarified as a result.
Treating Corticobasal Ganglionic Disease
Pharmacologic treatment of this class of disease has been proven to be difficult, as described by Kompoliti and colleagues (SEE ARTICLE) in a thorough and elegant study. Clearly, new treatments are needed to ameliorate the symptoms of this syndrome.
Apolipoprotein E and Dementia
Slooter and colleagues (SEE ARTICLE) reexamined using a population-based incidence study the apolipoprotein E genotype risk factor for dementia. Persons with the 4/4 genotype had a 10-fold higher risk of dementia, and subjects with the 3/4 genotype had a 1.7-fold increased risk for dementia, compared with persons with an 3/3 genotype. At the population level, it is clear that apolipoprotein E genotyping is of great value in determining the potential risk for developing Alzheimer disease.
Predicting Lewy Body Diseases
Litvan and colleagues (SEE ARTICLE) , in a thorough and elegant clinicopathologic study, show the important overlap of clinical features in the various presentations of disorders characterized by the presence of Lewy bodies. As the authors point out, predicting the future is risky and the accuracy of clinical diagnosis in these disorders is less than perfect. The lesson is that grouping Lewy body disorders into a single entity at present is premature and will compromise future research strategies.
Sensory Neuropathy Heralds Cancer
Camerlingo and colleagues (SEE ARTICLE) studied patients with sensory neuropathy, and 35% had cancer identified 3 to72 months from the onset of neuropathy. Sensory neuropathy takes on a new import from these observations.
Visualizing Myositis
Antimyosin scintigraphy and T2 magnetic resonance imaging of muscle are sensitive and diagnostic procedures to identify myositis and follow up patients' response to therapy. Löfberg et al (SEE ARTICLE) have developed a new approach to visualize myositis.
Thinking With Lewy Body Variant vs "Pure" Alzheimer Disease
Patients with "pure" Alzheimer disease (AD) or Lewy body variant (LBV) of AD with cortical and subcortical Lewy bodies were evaluated using cognitive profiles. The AD group performed significantly worse than the LBV group in memory testing. In contrast, the LBV group faired poorer on attention and construction subscales. It appears that Lewy body pathologic characteristics add significantly and uniquely to the cognitive profile, as described by Connor et al (SEE ARTICLE) .
Apolipoprotein E 4 and the Amyloid Burden
McNamara and colleagues (SEE ARTICLE) examined brain samples from individuals with Alzheimer disease with and without apolipoprotein E 4/4 for the amount of A 40, A 42, and total amyloid peptide. Persons with apolipoprotein 4/4 had increased concentrations of A 40 and A 42, and the explanation for this effect is elegantly weighed and discussed in this article.
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