 |
|
This Month in Archives of Neurology
Arch Neurol. 1998;55:597-598.
Corticobasal Disease With Colossal Callosal Atrophy
Yamauchi et al (SEE ARTICLE) describe progressive atrophy of the corpus callosum in corticobasal degeneration with cognitive loss and quantitative reduction in cortical metabolism measured using cerebral metabolic rate of glucose values determined with positron emission tomography. It is an elegant extension of our biochemical understanding of this complex disorder.
Cerebration After Grafting
A prospective controlled study of patients undergoing elective coronary artery bypass grafting was conducted with careful magnetic resonance imaging, electroencephalographic, neurologic, and neuropsychological assessments. Of 38 Finnish patients studied, along with 20 control patients, Vanninen and colleagues (SEE ARTICLE) found increased electroencephalographic alterations and ischemic cerebral lesions by magnetic resonance imaging without significant change in neuropsychological test performance. These findings are important and need to be compared and contrasted with parallel studies appearing in the literature.
The Personality of Myotonic Dystrophy
Genetic diseases of muscle may have correlative effects on brain function, including personality features. Delaporte (SEE ARTICLE) has assessed the personalities of 15 patients with myotonic dystrophy (DM) and found a homogeneous distinctive profile. A high incidence of avoidance personality is described in patients with DM. It appears that CTG repeats in the myotonin protein kinase gene and regulation of protein phosphorylation affect both central and peripheral functions. Behavioral genetics needs careful consideration even in the muscle clinic.
Withdraw Felbamate With Care
Withdrawal of felbamate therapy, as described by Welty and colleagues (SEE ARTICLE) , results in a significant increase in seizure frequency in the subsequent 3 months. Clearly, these findings are important for clinical trial design and clinical practice.
Swaying of the Vestibular or Cerebellar Type
The diagnostic usefulness of posturography has been studied in patients with vestibular loss and cerebellar disease. Sway amplitude and velocity were consistently increased in patients with vestibular disease and cerebellar atrophy, and it was difficult to distinguish between the 2 patient groups, as reported by Baloh and colleagues (SEE ARTICLE) .
Quality of Life in Epilepsy
Breier et al (SEE ARTICLE) contrast and compare self-reported quality of life in patients with intractable epilepsy and pseudoseizures. Self-perception in these 2 groups was similar but somewhat different, and to the degree that they were different is useful in appreciating their social stability.
Treating Childhood Epilepsy
Carpay et al (SEE ARTICLE) prospectively studied almost 500 children with a broad range of seizures and conclude that a substantial percentage of children with new-onset epilepsy did not need treatment with antiepileptic drugs. The details of this report provide interesting information about the natural history of seizures in children and put pharmacological therapy in a new light.
Thinking in and About Parkinson Disease
Cognitive performance in nondemented patients with Parkinson disease is controversial and needs further study. Goldman and colleagues (SEE ARTICLE) show that patients with Parkinson disease are predisposed to subclinical cognitive impairment. What the natural history of this mild impairment is will require longitudinal follow-up, but the issue is more pervasive than previously appreciated.
Blood Pressure Rhythm
Kukla et al (SEE ARTICLE) studied the relationship between circadian blood pressure rhythm and the type of lacunar infarction in some detail. It is interesting that a reduced circadian blood pressure variation due to an increased incidence of pathological nighttime blood pressure increases was noted. The reduced circadian blood pressure variation and other factors correlated with the occurrence of lacunar infarction. The dynamics of this entire process are clearly important to understand the interrelationships between these 2 features. Nighttime blood pressure normalization is a practical desired effect that needs further emphasis in patient treatment.
Presenilin Gene Expression
A large Swedish family with a His163Tyr mutation in the presenilin 1 gene is described by Axelman et al (SEE ARTICLE) . A large range in age of onset in this family with a uniform genetic basis supports the existence of unknown genetic or environmental factors responsible for the clinical expression of Alzheimer disease. The genetic aspects here are clear but the nongenetic influences remain elusive.
Doing Things With Alzheimer Disease
Giannakopoulos et al (SEE ARTICLE) studied patients with Alzheimer disease who had ideomotor and dressing apraxia, and the inability to perform these practical activities of daily living correlated with damage in the anterior cingulate cortex. The authors found that constructional apraxia, however, was related to specific disruption of cortical pathways mediating visuospatial behaviors. Of note, these correlations occurred between neurofibrillary tangle densities in these regions and did not correlate with neuritic plaque counts. This point is of considerable interest and is a unique biological observation.
Shunting Leukoencephalopathy
Patients with radiation-induced hydrocephalus benefit from ventriculoperitoneal shunting, as described by Thiessen and DeAngelis (SEE ARTICLE) . Reliable prediction of a successful shunt outcome in selected patients was not identified, but it is clear that some patients improve. It is an important observation, but the exact combination of factors involved in this complex disease process remains unknown.
|
