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Kornfeld and Evason review the cellular and molecular genetic events that affect biological aging from invertebrates to humans. They point out the factors that account for the slow progress in developing therapies that delay aging. They cite their and others’ exciting new data with the nematode worm Caenorhabditis elegans as a model system to study animal aging. They identified a group of anticonvulsant drugs that extend life span and delay age-related degenerative changes in C elegans. The ability of these drugs to delay the C elegans aging process raises the intriguing possibility that these drugs might also delay human aging.
Korth and Peters present a positive assessment of emerging new therapies for prion disease. They point out that huge progress has been achieved in demonstrating reversibility of the neuropathologic features and protection from clinical symptoms in animal models and in introducing potential pharmaceuticals. They discuss the role of antibodies that are protective against prion disease and heterocyclic small molecule compounds that have been proposed as antiprion lead compounds for clinical trials.
Josephs and colleagues provide convincing clinical and neuropathologic data indicating that frontotemporal lobar degeneration (FTLD) and motor neuron disease (MND) represent a spectrum of pathologic abnormalities. Of 17 cases of FTLD with MND proven neuropathologically, all had clinical evidence of FTLD while only 10 (59%) had clinical evidence of MND. Editorial perspective is provided by Christopher M. Clark, MD, and Mark S. Forman, MD.
Singer et al describe their experience showing that pyridostigmine bromide significantly improves standing blood pressure in patients with orthostatic hypotension without worsening supine hypertension. The data are convincing (Figure) and the clinical need for effective therapy for patients with orthostatic hypotension is real, making their contribution significant and welcome.
Diastolic blood pressure (DBP) before and after administration of study drug. Each group (placebo, pyridostigmine bromide, pyridostigmine and 2.5 mg of midodrine hydrochloride, pyridostigmine and 5.0 mg of midodrine hydrochloride) averaged for the supine position and standing position. Asterisk indicates P<.05; dagger, P<.01. Error bars represent mean ± SD.
Brewer and colleagues compared tetrathiomolybdate and trientine in treating patients with Wilson disease. Careful neurological assessments were conducted in a cohort of patients with Wilson disease with both drugs, and they concluded that tetrathiomolybdate is a better choice than trientine for preserving neurologic function in patients who present with neurologic disease.
Sheth and colleagues provide poignant information showing that protracted ictal confusion is often not considered in the ambulatory elderly patient with resulting delay in diagnosis. Electro encephalographic and videoelectroencephalographic studies performed while the patient is symptomatic are crucial to early diagnosis and timely management.
Mathiesen et al studied global brain N-acetyl aspartate as a better predictor of cognitive dysfunction in multiple sclerosis than conventional magnetic resonance imaging measures. Multi-slice echo-planar spectroscopic imaging measures distinguished between cognitively impaired and unimpaired patients with multiple sclerosis and correlated with a global cognitive measure. No significant correlations were found between cognitive dysfunction and conventional magnetic resonance imaging measures, ie, the brain parenchymal fraction and lesion volume.
Galimberti and colleagues determined chemokine levels in cerebrospinal fluid in patients with mild cognitive impairment and Alzheimer disease. They found that interferon-γ-inducible protein 10 is specifically increased in mild cognitive impairment and decreases with the progression of Alzheimer disease. Other important chemokine changes were noted in late stages of the disease. Thus, chemokines serve as a potential important biomarker both in the early and late stages of Alzheimer disease.
Clark et al determined the frequency and spectrum of parkin gene variants in early-onset Parkinson disease cases (aged ≤50 years) and in controls participating in a familial aggregation study. They report that the frequency of mutations among cases that were not selected based on family history of Parkinson disease is similar to what has previously been reported in sporadic Parkinson disease. The similar frequency of Leu261Leu in cases and controls indicates it is a normal variant rather than a disease-associated mutation. They confirm that heterozygous parkin mutations may increase susceptibility for early-onset Parkinson disease.
Coutinho and colleagues describe families with autosomal dominant ataxia with spasmodic cough as a new syndrome. In all reported families, attacks of spasmodic coughing preceded ataxia by 1 to 3 decades and were a reliable marker of the disease.
For exploratory, short phase II clinical trials, de Carvalho and Swash provide a strategy of including fast progressing patients with amyotrophic lateral sclerosis because it offers substantial savings in cost and time and could potentially accelerate the process of testing useful drugs for treatment.
Perrin et al recorded the auditory evoked potentials to the patient's own name and to 7 other equiprobable first names in 15 patients with brain damage. A P3 component was observed in response to the patient's name in all patients with locked-in syndrome, in all patients in a minimally conscious state, and in 3 of 5 patients in a vegetative state. These results support the view that partially preserved semantic processing can be observed in noncommunicative patients with brain damage, notably for the detection of very salient stimuli, such as the subject's own name.
Reitz and colleagues studied 1271 elderly persons without dementia for memory functions after having a stroke. Memory performance declined over time while abstract/visuospatial and language performance remained stable. The association between stroke and decline in memory performance was strongest for men and for persons without an apolipoprotein E ε4 allele.
Baba et al compared the phenotypes of familial and sporadic Parkinson disease. They studied 1277 sporadic and 40 familial patients with Parkinson disease. An increased incidence of parkinsonism in men with familial Parkinson disease suggested that the sex disparity is more likely the result of a protective effect against the development of Parkinson disease in women than of an increased risk in men associated with environmental factors.
Greffard and colleagues studied the relationship between the motor symptoms assessed by the Unified Parkinson Disease Rating Scale, neuronal loss in the substantia nigra, and the duration of the disease. Of interest, they found that the score on the motor section of the Unified Parkinson Disease Rating Scale is linearly linked to neuronal density. Each point added to the score corresponded to an estimated loss of 25 neurons per cubic millimeter.
This Month in The Archives of Neurology. Arch Neurol. 2006;63(4):484–485. doi:https://doi.org/10.1001/archneur.63.4.484
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