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The American Academy of Neurology endorses advance directives to improve care of patients with cognitive incapacity. When directives are lacking or ignored, end-of-life care is often unsatisfactory. Goldblatt (p. 148) asserts that patients who need the help of their advance directives are neurologic patients and that neurologists are uniquely prepared to assist in caring for them. In an accompanying Editorial, Bernat (p. 144) concludes that by discussing medical planning with their patients, neurologists can foster patient-centered medicine.
Emotional content: Salience affects sensory perception and motor ability
Vuilleumier and Schwartz (p. 153) studied neglect in patients with right hemispheric stroke and found that faces were less subject to extinction than shapes and that faces with happy or angry expressions were less extinguishable than neutral faces. Crucian et al. (p. 159) studied patients with PD to quantitate the effect of topics of emotional experience on verbal output. They found that number of words was increased when talking about past emotional experiences. They termed this response verbal kinesia paradoxica—by analogy to the heightened motor activation patients with PD exhibit when confronted with a stressful situation (kinesia paradoxica). The accompanying Editorial by Loring and Meador (p. 146) reviews emotional commun- ication—facial, prosodic, and lexical—and points out how the salience of stimuli affects the sensory and motor responses evoked.
Is mesial temporal sclerosis (MTS) hereditary?
Kobayashi et al. (p. 166) assessed 22 families in which two or more individuals had mesial temporal lobe epilepsy (MTLE) by clinical/EEG criteria. MTS as assessed by MRI was present in subjects without MTLE, and was not always present in patients with MTLE. Moreover, MTS appeared to be hereditary in certain cases.
Lamotrigine for thalamic pain
Vestergaard et al. (p. 184) conducted a double-blind controlled crossover trial of lamotrigine in 30 patients with central poststroke pain. Lamotrigine (200 mg/day) reduced pain score significantly although only 44% of patients improved. Skin rash (2/30) was the only side effect requiring withdrawal from the study.
CREB: Does its impaired phosphorylation affect cognition?
The study of rare genetic defects that affect the brain often provides a window into normal CNS development. Harum et al. (p. 207) studied fibroblasts from patients with the X-linked disorder Coffin–Lowry syndrome (CLS), characterized by cognitive disabilities, distinctive facial features, and bony abnormalities. CLS is caused by mutations in RSK2, which phosphorylates CREB. The phosphorylation of a CREB-like peptide was impaired in patients with CLS and correlated with the degree of cognitive disability of patients.
Prognostic and IgM M-proteins in neuropathies
Eurelings et al. (p. 228) quantitated antibodies to myelin-associated glycoprotein (MAG), sulfoglucuronyl paragloboside (SGPG), and sulfatides in 65 patients with peripheral neuropathy and IgM monoclonal protein spike who were also characterized clinically and electrophysiologically. They found that sensory symptoms in the feet predicted a less severe, more protracted course. MAG, SGPG, and sulfatides antibodies had no prognostic value in predicting neurologic deficits.
Adult Rasmussen’s enceph- alitis: Response to IVIg
Villani et al. (p. 248) report a 45-year-old woman with adult-onset aphasia, right hemiparesis, refractory epilepsy including epilepsia partialis continua (EPC), and left hemisphere atrophy of 27 years’ duration. She did not have GluR3 anti- body. After unsuccessful trials of corticosteroids and plasma exchange, IVIg treatment (continued monthly) resulted in major improvement: a 75% reduction in seizure frequency, cessation of EPC, and improvement in neurologic function.
Hereditary spastic paraplegia in dopa-responsive dystonia (DRD)
Furukawa et al. (p. 260) describe a 10-year-old boy whose initial findings were of spastic paraplegia before developing characteristic DRD. His father had milder symptoms. Novel mutations of the tyrosine hydroxylase gene were identified and may account for the unusual phenotype. Both patients responded to L-dopa.
Restless legs syndrome (RLS): Brain iron by MRI
In a pilot study, Allen et al. (p. 263) compared brain iron by MRI in five patients with RLS versus five controls. Iron was decreased in substantia nigra and putamen to an extent that correlated with RLS severity.
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