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Neurology 2001;56:697
© 2001 American Academy of Neurology

March 27 Highlights

Markers for treatment response to valproate and lamotrigine?

Petroff et al. (p. 709) assessed homocarnosine, GABA, and pyrrolidone by MRS in patients with complex partial seizures (CPS) and juvenile myoclonic epilepsy (JME). Low GABA levels were associated with poor seizure control in CPS. Increased homocarnosine levels were associated with improved seizure control in both JME and CPS.In the accompanying editorial (p. 698), Henry and Theodore note that MRS has the potential to provide a means of assessing adequacy of seizure treatment if it can be established that homocarnosine mediates antiseizure effects of antiepileptic drugs. MRS could serve as a surrogate marker preferable to the clinical estimate of seizure frequency, particularly in CPS in which patients and caregivers are not aware of the seizures.

Localization of pure alexia

Sakuri et al. (p. 778) report a man who developed an inability to read Japanese phonograms (kana) with preservation of the ability to read morphograms (kanji) after a discrete left posterior occipital lobe hemorrhage.As discussed in the accompanying editorial by Grossman and Nakada (p. 699), this unique case provides helpful insights into the alexias of English-speaking subjects. They review the anatomic features of reading and its disorders from a historical perspective, including current work with functional imaging.

Effect of deep brain stimulation (DBS) in Parkinson’s disease

DBS of the internal globus pallidus (Gpi) may benefit patients with PD. Notably, in patients on levodopa treatment DBS of the Gpi results in a reduction in dyskinesias. Chen et al. (p. 716) report that DBS in patients with PD on optimal dopaminergic medication normalized the silent period after repetitive transcranial magnetic stimulation. Therefore, DBS may alter the silent period in treated patients with PD and thereby prevent dyskinesia.

Parkinson’s disease: Effect on attention

Barrett et al. (p. 724) compare 11 patients with PD with healthy control subjects to determine whether PD is characterized by an abnormal "attentional flood light"—-do patients with PD have too narrow a focus of perception? The authors found that patients with PD perceived larger letters less rapidly than did control subjects whereas small and medium-sized letters were perceived normally. Pallidotomy appeared to improve the abnormality.

How fast to start gabapentin in epilepsy?

Fisher et al. (p. 743) compared rapid initiation (900 mg/d) with a three-day slow initiation (300 mg/d, 600 mg/d, 900 mg/d) in gabapentin treatment of partial seizures (both with and without generalized seizures). Of side effects, only dizziness was more frequent in the rapid group. Therefore, 900 mg/d initial treatment with gabapentin appears well tolerated.

SMA gene a prognostic factor for ALS

Veldink et al. (p. 749) studied 110 patients with sporadic ALS for deletions of exons 7 and 8 of the two survival motor neurons (SMN) genes. Spinal muscular atrophy (SMA) results from mutations of the telomeric SMN gene (SMN1), and patients who have milder SMA have more copies of the centromeric gene (SMN2) in addition to the SMN1 mutation. Homozygous deletions of SMN2 were four times as frequent in patients with ALS than in control subjects; in addition, homozygous deletion shortened ALS survival rate. None of the patients with ALS had the SMA gene defect (homozygous deletion of SMN1).

Intracerebral hemorrhage (ICH)

Two groups of authors consider ICH. Becker et al. (p. 766) note that because withdrawal of support in patients with ICH invariably leads to death, factors that influence the decision to withdraw support will bias predictive models of outcome. They also studied the attitudes of faculty from departments of neurology and neurosurgery about futility of care. The authors assessed the effect of withdrawal of support in the context of 87 consecutive patients with ICH. Based on experience at their institution, the investigators found that support was withdrawn from a number of patients in whom a reasonable outcome may have been achieved. Therefore, the decision to withdraw support based on preconceived notions about prognosis results in patient death.Bailey et al. (p. 773) reviewed 10 studies reporting follow-up of 1,880 survivors of ICH. Survivors of ICH developed recurrent stroke at a rate of 4%/year, and most recurrences were ICH rather than ischemic stroke. Patients with lobar hemorrhage were more likely to have recurrence than those with deep hemorrhage. A history of ICH should be a consideration in subsequent anticoagulant treatment of thrombotic disorders.

MS risk in sibs of patients with MS: Effect of consanguineous parentage

Sadovnick et al. (p. 784) studied 67 sibs of 22 patients with MS whose parents were related. They found a 9% (6/67) incidence of MS, lending support to the concept of multiple interacting genes increasing the risk of MS.





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