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June 13 Highlights

Larriviere and Bonnie found that current laws in over two-thirds of the states facilitate the withdrawal of artificial nutrition and hydration from patients diagnosed in a vegetative state, but pending legislation in several states could, if passed, make it much more difficult to do so.

see page 1624

The editorial by Bernat and Beresford discusses how the tragic case of Terri Schiavo raised to public consciousness the issue of whether a lawful surrogate is empowered to refuse artificial hydration and nutrition (AHN) on behalf of a patient in a persistent vegetative state (PVS) based on preferences she orally expressed while competent. This article illustrates why neurologists should understand how state laws regulate their medical practice—particularly important because a 1999 survey of neurologists uncovered a widespread lack of understanding of legal and ethical issues relating to stopping and withholding of life support. Second, it may stimulate neurologists to support state laws that protect the rights of patients to control their treatment once they have lost their capacity to consent or refuse. Moreover, the article highlights how important it is for neurologists to encourage their patients to execute advance directives that clearly designate who will be their surrogates in the event of incapacity and specify what treatments they want or do not want, including AHN.

see page 1618

Partial seizure symptom definitions for genetic studies

Standardized methods for classification are important for phenotype definition in the epilepsies. Choi et al. developed standardized definitions of 41 partial seizure symptoms for use in genetic research. For most definitions, inter-rater reliability of classification by two neurologists was "substantial" or "almost perfect."

see page 1648

The editorial by Gurnett and Trevathan first considers the disappointing lack of concordance between our current classification of the epilepsies and emerging genetic associations. They argue that a rethinking of our classification is necessary; by incorporating symptoms—as in the tool devised by Choi et al.—and treatment outcomes, newly identified genes can more easily be associated with epilepsy syndromes, thereby allowing clinicians a better basis for prognosis and treatment.

see page 1622

Levetiracetam in children with partial seizure

In a 14-week double-blind placebo-controlled study of 198 patients, Glauser et al. found that levetiracetam adjunctive therapy at 60 mg/kg/day is efficacious and well-tolerated in children (4 to 16 years old) with treatment-resistant partial seizures.

see page 1654

Genotype and chemosensitivity in oligodendroglial tumors

Walker et al. investigated the predictive and prognostic value of genotype in oligodendroglial tumors in a routine clinical setting. 1p/19q loss was associated with chemosensitivity and favorable outcome following PCV chemotherapy, supporting the use of genetic analysis to guide clinical management. However, 29% of cases with intact 1p/19q also responded.

see page 1661

Caudal paramedian midbrain syndrome

Caudal midbrain lesions involving the entire decussation of the superior cerebellar peduncles are rare, but have a distinctive clinical picture. Occasionally, unilateral lesions may produce a similar picture. Luigi Mossuto-Agatiello reports the spectrum of clinical and neuroimaging findings in patients with unilateral caudal midbrain infarcts.

Figure. T1 MRI: sagittal section showing caudal midbrain infarct.

see page 1668

Myelomeningocele in a Peruvian mummy from the Moche period

Moche was an ancient South American civilization that flourished in northern Peru. Carod-Artal and Vázquez-Cabrera describe a 1,700-year-old mummy of a Moche child with myelomeningocele.

see page 1775

The Case of the Reed in the Breeze

Neurology’s Newsletter Editor, Robert J. Joynt, turns his hand to fiction in this neurologic pastiche featuring Sherlock Holmes and Dr. Watson.

see page 1782

Late onset psychogenic nonepileptic attacks

This study by Duncan et al. identified a subgroup of patients with late onset psychogenic nonepileptic attacks (PNEA), in whom severe physical health problems and health-related traumatic experiences were common. Occurring more frequently in men, health-related psychological trauma played a prominent role in the development of PNEA.

see page 1644

Diagnosis of psychogenic nonepileptic status epilepticus

Holtkamp et al. demonstrate that younger age, presence of a venous port system, administration of larger amounts of benzodiazepines, and normal creatine kinase levels are helpful markers favoring the diagnosis of psychogenic rather than "true" status epilepticus.

Figure. Episodes of psychogenic nonepileptic status epilepticus (PNESE) vs refractory generalized convulsive status epilepticus (RGCSE).

see page 1727

Eye closure predicts nonepileptic seizures

Chung et al. report the association of eye closure during seizures and PNES. Eye closure was highly predictive for PNES while eye opening predicted true epileptic seizures.

see page 1730

The editorial by LaFrance and Benbadis about these three articles discusses the value of symptoms and signs that can help with the differential diagnosis between psychogenic non-epileptic attacks (PNES) and epileptic seizures. Using these signs and video-EEG monitoring will help make an early diagnosis of PNES, thus alleviating the considerable personal and societal costs—both monetary and psychosocial—attendant on the diagnosis and treatment of uncontrolled seizures.

see page 1620

Validity of measures of Friedreich ataxia

Lynch et al. found that multiple measures based both on the neurologic examination and quantitative performance tasks capture disease features of Friedreich ataxia.

see pages 1711 and 1717

A new rating scale for ataxia

Schmitz-Hübsch et al. tested a newly developed Scale for the Assessment and Rating of Ataxia (SARA) in two trials of 167 and 119 patients with spinocerebellar ataxia. SARA was easy to apply and satisfied accepted criteria of reliability. Further analyses demonstrated linearity and validity of the scale.

see pages 1717 and 1711

Treatment of refractory fever with cold saline infusion

Badjatia et al. used rapid infusion of large volume cold saline as an adjunctive therapy for inducing normothermia in a series of nine patients with refractory fever.

see page 1739

Altered tau phosphorylation in ALS

Strong et al. report tau hyperphosphorylation in ALS with cognitive impairment. Hyperphosphorylation was associated with increased insoluble tau and resistance to dephosphorylation.

see page 1770

Related Articles

The controversy over artificial hydration and nutrition

James L. Bernat and H. Richard Beresford
Neurology 2006 66: 1618-1619. [Full Text] [PDF]

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Search for Related Content Related Collections Related Articles