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- Lifeline. Interview with Christian Grefkes. [Interview]
- Lancet Neurol 2014 Feb; 13(2):139.
- Medications for sleep disturbances in children. [JOURNAL ARTICLE]
- Neurol Clin Pract 2014 Feb; 4(1):82-87.
At least 25% of infants, children, and adolescents have a sleep problem at some point during this developmental period. The management of pediatric sleep-related disorders often begins with behavioral strategies. While medications can be a useful adjunct, they are used off-label for sleep problems in this age group. When used, medications should be chosen carefully and targeted to specific outcomes as part of a comprehensive approach to management. This article reviews medications used for common pediatric sleep problems with a focus on pediatric insomnia and the importance of a multifactorial approach to evaluation and management.
- Health literacy and medication awareness in outpatient neurology. [JOURNAL ARTICLE]
- Neurol Clin Pract 2014 Feb; 4(1):71-81.
AS RESEARCHERS CONTINUE TO ILLUMINATE THE COMPLEXITIES OF NEUROLOGIC DISORDERS AND THEIR MANAGEMENT, THE CLINICIAN FACES AN EQUALLY INTENSIFYING BURDEN: how to communicate these advances effectively to patients. Health literacy (HL) refers to a person's ability to find, comprehend, and use basic information and resources in order to make appropriate decisions related to his or her health. We describe the first study of low HL prevalence using a validated measure in a cohort of adult neurology outpatients without dementia. Of 201 subjects, 20.5% had low HL and 26% were unable to name any of their medications. In a multivariate regression model controlling for demographic and clinical factors, low HL was associated with lower education, greater disease duration, each 1-point decrease in the Folstein Mini-Mental State Examination, and the presence of a caregiver. As clinicians, we must incorporate specific communication and patient education strategies into our daily practice to truly optimize our patients' care.
- Key sleep neurologic disorders: Narcolepsy, restless legs syndrome/Willis-Ekbom disease, and REM sleep behavior disorder. [JOURNAL ARTICLE]
- Neurol Clin Pract 2014 Feb; 4(1):16-25.
Sleep disorders are frequent comorbidities in neurologic patients. This review focuses on clinical aspects and prognosis of 3 neurologic sleep disorders: narcolepsy, restless legs syndrome/Willis-Ekbom disease (RLS/WED), and REM sleep behavior disorder (RBD). Narcolepsy causes pervasive, enduring excessive daytime sleepiness, adversely affecting patients' daily functioning. RLS/WED is characterized by an uncomfortable urge to move the legs before sleep, often evolving toward augmentation and resulting in daylong bothersome symptoms. RBD causes potentially injurious dream enactment behaviors that often signify future evolution of overt synucleinopathy neurodegeneration in as many as 81% of patients. Timely recognition, referral for polysomnography, and longitudinal follow-up of narcolepsy, RLS/WED, and RBD patients are imperatives for neurologists in providing quality comprehensive patient care.
- Cerebrovascular function and cognition in childhood: a systematic review of transcranial doppler studies. [JOURNAL ARTICLE]
- BMC Neurol 2014 Mar 6; 14(1):43.
The contribution of cerebrovascular function to cognitive performance is gaining increased attention. Transcranial doppler (TCD) is portable, reliable, inexpensive and extremely well tolerated by young and clinical samples. It enables measurement of blood flow velocity in major cerebral arteries at rest and during cognitive tasks.We systematically reviewed evidence for associations between cognitive performance and cerebrovascular function in children (0-18 years), as measured using TCD. A total of 2778 articles were retrieved from PsychInfo, Pubmed, and EMBASE searches and 25 relevant articles were identified.Most studies investigated clinical groups, where decreased blood flow velocities in infants were associated with poor neurological functioning, and increased blood flow velocities in children with Sickle cell disease were typically associated with cognitive impairment and lower intelligence. Studies were also identified assessing autistic behaviour, mental retardation and sleep disordered breathing. In healthy children, the majority of studies reported cognitive processing produced lateralised changes in blood flow velocities however these physiological responses did not appear to correlate with behavioural cognitive performance.Poor cognitive performance appears to be associated with decreased blood flow velocities in premature infants, and increased velocities in Sickle cell disease children using TCD methods. However knowledge in healthy samples is relatively limited. The technique is well tolerated by children, is portable and inexpensive. It therefore stands to make a valuable contribution to knowledge regarding the underlying functional biology of cognitive performance in childhood.
- Rotigotine transdermal system and evaluation of pain in patients with Parkinson's disease: a post hoc analysis of the RECOVER study. [JOURNAL ARTICLE]
- BMC Neurol 2014 Mar 6; 14(1):42.
Pain is a troublesome non-motor symptom of Parkinson's disease (PD). The RECOVER (Randomized Evaluation of the 24-hour Coverage: Efficacy of Rotigotine; Clintrials.gov: NCT00474058) study demonstrated significant improvements in early-morning motor function (UPDRS III) and sleep disturbances (PDSS-2) with rotigotine transdermal system. Improvements were also reported on a Likert pain scale (measuring any type of pain). This post hoc analysis of RECOVER further evaluates the effect of rotigotine on pain, and whether improvements in pain may be attributable to benefits in motor function or sleep disturbance.PD patients with unsatisfactory early-morning motor impairment were randomized to optimal-dose (up to 16 mg/24 h) rotigotine or placebo, maintained for 4 weeks. Pain was assessed in the early-morning using an 11-point Likert pain scale (rated average severity of pain (of any type) over the preceding 12 hours from 0 [no pain] to 10 [worst pain ever experienced]). Post hoc analyses for patients reporting 'any' pain (pain score >=1) at baseline, and subgroups reporting 'mild' (score 1-3), and 'moderate-to-severe' pain (score >=4) were performed. Likert pain scale change from baseline in rotigotine-treated patients was further analyzed based on a UPDRS III/PDSS-2 responder analysis (a responder defined as showing a >=30% reduction in early morning UPDRS III total score or PDSS-2 total score). As post hoc analyses, all p values presented are exploratory.Of 267 patients with Likert pain data (178 rotigotine, 89 placebo), 187 (70%) reported 'any' pain; of these 87 (33%) reported 'mild', and 100 (37%) 'moderate-to-severe' pain. Change from baseline pain scores decreased with rotigotine compared with placebo in patients with 'any' pain (-0.88 [95% CI: -1.56, -0.19], p = 0.013), and in the subgroup with 'moderate-to-severe' pain (-1.38 [-2.44, -0.31], p = 0.012). UPDRS III or PDSS-2 responders showed greater improvement in pain than non-responders.The results from this post hoc analysis of the RECOVER study suggest that pain was improved in patients with PD treated with rotigotine; this may be partly attributable to benefits in motor function and sleep disturbances. Prospective studies are warranted to investigate this potential benefit and the clinical relevance of these findings.
- Memory impairment caused by cerebral hematoma in the left medial temporal lobe due to ruptured posterior cerebral artery aneurysm. [JOURNAL ARTICLE]
- BMC Neurol 2014 Mar 7; 14(1):44.
Cognitive disorders, such as memory disturbances, are often observed following a subarachnoid haemorrhage. We present a very rare case where rupture of a posterior cerebral artery aneurysm caused restricted damage to the hippocampus unilaterally, and caused memory disturbances.A 56-year-old, right-handed man, with a formal education history of 16 years and company employees was admitted to our hospital because of a consciousness disturbance. He was diagnosed as having a subarachnoid haemorrhage due to a left posterior cerebral artery dissecting aneurysm, and coil embolization was performed. Subsequently, he had neither motor paresis nor sensory disturbances, but he showed disorientation, and both retrograde and anterograde amnesia. Although immediate recall and remote memory were almost intact, his recent memory was moderately impaired. Both verbal and non-verbal memories were impaired. Brain computed tomography (CT) and magnetic resonance imaging (MRI) revealed a cerebral hematoma in the left temporal lobe involving the hippocampus and parahippocampal gyrus, and single-photon emission computed tomography (SPECT) demonstrated low perfusion areas in the left medial temporal lobe.We suggest that the memory impairment was caused by local tissue destruction of Papez's circuit in the dominant hemisphere due to the cerebral hematoma.
- Role of the phosphoinositide phosphatase FIG4 gene in familial epilepsy with polymicrogyria. [JOURNAL ARTICLE]
- Neurology 2014 Mar 5.
The aim of this study was to identify the causal gene in a consanguineous Moroccan family with temporo-occipital polymicrogyria, psychiatric manifestations, and epilepsy, previously mapped to the 6q16-q22 region.We used exome sequencing and analyzed candidate variants in the 6q16-q22 locus, as well as a rescue assay in Fig4-null mouse fibroblasts and immunohistochemistry of Fig4-null mouse brains.A homozygous missense mutation (p.Asp783Val) in the phosphoinositide phosphatase gene FIG4 was identified. Pathogenicity of the variant was supported by impaired rescue of the enlarged vacuoles in transfected fibroblasts from Fig4-deficient mice. Histologic examination of Fig4-null mouse brain revealed neurodevelopmental impairment in the hippocampus, cortex, and cerebellum as well as impaired cerebellar gyration/foliation reminiscent of human cortical malformations.This study extends the spectrum of phenotypes associated with FIG4 mutations to include cortical malformation associated with seizures and psychiatric manifestations, in addition to the previously described Charcot-Marie-Tooth disease type 4J and Yunis-Varón syndrome.
- Pathogenic variants in TUBB4A are not found in primary dystonia. [JOURNAL ARTICLE]
- Neurology 2014 Mar 5.
To determine the contribution of TUBB4A, recently associated with DYT4 dystonia in a pedigree with "whispering dysphonia" from Norfolk, United Kingdom, to the etiopathogenesis of primary dystonia.High-resolution melting and Sanger sequencing were used to inspect the entire coding region of TUBB4A in 575 subjects with primary laryngeal, segmental, or generalized dystonia.No pathogenic variants, including the exon 1 variant (c.4C>G) identified in the DYT4 whispering dysphonia kindred, were found in this study.The c.4C>G DYT4 mutation appears to be private, and clinical testing for TUBB4A mutations is not justified in spasmodic dysphonia or other forms of primary dystonia. Moreover, given its allelic association with leukoencephalopathy hypomyelination with atrophy of basal ganglia and cerebellum and protean clinical manifestations (chorea, ataxia, dysarthria, intellectual disability, dysmorphic facial features, and psychiatric disorders), DYT4 should not be categorized as a primary dystonia.
- Treatment of primary CNS vasculitis with rituximab: Case report. [JOURNAL ARTICLE]
- Neurology 2014 Mar 5.
Primary CNS vasculitis (PCNSV) is an uncommon disorder of unknown cause that is restricted to brain and spinal cord. Glucocorticoids alone or in combination with cyclophosphamide achieve a favorable response in most patients.(1,2) However, some patients are intolerant or respond poorly to cyclophosphamide, therefore there is the need for new treatment options. We report a patient with PCNSV who appeared to respond to treatment with corticosteroids and rituximab. This study was approved by the Mayo Clinic Institutional Review Board and written informed patient consent to perform the study was obtained.