How Mead Has Muddled Judicial Review of Agency Action

When the Supreme Court decided United States v. Mead Corp. four years ago, Justice Scalia predicted that judicial review of agency action would devolve into chaos. This Article puts that prediction to the test by examining the court of appeals decisions applying the decision. Justice Scalia actually understated the effect of Mead. This Article suggests a remedy for the mess. In Mead, the Court held that an agency is entitled to deference under Chevron, U.S.A., Inc. v. NRDC only if Congress has delegated to that agency the authority to issue interpretations that carry the force of law, and the agency has used that authority in issuing a particular interpretation. Justice Scalia dissented, arguing that Mead makes an avulsive change in judicial review of agency action, the consequences of which will be enormous, and almost uniformly bad. On his reading, what was previously a general presumption of authority in agencies to resolve ambiguity in the statutes they have been authorized to enforce has been changed to a presumption of no such authority, which must be overcome by affirmative legislative intent. Lower courts, he warned, would not know what to make of the decision in practice: We will be sorting out the consequences of the Mead doctrine, which today has replaced the Chevron doctrine, for years to come. Notwithstanding Justice Scalia\u27s doomsday forecast, the majority believed that Mead was justified in principle. The Court stated that Mead tailors deference to [the] variety of administrative procedures that Congress envisions and agencies employ. An agency may receive Chevron deference as long as it chooses a proper procedure for issuing interpretations of the statute it administers. Thus, an agency may receive Chevron deference if it chooses a procedure that Congress generally intends to produce interpretations with the force of law - as with notice-and-comment rulemaking or formal adjudication. But an agency might not receive Chevron deference when it selects a more informal procedure unless the circumstances specifically suggest that Congress would have intended the resulting interpretation to carry the force of law. The agency may, however, still earn judicial respect under Skidmore v. Swift & Co., if it produces an interpretation that reflects \u27a body of experience and informed judgment \u27 upon which courts, though not required, may rely. This Article examines the effects of Mead by studying the court of appeals opinions that have purported to follow the decision.13 Years have passed since Mead was decided, and we still lack a clear answer to the question when an agency is entitled to Chevron deference for procedures other than notice-and-comment rulemaking or formal adjudication. Lower courts adopt inconsistent approaches. Many find ways to avoid the question altogether. Others use Mead in ways broader than the Court intended

The Future of Agency Independence

Independent agencies have long been viewed as different from executive-branch agencies because the President lacks authority to fire their leaders for political reasons, such as failure to follow administration policy. In this Article, we identify mechanisms that make independent agencies increasingly responsive to presidential preferences. We find these mechanisms in a context where independent agencies traditionally have dominated: financial policy. In legislative proposals for securing market stability, we point to statutorily mandated collaboration on policy between the Federal Reserve Board and the Secretary of the Treasury. In administration practices for improving securities regulation, we focus on White House coordination of, and Treasury Department involvement in, the policy of the Securities and Exchange Commission. We argue that these mechanisms undermine the conventional distinction between independent agencies and executive-branch agencies. Additionally, we argue that these mechanisms, though producing presidential involvement short of plenary control, are consistent with the strategic political interests of the President. We further contend that they promote political accountability, particularly because greater presidential control is unnecessary to align agency preferences with presidential preferences; indeed, such control might be counterproductive. In making this argument, we present a nuanced vision of accountability and update the standard justifications for independence. We also consider the constitutional implications of the new independence-accountability hybrids that we see, as well as possible applications in areas where executive-branch agencies traditionally have dominated. Our claim is not that these hybrids are part of law in any of these contexts; rather, we seek to highlight institutional relationships that outstrip conventional categories but fit with the development of the administrative state. In the future, agency independence will occur not at odds with political accountability but engaged with it along a spectrum of institutional structures

Speech-language therapy students' auditory-perceptual judgements of simulated concurrent hypernasality and articulation disorders

Auditory-perceptual judgements are regarded as the standard method for assessing speech disorders. However, the results of auditory-perceptual evaluations and rater reliability can be affected by various factors, such as concurrent problems in multiple speech subsystems. This study investigated the effect of a co-occurring articulation disorder on auditory-perceptual judgements of hypernasality and the effect of co-occurring hypernasality on judgements of an articulation disorder. The speech stimuli were sentences produced by a male speaker who simulated four levels of hypernasality (typical nasality, and mild, moderate, and severe hypernasality) at four levels of disordered articulation (typical articulation, and mild, moderate, and severe articulation disorder). Thirty speech and language therapy students used visual analogue scales to rate the severity of hypernasality and articulation disorder for each speech sample. Results showed that the hypernasality ratings were significantly higher when articulation disorder co-occurred compared to those without. However, there was no significant difference between mild, moderate and severe concurrent articulation disorder on hypernasality ratings. The speech samples with typical articulation and those with severe articulation disorder were rated as more severe in terms of articulation problem when combined with severe hypernasality. However, there was no significant hypernasality effect on articulation ratings for speech with mild or moderate articulation disorder. The present results generally agreed with previous findings regarding the effect of co-occurring speech problems on auditory-perceptual judgements. Clinicians are advised to be cautious of the potential impact. If possible, speech evaluation using instrumental techniques should be used to supplement auditory-perceptual judgements

Thalamocortical Connectivity Correlates with Phenotypic Variability in Dystonia

Dystonia is a brain disorder characterized by abnormal involuntary movements without defining neuropathological changes. The disease is often inherited as an autosomal-dominant trait with incomplete penetrance. Individuals with dystonia, whether inherited or sporadic, exhibit striking phenotypic variability, with marked differences in the somatic distribution and severity of clinical manifestations. In the current study, we used magnetic resonance diffusion tensor imaging to identify microstructural changes associated with specific limb manifestations. Functional MRI was used to localize specific limb regions within the somatosensory cortex. Microstructural integrity was preserved when assessed in subrolandic white matter regions somatotopically related to the clinically involved limbs, but was reduced in regions linked to clinically uninvolved (asymptomatic) body areas. Clinical manifestations were greatest in subjects with relatively intact microstructure in somatotopically relevant white matter regions. Tractography revealed significant phenotype-related differences in the visualized thalamocortical tracts while corticostriatal and corticospinal pathways did not differ between groups. Cerebellothalamic microstructural abnormalities were also seen in the dystonia subjects, but these changes were associated with genotype, rather than with phenotypic variation. The findings suggest that the thalamocortical motor system is a major determinant of dystonia phenotype. This pathway may represent a novel therapeutic target for individuals with refractory limb dystonia

The visual perception of natural motion: abnormal task-related neural activity in DYT1 dystonia

Although primary dystonia is defined by its characteristic motor manifestations, non-motor signs and symptoms have increasingly been recognized in this disorder. Recent neuroimaging studies have related the motor features of primary dystonia to connectivity changes in cerebello-thalamo-cortical pathways. It is not known, however, whether the non-motor manifestations of the disorder are associated with similar circuit abnormalities. To explore this possibility, we used functional magnetic resonance imaging to study primary dystonia and healthy volunteer subjects while they performed a motion perception task in which elliptical target trajectories were visually tracked on a computer screen. Prior functional magnetic resonance imaging studies of healthy subjects performing this task have revealed selective activation of motor regions during the perception of \u27natural\u27 versus \u27unnatural\u27 motion (defined respectively as trajectories with kinematic properties that either comply with or violate the two-thirds power law of motion). Several regions with significant connectivity changes in primary dystonia were situated in proximity to normal motion perception pathways, suggesting that abnormalities of these circuits may also be present in this disorder. To determine whether activation responses to natural versus unnatural motion in primary dystonia differ from normal, we used functional magnetic resonance imaging to study 10 DYT1 dystonia and 10 healthy control subjects at rest and during the perception of \u27natural\u27 and \u27unnatural\u27 motion. Both groups exhibited significant activation changes across perceptual conditions in the cerebellum, pons, and subthalamic nucleus. The two groups differed, however, in their responses to \u27natural\u27 versus \u27unnatural\u27 motion in these regions. In healthy subjects, regional activation was greater during the perception of natural (versus unnatural) motion (P \u3c 0.05). By contrast, in DYT1 dystonia subjects, activation was relatively greater during the perception of unnatural (versus natural) motion (P \u3c 0.01). To explore the microstructural basis for these functional changes, the regions with significant interaction effects (i.e. those with group differences in activation across perceptual conditions) were used as seeds for tractographic analysis of diffusion tensor imaging scans acquired in the same subjects. Fibre pathways specifically connecting each of the significant functional magnetic resonance imaging clusters to the cerebellum were reconstructed. Of the various reconstructed pathways that were analysed, the ponto-cerebellar projection alone differed between groups, with reduced fibre integrity in dystonia (P \u3c 0.001). In aggregate, the findings suggest that the normal pattern of brain activation in response to motion perception is disrupted in DYT1 dystonia. Thus, it is unlikely that the circuit changes that underlie this disorder are limited to primary sensorimotor pathways

Early onset torsion dystonia (Oppenheim's dystonia)

Early onset torsion dystonia (EOTD) is a rare movement disorder characterized by involuntary, repetitive, sustained muscle contractions or postures involving one or more sites of the body. A US study estimated the prevalence at approximately 1 in 30,000. The estimated prevalence in the general population of Europe seems to be lower, ranging from 1 in 330,000 to 1 in 200,000, although precise numbers are currently not available. The estimated prevalence in the Ashkenazi Jewish population is approximately five to ten times higher, due to a founder mutation. Symptoms of EOTD typically develop first in an arm or leg in middle to late childhood and progress in approximately 30% of patients to other body regions (generalized dystonia) within about five years. Distribution and severity of symptoms vary widely between affected individuals. The majority of cases from various ethnic groups are caused by an autosomal dominantly inherited deletion of 3 bp (GAG) in the DYT1 gene on chromosome 9q34. This gene encodes a protein named torsinA, which is presumed to act as a chaperone protein associated with the endoplasmic reticulum and the nuclear envelope. It may interact with the dopamine transporter and participate in intracellular trafficking, although its precise function within the cell remains to be determined. Molecular genetic diagnostic and genetic counseling is recommended for individuals with age of onset below 26 years, and may also be considered in those with onset after 26 years having a relative with typical early onset dystonia. Treatment options include botulinum toxin injections for focal symptoms, pharmacological therapy such as anticholinergics (most commonly trihexiphenydil) for generalized dystonia and surgical approaches such as deep brain stimulation of the internal globus pallidus or intrathecal baclofen application in severe cases. All patients have normal cognitive function, and despite a high rate of generalization of dystonia, 75% of those patients are able to maintain ambulation and independence, and therefore a comparatively good quality of life, with modern treatment modalities

GBA mutations are associated with Rapid eye movement sleep behavior disorder

Rapid eye movement sleep behavior disorder and GBA mutations are both associated with Parkinson’s disease. The GBA gene was sequenced in idiopathic rapid eye movement sleep behavior disorder patients (n = 265), and compared to controls (n = 2240). Rapid eye movement sleep behavior disorder questionnaire was performed in an independent Parkinson’s disease cohort (n = 120). GBA mutations carriers had an OR of 6.24 (10.2% in patients vs. 1.8% in controls, P < 0.0001) for rapid eye movement sleep behavior disorder, and among Parkinson’s disease patients, the OR for mutation carriers to have probable rapid eye movement sleep behavior disorder was 3.13 (P = 0.039). These results demonstrate that rapid eye movement sleep behavior disorder is associated with GBA mutations, and that combining genetic and prodromal data may assist in identifying individuals susceptible to Parkinson’s disease

High-throughput mutational analysis of TOR1A in primary dystonia

<p>Abstract</p> <p>Background</p> <p>Although the c.904_906delGAG mutation in Exon 5 of <it>TOR1A </it>typically manifests as early-onset generalized dystonia, DYT1 dystonia is genetically and clinically heterogeneous. Recently, another Exon 5 mutation (c.863G>A) has been associated with early-onset generalized dystonia and some ΔGAG mutation carriers present with late-onset focal dystonia. The aim of this study was to identify <it>TOR1A </it>Exon 5 mutations in a large cohort of subjects with mainly non-generalized primary dystonia.</p> <p>Methods</p> <p>High resolution melting (HRM) was used to examine the entire <it>TOR1A </it>Exon 5 coding sequence in 1014 subjects with primary dystonia (422 spasmodic dysphonia, 285 cervical dystonia, 67 blepharospasm, 41 writer's cramp, 16 oromandibular dystonia, 38 other primary focal dystonia, 112 segmental dystonia, 16 multifocal dystonia, and 17 generalized dystonia) and 250 controls (150 neurologically normal and 100 with other movement disorders). Diagnostic sensitivity and specificity were evaluated in an additional 8 subjects with known ΔGAG DYT1 dystonia and 88 subjects with ΔGAG-negative dystonia.</p> <p>Results</p> <p>HRM of <it>TOR1A </it>Exon 5 showed high (100%) diagnostic sensitivity and specificity. HRM was rapid and economical. HRM reliably differentiated the <it>TOR1A </it>ΔGAG and c.863G>A mutations. Melting curves were normal in 250/250 controls and 1012/1014 subjects with primary dystonia. The two subjects with shifted melting curves were found to harbor the classic ΔGAG deletion: 1) a non-Jewish Caucasian female with childhood-onset multifocal dystonia and 2) an Ashkenazi Jewish female with adolescent-onset spasmodic dysphonia.</p> <p>Conclusion</p> <p>First, HRM is an inexpensive, diagnostically sensitive and specific, high-throughput method for mutation discovery. Second, Exon 5 mutations in <it>TOR1A </it>are rarely associated with non-generalized primary dystonia.</p

TorsinA and the TorsinA-Interacting Protein Printor Have No Impact on Endoplasmic Reticulum Stress or Protein Trafficking in Yeast

Early-onset torsion dystonia is a severe, life-long disease that leads to loss of motor control and involuntary muscle contractions. While the molecular etiology of the disease is not fully understood, a mutation in an AAA+ ATPase, torsinA, has been linked to disease onset. Previous work on torsinA has shown that it localizes to the endoplasmic reticulum, where there is evidence that it plays roles in protein trafficking, and potentially also protein folding. Given the high level of evolutionary conservation among proteins involved in these processes, the ability of human such proteins to function effectively in yeast, as well as the previous successes achieved in examining other proteins involved in complex human diseases in yeast, we hypothesized that Saccharomyces cerevisiae might represent a useful model system for studying torsinA function and the effects of its mutants. Since torsinA is proposed to function in protein homeostasis, we tested cells for their ability to respond to various stressors, using a fluorescent reporter to measure the unfolded protein response, as well as their rate of protein secretion. TorsinA did not impact these processes, even after co-expression of its recently identified interacting partner, printor. In light of these findings, we propose that yeast may lack an additional cofactor necessary for torsinA function or proteins required for essential post-translational modifications of torsinA. Alternatively, torsinA may not function in endoplasmic reticulum protein homeostasis. The strains and assays we describe may provide useful tools for identifying and investigating these possibilities and are freely available.Howard Hughes Medical InstituteBachmann-Strauss Dystonia and Parkinson Foundatio