The Neural Correlates of Spatial Disorientation in Head Direction Cells.

While the brain has evolved robust mechanisms to counter spatial disorientation, their neural underpinnings remain unknown. To explore these underpinnings, we monitored the activity of anterodorsal thalamic head direction (HD) cells in rats while they underwent unidirectional or bidirectional rotation at different speeds and under different conditions (light vs dark, freely-moving vs head-fixed). Under conditions that promoted disorientation, HD cells did not become quiescent but continued to fire, although their firing was no longer direction specific. Peak firing rates, burst frequency, and directionality all decreased linearly with rotation speed, consistent with previous experiments where rats were inverted or climbed walls/ceilings in zero gravity. However, access to visual landmarks spared the stability of preferred firing directions (PFDs), indicating that visual landmarks provide a stabilizing signal to the HD system while vestibular input likely maintains direction-specific firing. In addition, we found evidence that the HD system underestimated angular velocity at the beginning of head-fixed rotations, consistent with the finding that humans often underestimate rotations. When head-fixed rotations in the dark were terminated HD cells fired in bursts that matched the frequency of rotation. This postrotational bursting shared several striking similarities with postrotational "nystagmus" in the vestibulo-ocular system, consistent with the interpretation that the HD system receives input from a vestibular velocity storage mechanism that works to reduce spatial disorientation following rotation. Thus, the brain overcomes spatial disorientation through multisensory integration of different motor-sensory inputs.

The Role of Electrodiagnostic Testing, Imaging, and Muscle Biopsy in the Investigation of Muscle Disease.

PURPOSE OF REVIEW: This article reviews the roles of electrodiagnostic testing, imaging studies (MRI and ultrasound), and muscle biopsy in evaluating patients for possible muscle diseases. RECENT FINDINGS: In addition to electrodiagnostic testing and muscle biopsy, muscle imaging is increasingly being used in the evaluation of patients with suspected muscle disease. MRI and ultrasound can help identify patterns of muscle involvement that may narrow the differential diagnosis and guide further testing. In addition, imaging can identify potential targets for muscle biopsy and can help evaluate for and exclude certain conditions that may mimic muscle disease. SUMMARY: This article provides a comprehensive overview of various testing modalities used in the evaluation of patients with suspected muscle disease, including electrodiagnostic studies, muscle imaging, and biopsy. In combination with a thorough history and clinical examination, these modalities can help narrow the differential diagnosis or, in certain cases, can confirm a specific etiology of muscle disease.

Infant botulism: review and clinical update.

Botulism is a rare neuromuscular condition, and multiple clinical forms are recognized. Infant botulism was first identified in the 1970s, and it typically occurs in infants younger than 1 year of age who ingest Clostridium botulinum spores. A specific treatment for infant botulism, intravenous botulism immunoglobulin (BIG-IV or BabyBIG®), was developed in 2003, and this treatment has substantially decreased both morbidity and hospital costs associated with this illness. This article will review the pathogenesis of infant botulism as well as the epidemiology, clinical manifestations, diagnosis, and treatment of this condition.

A volumetric MRI study of limbic, associative and sensorimotor striatal subregions in schizophrenia.

INTRODUCTION: Cognitive and emotional functioning is mediated by frontal-subcortical feedback loops. The striatum, a component of this circuitry, thus is a possible neural substrate of schizophrenia. Striatum volume, however, is believed to be differentially influenced by neuroleptic treatment due to an anterior-posterior D2 receptor density gradient. We thus rigorously parcellated it into subregions in order to assess whether neuroleptic effect on group differences is regionally specific. METHODS: 29 chronic, male, schizophrenia patients and 28 male, normal controls (NCs), group-matched for handedness, age, and parental SES, underwent structural brain imaging on a 1.5 Tesla GE system. We manually measured the volume, normalized for intracranial contents, of the striatum parcellated into anatomic subregions and their corresponding limbic, associative and sensorimotor functional subregions and performed clinical correlations. RESULTS: First, we found a localized bilateral enlargement of the posterior putamen in medicated chronic schizophrenia. Second, we showed associative striatal subregion volumes correlated with executive function in schizophrenia subjects and, to a lesser extent, in NCs. Third, we showed associative striatal subregions inversely correlated with negative symptoms but conversely, the ventral/limbic striatum did not correlate with positive or negative clinical symptoms. DISCUSSION: Our novel parcellation strategy, based on rigorous delineation of the ventral striatum, allowed for the demonstration of localized volumetric differences between schizophrenia and NCs. Furthermore, by parcellating the striatum into functional subregions we demonstrated significant positive correlations between the volume of the associative striatum and executive functioning in schizophrenia, adding further support to the importance of its role in the pathophysiology of schizophrenia.