An Overview of Gastrointestinal Dysfunction in Parkinsonian Syndromes.

Gastrointestinal (GI) dysfunction is a common nonmotor symptom in Parkinson's disease (PD) as well as other parkinsonian syndromes and may precede the onset of motor symptoms by decades. Involvement of all segments of the GI tract can lead to altered responses to medications and worsened quality of life for patients. While some GI symptoms occur in isolation, others overlap.

Movement Disorders and the Gut: A Review.

There is a close link between multiple movement disorders and gastrointestinal dysfunction. Gastrointestinal symptoms may precede the development of the neurologic syndrome or may arise following the neurologic presentation. This review will provide an overview of gastrointestinal accompaniments to several well-known as well as lesser known movement disorders.

Surrogates for rigidity and PIGD MDS-UPDRS subscores using wearable sensors.

Background: Telemedicine has the advantage of expanding access to care for patients with Parkinson's Disease (PD). However, rigidity and postural instability in PD are difficult to measure remotely, and are important measures of functional impairment and fall risk.

Research Question: Can measures from wearable sensors be used as future surrogates for the MDS-UPDRS rigidity and Postural Instability and Gait Difficulty (PIGD) subscores?

Methods: Thirty-one individuals with mild to moderate PD wore 3 inertial sensors at home for one week to measure quantity and quality of gait and turning in daily life.

Approach to Posture and Gait in Huntington's Disease.

Disturbances of gait occur in all stages of Huntington's disease (HD) including the premanifest and prodromal stages. Individuals with HD demonstrate the slower speed of gait, shorter stride length, and increased variability of gait parameters as compared to controls; cognitive disturbances in HD often compound these differences. Abnormalities of gait and recurrent falls lead to decreased quality of life for individuals with HD throughout the disease.

Subdural Hematoma as a Serious Complication of Huntington's Disease: An Observational Study.

Background: Persons with Huntington's disease (HD) are at increased risk for subdural hematomas (SDH) because of underlying brain atrophy and increased frequency of falls and head trauma. SDH can cause serious disability, but there is little information about the association of SDH with HD in the medical literature.

Objective: To review the occurrence and characteristics of SDH seen in clinics specializing in HD.

Monocular and binocular low-contrast visual acuity and optical coherence tomography in pediatric multiple sclerosis.

Background: Low-contrast letter acuity and optical coherence tomography (OCT) capture visual dysfunction and axonal loss in adult-onset multiple sclerosis (MS), and have been proposed as secondary outcome metrics for therapeutic trials. Clinical trials will soon be launched in pediatric MS, but such outcome metrics have not been well-validated in this population.

Objectives: To determine whether MS onset during childhood and adolescence is associated with measurable loss of visual acuity and thinning of the retinal nerve fiber layer (RNFL), whether such features are noted only in the context of clinical optic nerve inflammation (optic neuritis, ON) or are a feature of MS even in the absence of optic nerve relapses, and to define the optimal methods for such detection.

Visual pathway axonal loss in benign multiple sclerosis: a longitudinal study.

Background: Benign multiple sclerosis (MS), traditionally defined as Expanded Disability Status Scale (EDSS) score ≤3 and ≥15-year disease duration, is thought to follow a milder clinical course. We determined the extent of visual pathway axonal loss by optical coherence tomography (OCT) retinal nerve fiber layer (RNFL) thickness in a benign MS cohort and examined the relation to vision and quality of life (QOL).

Methods: In this longitudinal study of vision in MS at 3 academic centers, a subset of patients with EDSS, visual function, OCT, and QOL assessments was analyzed.

Cross-sectional evaluation of clinical neuro-ophthalmic abnormalities in an amyotrophic lateral sclerosis population.

Objective: Ocular motility abnormalities may be a marker of neuro-degeneration beyond motor neurons in amyotrophic lateral sclerosis (ALS). We formally compared clinical neuro-ophthalmic abnormalities in ALS patients and a control population.

Methods: Patients attending a multidisciplinary ALS clinic (n=63, age 60.

Retinal ganglion cell layer volumetric assessment by spectral-domain optical coherence tomography in multiple sclerosis: application of a high-precision manual estimation technique.

Background: Neuronal loss in the retina has been demonstrated pathologically in eyes of patients with multiple sclerosis (MS). In vivo, MS eyes have reduced total macular volumes by optical coherence tomography (OCT). Using a high-resolution spectral-domain OCT, this pilot study used a manual method to measure ganglion cell layer (GCL) volumes and to determine the relation of these volumes to visual function in MS eyes.

One eye or two: a comparison of binocular and monocular low-contrast acuity testing in multiple sclerosis.

Purpose: To determine the magnitudes of binocular summation for low- and high-contrast letter acuity in a multiple sclerosis (MS) cohort, and to characterize the roles that MS disease, age, interocular difference in acuity, and a history of optic neuritis have on binocular summation. The relation between binocular summation and monocular acuities and vision-specific quality of life (QoL) was also examined.

Design: Cross-sectional observational study.

Longitudinal study of vision and retinal nerve fiber layer thickness in multiple sclerosis.

Objective: Cross-sectional studies of optical coherence tomography (OCT) show that retinal nerve fiber layer (RNFL) thickness is reduced in multiple sclerosis (MS) and correlates with visual function. We determined how longitudinal changes in RNFL thickness relate to visual loss. We also examined patterns of RNFL thinning over time in MS eyes with and without a prior history of acute optic neuritis (ON).

Differential expression of neuronal ACE2 in transgenic mice with overexpression of the brain renin-angiotensin system.

Angiotensin-converting enzyme 2 (ACE2) is a newly discovered carboxy-peptidase responsible for the formation of vasodilatory peptides such as angiotensin-(1-7). We hypothesized that ACE2 is part of the brain renin-angiotensin system, and its expression is regulated by the other elements of this system. ACE2 immunostaining was performed in transgenic mouse brain sections from neuron-specific enolase-AT(1A) (overexpressing AT(1A) receptors), R(+)A(+) (overexpressing angiotensinogen and renin), and control (nontransgenic littermates) mice.