Patient Selection for Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration.

Clinical Vignette: A 23-year-old woman with pantothenate kinase-associated neurodegeneration (PKAN) presented with medication-refractory generalized dystonia and an associated gait impairment.

Clinical Dilemma: Bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) can be an effective treatment for dystonia. However, outcomes for PKAN DBS have been variable and there are no standardized criteria for patient selection.

Anticholinergic Medication Burden and Cognitive Subtypes in Parkinson's Disease without Dementia.

Objective: Cognitive changes are heterogeneous in Parkinson's disease (PD). This study compared whether anticholinergic burden drives differences in cognitive domain performance and empirically-derived PD-cognitive phenotypes.

Method: A retrospective chart review contained participants (n = 493) who had idiopathic PD without dementia.

Identification and Management of Persistent Stimulation-Induced Dyskinesia Associated with STN DBS: The See-Saw Dilemma.

Clinical Vignette: A 73-year-old woman with Parkinson's disease (PD) underwent implantation of bilateral subthalamic nucleus deep brain stimulators (STN-DBS) to address bilateral upper extremity medication-refractory tremor. Post-operatively, she experienced a "see-saw effect" where small increases in stimulation resulted in improvement in one symptom (tremor) with concurrent worsening in another (dyskinesia).

Clinical Dilemma: SID is usually considered a positive predictor of DBS outcome.

Illusory Responses across the Lewy Body Disease Spectrum.

Objective: This study was undertaken to study pareidolias, or perceived meaningful objects in a meaningless stimulus, in patients across the Lewy body (LB) disease spectrum, where most do not report hallucinations or delusions.

Methods: We studied illusory responses on the Noise Pareidolia Task in 300 participants (38 cognitively impaired LB, 65 cognitively unimpaired LB, 51 Alzheimer disease spectrum [AD-s], 146 controls). Pairwise between-group comparisons examined how diagnosis impacts the number of illusory responses.

Surgical Therapies for Parkinson Disease.

Purpose Of Review: Parkinson disease (PD) is a progressive neurodegenerative disorder that is often difficult to manage with medications alone. This article reviews the current therapeutic surgical interventions for PD, patient selection criteria, timing of patient referral to surgical services, procedure overview, and future directions.

Recent Findings: Adaptive, or closed-loop, deep brain stimulation is a promising therapy that can detect ongoing circuit changes and deliver appropriate stimulation based on the patient's dominant symptom and level of dopaminergic medication.

The chromatin repressors EZH2 and Suv4-20h coregulate cell fate specification during hippocampal development.

The cell fate transition from radial glial-like (RGL) cells to neurons and astrocytes is crucial for development and pathological conditions. Two chromatin repressors-the enhancer of zeste homolog 2 and suppressor of variegation 4-20 homolog-are expressed in RGL cells in the hippocampus, implicating these epigenetic regulators in hippocampal cell fate commitment. Using a double knockout mouse model, we demonstrated that loss of both chromatin repressors in the RGL population leads to deficits in hippocampal development.

Rare Etiology of Bow Hunter's Syndrome and Systematic Review of Literature.

Background: Bow Hunter's Syndrome is a mechanical occlusion of the vertebral artery which leads to a reduction in blood flow in posterior cerebral circulation resulting in transient reversible symptomatic vertebrobasilar insufficiency.

Case Description: We present a case of Bow Hunter's syndrome in a 53-year-old male that occurred after the patient underwent surgical correction of a proximal left subclavian artery aneurysm. Shortly after the surgery, the patient began to complain of transient visual changes, presyncopal spells, and dizziness upon turning his head to the left.

Repeated administrations of human umbilical cord blood cells improve disease outcomes in a mouse model of Sanfilippo syndrome type III B.

Sanfilippo syndrome type III B (MPS III B) is an inherited disorder characterized by a deficiency of α-N-acetylglucosaminidase (Naglu) enzyme leading to accumulation of heparan sulfate in lysosomes and severe neurological deficits. We have previously shown that a single administration of human umbilical cord mononuclear cells (hUCB MNCs) into Naglu knockout mice decreased behavioral abnormalities and tissue pathology. In this study, we tested whether repeated doses of hUCB MNCs would be more beneficial than a single dose of cells.

Blood-brain barrier impairment in an animal model of MPS III B.

Background: Sanfilippo syndrome type B (MPS III B) is caused by a deficiency of α-N-acetylglucosaminidase enzyme, leading to accumulation of heparan sulfate within lysosomes and eventual progressive cerebral and systemic multiple organ abnormalities. However, little is known about the competence of the blood-brain barrier (BBB) in MPS III B. BBB dysfunction in this devastating disorder could contribute to neuropathological disease manifestations.