Magnetic Resonance-Guided Focused Ultrasound without Anesthesiologist Support.

INTRODUCTION: Magnetic resonance-guided focused ultrasound (MRgFUS) is an effective treatment option for essential tremor (ET) and tremor dominant Parkinson's disease (TDPD), which is often performed with sedation or in the presence of an anesthesiologist in an effort to minimize adverse events and maximize patient comfort. This study explores the safety, feasibility, and tolerability of performing MRgFUS without an anesthesiologist. METHODS: This is a single academic center, retrospective review of 180 ET and TDPD patients who underwent MRgFUS treatment without anesthesiologist support. Patient demographics, intra-procedural treatment parameters, peri-procedural adverse events, and 3-month Clinical Rating Scale for Tremor Part B (CRST-B) scores were compared to MRgFUS studies that utilized varying degrees of anesthesia. RESULTS: There were no anesthesia related adverse events or unsuccessful treatments. There were no early treatment terminations due to patient discomfort, regardless of skull density ratio. 94.6% of patients would repeat the procedure again. The most common side effects during treatment were facial/tongue paresthesia (26.3%), followed by nausea (22.3%), dysarthria (8.6%), and scalp pain (8.0%). No anxiolytic, pain, or antihypertensive medications were administered. The most common early adverse event after MRgFUS procedure was gait imbalance (58.3%). There was a significant reduction of 83.1% (83.4% ET and 80.5% TDPD) of the mean CRST-B scores of the treated hand when comparing 3-month and baseline scores (1.8 vs. 10.9, n = 109, p < 0.0001). CONCLUSION: MRgFUS without intra-procedural anesthesiologist support is a safe, feasible, and well-tolerated option, without an increase in peri-procedural adverse events.

Increased plasmin-mediated proteolysis of L1CAM in a mouse model of idiopathic normal pressure hydrocephalus.

Idiopathic normal pressure hydrocephalus (iNPH) is a common neurological disorder that is characterized by enlarged cerebral ventricles, gait difficulty, incontinence, and dementia. iNPH usually develops after the sixth decade of life in previously asymptomatic individuals. We recently reported that loss-of-function deletions in CWH43 lead to the development of iNPH in a subgroup of patients, but how this occurs is poorly understood. Here, we show that deletions in CWH43 decrease expression of the cell adhesion molecule, L1CAM, in the brains of CWH43 mutant mice and in human HeLa cells harboring a CWH43 deletion. Loss-of-function mutations in L1CAM are a common cause of severe neurodevelopmental defects that include congenital X-linked hydrocephalus. Mechanistically, we find that CWH43 deletion leads to decreased N-glycosylation of L1CAM, decreased association of L1CAM with cell membrane lipid microdomains, increased L1CAM cleavage by plasmin, and increased shedding of cleaved L1CAM in the cerebrospinal fluid. CWH43 deletion also decreased L1CAM nuclear translocation, suggesting decreased L1CAM intracellular signaling. Importantly, the increase in L1CAM cleavage occurred primarily in the ventricular and subventricular zones where brain CWH43 is most highly expressed. Thus, CWH43 deletions may contribute to adult-onset iNPH by selectively downregulating L1CAM in the ventricular and subventricular zone.

Evidence for increased intraabdominal pressure as a cause of recurrent migration of the distal catheter of a ventriculoperitoneal shunt: illustrative case.

BACKGROUND: Placement of a ventriculoperitoneal (VP) shunt is an effective treatment for several disorders of cerebrospinal fluid flow. A rare complication involves postoperative migration of the distal catheter out of the intraperitoneal compartment and into the subcutaneous space. Several theories attempt to explain this phenomenon, but the mechanism remains unclear. OBSERVATIONS: The authors report the case of a 37-year-old nonobese woman who underwent placement of a VP shunt for idiopathic intracranial hypertension. Postoperatively, the distal catheter of the VP shunt migrated into the subcutaneous space on three occasions despite the use of multiple surgical techniques, including open and laparoscopic methods of abdominal catheter placement. Notably, the patient repeatedly displayed radiographic evidence of chronic bowel distention consistent with increased intraperitoneal pressure. LESSONS: In this case, the mechanism of catheter migration into the subcutaneous space did not appear to be caused by pulling of the catheter from above but rather by expulsion of the catheter from the peritoneum. Space in the subcutaneous tissues caused by open surgical placement of the catheter was permissive for this process. Patients with chronic increased intraabdominal pressure, such as that caused by bowel distention, obesity, or Valsalva maneuvers, may be at increased risk for distal catheter migration.

Idiopathic Normal Pressure Hydrocephalus With Stuttering: Report of Two Cases and Review of the Literature.

BACKGROUND: Idiopathic normal pressure hydrocephalus (iNPH) is a disorder of aging that is characterized by enlarged cerebral ventricles, gait apraxia, dementia, and urinary incontinence. iNPH is frequently misdiagnosed, in part because the symptoms resemble other neurological disorders, and because other associated symptoms have not been fully characterized. Importantly, iNPH has not previously been associated with stuttering, and shunting has not been shown to alleviate the symptom of stuttering. CASE DESCRIPTIONS: Here, we report 2 cases of patients with iNPH presenting with stuttering that resolved after ventriculoperitoneal (VP) shunt placement. Each patient presented with gait difficulty, incontinence, cognitive impairment, and stuttering. Lasting improvements of the symptoms (including stuttering) were seen in both patients after cerebrospinal fluid (CSF) drainage procedures that included lumbar puncture, extended lumbar CSF drainage, placement of a VP shunt, and VP shunt revision. CONCLUSIONS: These findings suggest that iNPH can present with stuttering or dysarthria. The significant improvement in stuttering and dysarthria, along with the improvements in gait difficulty, incontinence, and cognitive impairment that occurred after CSF drainage, suggests that the motor apraxia observed in iNPH can affect speech production. Practitioners should be aware that iNPH can present with stuttering, and that CSF drainage can improve stuttering in select circumstances.

Branch-specific plasticity of a bifunctional dopamine circuit encodes protein hunger.

Free-living animals must not only regulate the amount of food they consume but also choose which types of food to ingest. The shifting of food preference driven by nutrient-specific hunger can be essential for survival, yet little is known about the underlying mechanisms. We identified a dopamine circuit that encodes protein-specific hunger in Drosophila The activity of these neurons increased after substantial protein deprivation. Activation of this circuit simultaneously promoted protein intake and restricted sugar consumption, via signaling to distinct downstream neurons. Protein starvation triggered branch-specific plastic changes in these dopaminergic neurons, thus enabling sustained protein consumption. These studies reveal a crucial circuit mechanism by which animals adjust their dietary strategy to maintain protein homeostasis.