Relationship between cardiac parasympathetic dysfunction and the anteroposterior diameter of the medulla oblongata in multiple system atrophy.

PURPOSE: Neurodegeneration of the nucleus ambiguus and the dorsal vagal motor nucleus has been implicated in cardiac parasympathetic dysfunction in multiple system atrophy (MSA). The nucleus ambiguus and the dorsal vagal motor nucleus, which are located in the medulla oblongata (MO), control the autonomic-specifically, the parasympathetic-functions of the body. The aim of our study was to investigate the relationship between cardiac parasympathetic dysfunction and the anteroposterior diameter of the MO in MSA by quantitatively analyzing magnetic resonance imaging (MRI) outcome measures. METHODS: We retrospectively assessed 40 consecutive patients with probable MSA and 25 age- and sex-matched controls. The anteroposterior diameter of the MO at two locations (MO diameter-A and -B) and the diameters of the midbrain and pons were measured by conventional MRI. A cardiac parasympathetic function score (CP-score) and cardiac sympathetic function score (CS-score) were generated by calculating the z-scores of multiple autonomic function tests. The relationship between the scores and the measured diameters of the brainstem was also investigated. RESULTS: The CP-score and CS-score were significantly lower in the patients with MSA than in the controls (CP-score: 0.61 ± 0.75 vs. - 0.38 ± 0.52, p < 0.001; CS-score: 0.91 ± 1.06 vs. - 0.57 ± 1.07, p < 0.001). Also, in the patients with MSA, the CP-score was significantly correlated with MO diameter-A (r = 0.40, p = 0.010), and the CS-score was significantly correlated with the diameter of the midbrain (r = 0.33, p = 0.038). CONCLUSION: The anteroposterior diameter of the MO is a potential imaging marker of parasympathetic dysfunction in MSA.

[Anesthetic management of a patient with Maffucci syndrome for removal of a pituitary adenoma].

Maffucci syndrome is a rare syndrome characterized by multiple enchondromas and hemangiomas seen in various tissues and organs. We report anesthetic management of a 33-year-old man with multiple hemangiomas due to Maffucci syndrome undergoing removal of a pituitary adenoma. The preoperative examination revealed multiple hemangiomas in the head, neck, right upper and lower extremities, the tongue and the pharynx. In the operating room, we observed the locations of hemangiomas in the tongue and pharynx in detail using a fiberoptic bronchoscope before induction of anesthesia. Since there was a risk of rupture of a large hemangioma by using a laryngoscope, we planned fiberoptic bronchoscope-guided tracheal intubation without using a laryngoscope. After intravenous administration of propofol and rocuronium, a fiberoptic bronchoscope was orally introduced into the trachea and then the trachea was intubated with a flexible spiral endotracheal tube, preventing contact of the fiber or endotracheal tube with the hemangiomas. In addition, hemangiomas on the body surface were wrapped up with soft dressing to prevent rupture. Surgery was uneventfully completed. The trachea was carefully extubated, and bleeding from hemangiomas was not observed. Since patients with Maffucci syndrome have multiple hemangiomas, it is important to check for the presence of a hemangioma in the upper airway in anesthetic management.

A nerve conduction study predicts the prognosis of sporadic amyotrophic lateral sclerosis.

OBJECTIVE: To clarify the relationship between nerve conduction study (NCS) and prognosis in patients with amyotrophic lateral sclerosis (ALS). METHODS: We included 190 patients with sporadic ALS. We used onset age, sex, onset site (bulbar vs. spinal), revised El Escorial criteria category (definite vs. others), and the King's clinical systems, and the Milano-Torino (MiToS) functional staging systems, and decline rates of revised ALS functional rating scale (ALSFRS-R) as known prognostic factors. An NCS was performed on the median, ulnar, tibial, and sural nerves. The endpoint was death or the introduction of tracheostomy positive-pressure ventilation. Multivariate analysis for each NCS variable, known prognostic factors was performed using Cox stepwise proportional hazards analysis. Univariate analysis was performed for NCS variables that showed a significant association with prognosis in multivariate analysis. Survival was analyzed with a Kaplan-Meier curve and log-rank test. RESULTS: The Cox model identified the compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) amplitudes of the median nerve as prognostic factors. In the log-rank test, patients with higher median nerve CMAP amplitude had a significantly better prognosis than those with lower amplitude, regardless of age. And prognosis was better in the group with lower median nerve SNAP amplitude only in patients younger than the 25th percentile (~ 57 years). CONCLUSIONS: CMAP and SNAP amplitudes of the median nerve are considered to be independent prognostic factors of sporadic ALS.

Association of orthostatic blood pressure with the symptoms of orthostatic hypotension and cognitive impairment in patients with multiple system atrophy.

The degree and frequency of orthostatic hypotension (OH) are high in patients with multiple system atrophy (MSA); however, the association of orthostatic blood pressure (BP) with the symptoms of OH and cognitive impairment in these patients remains unclear. The aim of this study was to clarify whether absolute BP and/or changes in BP during standing are related to OH symptoms and cognitive impairment in patients with MSA. Thirty-two patients with MSA were examined using the head-up tilt and cognitive function tests. OH symptoms were evaluated using a patient-reported scale. The results were compared with those for 15 age- and sex-matched healthy controls. Seventeen of the 32 (53.1%) patients had OH, with eight of them exhibiting OH symptoms, which were related to the absolute BP value at 60° tilt. However, OH symptoms were not related to the degree of decrease in BP during the tilt test, and they were frequently observed in patients with a mean BP of <80 mmHg at 60° tilt (sensitivity, 67%; specificity, 91%). Cognitive dysfunction assessed by the Mini-Mental State Examination (MMSE; ≤ 26) was also associated with a low mean BP at 60° tilt (odds ratio, 1.32; 95% confidence interval, 1.04-1.67; p = 0.02). The upright BP value is associated with OH symptoms and the MMSE score in patients with MSA. Thus, careful observation of OH symptoms can enable early management of BP and the detection of cognitive impairment in these patients.

Difficulty with cuff deflation of reinforced tracheal tube caused by inflation line occlusion with silk thread ligation and fixation.

A reinforced tracheal tube, ligated with silk threads, was inserted into a tracheostomy orifice and fixed to the skin. The cuff inflation line of the reinforced tracheal tube became occluded. Reinforced 'armoured' tracheal tubes have a spiral of wire embedded into the wall of the tube to give strength and flexibility, and may be sharply bent without compromising the tube lumen. The tracheal cuff attached to the tube is inflated by injecting air through a narrow-diameter tube welded to the outside of the tracheal tube. When a reinforced tracheal tube is ligated and fixed with silk threads, it should be confirmed whether the tracheal tube cuff can be deflated and inflated after fixation. Moreover, because occlusion can be eliminated by removing all silk threads used to ligate a tracheal tube, they should be removed before extubation.