Co-administration of ephedrine prevents reductions in cardiac output and systemic oxygen delivery secondary to lung compression maneuvers during one-lung ventilation, without reducing arterial oxygenation.

PURPOSE: We previously showed that compression of the nondependent lung during one-lung ventilation (OLV) in patients undergoing esophagectomy improves arterial oxygenation but impairs cardiac output (CO) and systemic oxygen delivery (DO(2)). The objective of this study was to test the hypothesis that the combination of nondependent lung compression and ephedrine improves arterial oxygenation without compromising DO(2). METHODS: Twenty patients undergoing esophagectomy through a right thoracotomy were studied. Under general anesthesia, a left-sided double-lumen tube was placed, and the dependent lung was mechanically ventilated with a tidal volume of 8 ml/kg and a fraction of inspiratory oxygen of 0.8 during OLV. When nondependent lung was compressed by surgeons to improve surgical exposure, a randomly determined intravenous bolus of either ephedrine 4 mg (group E) or an identical volume of saline (group S) was administered. Arterial blood was sampled during two-lung ventilation (TLV), at 10 min of OLV (OLV1), and 5 min after nondependent lung compression (OLV2). RESULTS: The initiation of OLV resulted in a significant drop in PaO(2) at OLV1 (group E, 136 ± 69 mmHg; group S, 138 ± 83 mmHg; P < 0.01) compared with TLV (group E, 404 ± 44 mmHg; group S; 367 ± 51 mmHg) and tended to improve at OLV2 (group E, 170 ± 63 mmHg; group S; 196 ± 121 mmHg). However, although CO and DO(2) significantly decreased in group S at OLV2 (4.0 ± 0.8 l/min, 621 ± 116 ml/min; P < 0.01) compared with OLV1 (5.1 ± 0.7 l/min, 811 ± 140 ml/min), there was no significant difference in these parameters in group E for the two time points. CONCLUSION: Although arterial oxygenation was not significantly improved by the nondependent lung compression, the addition of intravenous ephedrine to nondependent lung compression prevented the decrease in systemic oxygen delivery without deterioration of arterial oxygenation during OLV in patients undergoing esophagectomy.

[A case of Bickerstaff brainstem encephalitis concomitant with axonal Guillain-Barre syndrome and ballism successfully treated with intravenous immunoglobulin treatment].

A 39-year-old man acutely developed diplopia, vertigo, unsteady gait, and disturbance of consciousness following an upper respiratory infection. Neurological examination showed ophthalmoplegia, facial paralysis, tetraplegia and loss of deep tendon reflexes. Babinski reflex was positive on the left and there were bilateral flexor withdrawal reflexes. He also developed ballism-like involuntary movements in all extremities, loss of proprioception predominantly on the left, and severe truncal ataxia. Anti-GQ1b IgG antibody was selectively elevated in serum, and CSF protein was elevated to 53 mg/dl with cell count of 12/mm3. Nerve conduction study showed decreased amplitude of compound motor action potentials in all extremities, and no response in facial muscles. Cranial MRI showed no abnormalities whereas EEG was severely abnormal with lack of posterior dominant rhythm and the presence of continuous diffuse theta-waves. This case presented clinical characteristics of three syndromes concurrently-Fisher syndrome, Bickerstaff brainstem encephalitis, and Guillain-Barré syndrome-that may be collectively called 'anti-GQ1b IgG antibody syndrome'. The unique feature of the present case was development of deep coma and ballism-like movements, associated with selective increase of serum anti-GQ1b IgG antibody. It is thus conceivable that anti-GQ1b IgG antibody might underlie the pathogenesis of all three conditions.

[A case presenting with both features of essential tremor and Parkinson tremor].

A 78-year-old woman had postural and action tremor in hands since age of late 20's, though without much difficulty in daily living. Since age 76, she has had an increasing difficulty in walking and postural balance, with some worsening of hand shaking as well. She also noted to have head shaking, involuntary movements in the mouth, and some difficulty in swallowing. Reportedly both of her parents and her brother had hand tremor. Neurologically she had resting, postural and action tremors in hands more on the left. She also had marked rigidity in the neck and moderate cogwheel rigidity in all limbs more on the left, moderate bradykinesia, markedly stooped posture with relatively wide base, slow and small-paced gait, and poor postural balance more posteriorly. Thus, it is most likely that the present case suffered from essential tremor and later developed Parkinson disease. Power spectrum analyses of surface electromyogram and accelerometer showed the peaks at 4.3 Hz for the resting tremor, and 3.1 and 5.2 Hz for the postural tremor. Furthermore, significant EEG-EMG coherence was seen at the peaks of 4.3 and 5.2 Hz, suggesting possible involvement of sensori-motor cortex for generation of both tremors. When the postural tremor was loaded with 500 g weight, the peak of power spectrum and EEG-EMG coherence showed the broader pattern with the maximal peak at 4.3 Hz, exactly the same frequency as that of the resting tremor. These findings may be explained by postulating that the 4.3 Hz peak of the resting tremor became apparent by loading because 5.2 Hz peak of the postural tremor was suppressed by the loading. The peak of the postural tremor at 3.1 Hz which was not detected by EEG-EMG coherence suggested that the tremor for 3.1 Hz was not involved to sensori-motor cortex for generation.

[Concurrence of myasthenia gravis, polymyositis, thyroiditis and eosinophilia in a patient with type B1 thymoma].

We presented a 43-year-old Japanese woman who acutely developed weakness of all extremities and difficulty in swallowing and drooping of eyelids, characterized by easy fatigability at the end of December, 2005. On general physical examination, she had moderate goiter. No cervical lymphadenopathy, cardiac murmur, or skin rash was noted. Neurologically, she had blepharoptosis, more on the right, only in the upright position with easy fatigability and marked weakness in the neck flexor, trunk, and all limb muscles much more proximally than distally. She had neither muscular atrophy nor upper motor neuron sign. Laboratory data showed slight leukocytosis with eosinophilia (up to 31%), and serum creatine kinase was markedly increased to over 2,000 IU/l. TSH receptor antibody (11.9%) and anti-acetylcholine receptor antibody (46.6 nmol/L) were also increased. Edrophonium test was positive. Electrophysiologically, muscle evoked potentials by repetitive motor nerve stimulation showed 13% and 50% waning in abductor pollicis brevis and deltoid muscle, respectively, at low frequency and no waxing at high frequency. Needle EMG showed fibrillation potentials and positive sharp waves in proximal muscles. Polymyositis was diagnosed by muscle biopsy which showed infiltration of lymphocytes in the endomysium and around non-necrotic muscle fibers. Upper arm muscle MRI showed multifocal high signal intensity lesions on T2-weighted images which were likely related to myositis. This finding is atypical for polymyositis. X-ray and CT of chest showed a mass lesion in the left pulmonary hilum, which was histologically diagnosed as type B1 thymoma. Thus, the present case had myasthenia gravis, polymyositis, thyroidititis and eosinophilia associated with type B1 thymoma. After the thymectomy, corticosteroid administration and immunoadsorption therapy, clinical symptoms and all laboratory abnormalities markedly improved.

[Case presenting both clinical features of progressive supranuclear palsy and amyotrophic lateral sclerosis].

A 70-year-old Japanese male farmer, born and living in Kyoto prefecture, developed gait disturbance, with tendency to fall at age 68, and muscle atrophy and weakness of the right shoulder and arm a year and half later. All symptoms have been progressive ever since. The patient manifested marked dementia, parkinsonism associated with limitation of ocular movements in all directions with slow saccade, loss of startle reflex, asymmetric muscle atrophy and weakness in shoulder girdles and upper limbs with hyperreflexia, and positive Babinski reflexes. The needle electromyogram showed evidence of active denervation. Brain MRI showed cortical atrophy in the frontal and temporal lobes, and midbrain tegmentum. Cerebral blood flow image on SPECT suggested hypoperfusion in the frontal, temporal and parietal cortices and basal ganglia bilaterally. Thus, it is most likely that the present case suffered from clinical features of amyotrophic lateral sclerosis (ALS) and progressive supranuclear palsy at the same time. Relation to the ALS/Parkinsonism/Dementia complex reported from Kii peninsula and Guam was discussed.

Gluten sensitivity in Japanese patients with adult-onset cerebellar ataxia.

OBJECTIVE: Gluten sensitivity is associated with multiple neurological abnormalities including gluten ataxia, motor neuron disease-like neuropathy, small fiber type neuropathy, cognitive impairment, and even parkinsonism. We investigated whether or not gluten sensitivity is involved in Japanese patients with idiopathic cerebellar ataxia with extracerebellar presentation. PATIENTS OR MATERIALS: Fourteen patients with idiopathic cerebellar ataxia with extracerebellar presentation (autonomic instability, parkinsonism, or pyramidal dysfunction in varying combinations) were screened for anti-gliadin antibodies (AGA) to analyze for the presence or absence of gluten sensitivity. Patients with typical MR findings of multiple system atrophy of the cerebellar type were excluded. As disease controls without cerebellar ataxia, 9 patients with Parkinson's disease and 18 patients with amyotrophic lateral sclerosis were screened for AGA. Forty-seven normal controls were also screened for AGA. RESULTS: We found a high prevalence of AGA in 5 (36%) of 14 cerebellar ataxia patients, but in only 1 (4%) of 27 disease controls without cerebellar ataxia (odds ratio, 14.4; 95% CI, 1.41147; p<0.05) and in only 1 (2%) of 47 normal controls (odds ratio, 25.6; 95% CI, 2.66246; p<0.001). Among the cerebellar ataxia patients, atypical features such as sensorimotor neuropathy and/or mild cognitive impairment were more prevalent in the AGA-positive group (60%) than in the AGA-negative group (0%). In one of the ataxic patients with AGA, a gluten-free diet had positive effects on neurological symptoms and nutritional status. CONCLUSION: Gluten sensitivity is involved in at least some of the unexplained neurological symptoms of Japanese patients with adult-onset, sporadic cerebellar ataxia.