1/4. Unusual association of sporadic olivopontocerebellar atrophy and motor neuron disease.Sporadic olivopontocerebellar atrophy (OPCA) is a neurodegenerative disorder that presents a wide clinical spectrum. motor neuron disease (MND) is characterized by a selective degeneration of motor neurons. A 60-year-old man developed slurred speech and unsteadiness of gait. He had also noticed difficulty in holding his head upright and shoulder weakness. The disease had a rapid progression. At the age of 63 years, magnetic resonance imaging supported a diagnosis of OPCA, and a diagnosis of MND was suggested by clinical and electrophysiological findings. He also had upward gaze palsy. A muscular biopsy showed sporadic ragged red and Cox deficient fibers. The present case could define a unique disorder, as the occasional occurrence of two degenerative disorders appears unlikely.- - - - - - - - - - ranking = 1keywords = palsy (Clic here for more details about this article) |
2/4. multiple system atrophy with retinal degeneration in a young child.A 4-year-old girl with multiple system degeneration and retinal degeneration was presented. There was onset of an ataxic gait at two years and rapid progression of retinal degeneration, myoclonus and cranial nerve palsy. Neuropathological examination revealed severe degeneration of the cerebellar cortex and the pathways of auditory and deep sensation, as well as degeneration of the cerebellar efferent fibers, the striatonigral system, the cerebellar afferent fiber system and lower motor neurons. Cases of young children with spinocerebellar degeneration have been reported in several families of olivopontocerebellar atrophy (OPCA), but degenerative changes in our case were more widespread than those in OPCA cases. The multiple system lesions in the central nervous system and retina of this child are different from those of any other previously reported cases.- - - - - - - - - - ranking = 1keywords = palsy (Clic here for more details about this article) |
3/4. sleep-related breathing disorders in patients with multiple system atrophy and vocal fold palsy.We performed sleep studies in eight patients with multiple system atrophy (MSA) and three patients with peripheral bilateral vocal fold palsy (PBVFP) and investigated stenosis of the upper airway tract during sleep in MSA patients with vocal fold palsy. Among the eight MSA patients in this study, five had definite glottic snoring and two others were suspected of having glottic snoring. Of the PBVFP patients, two had glottic snoring. Three of 11 patients died, and two of the three deaths occurred during sleep. Glottic snoring indicated a high degree of negative esophageal pressure. High negative esophageal pressure demonstrates severe narrowing of the upper airway tract. Therefore, glottic snoring should be considered a risk factor for sudden death in sleep. Repeated laryngoscopic examination is useful in evaluating the progressive process of vocal fold palsy while awake, but this examination performed only while awake is not enough to evaluate narrowing of the upper airway during sleep. sleep studies that include the measurement of esophageal pressure can be very useful in evaluating the severity of narrowing in the upper airway tract. It is suspected that sudden nocturnal death in MSA patients is caused not only by abnormal respiration resulting from impairment of the respiratory center, but also by glottic obstruction caused by sputum or by edema of the vocal folds. We recommend treatment of respiratory disorders when loud laryngeal snoring occurs in patients with MSA, even if they do not complain of dyspnea while awake.- - - - - - - - - - ranking = 7keywords = palsy (Clic here for more details about this article) |
4/4. Vecuronium dose requirement and pupillary response in a patient with olivopontocerebellar atrophy (OPCA).PURPOSE: Olivopontocerebellar atrophy (OPCA), a variant of spinocerebellar degeneration (shy-drager syndrome), is a systemic degenerative disorder affecting the neurons of multiple nuclei. We investigated the sensitivity to vecuronium and the pupillary responses to various stresses in a patient with OPCA. CLINICAL FEATURES: A 65-yr-old woman with a six-month history of OPCA underwent a left upper lobectomy for lung cancer under propofol-N2O anaesthesia. She had symptoms of dysarthria, bulbar palsy, cerebellar ataxia, Parkinsonism, myosis, pyramidal signs and muscular atrophy of the distal extremities. A cumulative dose-response curve for vecuronium was constructed, and pupillary changes in response to various noxious stimuli were evaluated with concomitant recording of the Spectral-Edge-Frequency 90% (SEF90; the frequency below which 90 percent of the EEG power is located). The dose-response curve for vecuronium and the estimated ED50 value (the 50% blocking dose of vecuronium) in this patient with OPCA were almost identical with those of five ASA I-II patients (27 micrograms.kg-1 vs 31 micrograms.kg-1). The pupil size and the SEF90 did not change after tracheal intubation or surgical stimulation in this patient, while in the control subjects (n = 3), these measures increased in response to both stresses. CONCLUSIONS: The absence of pupillary and SEF90 responses to noxious stimuli suggests a sensitivity to propofol and/or central autonomic dysfunction in patients with OPCA. Although the dose requirement of vecuronium in this patient was similar to that of the control patients, the effects of neuromuscular blockers may vary depending on the severity of muscle atrophy.- - - - - - - - - - ranking = 1keywords = palsy (Clic here for more details about this article) |