Taneyoshi Nozawa

Development of ophthalmoplegia in amyotrophic lateral sclerosis during long-term use of respirators

Patients with amyotrophic lateral sclerosis (ALS), who survive longer on a life-support system, exceeding the natural course of this disease, show new features of ALS. We report here a clinico-pathologic study of a 51-year-old patient with sporadic ALS who developed progressive external ophthalmoplegia 3 years after he remained on a respirator and died 5 years later, 13 years after the onset of his illness. The external ophthalmoplegia was initially accompanied by preserved dolls eye phenomenon, which later became absent. Autopsy revealed not only degeneration of the upper and lower motor neuron systems typical of ALS, but also degeneration of the Clarkes dorsal nuclei, spinocerebellar tracts, substantia nigra and inferior olives in addition to intracytoplasmic neuronal inclusion bodies in various areas. The oculomotor and abducens nuclei were variably involved, accompanied by neurogenic atrophy of the extraocular muscles. Our case report is consistent with the idea that ALS comprises a heterogeneous group of disorders, and also indicates that long-term use of respirators may make some patients with this illness prone to developing atypical clinical and neuropathologic features which are not observed during the natural course of ALS.

Acute Spinal Epidural Abscess and Spinal Leptomeningitis: Report of 2 Cases with Comparative Neuroradiological and Autopsy Study

A 21-year-old male developed back pain, fever, and rapidly progressive quadriparesis. Lumbar tap yielded frank pus which was confirmed on magnetic resonance imaging (MRI) to be located mainly in the cervical epidural space. Conservative antibiotic remedy was partially effective for restoration of the neurological deficits. A 82-year-old female noticed low-back pain which was rapidly accompanied with clouding of consciousness, paraplegia, and sphincter disturbances. Lumbar puncture revealed thick pus which was best depicted on MRI in the thoracolumbar subarachnoid space. At autopsy, spinal subarachnoid abscess or leptomeningitis was confirmed, and a spinal infarction previously unrecognized on MRI was found. Usefulness and shortcomings of MRI in the diagnosis of paraspinal infections are discussed.

Periodic limb movements and sleep-wake disorder

Abstract The relationship of periodic limb movements (PLM) and Sleep‐wake disorders in 11 patients was investigated. Two patients complained of insomnia. A patient with cervical spinal canal stenosis had a complaint of difficulty in initiating sleep. Movement index (MI) was 51 and PLM arousal index was 8. A patient with chronic hemodialysis had a complaint of difficulty in initiating and maintaining sleep. MI was 79 and PLM arousal index was 51. One patient with myotonic dystrophy showed 79 in MI and 3 in PLM arousal index. It is suspected that myotonic dystrophy is less sensitive to stimuli during sleep (i.e. PLM). These results suggest that the sleep‐wake disorders associated with PLM relate to the threshold of awakening.

12. Factors associated with effectiveness of CPAP therapy on central sleep apnea – Influence of body position during sleep and underlying cardiac dysfunction

The effectiveness of CPAP therapy on central sleep apnea (CSA) remains controversial and factors associated with such effectiveness are still unclear. We investigated effectiveness of CPAP on CSA and related factors, focusing on body position during sleep and presence or absence of underlying cardiac dysfunction. Among 7268 subjects who underwent polysomnography, 115 patients who had central apnea hypopnea index (CAHI) P15 were analyzed. We excluded 47 patients who had rarely lateral position during sleep ( 80% reduction from the diagnostic AHI) whereas residual 34 subjects did not show such reductions (i.e. ineffective group). Ineffective group showed significantly greater AHI on lateral position (L-AHI) and lower % of slow wave sleep (%SWS) on diagnostic study, and more frequently had cardiac dysfunction as compared with effective group. Multivariate logistic regression analysis showed greater LAHI, lower %SWS and presence of cardiac dysfunction are independently associated with the CPAP-ineffectiveness. Our findings suggest that in addition to the underlying cardiac dysfunction, CSA on lateral position may play a role to determine CPAP effectiveness on CSA.

118. A case with abnormal nocturnal behavior during EEG-wakefulness

We present a 35-year-old man with abnormal nocturnal behavior during EEG-wakefulness. He has been diagnosed as depression and panic disorder which were treated with several tranquilizers. His wife complained about his abnormal behaviors during sleep period, such as screaming, walking and going outside with opening locked door. However, he was always completely amnestic for such behaviors. Though he was young, he was initially suspected to have REM sleep behavior disorder. However, in the polysomnography, such abnormal behaviors occurred during EEG-wakefulness just after short-term REM sleep. Next morning, he was totally amnestic about such behaviors. Therefore, he was diagnosed as sleep related dissociative disorder. This case reminds us to perform polysomnography for all cases with psychiatric disorders in order to diagnose such nocturnal behavior disorder, appropriately.