Previously it was reported that some patients with tremor had the same electrophysiological findings as those seen in patients with cortical reflex myoclonus, and consequently the tremor was named "cortical tremor." In the present study, we examined six patients from three families with cortical tremor of relatively late onset. The inheritance pattern of cortical tremor was compatible with autosomal dominant trait. Those patients had relatively rhythmic involuntary movements (tremor) in the distal upper and lower limbs, especially during posture and/or fine movements. There was no cerebellar ataxia or dementia, and fits of loss of consciousness occurred only infrequently. Electrophysiologically, they had generalized spikes on electroencephalogram (EEG), giant cortical components of somatosensory evoked potential, an enhanced long-loop reflex (C-reflex), and cortical spikes preceding the rhythmic jerk demonstrable by the jerk-locked back averaging method, thus fulfilling the criteria of cortical reflex myoclonus. Furthermore, they had slow negative EEG shift starting 1-2 s prior to voluntary movements, suggesting that, as opposed to the conventional form of progressive myoclonus epilepsy (PME), the cerebellar efferent input to the motor cortices was normal. These clinical and electrophysiological pictures are distinct from those of familial essential tremor, familial essential myoclonus, or the conventional form of PME, and the term "familial cortical myoclonic tremor" will represent the clinical and electrophysiological features of this unique entity most appropriately.

Objectives: There are two types of late coronary dilated lesions after Kawasaki disease: new aneurysms and expanding aneurysms. The development of coronary dilated lesions late after Kawasaki disease was investigated. Methods: Between 1978 and 2003, 562 patients with coronary arterial lesions underwent selective coronary angiography on at least two occasions. Results: Of the 562 patients studied, 17 new dilated or expanding lesions were found in 15 patients (3%, 11 boys, four girls). The time of detection of new aneurysms after Kawasaki disease ranged from 1.9-19.2 years (median 11.4 years) and their diameters ranged from 2.0-6.5 mm (median 4.4 mm). Thirteen new aneurysms occurred in vessels in which previous aneurysms had regressed and all new aneurysms were associated with localised stenosis. A new aneurysm at the bifurcation or in the branches was seen in 14 (93%) and 13 were eccentric (87%). Of two expanding aneurysms, one involved the right coronary artery in one patient and the other the left anterior descending coronary artery. One expanding aneurysm increased from 4.4 mm to 19.5 mm over 17 years, and the other expanding aneurysm increased from 10 mm to 15 mm in one year. Conclusions: Neither new nor expanding aneurysms have caused cardiac events. New aneurysms often develop as a pre-stenotic or post-stenotic dilatation secondary to localised stenosis. New and expanding aneurysms may be caused by haemodynamic factors in addition to the abnormality of the coronary arterial wall after severe acute vasculitis. Coronary arterial wall abnormalities were stenosis as well as, rarely, dilatation of the vessels in the late period. It is important to recognise that the changes of the coronary arterial wall persist late after regression of a large aneurysm.

Eighteen amyotrophic lateral sclerosis (ALS) patients having neither respiratory complaints nor subjective symptoms of sleep disturbance were studied by using an ambulatory multi-parameter monitoring system during sleep. They were divided into two groups: 11 patients with predominantly bulbar form and seven with non-bulbar form. After performing daytime pulmonary function tests, the presence of sleep-disordered breathing (SDB) was evaluated by using a portable device. ALS patients did not show significant SDB as a whole, and the respiratory disturbance index (RDI) was not significantly different between the bulbar group and the non-bulbar group. However, three patients of the bulbar group showed significant SDB, and the patterns of apnea/hypopnea suggested that both bulbar weakness and minimal diaphragmatic weakness might cause SDB in ALS patients at an early clinical stage. Multi-parameter respiratory monitoring during sleep should be included in the routine evaluation of ALS patients at an early clinical stage, especially those with predominantly bulbar involvement, in order to predict early respiratory failure.

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