Use of the triaxial microcatheter method in super-selective transcatheter arterial chemoembolisation for hepatocellular carcinoma.

OBJECTIVES: Transcatheter arterial chemoembolisation (TACE) has been widely used for inoperable hepatocellular carcinoma (HCC). Super-selective TACE is preferable to non-selective therapy, because it maximises the impact of treatment on the tumour while minimising damage to tumour-free liver parenchyma. It is therefore important to advance the catheter tip as close as possible in the feeding artery. There is now a new microcatheter with a 1.9-Fr tip with no taper, which can be inserted into a 2.7-Fr microcatheter. In this study we describe the new technique of using the two microcatheters called the triaxial microcatheter method. METHODS: We evaluated 30 TACE procedures to investigate whether or not the catheter tip could be advanced closer to HCC with the triaxial microcatheter method than with previous TACE using a conventional microcatheter. RESULTS: With conventional microcatheters, the level of embolisation was a lobar artery in 4 cases, segmental in 8 cases, subsegmental in 15 cases and sub-subsegmental in only 1 case. TACE could not be performed in two cases. When using the triaxial microcatheter method the level of embolisation was subsegmental in 8 cases, including 2 in which the level was the same as that with a conventional microcatheter, sub-subsegmental in 13 cases and more distal in 7 cases. In the two cases in which TACE could not be performed with the conventional microcatheter, it could be performed sufficiently using the new method. As a whole, in 28 of the 30 procedures (93%) we could successfully advance a catheter tip closer than with the previous TACE. CONCLUSION: The triaxial microcatheter method appears to be useful.

Embolization for asymptomatic aneurysms of the first jejunal artery.

In a 71-year-old man with a history of coronary artery bypassing using the left internal thoracic and gastroepiploic arteries, the first jejunal artery aneurysms were found by chance at 3D-CT performed to evaluate conditions of the grafts. He was successfully treated by transcatheter embolization using interlocking detachable coils. During a follow-up period of 5 months, the patient did well and had no sign of intestinal ischemia.

Convulsive episodes in patients with group A xeroderma pigmentosum.

OBJECTIVES: To clarify the incidence of convulsive episodes in patients with group A xeroderma pigmentosum (XPA). MATERIALS AND METHODS: By investigating the history of convulsive episodes of our 33 XPA patients through either their medical charts or direct interviews with their caretakers. RESULTS: Five patients had several episodes of afebrile convulsion at ages older than 12. With the exception of one patient who began to show convulsive episodes at 13, no other XPA patients exhibited febrile seizures. As far as our 33 XPA patients were concerned, 15% exhibited epilepsy, and 3% experienced febrile seizures. CONCLUSIONS: Japanese XPA patients showed a lower incidence of febrile seizures, while exhibiting a higher incidence of epilepsy. It is assumed that the brain of young patients with XPA is difficult to develop convulsions.

Tuberous sclerosis: epileptogenicity and multimodal presurgical evaluations.

We report on a patient with tuberous sclerosis-related epilepsy who benefited from surgical treatment. Various presurgical evaluations, including positron emission tomography (PET), made it possible for us to localize the epileptic focus accurately. In this paper, we stress the importance of performing multimodal evaluations to determine which tubers really possess epileptogenicity. In addition, the implications of PET in tuberous sclerosis-related epilepsy are described.

Asynchronous breathing during sleep.

BACKGROUND: Children rarely complain of symptoms associated with sleep disordered breathing (SDB). Paradoxical inward rib cage movement (PIRCM) during sleep might prove useful for detecting SDB. AIMS: (1) To determine the correlation between the degree of PIRCM and other measures of disordered breathing during sleep. PIRCM occurs physiologically throughout rapid eye movement sleep in neonates, while no PIRCM has been reported during sleep in adolescents. (2) To determine the chronological changes in the degree of PIRCM. METHODS: PIRCM was quantified by means of the laboured breathing index (LBI). LBI was determined by respiratory inductive plethysmography; PIRCM accompanies a high LBI. Sleep recordings obtained for 101 subjects for various reasons (aged from 3.5 months to 19 years) were analysed. RESULTS: In 22 records, the minimum SaO2 value was 90% or more and no obstructive apnoea of more than 10 seconds was observed. In these 22 records, LBI during rapid eye movement sleep decreased significantly with age, reaching the mature low level at 3.3 years of age. In the other 79 records, LBI correlated well with measures of obstructed breathing during sleep. CONCLUSIONS: By paying more attention to PIRCM, more obstructed breathing during sleep might be found among children aged 3 years or more.

Nocturnal enuresis and the pontine reticular formation.

OBJECTIVES: To assess the previously proposed hypothesis that enuretic patients have a dysfunction in the pontine reticular formation. METHODS: In 18 patients with intractable nocturnal enuresis, rapid eye movement (REM)-related phasic chin muscle activity loss in REM sleep was examined by means of a single-night polysomnography. RESULTS: In 5 of 18 patients, this physiologically seen phenomenon was found to be disturbed. CONCLUSIONS: Since REM-related phasic chin muscle activity loss is disturbed by the functional impairment in the pontine reticular formation, some enuretic patients could be considered as presenting a dysfunction in this structure.

Rapid eye movement sleep in breath holders.

One-night polysomnography was performed on seven subjects suffering from breath-holding spells, including one whose death was suggested to be a consequence of a breath-holding spell. The fatal case showed no rapid eye movements (REMs) during REM sleep, although he exhibited REMs during wakefulness. The average numbers of both REMs and bursts of REMs in REM sleep in the other six breath holders were significantly lower than those in age-matched controls. The breath holders showed no airway obstruction, desaturation, or sleep fragmentation. Since the rapid ocular activity in REM sleep is generated in the brain stem, we hypothesized that a functional brainstem disturbance is involved in the occurrence of breath-holding spells.

A patient with mitochondrial myopathy associated with isolated succinate dehydrogenase deficiency.

We report on a boy with normal mental development who had muscle hypotonia and congenital dislocation of the hip and knee joints. Histochemical and biochemical examinations of his muscle specimen revealed no succinate dehydrogenase (SDH) activity. Since the NADH cytochrome c reductase and cytochrome c oxidase activities were normal, we concluded that he had an isolated SDH deficiency. Our patient provides further evidence for the clinical variability of this disorder.

ACTH activates rapid eye movement-related phasic inhibition during REM sleep in patients with infantile spasms.

OBJECTIVES: Phasic inhibition index (PII) is the rate of the simultaneous occurrence of phasic chin muscle activity (PCMA) and rapid eye movement bursts during rapid-eye-movement sleep (REMS). In naive patients with infantile spasms (IS), the PII value was found to reflect their prognosis. We studied the effects of adrenocorticotropic hormone (ACTH) on REMS components including PII in IS. METHODS: REMS parameters were examined in 18 IS patients before and after ACTH treatment. The effects of corticosteroids (CSs) were examined in 3 patients with congenital adrenal hyperplasia (CAH) and 3 with nephrotic syndrome (NS). RESULTS: ACTH decreased PII and PCMA in IS patients. In CAH patients, physiological doses of CSs corrected the increased intrinsic ACTH level and increased PII. In NS patients, therapeutic doses of CSs suppressed PCMA without affecting PII. CONCLUSION: ACTH suppressed PCMA through CSs, and reduced PII directly. ACTH was hypothesized to eliminate IS through these dual modes of action.

Quantitative analysis of paradoxical inward rib cage movement during sleep in children.

Paradoxical inward rib cage movement in children is quantified by the labored breathing index (LBI) on the respiratory inductive plethysmography. Labored breathing index during rapid eye movement sleep (REMS) in 59 children without obvious sleep disordered breathing (SDB) declined with age, and decreased to the mature low level at 35 months of age. The LBI was also found to reflect well the severity of SDB. Paradoxical inward rib cage movement, which was quantified by LBI, is concluded to be an important finding in diagnosing SDB in child patients.

REM sleep components predict the response to initial treatment of infantile spasms.

PURPOSE: The phasic inhibition index (PII) is the rate of the simultaneous occurrence of rapid eye movement bursts (RBs) and phasic chin muscle activity (PCMA) during rapid eye movement sleep (REMS). PII is low insofar as physiologically occurring REM-related phasic inhibition acts on chin muscles. Previously we found that PII was significantly higher in patients with infantile spasms (ISs) who had a recurrence of convulsions than in patients with ISs who exhibited no recurrence. We aimed to predict the response of patients with ISs to conventional anticonvulsants (AEDs) by means of REMS components including PII, expecting to facilitate avoidance of potentially hazardous hormonal therapy. METHODS: REMS, recorded before the beginning of any medication, was retrospectively examined in 15 patients with ISs. The patients were classified into two groups according to the response to initial treatment with conventional AEDs. Conventional AEDs were enough to control the spasms in six good responders (GRs), whereas further hormonal therapy was required in nine poor responders (PRs) to control the spasms. RESULTS: The amount of REMS was significantly lower in patients with ISs than in controls. GRs had less REMS than did PRs, although no significant difference was observed. Although the frequencies of RB and PCMA showed no significant differences among GRs, PRs, and controls, the average PII value in PRs (12.6+/-3.4; mean+/-SD) was significantly (p < 0.001) higher than that in GRs (6.1+/-1.7). CONCLUSIONS: PII is a useful parameter for differentiating GRs from PRs.

[Auditory brainstem responses in group A xeroderma pigmentosum].

Xeroderma pigmentosum has been known to result from disturbance in the repair of injured DNA caused by the ultra violet light. According to cell fusion studies, this disease is classified into 8 groups. Among these groups, group A (A-XP) shows the most severe type of neurological disturbance. Slowly progressive diffuse impairment both in the central and the peripheral nervous systems has been reported. Although hearing loss occurs in all the patients, few papers have described the chronological changes of this disability. This study aimed to clarify the development of audiological abnormalities in A-XP patients. We recorded auditory brainstem evoked responses (ABRs) in 20 Japanese children with A-XP. All patients had homozygous intron 3 splicing mutations of xeroderma pigmentosum group A complementing gene, the most common type mutations in Japan. ABR threshold, peak latency of 5th wave and the peak interval latency between the 1st and 5th waves (I-V interpeak latency) were measured, and were compared with those obtained from age-matched controls. ABRs were well detected in all patients examined under 4 years old. The I-V interpeak latencies became shorter with age as in the controls. In 3 ears, the 5th wave was recorded without 1st wave. In 4 ears which no ABRs were detected with 90 dBHL stimulation, the only 5th wave was detected. No waves were obtained from the patients aged over 10 years. The current study revealed that ABR disturbance in A-XP patients became obvious after 4 years of age. In addition, no ABR was found to be identified after 10 years of age. During 4 to 10 years of age, some patients showed the elevation of the threshold of the 5th wave. In A-XP patients, the peripheral nerve was hypothesized to be affected earlier than the central pathway involved in ABR.

Video analysis of gross body movements during sleep.

Gross body movements (GM) during sleep, classified into four GM types by the involved parts of body, were evaluated using two-dimensional video analysis in five normal children aged 4-12 years. The rate of occurrence of all GM types showed apparent sleep stage dependency. Among four GM types, GM-1 (GM with axial rotation) was the most frequent. Averaged duration of GM-1 was the longest. A total of 77.2% of GM-1 started with the contraction of chin muscle and 35% of total sleep changes (tSC) were related to GM. A total of 5.6% of tSC following GM shifted to the deeper stage. Further evaluation is necessary in order to understand the physiological mechanism of GM.

Maturation of motility and motor inhibition in rapid-eye-movement sleep.

OBJECTIVE: To describe the age-related changes in the number of movements in rapid eye movement (REM) sleep, and to quantify the functional maturation of motor inhibition. STUDY DESIGN: Gross movements, phasic mentalis muscle activity (PMMA), and a new index that expressed the shortness of PMMA (the proportion of short PMMA among all PMMA) were examined cross-sectionally in 87 healthy children from premature babies to preadolescents by means of a single (all-night) polysomnography. RESULTS: The incidence of gross movements and long PMMA decreased with age, whereas that of short PMMA increased with age. The new index exhibited an age-related increase, with the highest correlation with age among sleep parameters examined, and reached an adult level after 6 years of age. CONCLUSION: We found that the age-related reduction of PMMA duration, which was expressed by a new index, occurred in parallel with the maturation of the inhibitory system that is tonically activated during REM sleep. We named this index the tonic inhibition index and concluded that the neuronal system involved in motor inhibition during REM sleep was still maturing during early childhood. We propose the tonic inhibition index as a useful quantitative indicator for the maturity of the inhibitory system.

[Disturbance of active sleep components in neonates with congenital hypothyroidism].

In order to investigate the effect of hypothyroidism during the early development on the functional brainstem maturation, polysomnograms were recorded on 7 patients with congenital hypothyroidism detected by neonatal screening before treatment. The following two sleep indices for phasic muscle activity during rapid eye movement (REM) sleep were evaluated: dissociation index (DI) and % body movements in REMs burst (% BM). The DI is defined as the ratio of the number of twitch movements (TMs) during REM sleep to the sum of TMs and localized movements (LMs) during REM sleep. The % BM is the percentage of TMs and LMs which occur during bursts of REMs in relation to the sum of TMs and LMs during REM sleep. The DI and % BM can reflect maturation of the tonic and phasic inhibitory system functioning during REM sleep, respectively. In congenital hypothyroidism, DI was lower than that in controls, while % BM was identical. The tonic inhibitory system was specifically involved, whereas the phasic one was preserved. It is suggested that thyroid hormone could play an important role on the functional brainstem maturation. We propose to investigate their neuropsychological development over the long term to elucidate the influence of hypothyroidism.

Phasic motor activity reduction occurring with horizontal rapid eye movements during REM sleep is disturbed in infantile spasms.

Single polysomnography was performed before treatment in 17 patients with infantile spasms (IS) (13 with a cryptogenic type and 4 with a symptomatic one). Their sleep components during rapid eye movement (REM) sleep were compared with those in 22 age-matched controls. The tonic muscle atonia during REM sleep was observed in all IS patients as in controls. The amount of REM sleep in IS patients was significantly lower, while the incidences of gross movements, phasic chin muscle activity, and bursts of horizontal rapid eye movements were identical with those in controls. The phasic inhibition index (PII), i.e., the rate of simultaneous occurrence of phasic chin muscle activity and bursts of horizontal rapid eye movements, was significantly higher in IS than in controls. The PII value was the only parameter that reflected our patients' prognosis among the obtained REM sleep parameters. We presume that the elevated PII in IS reflects the weakness of the phasic motor activity reduction occurring with horizontal rapid eye movements, and attribute this disturbance to a functional instability of the rostral pontine tegmentum. We propose that PII is a useful parameter for assessing the prognosis of IS. Considering the neural basis for elevated PII in IS, this index is expected to provide a clue for explaining the pathophysiology of IS.

Motor disturbance during REM sleep in group A xeroderma pigmentosum.

We investigated motor phenomena during rapid eye movement (REM) sleep in 13 patients with group A xeroderma pigmentosum aged from 11 to 39 months, and compared them with those obtained from 12 age-matched controls. At the time of sleep study, they had no abnormality on routine electrophysiological examinations. The amount of REM sleep and the incidence of motor phenomena during REM sleep in patients were similar to those in age-matched controls. However using the newly designated indices, we demonstrated disturbance on both the tonic motor inhibition occurring during the whole REM sleep period and the phasic one acting simultaneously with horizontal rapid eye movements in these patients. Since the motor inhibition during REM sleep is mediated by the subcortical structures, our study indicate that these structures are functionally impaired in group A xeroderma pigmentosum even during the early stage of the illness.

Impairment of respiratory rhythmogenesis and sequelae of bacterial meningitis.

A 9-year-old boy with respiratory disturbance associated with medullary lesions after pneumococcal meningitis is reported. Although he lives a normal daily life, he cannot cough or sneeze. A polysomnographic study revealed a low respiration rate and an irregular respiratory rhythm not only during REM sleep but also during slow wave sleep, and marked desaturation during sleep. Respiratory function tests including CO2 response revealed normal values. Magnetic resonance imaging demonstrated bilateral small lesions in the medulla. This patient is unusual because respiratory rhythm is impaired, without decreased ventilatory capacity or CO2 response, supporting the possibility that rhythmogenetic respiratory neurons are located in a limited area of the human medulla.

Phasic motor activity reduction occurring with horizontal rapid eye movements during active sleep in human.

We describe the phasic reduction of motor activity occurring with horizontal rapid eye movements (REMs) during active sleep in 15 children (12 healthy children and 3 patients with severe brain damage). A REM-related decrease in intercostal muscle activity was demonstrated by averaging integrated surface electromyograms. In the healthy subjects, this reduction had a mean latency from the REM onset of 37.1 ms and a duration of 225.9 ms. This phenomenon was also observed in the 3 patients who had lost cerebral function. We hypothesized a brainstem origin for the effect. A REM-related mentalis muscle activity loss, detected by averaging mentalis muscle twitches, was observed in 10 healthy children among the subjects. This loss began at 59.1 ms before the onset of REMs and lasted for 230.2 ms on average. In addition, a transient decrease in integrated REM activity surrounding mentalis muscle twitches (a twitch-related reduction of REMs) was observed. We discuss the similarity between REM-related phasic reduction of muscle activity obtained for intercostal and mentalis muscles and pontogeniculo-occipital (PGO) wave-related inhibitory postsynaptic potentials reported for feline lumbar and trigeminal motoneurons, respectively. We then assume the presence of a phasic event generator, functioning during active sleep in healthy humans, which triggers at least three generators; that is, the generator of PGO waves (or REMs), motor inhibition, and of motor excitation including muscle twitches.