[Three cases with acute encephalopathy related with adenovirus type 7 infection].

We studied the clinical and CT findings of 3 children with acute encephalopathy associated with adenovirus type 7 (AD-7) infection. Seizures in all the patients developed from 8 to 10 days after the onset of pyrexia. The values of serum AST, LDH and CRP elevated and those of WBC and serum protein decreased at the onset of encephalopathy.

[Efficacy of methylprednisolone pulse and mild hypothermia therapies in patients with acute encephalopathy].

Five patients with acute encephalopathy underwent methylprednisolone pulse (mPSL-P), hypothermia and their combination therapies (3 cases, 1 case and 1 case, respectively), with excellent outcome. Two cases with severe brain edema survived. One had severe brain damage as a sequelae.

Extraocular muscle responses to high dose intravenous methylprednisolone in myasthenia gravis.

Three patients with generalised myasthenia gravis and three with ocular myasthenia gravis received two to five courses of high dose intravenous methylprednisolone because of the failure of standard immunomodulating therapies. Changes in myasthenic signs were assessed using a four step system for grading muscle weakness and fatiguability in 10 test items. Although a brief and modest amelioration was found from day 1 to day 2 after the initial infusion in two patients with generalised myasthenia gravis, all three experienced a prolonged phase of worsening followed by improvement before the next course.

Algorithm for the treatment of rapid cycling.

Rapid cycling is a clinical subtype of bipolar mood disorder that has four or more episodes during the previous 12 months and has poor response to lithium (Li) prophylaxis treatment. Therefore it is said the refractory mood disorder and algorithm for treatment of rapid cycling is to be expected. The first choice of drug for rapid cycling is Li, the second choice is carbamazepine (CBZ) or valproic acid (VPA), in addition to Li, the third choice is clonazepam (CNZP) in addition to Li, and CBZ or VPA, the fourth choice is levothyroxine or bromocriptine in addition to them, and the fifth choice is electroconvulsive therapy.

[A study of aggravation of atopic dermatitis during Japanese cedar pollen season--correlation with grades of dermatitis on face and Cry j 1 specific IgE].

We studied influence of Japanese cedar pollen (Jcp) on aggravation of atopic dermatitis (AD) during the pollination season. 48.5% of 97 patients with atopic dermatitis showed aggravation of dermatitis during the pollination season and 85% of them had Japanese cedar pollinosis, whereas only 44% of AD patients without the aggravation had the pollinosis.

Topographical differences in the developmental profile of central motor conduction time.

Objectives: To study the topographical difference in the developmental profile of the central motor conduction time (CMCT) in upper extremity muscles, electromyographic (EMG) responses to transcranical magnetic stimulation (TMS) were examined in the first dorsal interosseous, extensor carpi radialis (ECR), biceps (BCP), and deltoid (DT) muscles of 25 neurologically normal subjects aged from 2 to 26 years.

Methods: The motor cortex and cervical spinal roots were magnetically stimulated, and CMCT was measured as the onset latency difference between these EMG responses.

Results: CMCT in children was shorter in the more proximal muscle of each adjacent muscle pair, despite the tendency of a higher threshold intensity for TMS of the more proximal muscle.

[Acute shock encephalopathy syndrome in a girl with Sotos syndrome--with special reference to differentiation from Reye syndrome].

We reported here a 5-year-old girl with Sotos syndrome who developed acute shock encephalopathy syndrome (ASES), and differentiated ASES from Reye syndrome (RS). Abrupt onset of shock and status epilepticus developed and these were followed by disseminated intervascular coagulation (DIC) and liver damage. Gradual elevation of hepatic enzymes, high serum bilirubin value, and normal serum ammonia value in acute phase were incompatible with typical RS.

[Clinical study in 28 patients with infection-related acute onset encephalitis].

Of the 75 patients with infection-related acute encephalopathy or encephalitis treated by us in the last 10 years, 28 had acute onset encephalitis. The results of clinical studies on these 28 patients were as follows: (1) The number of cases who exhibited CNS manifestations during (A) and after pyrexia (B) were 15 and 13, and the ages of predilection were infancy and school age, respectively. (2) MRI studies in cases of A revealed multifocal CNS lesions in 1 case, localized lesions in 7 and normal findings in 7.

Functional recovery in hemiplegic cerebral palsy: ipsilateral electromyographic responses to focal transcranial magnetic stimulation.

The patterns of functional recovery after unilateral cerebral damage occurring in the prenatal to infantile periods were studied in nine patients with hemiplegic cerebral palsy. Motor evoked potentials (MEPs) recorded from the small hand muscles were investigated using focal transcranial magnetic stimulation (TMS). The MEPs findings could be separated into three subtypes based on the features of ipsilateral MEPs elicited by TMS over the unaffected motor cortex.

Relation between body fat-corrected ECG voltage and ambulatory blood pressure in patients with essential hypertension.

Because adipose tissue has high electric resistance, the amount of body fat influences ECG voltage. In this study, body fat weight of patients with essential hypertension was measured by means of the impedance method and was used to correct mean ECG voltage. Then the relation between body fat-corrected mean ECG voltage (Vfm) and ambulatory blood pressure (BP) was investigated.

Edrophonium-induced macrosaccadic oscillations in myasthenia gravis.

In a myasthenia gravis patient with profound ophthalmoplegia who was given edrophonium during forward fixation or horizontal/vertical saccades, horizontal macrosaccadic oscillations occurred that were conjugate, 2 to 3 Hz, and of peak-to-peak amplitude up to 50 degrees. The intersaccadic interval averaged 129.5 msec as measured by electro-oculography.

Electromyographic study in an infant with benign paroxysmal torticollis.

The findings of surface electromyography (s-EMG) in a female with benign paroxysmal torticollis in infancy (BPT) are reported. This disease is characterized by (1) a self-limiting condition that appears in early infancy and disappears spontaneously, (2) recurrent episodes of sudden, stereotypic torticollis, usually alternating from side to side, and (3) episodes that usually continue for several hours to a few days. The pathogenesis of this disease has not been determined.

Magnetic stimulation of the corticospinal tracts in Pelizaeus-Merzbacher disease.

To evaluate conduction abnormalities of the corticospinal tracts (CSTs) in Pelizaeus-Merzbacher disease (PMD), magnetic stimulation at three levels was carried out in 3 boys with PMD aged between 9 and 12 years. They were all diagnosed as having a duplicated proteolipid protein gene. The motor cortex and cervical spinal roots were stimulated with a round coil, whereas a double cone coil was used for brain-stem stimulation.

Characteristic response to transcranial magnetic stimulation in Rett syndrome.

To pathophysiologically evaluate the corticospinal tracts (CSTs) in Rett syndrome (RS), transcranial magnetic stimulation (TMS) was performed in 3 patients aged 4, 6 and 13 years. The two younger cases exhibited the clinical characteristics of the pseudostationary stage (stage III), while ambulation was lost in the oldest case at the age of 11 years (stage IV). The motor cortex and cervical spinal roots were magnetically stimulated to obtain motor evoked potentials (MEPs) from the relaxed first dorsal interosseous muscle.

Statistical base value of 24-hour blood pressure distribution in patients with essential hypertension.

The purpose of this study was to calculate statistically the minimum (base) blood pressure (BP) of nighttime (sleep-time) BP values obtained by ambulatory BP monitoring (ABPM) and to investigate its clinical significance. Twenty-four-hour recording of ECG with ABPM was performed directly (n=89) or indirectly (n=117) in 206 patients with essential hypertension. A telemeter was used for the direct method and a multi-biomedical recorder (TM2425) was used for indirect measurement.

Clinical and radiological variability of influenza-related encephalopathy or encephalitis.

Background: Influenza-related encephalopathy or encephalitis is not rare in children. However, it is not well understood why the brain lesion develops from influenza infection. The purpose of this study was to clarify its pathogenesis by analyzing the clinical and neuroradiological findings in patients having influenza-related brain lesions.

[Clinical studies on 35 patients with infection-related acute encephalopathy].

The findings on magnetic resonance imaging (MRI) or computed tomographic scan in 35 children with acute encephalopathy (AE) induced by infections were categorized into five groups: (1) normal findings (11 cases), (2) normal findings in the acute phase followed by mild brain atrophy (1 case), (3) severe brain edema which developed within 48 hours after the onset of AE (7 cases), (4) diffuse cortical necrosis which appeared around 4 days after the onset of AE (9 cases), and (5) symmetric thalamic lesions (7 cases). Their outcomes were as follows: three cases developed epilepsy in (1) and (2); six died and one had a sequel of severe brain damage (SBD) in (3); all cases survived but had SBD in (4), and four died, one had SBD, and 2 recovered in (5). Serum AST were elevated in 7.

[Focal brain lesion accompanied by exanthema subitum: report of two cases].

We report two cases with exanthem subitum in which hemioaresis developed during the acute infectious phase. Magnetic resonance imaging (MRI) demonstrated a cerebral ischemic infarct in the territory of the right lenticulostriate artery in one patient. Along the right temporal and parieto-occipital cortex of the other patient, there were high-signal-intensity lesions on T2-weighted images, which showed marked enhancement by gadolinium on T1-weighted images.

Circadian variation of hemodynamics and baroreflex functions in patients with essential hypertension.

It is well known that cardiovascular accidents such as myocardial infarction frequently occur in the morning, but their triggering mechanisms are not clear. The present study investigated circadian variations of hemodynamics and baroreflex functions. Twenty-three patients with essential hypertension were studied.

Two cases of essential myoclonus, epilepsy, mental retardation and anxiety disorders.

We examined an 18-year-old female and an 18-year-old male with mild mental retardation who suffered from the oscillatory form of sporadic essential myoclonus from an age of 3 years. Although the generalized oscillatory myoclonus resembled severe essential tremor, surface electromyography revealed small myoclonic jerks with frequencies of 6-8 Hz. As concomitant symptoms, the female case exhibited overanxious irritability from early childhood and generalized epileptic seizures occurred from the age of 4 years.

Alternating hemiplegia of childhood: report of a case having a long history.

We examined a patient with alternating hemiplegia of childhood (AHC) who had over a 23-year history of AHC to investigate the origin of the neurological deterioration with increasing age. Hemiplegic attacks had occurred consistently at a frequency of a few per week since infancy, and he first experienced attacks of cerebellar ataxia at the age of 23 years. Intellectual impairment, dysarthria, dystonic posturing, and a wide-based gait had been slowly progressive, but they had been stable since he turned twenty.

Acute confusional migraine and migrainous infarction in childhood.

We report two children with acute confusional migraine (ACM) and another with migrainous infarction (MI), aged 7-12 years. There was a family history of migraine in all patients. The patients, who were all right-handed, all manifested sudden onset of consciousness disturbance and other neurological deficits as the first aura in their life.

Transcranial magnetic stimulation in benign childhood epilepsy with centro-temporal spikes.

Motor cortical excitability was studied using transcranial magnetic stimulation (TMS) in 10 age-matched controls, and 13 children with benign childhood epilepsy with centro-temporal spikes (BECT), with a mean age of 11.2 +/- 2.0 years (five untreated, and eight treated with sodium valproate (VPA) and well controlled).