以前、知能障害が全くない子もいた。核黄疸。

《1》アテトーゼ型脳性麻痺児の構音分析
Articulation analysis of athetoid children
音声言語医学Vol.33(1992)No.3p221-226
https://www.jstage.jst.go.jp/article/jjlp1960/33/3/33_3_221/_pdf
The articulation impairments of 13 athetoid children were investigated using an utterance test consisting of monosyllables and 50 words.

Results were as follows.

Production of the sounds /b//m//k//p//n/ was correct, while that of the sounds /s//r//z//ts//∫/ was unclear.

In place categories, dentals and alveolars were pronounced incorrectly.

In manner categories, the least acurrate production was of liquids, fricatives and affricates.

The characteristics of the errors were as follows: production of stops (instead of liquids, fricatives and affricates) ; no control of voicing and devoicing method; distortion by the tongue, transferring backward and touching too wide.

In the less intelligible group, incorrect sounds tended to appear within a word, although they were successfully made in single syllables.



《2》不随意運動の臨床
Pathophysiology of involuntary movement in adults
脳と発達Vol.29(1997)No.3p193-198
https://www.jstage.jst.go.jp/article/ojjscn1969/29/3/29_3_193/_pdf
Inhibitory control of basal ganglia output to thalamocortical projection plays an important role in normal cortical activity in the current model of the basal ganglia motor circuit.

Hypokinetic and hyperkinetic movement disorders of basal ganglia origin can be explained by excess or collapse of the basal ganglia output.

An abundance of evidence indicates that parkinsonian akinesia results from hyperactivity of the basal ganglia output.

Reversal of akinesia by lesions of the internal division of the globus pallidus GPi or its excitatory source, the subthalamic nucleus, agrees with this pathological schema.

Ballism associated with subthalamic lesions, and dopa-induced dyskinesia are regarded as hyperkinetic disorders resulting from suppressed subthalamopallidal projection.

Decreased firing rate in GPi was reported in both disorders.

However, pallidotomy has recently been postulated to abolish both ballism and dopa-induced dyskinesia.

A possible mechanism for the effect of GPi destruction in these hyperkinetic disorders may be blockade of the generation or conduction of phasic neuronal activities driving choreic movements.

Symptomatologically, dystonia has aspects of both hypokinetic and hyperkinetic disorders. Overactivity of the premotor cortices, which receive projections from the basal ganglia via the ventral thalamus, was found both at rest and on movement in idiopathic dystonia.

This abnormal cortical activity may arise from underactivity of basal ganglia output; however, the amelioration of dystonia with pallidotomy suggests a complex pathomechanism of the pallidothalamic system in dystonia.


《3》中枢神経の障害(4)不髄意運動:特に脳性麻痺アテトーゼ型について
日本義肢装具学会誌Vol.16(2000)No.1p60-68
https://www.jstage.jst.go.jp/article/jspo1985/16/1/16_1_60/_pdf



《4》重度仮死後緊張型アテトーゼ型脳性麻痺の頭部MRI所見
脳と発達Vol.26(1994)No.3p276-277
https://www.jstage.jst.go.jp/article/ojjscn1969/26/3/26_3_276/_pdf



《5》MRIで淡蒼球・視床下核・海馬に限局性病変を認めた重症心身障害者の臨床的検討
Localized lesions on MRI in the globus pallidus, subthalamic nuclei and hippocampus in patients with severe intellectual and motor disabilities
脳と発達Vol.29(1997)No.6p449-454
https://www.jstage.jst.go.jp/article/ojjscn1969/29/6/29_6_449/_pdf
We examined the clinical picture of eight patients with severe intellectual and motor disabilities, who had experienced prolonged and severe neonatal jaundice, and showed localized lesions in the globus pallidus, subthalamic nuclei and hippocampus on MRI.

All patients had athetoid tetraplegia, and five patients showed disturbed ocular movements and seven showed dysphagia.

Five patients could communicate with others or utter words, and all showed mental retardation.

Auditory brainstem responses were abnormal in seven, and the percentage of REM sleep on all-night polysomnography was reduced in three.

Neither CT nor T1-weighted MR images could detect any changes in the pallidum or subthalamic nuclei, while T2-weighted MR images disclosed bilateral high signals in the pallidum, especially in the internal segment, in all patients.

Five of the 7 patients, in whom coronal T2-weighted MR imagings were obtained, showed high signals in the subthalamic nuclei.

The hippocampus showed atrophy and/or T2-prolongation in seven patieits.

In one autopsy case, these MRI changes were concordant with pathological lesions.

In patients with athetoid cerebral palsy, brainstem dysfunctions, and abnormal ABR, localization of MRI lesions to the pallidum and subthalamic nuclei is evidence for neonatal bilirubin encephalopathy



《6》不随意運動と大脳基底核遠心路
Roles of the basal ganglia outputs in movement disorders:
A viewpoint based on experiences of stereotactic surgery for idiopathic dystonia
脳と発達Vol.29(1997)No.3p206-212
https://www.jstage.jst.go.jp/article/ojjscn1969/29/3/29_3_206/_pdf
Ventrolateral (VL)-thalamotomy and posteroventral pallidotomy were carried out in 37 patients with idiopathic dystonia, including 23 with dystonia musculorum deformans, 12 with Meige syndrome and 2 with focal dystonia.

There were 11 patients with the onset age of dystonia below 10 years and 26 with the age above 11 years.

Dystonia tended to generalize in the pediatric patients and to localize to the palmo-mental region in the adult patients.

VL-thalamotomy in 30 patients improved the upper body dystonia especially in the adult patients, but, often failed to benefit the pediatric patients.

Posteroventral pallidotomy in 18 patients dramatically alleviated the generalized dystonia especially in the pediatric patients.

In one adult patient, pallidotomy caused hemiparesis due to intracerebral hemorrhage.

The internal pallidum projects the efferents to the thalamic motor nuclei and also to the brainstem reticular formation that connects to the spinal motoneurons.

The present findings implicate the descending pallido-reticular pathway in the mechanism of dystonia of pediatric patients, and the ascending pallido-thalamic pathway in that of adult patients



《7》重症心身障害児 (者) における突然死例の臨床的検討
A clinical study of sudden death in the severely handicapped persons
脳と発達Vol.27(1995)No.6p466-472
https://www.jstage.jst.go.jp/article/ojjscn1969/27/6/27_6_466/_pdf
A clinical study was performed on 8 cases of sudden death (9.0%) selected from a series of 89 patients with severe handicaps who died at the Metropolitan Medical Center of Severely Handicapped during the period from 1968 through 1986.

1) All suffered from profound mental retardation with motor disturbance, but were able to react to environmental stimuli.

2) Most had mixed quadriplegia showing athetosis-related hypertonus.

3) Severe physical deformation and chronic respiratory symptoms were present in 6 of 8 patients.

4) Most of them were adolescent.

5) Autopsy revealed no particular findings to which the expected death could be attributed except for the right cardiac enlargement in 5 cases.

Four of the 8 patients were recovering from respiratory infection or were in the process of having their anticonvulsant doses tapered.

These four cases had shown recent improvement in their general systemic condition.

Sudden death occurred mainly during limited time periods in the early morning or in the evening and was not related to sleep.

The authors suggest that the changes in the autonomic nervous system may precipitate them into sudden death.



《8》けいれん重積型急性脳症における神経学的後遺症の長期経過
Neurological sequelae of acute encephalopathy with febrile convulsive status epilepticus
脳と発達Vol.45(2013)No.4p.294-298
https://www.jstage.jst.go.jp/article/ojjscn/45/4/45_294/_pdf
The clinical characteristics of neurological sequelae in patients with acute encephalopathy with febrile convulsive status epilepticus (AEFCSE) was elucidated.

Methods:

We retrospectively reviewed 8 patients, which were admitted to our hospital from 2002 to 2011.

Results:

In the subacute phase, transient neurological symptoms, such as dystonia (n=3), choreoathetosis (n=2), oral tendency (n=5) and unilateral spatial neglect (n=6), appeared from 3 weeks after onset.

Then, severe intellectual disability (n=7), attention deficit (n=7), disturbance of communication skill (n=7) and emotional disturbance (n=2), persisted from one month after onset.

Although seven patients resumed ambulatory abilities, six exhibited unstable gait without ataxia or muscular weakness.

The neuroradiological findings on MRI corresponded to the clinical course.

In the subacute phase, reversible bilateral signal changes were noted in the subcortical white matter (n=8), caudate nuclei (n=2), putamen (n=1) and thalamus (n=1).

In the chronic phase, diffuse cortical atrophy, predominantly in the fronto-temporal lobes.

Diffuse cortical atrophy suggested that the persistent neurological sequelae of AEFCSE represent cortical dysfunction.

Therefore, we propose that the unstable gait in our patients was gait ataxia, being related to the frontal lobe dysfunction.

Conclusions:

These neurological findings of AEFCSE showed characteristic temporal changes, which should be considered in the development of rehabilitation programs



《9》周産期脳障害の形成機序と早期診断
リハビリテーション医学Vol.31(1994)No.8p578-582
https://www.jstage.jst.go.jp/article/jjrm1964/31/8/31_8_578/_pdf



《10》脳性麻痺
発生言語医学Vol.29(1988)No.4p374-376
https://www.jstage.jst.go.jp/article/jjlp1960/29/4/29_4_374/_pdf



《11》摂食動作時における口と手の協調運動‐手づかみ食べにおけるpick upから口唇での摂りこみまで‐
Mouth to hand coordination during finger feeding
昭和歯学会誌Vol.17(1997)No.1p.13-22
https://www.jstage.jst.go.jp/article/dentalmedres1981/17/1/17_13/_pdf
Hand to mouth coordination is one of the important behaviors when eating.

However, some people with feeding disorders are unable to coordinate well the mouth with the hand when feeding themselves, causing fatigue and aspiration.

The subjects were 20 healthy volunteers aged 22 to 35 with normal upper limb function and normal dental occlusion.

The food item used was a very small, round bite-sized cookie ("bolo" in Japanese).

The process of finger feeding was video-taped by two CCD cameras.

The picture was recorded to a Motion Grabber, an image processing system established by Ouyou Keisoku Corporation.

The movements were broken down into 1/60 seconds and the following segments were analyzed :

1. From picking up the food to placing it on the lip (P-I), 2. from picking up the food to opening of the mouth (P-O), 3. from opening the mouth to placing the food on the lip (O-I), 4. from picking up the food to pointing the food and the index finger perpendicular to the lip (P-V), and 5. from pointing the food and the index finger perpendicular to the lip to placing the food on the lip (V-I).

The results obtained were as follows :

1. There were statistically significant differences in length of movement, time length and speed between P-V and V-I.

2. The subjects could be classified into the following 3 groups:

Group 1) the length of movement for P-V is longer than P-O (13 subjects); Group 2) the length of movement for P-O is longer than P-V (2 subjects); and Group 3) the length of movement for P-V and P-O was changed by food locations (5 subjects).

3. Time length and speed for P-I in Group 2 were significantly longer and slower than those in Group 1



《12》ジストニアの診断と治療‐病態生理的アプローチ
Diagnosis and treatment of dystonia
https://www.jstage.jst.go.jp/article/clinicalneurol/48/11/48_11_844/_pdf
Diagnosis of dystonia is not difficult by recognizing the pattern of clinical presentation.

Dopa-responsive dystonia (DRD) and Wilson disease are important in differential diagnosis because of their specific treatment.

The most common are the focal dystonias, including blepharospasm and spasmodic torticollis.

Dystonia comprises mobile involuntary movements and abnormal postures, the latter is better described as hypokinetic disorder.

The pathogenesis of dystonia is now being clarified, and includes abnormal neuroplasticity caused by the relative excess of dopamine in the matrix compartment of the striatum, the possible primary lesion being the striosome.

In a dopa-responsive dystonia model, dopaminergic projection is more deficient to the striosome than to the matrix, which could produce imbalance between the direct versus. indirect pathway activities.

The treatment options include trihexyphenidyl, minor tranquilizers, botulinum toxin injection, and deep brain stimulation.



《13》中枢神経の障害(4)不髄意運動:特に脳性麻痺アテトーゼ型について
日本義肢装具学会誌Vol.16(2000)No.1p60-68
https://www.jstage.jst.go.jp/article/jspo1985/16/1/16_1_60/_pdf


《14》重度仮死後緊張型アテトーゼ型脳性麻痺の頭部MRI所見
脳と発達Vol.26(1994)No.3p276-277
https://www.jstage.jst.go.jp/article/ojjscn1969/26/3/26_3_276/_pdf


《15》脳性小児麻痺における筋拘縮と体幹四肢の変形
医療Vol.34(1980)No.Supplement P447-448
https://www.jstage.jst.go.jp/article/iryo1946/34/Supplement/34_Supplement_447/_pdf

・・・・・・・・・・

先週健診受けました
血圧107/73
B型肝炎、C型肝炎、梅毒、全て陰性
血糖値、脂質系、正常値