Introduction: Schizencephaly is a rare congenital malformation of cerebral cortical development. Epilepsia partialis continua and movement disorders often display abnormal movements with overlapping phenomenology in schizencephaly Case Report: A 6-year-old-girl with normal prenatal and labor history, presented with continuous left hand movement since 1-year-old. Neurological examination showed left spastic hemiparesis. Electroencephalography (EEG) showed sharp and spike wave in right temporoparietooccipital, frontocentrotemporal and centroparietal region. Cerebral magnetic resonance imaging (MRI) showed a cleft in right frontal lobe extending to the right lateral ventricle classified as open-lip schizencephaly, and an agenesis of septum pellucidum leads to monoventricular features, and polymicrogyria. She was treated with valproic acid, haloperidol, and regular physiotherapy. Conclusion This patient was diagnosed with schizencephaly from the anamnesis, physical examination, EEG, and cerebral MRI. The therapy of this patient was pharmacological treatment and physiotherapy.
Athetosis

Our experienced is based on a series of 5 cases of dystonia who underwent stereotaxic thalamotomy. The immediate good results have been obtained by thalamotomy. We have been able to estimate secondary deterioration on long term results in patients examined 2-5 years postoperatively. Some deterioration is noted patients operated on for athetosis over the age of 20. A pallido-subthalamic lesion is efficient at the beginning of the disease course since it improves motor performance and thereby helps possiblities of intellectual acquirement. Bilateral lesions have done after improved. I.Q. Effects of this treatment specially on spasticity must be discussed among other types of surgery.
Athetosis ; Dystonia* ; Humans ; Muscle Spasticity

The purpose of this study is to evaluate the effects of intramuscular botulinum toxin A injection in cerebral palsy. We studied 25 children with cerebral palsy(age 3 to 20 years old). Among them, 14 children were spastic diplegia; 5 were athetoid quadriplegia; 3 were spastic hemiplegia; and 3 were mixed type(spasticity and athetosis). Botulinum toxin A was injected into the target muscle groups with electromyographic guidance. The dose was calculated in unit/body weight basis. Followup assessments were performed at 1 and 3 months after injection. After injection, 11 out of 16 children(68.8%) had a one-level improvement in ambulatory status. The passive range of joint motion increased significantly after injection. Modified Ashworth scale decreased significantly after injection. In 23 out of 25 children, there were a significant increase of the mean GMFM(gross motor function measure) total score and mean GMFM scores for all dimensions at three months after injection. There were the distant effects after injection in spastic diplegia. In conclusion, botulinum toxin A tretment would improve the motor function and ambulatory status in cerebral palsy by reducing hypertonicity, spasticity, dynamic contracture and athetoid movement.
Athetosis ; Botulinum Toxins* ; Cerebral Palsy* ; Child ; Contracture ; Follow-Up Studies ; Hemiplegia ; Humans ; Joints ; Muscle Spasticity ; Quadriplegia

OBJECTIVE: To evaluate the relationship of the respiration patterns and the abdominal muscle activities during various speech productions in the adults with athetoid cerebral palsy (CP). METHOD: Subjects were 7 athetoid CPs and 7 healthy controls. Respirography and abdominal muscle activities were recorded by use of the pneumobelt and the surface EMG during their performances of six kinds of non-speech and speech tasks. RESULTS: The followings were observed in the athetoid CP. 1) Respiratory phase was irregular and variable in the intensity and period of revolution. The frequency of the respiratory phase was not different from the control in usual respiration, but higher in deep breathing. The time to persist one vowel phonation was shorter. 2) Inappropriate and frequent inspirations were occurred during speech task and the number of syllables was fewer and irregular for one speech breath. 3) The abdominal muscle activities were abrupt, irregular, inconsistent with the respiratory phase during all kinds of non-speech and speech tasks. CONCLUSION: These findings suggest that the abnormal respiration patterns and abdominal muscle activities contribute to the abnormal speech production of athetoid CP and their incoordination is similar to athetoid movement of the extremities. We should consider the athetoid speech production as well as the dysarthria in the athetoid CP.
Abdominal Muscles* ; Adult ; Ataxia ; Athetosis ; Cerebral Palsy ; Dysarthria ; Extremities ; Humans ; Phonation ; Respiration*

Athetosis ; chemically induced ; therapy ; Benzene ; poisoning ; Humans ; Male ; Nitriles ; poisoning ; Pyrethrins ; poisoning ; Young Adult

Paroxysmal kinesigenic choreoathetosis (PKC) is characterized by short paroxysms of focal or generalized involuntary movement induced by sudden movements, and is a well-known disease in the neurologic literature, but only 4 cases have been reported in Korea. The purpose of the presentation is to clarify the clinical features of PKC in Korea. We clinically analyzed 20 patients with PKC between 1986 and 1994 at Yongdong Severance Hospital, Yonsei Medical Center, with a minimum of a 1 to 2 year follow-up period. There were 14 men and 6 women. The age at onset of the condition ranged from 8 to 17 years (mean, 13.1 years). Six patients (30%) had a family history of the condition and the mode of inheritance was suggestive of an autosomal recessive pattern. The involuntary movements seemed to be dystonic rather than choreoathetonic upon a mild attack, and the paroxysms were precipitated by sudden movements. The attacks occurred on one or both sides, and were often associated with dysarthria, upward gaze and sensory aura. Consciousness was never lost. Their duration were usually 10 to 30 seconds, and never more than two minutes. All laboratory tests including electroencephalographic and neuroimaging studies showed no abnormality. All patients responded well to diphenylhydantoin. PKC is not rare in Korea and has a benign course.
Adolescent ; Athetosis/drug therapy/*physiopathology ; Child ; Chorea/drug therapy/*physiopathology ; Female ; Human ; Male ; *Movement ; Phenytoin/therapeutic use ; Recurrence

Neurologic complications such as mental status changes, incontinence, gait disturbances, extrapyramidal symptoms may be delayed days or weeks after exposure to carbon monoxide but hyperkinesia such as choreoballism and athetosis rarely occurs as symptoms of delayed neurologic complication of carbon monoxide intoxication. Neuroleptic malignant syandrome (NMS) is a rare, potentially fatal drug-induced disorder associated with hyperthermia, muscle rigidity, autonomic instability, mental dysfunction and various dyskinesia. Various major neuroleptic drugs seem to be its culprits. We describe a 24-year-old female patient who presented with NMS following administration of haloperidol for choreoballism, a symptom of delayed neurologic sequelas after carbon monoxide intoxication, with a review of the literature.
Antipsychotic Agents ; Athetosis ; Carbon Monoxide* ; Carbon* ; Dyskinesias ; Female ; Fever ; Gait ; Haloperidol ; Humans ; Hyperkinesis ; Muscle Rigidity ; Neuroleptic Malignant Syndrome* ; Young Adult

Brain-lung-thyroid syndrome is a rare autosomal dominant disorder. More than 100 cases have been reported worldwide, but few cases have been reported in China. In December 2018, a boy with brain-lung-thyroid syndrome, aged 3 years and 10 months, was admitted to Xiangya Hospital of Central South University due to repeated cough for more than 3 years. In infancy of the boy, psychomotor retardation, repeated cough, and hypothyroidism were found. Gene detection showed that there was c.927delc heterozygous variation in NKX2-1 gene (NM-001079668: exon3: c.927delC). The variation of this gene locus has not been reported in relevant literature so far, which indicates a new mutation. According to the above clinical manifestations and examination results, the boy was diagnosed as brain-lung-thyroid syndrome, which mainly characterized by nervous system disorders, accompanied by respiratory manifestations and hypothyroidism. The boy was treated with oral dopasehydrazine to relieve tremor and levothyroxine sodium tablets to relieve hypothyroidism. Anti-infection, atomization, rehabilitation training and other symptomatic supporting treatment were also administered. The boy's language and movement have improved, the thyroid hormone level is normal, and there are still repeated respiratory tract infections.
Athetosis/genetics* ; Chorea ; Congenital Hypothyroidism/genetics* ; Cough ; Humans ; Male ; Respiratory Distress Syndrome, Newborn ; Thyroid Nuclear Factor 1/genetics*

Extrapyramidal tract motor disorder in calcification of basal ganglia probably occurs when the deposition of acid mucopolysaccharides in the basal ganglia is severe enough to lead to neuronal loss. Basal ganglia calcification has been noted to occur with higher incidence and intensity in encephalitis lethargica, carbon monocide intoxication, anoxia, tuberous sclerosis, toxoplasmosis, hypothyroidism and hypoparathyroidism. The neurologic disorder is frequently reversible with treatment in patients with basal ganglia calcification who have hypoparathy-roidism. We report a patient with idiopathic hypoparathyroidism associated with bilateral calcification of the basal ganglia and athetoid movement. This is the first case report in Korea.
Anoxia ; Athetosis* ; Basal Ganglia* ; Carbon ; Cefonicid ; Encephalitis ; Extrapyramidal Tracts ; Glycosaminoglycans ; Humans ; Hypoparathyroidism* ; Hypothyroidism ; Incidence ; Korea ; Nervous System Diseases ; Neurons ; Toxoplasmosis ; Tuberous Sclerosis

Pelizaeus-Merzbacher disease (PMD) is a rare sudanophilic leukodystrophy with a reduced number of mature oligodendrocytes as well as diffuse central nervous system hypomyelination (dysmyelination) due to abnormal synthesis of proteolipid protein. PMD is characterized with pendular nystagmus, stridor, delay in psychomotor development, hypotonia, ataxia, athetosis and extrapyramidal signs. Abnormal high signal intensity is shown in the entire white matter of cerebrum and cerebellum at early stage by T2-weighted magnetic resonance imaging (MRI). We report two cases of PMD diagnosed with characteristic clinical manifestations and brain MRI findings.
Ataxia ; Athetosis ; Brain ; Central Nervous System ; Cerebellum ; Cerebrum ; Magnetic Resonance Imaging ; Muscle Hypotonia ; Nystagmus, Pathologic ; Oligodendroglia ; Pelizaeus-Merzbacher Disease* ; Respiratory Sounds