Sample records for athetosis

  1. 综合手法治疗痉挛及手足徐动型脑性瘫痪的初探%Comprehensive manual treatment of cerebral palsy with spasm and athetosis

    Institute of Scientific and Technical Information of China (English)

    张华; 李初阳


    @@ Background: Comprehensive manual treatment consists of Shangtian Zheng therapy and posture correction used in China ,which is effective in relieving cerebral palsy with spasm and athetosis. According to Shangtian Zheng principle, spasm and extreme tension are the main causes underlying cerebral palsy. So, extreme tension in hands and feet should be delimitated.

  2. Kinetografia: interpretação dos kinetogramas em um caso de atetose unilateral Kinetography: interpretation of the kinetograms in a case of unilateral athetosis

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    Milton B. Moreira


    Full Text Available O autor define e explica o que é kinetograma e a técnica de sua realização. Mostra que a movimentação exigida resulta do funcionamento de quase todo o sistema nervoso central através de um mecanismo interno complexo do qual participam vários circuitos nervosos. Adverte que as lesões localizadas nestes últimos acarretam deformações e desorganizações kinetográficas peculiares. Informa que a observação vem demonstrando que desde o início de algumas enfermidades sistematizadas do sistema nervoso central existem variações características nos traçados. Em um caso de atetose unilateral, por lesão do striatum contralateral, o autor procura explicar a ação vicariante do hemisfério cerebral homolateral íntegro, por ocasião dos movimentos simultâneos e sinérgicos das duas mãos. Responsabiliza a comissura de Meynert pela condução interpalidal da suplência verificada mediante os kinetogramas e expõe o circuito reverberante aberrante (extrapiramidal que entra em funcionamento nestas condições excepcionais.The author explains the kinetogram's technique and says that the necessary movimentation results from a complex mechanism where various nervous circuits participate, emphasizing that lesions located on these circuits produce peculiar kinetographic distortions. He points out that from the beginning of some diseases of the central nervous system there are characteristic changes on the tracings. In one case of unilateral athetosis the author tries to explain the vicariant action of the normal homolateral cerebral hemisphere during simultaneous movements of both hands. Probably the Meynert's commissure is responsible for the interpallidal conduction of the vicariant control verified through the kinetograms. The reverberating and aberrant circuit which takes action on these conditions is discussed.

  3. Genetics Home Reference: aromatic l-amino acid decarboxylase deficiency (United States)

    ... the limbs (athetosis). They may be lacking in energy (lethargic), feed poorly, startle easily, and have sleep disturbances. People with AADC deficiency may also experience episodes called oculogyric crises that involve abnormal rotation of the eyeballs; extreme ...

  4. Analysis of cranial CT-scan findings in cerebral palsy

    Energy Technology Data Exchange (ETDEWEB)

    Wada, F.; Andoh, T.; Une, K.; Takamatsu, T. (Kitakyushu Municipal Sogo-Ryoiku Center (Japan))


    CT-scan findings of 87 cerebral palsied children were studied. They consist of 23 cases of spastic quadriplegia, 9 cases of diplegia, 12 cases of paraplegia, 24 cases of athetosis and mixed type, and 19 cases of hemiplegia. In the former four types, ventricular dilatation and cortical atrophy were measured and abnormal changes in cerebral substance and cerebellar atrophy were observed. Spastic quadriplegia showed most intense changes in every aspect of the abnormalities, while paraplegia had almost normal appearance. Athetosis and mixed type had moderate changes. Hemiplegia always showed asymmetrical view on CT-scan, dilatation of lateral ventricle or atrophy of hemisphere in contralateral side being observed.

  5. Development of postural adjustments during reaching in infants with CP

    NARCIS (Netherlands)

    Hadders-Algra, M; van der Fits, IBM; Stremmelaar, EF; Touwen, BCL


    The development of postural adjustments during reaching movements was longitudinally studied in seven infants with cerebral palsy (CP) between 4 and 18 months of age. Five infants developed spastic hemiplegia, one spastic tetraplegia, and one spastic tetraplegia with athetosis. Each assessment consi

  6. Polyarticular juvenile idiopathic arthritis associated with Fahr′s syndrome

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    U Dundar


    Full Text Available Bilateral symmetric calcification involving striatum pallidum with or without deposits in the dentate nucleus, thalamus and white matter is commonly referred to as Fahr′s syndrome. Symptoms of the disorder may include deterioration of motor function, spasticity, spastic paralysis, dysarthria, dementia, seizures, headache and athetosis. The clinical and imaging abnormalities are restricted to the central nervous system (CNS. We report an unusual association of Fahr′s syndrome with polyarticular juvenile idiopathic arthritis in a girl.

  7. Biomechanical analysis of countermovement jump in people with cerebral palsy Análisis biomecánico del salto vertical con contramovimiento en personas con parálisis cerebral


    K. Gianikellis; Bote, A.; J. M.ª Pulido; Pérez, A.


    The main purpose of the study was to evaluate the patterns of the developed ground reaction forces in the two – legged countermovement jumping, performed by persons affected by tetraparesis with ataxia, tetraparesis with athetosis, tetraparesis with spasticity, diplegia with spasticity, right and left hemiplegia and, finally, right and left hemiparesis. After twenty subjects jumped on the surface of a force plate analysis of the ground reaction force &nd...

  8. CT cold areas in both putamens in cases with history of perinatal asphyxia

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    Ishizaki, Asayo; Maruyama, Hiroshi (Tokyo Women' s Medical Coll. (Japan))


    CT bilaterally showed a cold area in the putamen of 5 infants with cerebral palsy who had had asphyxia at birth. The etiology was discussed, and 4 of the cases were clinically studied. All four patients had convulsive tetraplegia, or convulsive bilateral paralysis with the element of athetosis. Three of them had a history of infantile epilepsy, accompanied by abnormal ocular movement. Two patients with tetraplegia showed marked hypotonia of the trunk in ventral support (Landau). Impairment of the bilateral putamens in the abnormal muscle tone was inferred.

  9. 颈总动脉交感神经网剥脱术治疗脑性瘫痪的探讨%Discussion of common carotid artery sympathetic nerve net exfoliation therapy treating cerebral palsy

    Institute of Scientific and Technical Information of China (English)

    尹彪中; 李如求; 成顺成


    Objective To discuss mechanism of common carotid artery sympathetic nerve net exfoliation therapy treating sequela of cerebral palsy. Methods Examing and assessing before and after operation, curative effect analysis, Wals value assay and 1 year follow up to 124 patients. Result Score of patients in this group reached excellent 1 week after operation, reached good 1 year after operation, Wals value measure P≤ 0.05. Conclusion This operation had an effect on the promotion of mixed type; athetosis type cerebral palsy, upper limb function disorder and malfunction.

  10. Movement disorders in cerebrovascular disease. (United States)

    Mehanna, Raja; Jankovic, Joseph


    Movement disorders can occur as primary (idiopathic) or genetic disease, as a manifestation of an underlying neurodegenerative disorder, or secondary to a wide range of neurological or systemic diseases. Cerebrovascular diseases represent up to 22% of secondary movement disorders, and involuntary movements develop after 1-4% of strokes. Post-stroke movement disorders can manifest in parkinsonism or a wide range of hyperkinetic movement disorders including chorea, ballism, athetosis, dystonia, tremor, myoclonus, stereotypies, and akathisia. Some of these disorders occur immediately after acute stroke, whereas others can develop later, and yet others represent delayed-onset progressive movement disorders. These movement disorders have been encountered in patients with ischaemic and haemorrhagic strokes, subarachnoid haemorrhage, cerebrovascular malformations, and dural arteriovenous fistula affecting the basal ganglia, their connections, or both.

  11. CT findings of cerebral palsy and behaviour development

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    Sakamoto, Zenji


    It is well recognized that CT scan is very useful in the early diagnosis of cerebral palsy. The author has studied this time the CT scan findings of cerebral palsy children in their relations to the type of palsy, cause of palsy, complications in the central nervous system, and prognosis of behaviour development, in order to predict the prognosis of behaviour development. Dilatation of the contralateral cerebral ventricle was found in 82 % of hemiplegic type. Abnormal EEG was found in 73 %, but their behaviour development was satisfactory, with good development of speech regardless to the side of palsy. This might be helped by compensational function of the brain due to plasticity. Diplegia presented bilateral moderate dilatation of ventricles with favorable prognosis. Tetraplegia was caused mostly by asphyxia or congenital anomaly and revealed marked dilatation of ventricles or severe cortical atrophy. Some cases presented diffuse cortical low-density, often associated with abnormal EEG, and their prognosis was worst. Athetosis had normal CT finding or mild ventricular dilatation, but all cases of ataxia presented normal CT findings. Hypotonia had mild ventricular dilatation. Two of three mixed type cases had normal CT findings and another had mild ventricular dilatation. No correlation was found between ventricular dilatation and behaviour development, but statistically significant difference was found in the cases with 30 % or more Evans' ratio (P < 0.05). Prognosis of severe ventricular dilatation cases was poor.

  12. CT findings of hereditary dentatorubral-pallidoluysian atrophy (DRPLA)

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    Tokiguchi, Susumu; Kurashima, Akihiko; Tsuchiya, Toshiaki; Ito, Jusuke; Naito, Haruhiko; Nagai, Hiroko; Wakabayashi, Masatoshi; Morita, Masahiro


    Hereditary dentatorubral-pallidoluysian atrophy (DRPLA) has recently been recognized as a clinicopathological entity. It may be defined as a multisystem degenerative disease of dominant inheritance, and characterized clinically by a combination of epilepsy, myoclonus, ataxia, dementia, and choreo-athetosis. This paper reports on the CT findings of ten patients (in four families) with DRPLA. In two families, the diagnosis was established on the basis of the clinicopathological findings, while in the other two, the diagnosis was made clinically. Although the CT findings were not identical in all patients, some degree of atrophic change was always observed in the cerebellum, brainstem, and cerebral cortex. Cerebellar atrophy was always accompanied by a dilatation of the fourth ventricle. Midbrain atrophy was characterized by a prominent tegmental atrophy and aqueductal dilatation, such as is seen in progressive supranuclear palsy. Of the four patients over 40 years of age, three had a diffuse hypodensity of the cerebral white matter on CT. To our knowledge, there have been no previous reports on this hypodensity in patients with spino-cerebellar degeneration or Huntington's chorea. CT may be helpful in the differential diagnosis of progressive neuro-degenerative disorders.

  13. Case of dentato-rubro-pallido-luysian atrophy. MRI findings and disturbance of ocular movement

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    Usui, Sadanari; Komiya, Tadatoshi


    A clinical case of dentato-rubro-pallido-luysian atrophy (DRPLA) was reported. We established several aspects on the basis of MRI findings and a neuro-otological study. A 47-year-old woman had gait disturbance, involuntary movements, speech disturbance, and memory disturbance at the age of 42. She was admitted to the hospital because of worsening of the gait disturbance. Neurological examinations showed choreo-athetosis of the face, neck and upper extremities, mental disturbance, and scanning speech. However, she had neither ocular disturbance nor epilepsy or myoclonus. On the MRI-CT, an atrophy of midbrain and pontine tegmentum was observed. The neuro-otological study showed gaze nystagmus at the horizontal gaze, rebound nystagmus, hypometria of the saccade, saccadic pursuit, reduction of the optokinetic nystagmus, and increase in caloric nystagmus by means of visual input. A severe atrophy of the brainstem tegmentum and a mild atrophy of the cerebellar hemisphere and cerebral cortex are regarded as neuro-radiological features of DRPLA. Moreover, tegmental atrophy is related to ocular disturbance as a clinical feature. Various neuro-otological findings reveal many systems of ocular movements, i.e., a smooth pursuit system, a saccade system, and a vestibulo-ocular reflex system, involving flocculus. DRPLA can be clinically diagnosed by means of clinical features, MRI findings, and neuro-otological findings. A variety of neuro-otological abnormalities may indicate a progression of the ocular disturbance and a variety of lesions.

  14. MR imaging of cerebral palsy

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    Saginoya, Toshiyuki [Urasoe General Hospital, Okinawa (Japan); Yamaguchi, Keiichiro; Kuniyoshi, Kazuhide [and others


    We evaluated 35 patients with cerebral palsy on the basis of MR imaging findings in the brain. The types of palsy were spastic quadriplegia (n=11), spastic diplegia (n=9), spastic hemiplegia (n=2), double hemiplegia (n=1), athetosis (n=10) and mixed (n=2). Of all patients, 28 (80%) generated abnormal findings. In spastic quadriplegia, although eight cases revealed severe brain damage, two cases showed no abnormal findings in the brain. One of the three had cervical cord compression caused by atlanto-axial subluxation. In spastic diplegia, the findings were divided according to whether the patient was born at term or preterm. If the patient had been born prematurely, the findings showed periventricular leukomalacia and abnormally high intensity in the posterior limbs of the internal capsule on T2-weighted images. MR imaging in spastic hemiplegia revealed cerebral infarction. In the athetoid type, half of all cases showed either no abnormal findings or slight widening of the lateral ventricle. Three cases showed abnormal signals of the basal ganglia. The reason why athetoid-type palsy did not show severe abnormality is unknown. We believe that MR imaging is a useful diagnostic modality to detect damage in the brain in cerebral palsy and plays an important role in the differentiation of cerebral palsy from the spastic palsy disease. (author)

  15. Selective lumbosacral posterior and anterior rhizotomy for mixed cerebral palsy%选择性腰骶脊神经后根+前根切断术治疗混合型脑瘫

    Institute of Scientific and Technical Information of China (English)

    王逢贤; 徐林; 曹旭; 俞兴; 穆晓红; 吴坤懂


    Objectives: To investigate the efficacy of selective posterior rhizotomy(SPR) with selective anterior rhizotomy (SAR) for the treatment of mixed cerebral palsy. Methods: 48 patients with an average age of 8.8 years old(3-22 years) were reviewed retrospectively from January 2004 to January 2010. There were 36 males and 12 females. All cases had mixed cerebral palsy, and all patients presented with spasm compliacted with lower limb athetosis, the muscular tension according to Ashworth spasticity scale was grade 3. Preopera-tive lower limb dysfunction included: walking independently in 36 cases, walking with assistance in 8 cases and standing with assistance in 4 cases. SPR with SAR was applied in all cases. The L5 and SI posterior roots in 38 cases and L4, L5 and SI posterior roots in 10 cases were cut off partly in accordance with the degree of lower limb spasticity by preoperativc physical exam. 30-50 percent of the posterior nerve roots were cut off. The L5 anterior roots in 28 cases and S1 anterior roots in 20 cases were cut off partly in accordance with the degree of athetosis. 30 percent of the anterior nerve roots were cut off. Laminae were maintained as far as possible so as not to violate the stability of spine. Results: Postoperative leg or foot numbness were noted in 2 cases, lower limb weakness in 3 cases, low back pain in 2 cases, lower back weakness in 5 cases, urine dysfunction in 1 case, all these were resolved by the corresponding intervention in six months. The average follow-up was 18 months, 48 cases with muscle tension at 1 week, 6 months, 18 months after operation improved significantly compared with preopeiation, and the spasm relief rate reached 100%. As for the lower limb athctosis: after 1 week, deterioration was noted in 1 case, which was controlled satisfactively after administration of sedative drugs and baclofen; unchanged in 4, and excellent in 43 cases; after 6 months, unchanged in 5 cases and excellent in 43 cases, no case was noted

  16. Wilson's disease: two treatment modalities. Correlations to pretreatment and posttreatment brain MRI

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    Leiros da Costa, Maria do Desterro [Federal University of Paraiba, Movement Disorders Unit, Paraiba (Brazil); Spitz, Mariana; Bacheschi, Luiz Alberto; Barbosa, Egberto Reis [University of Sao Paulo, Movement Disorders Unit, Sao Paulo (Brazil); Leite, Claudia Costa; Lucato, Leandro Tavares [University of Sao Paulo, Department of Radiology, Sao Paulo (Brazil)


    Brain magnetic resonance imaging (MRI) studies on Wilson's disease (WD) show lack of correlations between neurological and neuroimaging features. Long-term follow-up reports with sequential brain MRI in patients with neurological WD comparing different modalities of treatment are scarce. Eighteen patients with neurological WD underwent pretreatment and posttreatment brain MRI scans to evaluate the range of abnormalities and the evolution along these different periods. All patients underwent at least two MRI scans at different intervals, up to 11 years after the beginning of treatment. MRI findings were correlated with clinical picture, clinical severity, duration of neurological symptoms, and treatment with two different drugs. Patients were divided into two groups according to treatment: d-penicillamine (D-P), zinc (Zn), and Zn after the onset of severe intolerance to D-P. MRI scans before treatment showed, in all patients, hypersignal intensity lesions on T2- and proton-density-weighted images bilaterally and symmetrically at basal nuclei, thalamus, brain stem, cerebellum, brain cortex, and brain white matter. The most common neurological symptoms were: dysarthria, parkinsonism, dystonia, tremor, psychiatric disturbances, dysphagia, risus sardonicus, ataxia, chorea, and athetosis. From the neurological point of view, there was no difference on the evolution between the group treated exclusively with D-P and the one treated with Zn. Analysis of MRI scans with longer intervals after the beginning of treatment depicted a trend for neuroimaging worsening, without neurological correspondence, among patients treated with Zn. Neuroimaging pattern of evolution was more favorable for the group that received exclusively D-P. (orig.)

  17. Disorders of Upper Limb Movements in Ataxia-Telangiectasia.

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    Aasef G Shaikh

    Full Text Available Ataxia-telangiectasia is known for cerebellar degeneration, but clinical descriptions of abnormal tone, posture, and movements suggest involvement of the network between cerebellum and basal ganglia. We quantitatively assessed the nature of upper-limb movement disorders in ataxia-telangiectasia. We used a three-axis accelerometer to assess the natural history and severity of abnormal upper-limb movements in 80 ataxia-telangiectasia and 19 healthy subjects. Recordings were made during goal-directed movements of upper limb (kinetic task, while arms were outstretched (postural task, and at rest. Almost all ataxia-telangiectasia subjects (79/80 had abnormal involuntary movements, such as rhythmic oscillations (tremor, slow drifts (dystonia or athetosis, and isolated rapid movements (dystonic jerks or myoclonus. All patients with involuntary movements had both kinetic and postural tremor, while 48 (61% also had resting tremor. The tremor was present in transient episodes lasting several seconds during two-minute recording sessions of all three conditions. Percent time during which episodic tremor was present was greater for postural and kinetic tasks compared to rest. Resting tremor had higher frequency but smaller amplitude than postural and kinetic tremor. Rapid non-rhythmic movements were minimal during rest, but were triggered during sustained arm postures and goal directed arm movements suggesting they are best considered a form of dystonic jerks or action myoclonus. Advancing age did not correlate with the severity of involuntary limb movements. Abnormal upper-limb movements in ataxia-telangiectasia feature classic cerebellar impairment, but also suggest involvement of the network between the cerebellum and basal ganglia.

  18. Diagnosis, treatment, and prevention of cerebral palsy. (United States)

    O'Shea, Thomas Michael


    Cerebral palsy is the most prevalent cause of persisting motor function impairment with a frequency of about 1/500 births. In developed countries, the prevalence rose after introduction of neonatal intensive care, but in the past decade, this trend has reversed. A recent international workshop defined cerebral palsy as "a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain." In a majority of cases, the predominant motor abnormality is spasticity; other forms of cerebral palsy include dyskinetic (dystonia or choreo-athetosis) and ataxic cerebral palsy. In preterm infants, about one-half of the cases have neuroimaging abnormalities, such as echolucency in the periventricular white matter or ventricular enlargement on cranial ultrasound. Among children born at or near term, about two-thirds have neuroimaging abnormalities, including focal infarction, brain malformations, and periventricular leukomalacia. In addition to the motor impairment, individuals with cerebral palsy may have sensory impairments, cognitive impairment, and epilepsy. Ambulation status, intelligence quotient, quality of speech, and hand function together are predictive of employment status. Mortality risk increases incrementally with increasing number of impairments, including intellectual, limb function, hearing, and vision. The care of individuals with cerebral palsy should include the provision of a primary care medical home for care coordination and support; diagnostic evaluations to identify brain abnormalities, severity of neurologic and functional abnormalities, and associated impairments; management of spasticity; and care for associated problems such as nutritional deficiencies, pain, dental care, bowel and bladder continence, and orthopedic complications. Current strategies to decrease the risk of cerebral palsy include interventions to

  19. Recessive C10orf2 mutations in a family with infantile-onset spinocerebellar ataxia, sensorimotor polyneuropathy, and myopathy. (United States)

    Park, Mi-Hyun; Woo, Hae-Mi; Hong, Young Bin; Park, Ji Hoon; Yoon, Bo Ram; Park, Jin-Mo; Yoo, Jeong Hyun; Koo, Heasoo; Chae, Jong-Hee; Chung, Ki Wha; Choi, Byung-Ok; Koo, Soo Kyung


    Recessive mutations in chromosome 10 open reading frame 2 (C10orf2) are relevant in infantile-onset spinocerebellar ataxia (IOSCA). In this study, we investigated the causative mutation in a Korean family with combined phenotypes of IOSCA, sensorimotor polyneuropathy, and myopathy. We investigated recessive mutations in a Korean family with two individuals affected by IOSCA. Causative mutations were investigated using whole exome sequencing. Electrophysiological analyses and muscle and nerve biopsies were performed, along with magnetic resonance imaging (MRI) of the brain and lower extremities. Compound heterozygous mutations c.1460C>T and c.1485-1G>A in C10orf2 were identified as causative of IOSCA. Skeletal muscle showed mitochondrial DNA (mtDNA) deletions. Both patients showed a period of normal development until 12-15 months, followed by ataxia, athetosis, hearing loss, and intellectual disability. Electrophysiological findings indicated motor and sensory polyneuropathies. Muscle biopsy revealed variations in the size and shape of myofibers with scattered, small, and angulated degenerating myofibers containing abnormal mitochondria; these observations are consistent with myopathy and may be the result of mtDNA deletions. Sural nerve biopsy revealed an axonal neuropathy. High-signal-intensity lesions in the middle cerebellar peduncles were correlated with clinical severity, and MRI of the lower legs was compatible with the hypothesis of length-dependent axonal degeneration. We identified novel compound heterozygous mutations of the C10orf2 gene as the cause of IOSCA with sensorimotor polyneuropathy and myopathy. Signs of motor neuropathy and myopathy were discovered for the first time in IOSCA patients with C10orf2 mutations. These results suggest that the clinical spectrum of IOSCA caused by C10orf2 mutations may be more variable than previously reported.

  20. 选择性腕掌部肌力平衡术治疗脑瘫手部畸形%Muscle balance operation for the treatment of spastic cerebral palsy of hand deformity

    Institute of Scientific and Technical Information of China (English)

    孙立泉; 于嘉智; 刘涛


      目的:探讨痉挛性脑瘫手部畸形的手术改进方法,最大限度改善患儿手部的正常功能。方法:采取患儿手部本身原有软组织的肌腱滑行延长和肌内延长方法,达到伸屈腕肌力的平衡恢复手的基本功能位。结果:2006~2011年收治24例手畸形患儿,术后随访1~4年,效果良好,无复发,家长满意。结论:肌力平衡术治疗痉挛性脑瘫手部畸形适合4岁以上并且能主动配合功能锻炼的4岁以上患儿,手足徐动型不排除在指征之外。%Objective: To study the effect of Improved surgery methods of spastic cerebral palsy in children with hand deformity , which may improve hand function maximatily. Methods:24 infants with hand deformity, who attended our hospital from 2006 to 2011, were treated by surgical methods of autologous tendon gliding extended and intramuscular lengthening,with a purpose of muscle strength balance of wrist and hand basic function recovery. Results:All of the infants had good effect after treatment, and none of the babies had recurrence, which were followed up for 1-4 years. Conclusion:Muscle balance operation for the treatment of spastic cerebral palsy of hand deformity were suitable for patients who were more than 4years old and could take the initiative to cooperate with functional exercise. Athetosis was not excluded from the indications.

  1. Fahr’s disease: a case series

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    Osama Shukir Muhammed Amin


    Full Text Available Fahr’s disease is a rare idiopathic bilateral and symmetrical calcification of the basal ganglia, thalami, subcortical hemispheric white matter, and deep cerebellar nuclei that usually presents between the 4nd and 6th decade of life with a variable combination of involuntary movements, Parkinsonism, presenile subcortical dementia, seizures, and ataxia. This longitudinal observational case series was conducted at the neurology outpatients’ department of Sulaimaniya general teaching hospital, Iraq. Three patients were diagnosed with Fahr’s disease. Their chief presenting complaint and other coexistent clinical features were noted and followed-up for at least one year. The ages of those consecutive patients were 25, 34, and 21 years, respectively. Two were females and the other patient was a male. The chief presenting complaint among the 3 patients was heterogeneous; cognitive impairment, seizures, and chorea, respectively. At the time of diagnosis, Parkinsonism and cognitive decline were present in all patients. The 3 patients never developed dystonia, dyskinesia, or athetosis and one patient only had mild cerebellar ataxia. Seizures were the presenting feature in one patient and they never developed in the other 2 patients. All patients had a variable degree of intracerebral calcification. Fahr’s disease has heterogeneous phenotypes and the brain radiological findings do not predict the clinical presentation and course. Although Parkinsonism was not the presenting feature, it was found in all patients at the time of diagnosis. Involuntary movements and cerebellar dysfunction were uncommon and the cognitive impairment was of the frontal lobe subcortical dysfunction. [Cukurova Med J 2013; 38(4.000: 823-831

  2. Door-to-door survey of major neurological disorders (project in Al Quseir City, Red Sea Governorate, Egypt

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    El Tallawy HN


    Full Text Available Hamdy NA El Tallawy,1 Wafaa MA Farghaly,1 Tarek A Rageh,1 Ghaydaa A Shehata,1 Reda Badry,1 Nabil A Metwally,2 Esam A El Moselhy,2 Mahmoud Hassan,2 Mohamed A Sayed,3 Ahmed A Waris,1 Yaser Hamed,2 Islam Shaaban,2 Mohamed A Hamed,1 Mahmoud Raafat Kandil11Department of Neurology, Faculty of Medicine, Assiut University, Assiut, Egypt; 2Department of Neurology and Public Health, Faculty of Medicine, Al-Azhar University (Assiut branch, Assiut, Egypt; 3Department of Neurology, Faculty of Medicine, Sohag University, Sohag, EgyptAbstract: A door-to-door survey, including every household, was conducted for all inhabitants of Al Quseir City (33,283, Red Sea Governorate, Egypt by three specialists of neurology as well as nine senior staff members of neurology and 15 female social workers to assess the epidemiology of major neurological disorders. Over six phases, from July 1, 2009 to January 31, 2012, screening of all eligible people in the population was carried out, by which case ascertainment of all major neurological disorders included in the study was done according to the accepted definitions and diagnostic criteria of the World Health Organization. The order of frequency of prevalence of the studied neurological disorders was dementia (3.83% for those aged > 60 years, migraine (2.8% for those aged > 8 years, stroke (6.2/1000 for those aged > 20 years, epilepsy (5.5/1000, Parkinson’s disease (452.1/100,000 for those aged > 40 years, cerebral palsy (3.6/1000 among children 37 years, chorea (21.03/100,000, athetosis (15/100,000, and multiple sclerosis (13.74/100,000. The incidence rates of stroke, epilepsy, and Bell’s palsy were 181/100,000, 48/100,000, and 98.9/100,000 per year, respectively.Keywords: prevalence, incidence, neurological disorders

  3. [NMDA receptor encephalitis in the course of recurrent CNS demyelinating disorders: a case report]. (United States)

    Yamamoto, Masanari; Kokubun, Norito; Watanabe, Yuka; Okabe, Ryuta; Nakamura, Toshiki; Hirata, Koichi


    We present the case of a 31-year-old woman who developed N-methyl-d-aspartate (NMDA) receptor encephalitis during the course of relapsing and remitting multiple brain lesions. The patient developed a tingling sensation in the left upper and lower extremities, and was first admitted to our hospital at age 27. She was tentatively diagnosed with multiple sclerosis on the basis of multiple lesions with Gd-enhancement in the brainstem, and 2 separate clinical relapses by age 28. At age 31, she developed a headache and pyrexia, followed by confusion and abnormal behavior. Her symptoms acutely progressed to stupor, and subsequently, she developed oral dyskinesia and athetosis-like involuntary movement of the left arm. The stupor state continued over 2 months. However, she had completely recovered by 3 months after the onset of psychiatric symptoms. Her serum and CSF samples tested positive for anti-NMDA receptor antibodies, and she was diagnosed with NMDA receptor encephalitis. Her serum was negative for anti-AQP4 antibody, but showed weak positivity for antinuclear antibody. Between ages 32 and 34, she experienced 2 clinical relapses, including right-hand clumsiness, confusion, aphasia, and dysphagia. FLAIR images showed a high-intensity area in the brain stem, thalamus, and subcortical white matter. No tumors were found throughout the course. A clinical entity of NMDA receptor encephalitis can include various neurologic disorders, such as the development of recurrent demyelinating brain lesions. Further investigation is required to clarify the pathophysiological role of anti-NMDA receptor antibody in our patient.

  4. Biomechanical analysis of countermovement jump in people with cerebral palsy Análisis biomecánico del salto vertical con contramovimiento en personas con parálisis cerebral

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    K. Gianikellis


    Full Text Available

    The main purpose of the study was to evaluate the patterns of the developed ground reaction forces in the two – legged countermovement jumping, performed by persons affected by tetraparesis with ataxia, tetraparesis with athetosis, tetraparesis with spasticity, diplegia with spasticity, right and left hemiplegia and, finally, right and left hemiparesis. After twenty subjects jumped on the surface of a force plate analysis of the ground reaction force – time trend took place. The obtained results confirm the accomplishment of the biomechanical principle of the initial force as in the case of persons not affected by neurological disorders. Finally the calculated ratio between the breaking impulse and the acceleration impulse is very near to optimum values.
    KEY WORDS: cerebral palsy, biomechanics, countermovement jump.


    El valor óptimo del impulso de aceleración en el salto vertical con contra -movimiento, es aproximadamente tres veces superior al impulso de “frenaje”. El objetivo de este estudio ha sido investigar el modo de actuación de la cadena biocinemática del tren inferior en el salto vertical con contra -movimiento para personas afectadas por parálisis cerebral, concretamente, tetraparesia con ataxia, tetraparesia con atetosis, tetraparesia con espasticidad, diplegia con espasticidad, hemiplegia izquierda y derecha, y finalmente, hemiparesia izquierda y derecha. Para ello, se han analizado las fuerzas de reacción de veintitrés sujetos que han realizado saltos con contramovimiento sobre una plataforma de fuerzas. El hallazgo de un valor medio de la ratio entre el impulso de “frenaje” y el de aceleración muy próximo al valor teórico para los veintitrés sujetos con diferentes síntomas de parálisis cerebral, confirma el principio de la fuerza inicial mientras que el alto coeficiente de variación de los parámetros utilizados en este estudio confirma la esperada

  5. 小儿脑瘫危险因素与合并症相关分析%Correlation Analysis of Risk Factors and Complications on Children Cerebral Palsy

    Institute of Scientific and Technical Information of China (English)

    王军英; 伍俊妮; 张惠佳; 汤清波; 覃蓉; 颜华; 熊毛伟; 易慧娟; 肖曙光; 王跑球


    Objective To investigate the risk factors related with children cerebral palsy (CP), the complications and the constituent ratio of types of cerebral palsies.Methods Retrospectively study 1204 cases of affected children' s clinical data from January 2005 to December 2009, using the statistical method of chi-square criterion, the relationships of the high risk factors and the complications resulted in children cerebral palsies were investigated, and the relationships of the incidence rate of types of CP constituent ratio and the complications were studied as well.Results There presented risk factors of 1044 in 1204 cases (86.71%).The major risk factors by turns were asphyxiation, preterm low weight, other un-definite factors, choloplania, intrauterine infection, superfoetation/twins, intracranial hemorrhage and the abnormal umbilical core.The rate of the CP children accompanied with complications was 91.35% (1102/1204), the incidence rate with mental retardation was 72.09% (868 cases), with language disturbance 43.52% (524 cases), with epilepsy 22.26% (268 cases), with hearing disturbance 22.09% (266 cases), with ingestion difficulty 58.47% (702 cases),with optic atrophy 25.75 % (310 cases), with hydrostomia 75.91% (914 cases), and there were statistical differences among different types of CP (P< 0.05), the three highest incidence rates was spasmus, athetosis and nixed types of CP by turns.And there were significant difference in statistics ( P < 0.01 ).Conclusions The CP was a kind of sophisticated disorder resulted from the lesion of brain, the affected presented a series of developmental disturbance.During the treatment, we should be pay attention to the entire rehabilitation for affected children.%目的 分析小儿脑瘫危险因素、脑瘫分型与合并症相关性.方法 回顾性调查2005年1月-2009年12月期间的1 204例脑瘫患儿临床资料,了解脑瘫高危因素与合并症的关系以及与脑瘫分型的发生率,用

  6. 脑性瘫痪的临床与MRI表现分析%Clinical and magnetic resonance imaging features findings of cerebral palsy

    Institute of Scientific and Technical Information of China (English)

    周陶成; 童光磊; 张敏; 李司南; 李红


    [Objective] To investigate the relationship between the brain MRI and cerebral palsy (CP) types, gesta-tional age,age and risky factors. [Method] All the 184 hospitalized CP cases were examined neurologically and their perinatal history were reviewed. [Results] MRI abnormalities were found in 163 of the 184 cases and abnormal rates of spastic diplegia,tetraplegia,hemiplegia,athetosis,hypotonia,ataxia and mixed were 82. 1 % , 100% , 100% ,80. 0%,80. 6%, 100% and 81.8%, respectively. Spastic tetraplegia was associated with extensive, bilateral, diffuse brain damage. 38 of 55 (69. 1%) children with spastic diplegia had significant periventricular leukomalacia(PVL). Of the 20 children with hemiple-gia,18 had unilateral lesions on neuroimaging. In different age groups,children less than 1 year of age had the highest rate of MRI abnormalities (93. 6%) ,brain MRI abnormality rate decreased gradually with age. Risky factors in the higher rate of MRI abnormalities were:low birth weight,prematurity and hypoxia asphyxia. [Conclusion] MRI changes are closely related to CP types,gestational age,age and risky factors.%[目的] 探讨脑性瘫痪(脑瘫)患儿脑MRI的表现及其与脑瘫类型、出生胎龄、年龄、高危因素之间的关系.[方法] 回顾性分析184例脑瘫患儿的临床与MRI表现. [结果] 184例脑瘫患儿MRI异常率为88.6%,早产和足月儿组MRI异常率分别为95.9%和83.6%.痉挛型双瘫、四肢瘫、偏瘫、肌张力低下型、不随意运动型、共济失调型和混合型脑瘫MRI异常率分别为82.1% 、100%、100%、80.6%、80.0% 、100%和81.8%.各类型脑瘫的MR1异常表现不同:痉挛性四肢瘫的异常MRI表现广泛、弥漫的两侧脑损伤,痉挛性双瘫以脑室周围白质软化(periventricular leukomala-cia,PVL)为主(56.7%),不随意运动型多有基底节区病变(75%),共济失调型均有小脑发育不良,偏瘫型多为单侧脑损伤(90%).不同出生胎龄的MRI特点不

  7. 戊二酸尿症1型28例的临床与实验室特征%Clinical and laboratory studies on 28 patients with glutaric aciduria type 1

    Institute of Scientific and Technical Information of China (English)

    王峤; 丁圆; 刘玉鹏; 李溪远; 吴桐菲; 宋金青; 王玉洁; 杨艳玲


    Objective To investigate the clinical,biochemical and genetic profiles of 28 Chinese patients with glutaric aciduria type 1.Method Twenty-eight patients with glutaric aciduria type 1 seen in the Department of Pediatrics,Peking University First Hospital from July 2003 to October 2013 were studied.The data of clinical course,laboratory examinations,cranial MRI and GCDH gene mutations of the patients were analyzed.Result (1) Three cases were detected by newborn screening,and the other patients were diagnosed at the age of 2 months to 17 years.(2) 22 patients (79%) were infant onset cases with psychomotor retardation,dystonia,seizures,athetosis,recurrent vomiting,drowsiness or feeding difficulty.Only two of the 22 patients with infant onset got normal intelligence and movement after treatment.Twenty of them were improved slowly with delayed development,dystonia and other neurological problems.Three patients (11%) had late onset.They had motor regression,headache and seizure at the age of 8,9 and 17 years,respectively.Rapid improvement was observed after treatment.(3) Cranial MRI has been checked in 23 patients ; 22 of them showed characteristic widening of the Sylvian fissure,abnormalities of the basal ganglia,leukoencephalopathy and brain atrophy.Thirty-five mutations in GCDH gene of the patients were identified; c.148T > C (p.W50R)was the most common mutation with the frequency of 7.7% ; 6 mutations (c.628A > G,c.700C >T,c.731G > T,c.963G > C,c.1031C > T and c.1109T > C) were novel.Conclusion Glutaric aciduria type 1 usually induced neurological deterioration resulting in severe psychomotor retardation and dystonia.Most of our patients were clinically diagnosed.Patients with early onset usually remained having neurological damage.Phenotype and genotype correlation has not been found in the patients.Neonatal screening for organic acidurias should be expanded in China.%目的 分析戊二酸尿症1型患者的临床、生

  8. Associations between Manual Abilities, Gross Motor Function, Epilepsy, and Mental Capacity in Children with Cerebral Palsy

    Directory of Open Access Journals (Sweden)



    Full Text Available How to Cite This Article: Gajewska E, Sobieska M, Samborski W. Associations between Manual Abilities, Gross Motor Function, Epilepsy, and Mental Capacity in Children with Cerebral Palsy. Iran J Child Neurol. 2014 Spring 8(2:45-52.ObjectiveThis study aimed to evaluate gross motor function and hand function in children with cerebral palsy to explore their association with epilepsy and mental capacity. Material & MethodsThe research investigating the association between gross and fine motor function and the presence of epilepsy and/or mental impairment was conducted on a group of 83 children (45 girls, 38 boys. Among them, 41 were diagnosedwith quadriplegia, 14 hemiplegia, 18 diplegia, 7 mixed form, and 3 athetosis.A neurologist assessed each child in terms of possible epilepsy and confirmed diagnosis in 35 children. A psychologist assessed the mental level (according toWechsler and found 13 children within intellectual norm, 3 children with mild mental impairment, 18 with moderate, 27 with severe, and 22 with profound.Children were then classified based on Gross Motor Function Classification System and Manual Ability Classification Scale.ResultsThe gross motor function and manual performance were analysed in relation to mental impairment and the presence of epilepsy. Epilepsy was found to disturb conscious motor functions, but also higher degree of mental impairment wasobserved in children with epilepsy.ConclusionThe occurrence of epilepsy in children with cerebral palsy is associated with worse manual function. The occurrence of epilepsy is associated with limitations in conscious motor functions. There is an association between epilepsy in children with cerebral palsy and the degree of mental impairment.The occurrence of epilepsy, mainly in children with hemiplegia and diplegia is associated with worse mental capacities.ReferencesRichards CL, Malouin F. Cerebral palsy: definition, assessment and rehabilitation. Handb Clin Neurol

  9. 苯海索治疗不同亚型不随意运动型脑瘫患儿运动功能的疗效观察

    Institute of Scientific and Technical Information of China (English)

    张峰; 冯珍


    the target dose of 4 mg / d, daily dose divided into 2 or 3 times service. Respectively into the group, after treatment (6 months after), the were GMFM, GMFCS, FMFM and WeeFIM assessment, and record the salivation and drug resistance. Results The three groups after treatment in children with GMFM score [group A (35.490±19.327), group B (29.110±11.864), group C (15.540±11.598)] have significant difference (F=4.988, P<0.05), group AB and group C comparison were statistically significant (P<0.05), between the group AB compared with no significant difference. Have statistical difference (F=4.319, P<0.05) of three groups of children with WeeFIM scores [group A (49.000±22.520), group B (38.780±11.914), group C (27.000±12.179)], group A and group B with the score of patients compare with group C had significant difference (P<0.05), and in the groups A and B of children with no significant difference. There was no significant difference in GMFCS and FMFM between the three groups. Three groups of children with salivation were improved, no significant difference. The athetoid-spastic cerebral palsy children can be observed exacerbated with spastic. Conclusion Benzhexol hydrochloride in the treatment of chorea-athetoid and athetosis type of dyskinetic cerebral palsy (CP) is helpful to improve the motor function and the ability to live independently, curative effect is better than that of dystonic cerebral palsy children. There was no significant difference between the three groups in the classification of General motor function and the improvement of the Fine motor function.