Sample records for athetosis
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Directory of Open Access Journals (Sweden)
Douglas J. Lanska
2013-03-01
Full Text Available Background: Since the description of athetosis in 1871 by American neurologist William Alexander Hammond (1828-1900 the disorder has been a source of controversy, as were many aspects of Hammond’s career. Methods: Review of controversies in the semi-centennial since the description of athetosis. Results: Hammond struggled to establish athetosis as a distinct clinic-pathological entity, and had successfully predicted the striatal pathology in his initial case (albeit somewhat serendipitously. Athetosis was, nevertheless, considered by many neurologists to be a form of post-hemiplegic chorea or part of a continuum between chorea and dystonia. European neurologists, and particularly the French, initially ignored or discounted the concept. Additional controversies arose over whether the movements persisted during sleep, whether athetosis was, or could be, associated with imbecility or insanity, and how it should be treated. Discussion: Some controversies concerning athetosis served to identify areas where knowledge was insufficient to make accurate statements, despite prior self-assured or even dogmatic statements to the contrary. Other controversies illustrated established prejudices, even if these biases were often only apparent with the greater detachment of hindsight.
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Analysis of cranial CT-scan findings in cerebral palsy
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Wada, F.; Andoh, T.; Une, K.; Takamatsu, T. (Kitakyushu Municipal Sogo-Ryoiku Center (Japan))
1981-06-01
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.
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Analysis of cranial CT-scan findings in cerebral palsy
International Nuclear Information System (INIS)
Wada, Fumio; Andoh, Tadashi; Une, Koji; Takamatsu, Tsurukichi
1981-01-01
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. (author)
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Glossary of ALS-Related Medical and Scientific Terms
... dyskinesia An involuntary movement including athetosis and chorea. dysphagia Difficulty in swallowing. dystonia A slow movement or ... Paralysis of a muscle or group of muscles. Parkinson's Disease The most common form of Parkinson's is ...
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Full Text Available ... disease Arrhythmias Atherosclerosis Athetosis resulting from basal ganglia injury Balloon angioplasty - short segment Bladder function - neurological control Blinking Blood clotting Blood flow Blood pressure Brain components Breast lift Breathing Bunion Cancer of ...
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Full Text Available ... and how diseases and conditions affect them. Allergies Alzheimer disease Arrhythmias Atherosclerosis Athetosis resulting from basal ganglia ... any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should ...
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Full Text Available ... Allergies Alzheimer disease Arrhythmias Atherosclerosis Athetosis resulting from basal ganglia injury Balloon angioplasty - short segment Bladder function - neurological control Blinking Blood clotting Blood flow Blood pressure Brain components Breast lift Breathing Bunion Cancer of the ...
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Development of postural adjustments during reaching in infants with CP
Hadders-Algra, M; van der Fits, IBM; Stremmelaar, EF; Touwen, BCL
1999-01-01
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
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Jenner, P.; McCreary, A. C.; Scheller, D. K. A.
2011-01-01
The treatment of the motor symptoms of Parkinson's disease (PD) is dependent on the use of dopamine replacement therapy in the form of l-dopa and dopamine agonist drugs. However, the development of dyskinesia (chorea, dystonia, athetosis) can become treatment limiting. The initiation of dyskinesia
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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))
1982-12-01
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.
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[Vegetative disorders in children with cerebral palsy. Results of an inquiry of parents].
Feldkamp, M; Bartmann, D; Süreth, H; Steinhausen, D
1976-08-01
Many of the disturbances resulting from dysregulations in the autonomous nervous system of children with cerebral palsy are rarely discussed in the doctor's praxis. Nevertheless, they are causes of trouble and worry for the parents. For this reason we started an inquiry into this matter. Questionnaires were sent to the parents of 452 C.P. patients. 374 were answered with sufficient care. The following factors were evaluated: sleep, bladder and bowel activity, temperature regulation, vomiting, sweating, blood circulation, growth. The C.P. children were compared to their own siblings especially to the next younger ones. The diagnoses were as follows: Spastic tetraplegia 197 patients. Spastic hemiplegia 44 patients, Athetosis 33 patients, Mixed cases of spasticity and athetosis 82 patients, Other 15 patients. The degrees of handicap in terms of motor development were: severe (unability to sit unsupported) 166 cases, moderate (unability to kneel or walk unsupported) 118 cases, mild (ability to kneel and/or walk unsupported) 87 cases. Summarized, the statements of the parents gave the following results: sleep disturbances: 169 cases (46%), constipation: 145 cases (39%), tendency towards temperature dysregulation: 112 cases (30%) , tendency towards increased vomiting: 91 cases (25%), sweating increased or decreased: 110 cases (30%), irregular and frequent voiding of bladder: 92 cases (25%), unstable regulation of blood circulation: 101 cases (27%), cold skin: 264 cases (71%), body-length deficit: 119 cases (32%), low-weight: 177 cases (48%), feet too small for age: 252 cases (68%). Results are related to diagnosis and severeness of handicap. In addition, it is discussed, whether there are relations between several of the investigated factors. The influence of the patients sex is discussed.
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Clinical features of movement disorders.
Yung, C Y
1983-08-01
The descriptive aspects of all types of movement disorders and their related syndromes and terminologies used in the literature are reviewed and described. This comprises the features of (a) movement disorders secondary to neurological diseases affecting the extrapyramidal motor system, such as: athetosis, chorea, dystonia, hemiballismus, myoclonus, tremor, tics and spasm, (b) drug induced movement disorders, such as: akathisia, akinesia, hyperkinesia, dyskinesias, extrapyramidal syndrome, and tardive dyskinesia, and (c) abnormal movements in psychiatric disorders, such as: mannerism, stereotyped behaviour and psychomotor retardation. It is intended to bring about a more comprehensive overview of these movement disorders from a phenomenological perspective, so that clinicians can familiarize with these features for diagnosis. Some general statements are made in regard to some of the characteristics of movement disorders.
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Holmes' tremor as a delayed complication of thalamic stroke.
Martins, William Alves; Marrone, Luiz Carlos Porcello; Fussiger, Helena; Vedana, Viviane Maria; Cristovam, Rafael do Amaral; Taietti, Marjorye Z; Marrone, Antonio Carlos Huf
2016-04-01
Movement disorders are not commonly associated with stroke. Accordingly, thalamic strokes have rarely been associated with tremor, pseudo-athetosis and dystonic postures. We present a 75-year-old man who developed a disabling tremor 1 year after a posterolateral thalamic stroke. This tremor had low frequency (3-4 Hz), did not disappear on focus and was exacerbated by maintaining a static posture and on target pursuit, which made it very difficult to perform basic functions. MRI demonstrated an old ischemic lesion at the left posterolateral thalamus. Treatment with levodopa led to symptom control. Lesions in the midbrain, cerebellum and thalamus may cause Holmes' tremor. Delayed onset of symptoms is usually seen, sometimes appearing 2 years after the original injury. This may be due to maturation of a complex neuronal network, leading to slow dopaminergic denervation. Further studies are needed to improve our understanding of this unique disconnection syndrome. Copyright © 2015 Elsevier Ltd. All rights reserved.
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Gasquoine, Philip Gerard
2017-06-01
Distinct forms of acquired neurocognitive impairment are often described by "a" prefixed terms that derive from ancient Greek (and in one case Latin). Two modern English language neurological and neuropsychological reference books were searched to identify 17 such terms in contemporary usage: amnesia, akinesia, ataxia, aphasia, agraphia, anosmia, apraxia, athetosis, ageusia, achromatopsia, agnosia, alexia, amusia, anomia, anarthria, anosognosia, and acalculia. These were traced to their initial association with acquired neurocognitive impairment in German, English, and French language medical publications from the late 18th, 19th, and early 20th centuries (1770 through 1920). Some of these terms (e.g., agnosia) were used in ancient Greek, although not associated with neurocognitive impairment. The remainder constitute novel semantically plausible (e.g., anosmia) and unclear (e.g., alexia) formulations. In the localizationist thinking of the time, neurocognition was conceived as being organized within specialized "centers" in specific locations connected by pathways within the brain.
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Auto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports.
Bashiri, Fahad A; Al-Rasheed, Abdulrahman A; Hassan, Saeed M; Hamad, Muddathir H A; El Khashab, Heba Y; Kentab, Amal Y; AlBadr, Fahad B; Salih, Mustafa A
2017-08-01
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently identified auto-immune disorder characterised by severe memory deficit, a decreased level of consciousness, seizures, autonomic dysfunction and movement disorders. Three girls with the disorder are reported; they were aged 4 years, 5 years and 10 months. The 10-month-old infant who is one of the youngest patients reported with anti-NMDAR encephalitis worldwide, had MRI features suggestive of herpes simplex encephalitis (known to trigger anti-NMDAR encephalitis), but CSF PCR for herpes simplex was negative. All the patients presented with seizures, behavioural change, regression of speech, dystonia and choreo-athetosis. Anti-NMDAR antibodies were detected in all patients' sera and cerebrospinal fluid (CSF). Intravenous immunoglobulin, corticosteroids and rituximab were administered at different intervals. Cases 1 and 2 made a full recovery, but case 3 has mild motor and speech delay. Patients who present with encephalopathy, seizures and movement disorders should be tested for anti-NMDAR antibodies in serum and CSF in addition to being screened for herpes simplex encephalitis.
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Directory of Open Access Journals (Sweden)
Angelika eRichter
2015-11-01
Full Text Available Dystonia is defined as a neurological syndrome characterized by involuntary sustained or intermittent muscle contractions causing twisting, often repetitive movements and postures. Paroxysmal dyskinesias are episodic movement disorders encompassing dystonia, chorea, athetosis and ballism in conscious individuals. Several decades of research have enhanced the understanding of the etiology of human dystonia and dyskinesias that are associated with dystonia, but the pathophysiology remains largely unknown. The spontaneous occurrence of hereditary dystonia and paroxysmal dyskinesia is well documented in rodents used as animal models in basic dystonia research. Several hyperkinetic movement disorders, described in dogs, horses and cattle, show similarities to these human movement disorders. Although dystonia is regarded as the third most common movement disorder in humans, it is often misdiagnosed because of the heterogeneity of etiology and clinical presentation. Since these conditions are poorly known in veterinary practice, their prevalence may be underestimated in veterinary medicine. In order to attract attention to these movement disorders, i.e. dystonia and paroxysmal dyskinesias associated with dystonia, and to enhance interest in translational research, this review gives a brief overview of the current literature regarding dystonia/paroxysmal dyskinesia in humans, and summarizes similar hereditary movement disorders reported in domestic animals.
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International Nuclear Information System (INIS)
Saginoya, Toshiyuki; Yamaguchi, Keiichiro; Kuniyoshi, Kazuhide
1996-01-01
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)
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Saginoya, Toshiyuki [Urasoe General Hospital, Okinawa (Japan); Yamaguchi, Keiichiro; Kuniyoshi, Kazuhide [and others
1996-06-01
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)
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MUTATIONS IN THE ARX GENE: CLINICAL, ELECTROENCEPHALOGRAPHIC AND NEUROIMAGING FEATURES IN 3 PATIENTS
Directory of Open Access Journals (Sweden)
I. V. Ivanova
2017-01-01
Full Text Available The Aristaless-related homeobox (ARX gene is a member of the paired-type homeodomain transcription factor family with critical roles in embryonic development, particularly in the developing brain. Mutations in ARX gene demonstrate striking intra- and interfamilial pleiotropy together with genetic heterogeneity and lead to a broad spectrum of diseases. They give rise to 4 key phenotypic features: a different types of brain malformation, abnormal genitalia, epilepsy and intellectual disability. Authors present 3 clinical cases: a girl with duplication on the short arm of X-chromosome (Xp11.22-p22.33, which include genes ARX and CDKL5; a girl and a boy with a missense mutation in ARX gene that have not been previously described (chrX:25031522C>A, causes the substitution of an amino acid in the 197 protein position (p.Gly197Val, NM_139058.2. All patients suffer from severe epilepsy, that is refractory to antiepileptic drugs, and all of them have different degrees of psychomotor delay. The patients with missense mutation also have movement disorders: stereotypic movements in the girl and choreo athetosis and dystonia in the boy. Electroencephalographic abnormalities have been identified in all patients, and there were not significant abnormalities on magnetic resonance imaging in all cases. The described cases broaden the clinical spectrum of mutations in ARX gene.
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CT findings of cerebral palsy and behaviour development
International Nuclear Information System (INIS)
Sakamoto, Zenji
1987-01-01
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. (author)
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A case of dentato-rubro-pallido-luysian atrophy
International Nuclear Information System (INIS)
Usui, Sadanari; Komiya, Tadatoshi
1988-01-01
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. (author)
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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)
2009-10-15
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.)
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A case of idiopathic intracranial calcifications - Hahr syndrome
International Nuclear Information System (INIS)
Georgiev, R.; Balev, B.; Georgieva, M.; Georgiev, J.
2006-01-01
Full text: The purpose of the study is to review the clinical manifestation, imaging characteristics and pathophysiology of the Fahr syndrome and to present a case of the Fahr syndrome from our clinic. The Fahr syndrome is a rare neurodegenerative disorder, characterized by seizures, tetany, psychomotor retardation, development of a spastic paralysis, athetosis and parkinson like syndrome. It is inherited by an AR way but in affected families (relatives) an AD way is also possible. Sporadic cases have been known. Recently a possible chromosome locus on 14q was proved. Probably the case in point is a group of anomalies, in which symmetrically and bilaterally significant calcifications in the region of the basal ganglia, dentate nuclei in cerebellum and centrum semiovale are found. It is not clear yet whether these calcifications are a result from a 'metastatic' deposition because of a local destruction of the blood-brain barrier or are due to a disturbance in the neuronic calcium metabolism. The X-ray findings could be accidental in an asymptomatic patient but a progressive development of an extrapyramidal syndrome may be also observed. Our case is a 37 years old woman with seizures with loss of consciousness, convulsions and urine incontinence. The complaints are dated from the age of 5 years old. The X-ray images disclosed striking non-natural calcifications in globus pallidus, putamen, n.caudatus, thalami, n.dentati, cerebellum.The blood test revealed normal serum levels of calcium, phosphorus, alkaline phosphatase. The CT findings put together with the typical clinical history and the normal blood test were a prerequisite for this diagnosis
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Orthopedic surgery in cerebral palsy: Instructional course lecture
Directory of Open Access Journals (Sweden)
Deepak Sharan
2017-01-01
Full Text Available Orthopedic surgery (OS plays an important role in the management of cerebral palsy (CP. The objectives of OS are to optimize functions and prevent deformity. Newer developments in OS for CP include emphasis on hip surveillance, minimally invasive procedures, use of external fixators instead of plates and screws, better understanding of lever arm dysfunctions (that can only be corrected by bony OS, orthopedic selective spasticity-control surgery, and single-event multilevel lever arm restoration and anti spasticity surgery, which have led to significant improvements in gross motor function and ambulation, especially in spastic quadriplegia, athetosis, and dystonia. The results of OS can be dramatic and life altering for the person with CP and their caregivers if it is performed meticulously by a specialized surgical team, at the appropriate age, for the correct indications, employing sound biomechanical principles and is followed by physician-led, protocol based, intensive, multidisciplinary, institutional rehabilitation, and long term followup. However, OS can be a double-edged sword, and if performed less than optimally, and without the supporting multidisciplinary medical and rehabilitation team, expertise and infrastructure, it often leads to significant functional worsening of the person with CP, including irretrievable loss of previous ambulatory capacity. OS must be integrated into the long term management of the person with CP and should be anticipated and planned at the optimal time and not viewed as a “last resort” intervention or failure of rehabilitation. This instructional course lecture reviews the relevant contemporary principles and techniques of OS in CP.
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Wilson's disease: two treatment modalities. Correlations to pretreatment and posttreatment brain MRI
International Nuclear Information System (INIS)
Leiros da Costa, Maria do Desterro; Spitz, Mariana; Bacheschi, Luiz Alberto; Barbosa, Egberto Reis; Leite, Claudia Costa; Lucato, Leandro Tavares
2009-01-01
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.)
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USE OF BOTULINUM TOXIN TYPE A IN THE TREATMENT OF SPASTICITY IN CHILDREN WITH CEREBRAL PALSY
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Ljiljana Lazić
2011-06-01
Full Text Available Cerebral palsy has an incidence of about 1-2 per 1000 live births, and in spite of the progress of neonatal medicine, it seems that the incidence will not subside in the near future. The most important characteristic of cerebral palsy is movement abnormality: spasticity, chorea, athetosis, ataxia, dystonia, as well as their different combinations. About 70% of children who suffer from cerebral palsy also suffer from some form of spasticity. Spasticity is a type of muscle hypertonicity characterized by rapid increase in resistance to passive stretching of muscles. The interest for botulinum toxin application in the treatment of spasticity has dramatically increased in the last 10 years. Botulinum toxin is the most powerful neurotoxin that is found in nature. It is produced by anaerobic bacteria – clostridium botulinum. It is produced in eight serotypes of which type A is the most commonly used. Botulinum toxin blocks neuromuscular transmission and causes irreversible weakness of the treated muscle. It has been used since 1993 in the treatment of cerebral palsy in children. The toxin effect is permanent and it results in irreversible denervation. Functional recovery is possible after 2-4 months, due to sprouting of nerve endings and the formation of new synaptic contacts. Treatment with botulinum toxin is safe. Adverse effects are rare, temporary and completely reversible. Application of botulinum toxin prevents or reduces contractures and deformities, and thus delays or avoids surgical treatment. Yet, physical therapy, which prolongs and improves the effects of botulinum toxin, remains an essential and most important form of therapy in the treatment of children with cerebral palsy.
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Directory of Open Access Journals (Sweden)
El Tallawy HN
2013-05-01
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
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CT of the infants and children with mental and/or physical handicaps
International Nuclear Information System (INIS)
Okada, Junichiro; Takeuchi, Kazuo
1980-01-01
Computed tomography (CT) was performed on 47 children and adolescents with mental and/or physical handicaps. Of these series, 22 cases of morphological change were noted. Another 25 cases showed no overt CT abnormality. These 47 cases were divided into three groups in the following manner. Group 1, with no CT abnormality; Group 2, with ventricular dilatation and/or cerebral atrophy, and Group 3, with a major morphological anomaly of the brain. Group 1 (25 cases) showed a marked dissociation between the CT findings and the IQ. EEG showed normal findings in two cases, diffuse abnormality in 5 cases, and focal abnormality in 9 cases. This group alone included 8 cases of athetosis. Group 2 (14 cases). Seven cases of EEG showed diffuse abnormality in 3 cases and focal abnormality in 4 cases. So-called cerebral palsy was noted in 11 cases. Group 3 (8 cases). This group included cases of hemihydranencephaly, porencephaly, agenesis of the corpus callosum, and arachnoid cyst. The mean and standard deviations of the IQ's in the groups are 57.1 +- 21.6, 65.2 +- 20.5, and 72.0 +- 8.0. That is, an inverted correlation between the CT abnormality and the IQ was noted. CT is a noninvasive study and a reasonable method of investigation for mentally handicapped children. DeMyer gave three categories of cerebral malformation: cytogenetic malformations, organogenetic disorders, and histogenetic disorders. On the other hand, EEG aimed at evaluating cerebral function and CT undertaken for morphological evaluation reveal no intimate correlation with one another. Rather, these two procedures each have their one value for the evaluation of the function and the structure of the brain. Mentally and/or physically handicapped patients without any overt cerebral anomaly have been found to be as follows: Murobushi, 12.29%; Malamud, 34%; Gross, 15.8%; Benda, 15%, and Hamada, 45.4%. (author)
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Prevalence of neurological disorders in Al Quseir, Egypt: methodological aspects
Directory of Open Access Journals (Sweden)
El-Tallawy H
2013-09-01
Full Text Available Hamdy El-Tallawy,1 Wafa Farghaly,1 Nabil Metwally,2 Tarek Rageh,1 Ghaydaa A Shehata,1 Reda Badry,1 Esam El Moselhy,2 Mahmoud Hassan,2 Mohamed M Sayed,3 Ahmed A Abdelwarith,1 Y Hamed,2 I Shaaban,2 Talal Mohamed,4 Mohamed Abd El Hamed,1 MR Kandil1 1Department 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, Egypt; 4Department of Neurology, Faculty of Medicine, Qena University, Qena, Egypt Abstract: Methodology and strategy play a very important role in epidemiological studies. Determination of the study area, geographical features, facilities, difficulties, and key personnel from the same area are important factors for successful methodology. Over 31 months (July 1, 2009 to January 31, 2012, a screening and an examination survey were carried out to ascertain the prevalence of epilepsy, stroke, dementia, cerebellar ataxia, migraine, cerebral palsy, Parkinsonism, chorea, athetosis, dystonia, trigeminal neuralgia, Bell's palsy, multiple sclerosis, spinal cord disorders, and attention deficit hyperactivity disorders in Al Quseir, Red Sea Governorate, Egypt. A total of 33,285 people were screened by three neurologists in a door-to-door manner, including every door, using a standardized Arabic questionnaire to detect any subject with a neurological disorder. The methodological aspects of this project were carried out through eight phases: (1 data collection; (2 preparation; (3 screening; (4 case ascertainment; (5 investigations; (6 classifications; (7 data entry; and (8 statistics and tabulations. The results of this study reveal that the total prevalence of neurological disorders in Al Quseir was 4.6% and higher among females (5.2% than males (3.9%. The highest prevalence was recorded in the elderly population (60+ years [8.0%] and among the age
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Diagnosis and management of acute movement disorders.
Dressler, D; Benecke, R
2005-11-01
Most movement disorders, reflecting degenerative disorders, develop in a slowly progressive fashion. Some movement disorders, however, manifest with an acute onset. We wish to give an overview of the management and therapy of those acute-onset movement disorders.Drug-induced movement disorders are mainly caused by dopamine-receptor blockers (DRB) as used as antipsychotics (neuroleptics) and antiemetics. Acute dystonic reactions usually occur within the first four days of treatment. Typically, cranial pharyngeal and cervical muscles are affected. Anticholinergics produce a prompt relief. Akathisia is characterized by an often exceedingly bothersome feeling of restlessness and the inability to remain still. It is a common side effect of DRB and occurs within few days after their initiation. It subsides when DRB are ceased. Neuroleptic Malignant Syndrome is a rare, but life-threatening adverse reaction to DRB which may occur at any time during DRB application. It is characterised by hyperthermia, rigidity, reduced consciousness and autonomic failure. Therapeutically immediate DRB withdrawal is crucial. Additional dantrolene or bromocriptine application together with symptomatic treatment may be necessary. Paroxysmal dyskinesias are childhood onset disorders characterised by dystonic postures, chorea, athetosis and ballism occurring at irregular intervals. In Paroxysmal Kinesigenic Dyskinesia they are triggered by rapid movements, startle reactions or hyperventilation. They last up to 5 minutes, occur up to 100 times per day and are highly sensitive to anticonvulsants. In Paroxysmal Non-Kinesiogenic Dyskinesia they cannot be triggered, occur less frequently and last longer. Other paroxysmal dyskinesias include hypnogenic paroxysmal dyskinesias, paroxysmal exertional dyskinesia, infantile paroxysmal dystonias, Sandifer's syndrome and symptomatic paroxysmal dyskinesias. In Hereditary Episodic Ataxia Type 1 attacks of ataxia last for up to two minutes, may be accompanied
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Directory of Open Access Journals (Sweden)
K. Gianikellis
2010-09-01
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