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At least 685 records · Page 38Linked to original sources
PubMed1976

Sequelae of head and neck positions on auditory performance.

Stapedius reflex level, slow auditory evoked potential (AEP), and hearing threshold level of 18 normal adult probands (11 female, 7 male) as well as of 14 normal hearing cerebral palsied children between 4 and 12 years of age (5 female, 9 males)--were determined in different postures which correspond to the influence of tonic reflex activities in supine and prone, and in the so-called "reflex inhibiting pattern" (Bobath) and in a symmetrical relaxed position of neck, trunk and extremities in supine (the last one could only be maintained by the normal volunteers). Influence of the postures was small but significant for all parameters in the volunteers group as well as for AEP in the patients group. In the latter group the influence of tonic reflex activities on auditory evoked potential was independent from their strength. The strength of tonic reflex activity had been graded from 1-5 as judged from clinical impression by observation and palpation.

Adult↗
PubMed1976

Bobath-therapy, tonic reflex--activities and processing of acoustic stimuli.

The influence of tonic reflex activities on the slow auditory evoked potential (AEP) as well as the influence of Bobath's reflex inhibiting patterns (r.i.p.) in a group of 14 cerebral palsied children is compared under the same conditions with simulated tonic reflex patterns in 18 adult normal volunteers and 15 cerebral palsied children. In the adult volunteers group the effect of a relaxed symmetrical position of neck, trunk, and extremities in supine on the AEP is also evaluated. There are none but age specific differences in the latence of AEP between both groups but clear cut differences in the amplitudes and in the specific effect of r.i.p. in both groups.

Adult↗
PubMed2001

Phenytoin-induced choreoathetosis in patients with severe myoclonic epilepsy in infancy.

We describe three patients with severe myoclonic epilepsy in infancy (SME) who suffer from choreoathetosis due to the adverse effect of phenytoin. Choreoathetosis appeared when these patients were 8, 19, and 21 years old, 2 days to 6 months after increasing the phenytoin dosage. Choreoathetosis disappeared when the phenytoin dosage was decreased. The two elder patients experienced episodic and rather paroxysmal onset of long-lasting choreoathetosis, requiring the differential diagnosis from degenerative disease. In one of the patients, an ictal SPECT revealed decreased perfusion in the basal ganglia contralateral to the unilateral choreoathetosis. Polypharmacy, including carbamazepine and zonisamide, may have facilitated the onset of choreoathetosis. Phenytoin-induced choreoathetosis in the patients with SME is an important differential diagnosis among degenerative disorders involving involuntary movements. The episodic and paroxysmal nature of this movement disorder can delay its diagnosis and effective treatment. Patients with SME appear to be particularly vulnerable to this side effect of phenytoin, indicating the possible involvement of basal ganglia in the pathophysiology of this type of epilepsy.

Adolescent↗
PubMed2002

The relationship between paroxysmal kinesigenic choreoathetosis and epilepsy.

PURPOSE: To clarify the relationship between paroxysmal kinesigenic choreoathetosis (PKC) and epilepsy, we investigated the clinical and electroencephalographic (EEG) findings of patients with familial PKC and epilepsy, as well as sporadic cases with both PKC and epilepsy. PATIENTS AND METHODS: Patients consisted of 12 familial cases from seven families and three sporadic cases. The period of follow-up ranged from 17 months to 33 years, 7 months (average: 16 years, 8 months). During the follow-up, a total of 163 EEGs (11 EEGs per subject) were studied, including interictal and ictal EEGs. RESULTS: Transient epileptic discharges were found in ten of the 15 patients (66.7 %) during the clinical course. As for focus, centro-midtemporal and frontal spikes were most often observed. The ictal EEG of an afebrile convulsion in one patient showed a partial seizure with secondary generalization which originated from the frontal area. CONCLUSIONS: It appears that patients who suffer from both PKC and epilepsy have a functional abnormality of the cerebral cortex, particularly in the perirolandic and frontal regions.

Adolescent↗
PubMed2002

Paroxysmal kinesigenic dyskinesia and generalized seizures: clinical and genetic analysis in a Spanish pedigree.

Familial paroxysmal kinesigenic dyskinesia (PKD) is a rare disorder featuring brief, dystonic or choreoathetotic attacks, typically triggered by sudden movements. Symptoms usually start in mid-childhood, although in several pedigrees infantile convulsions have been reported as the presenting sign. Previous linkage studies have identified two PKD loci on 16 p12.1-q21. We report here the clinical features of a Spanish kindred with autosomal dominant PKD, in which haplotype data are compatible with linkage to the pericentromeric region of chromosome 16 and exclude linkage to the locus for Paroxysmal Non Kinesigenic Dyskinesia (PNKD) on chromosome 2 q35. In this family, the conservative candidate region for the disease lies between markers D16S3145 and GATA140E03 on 16 p12.1-q21 and partially overlaps with both the Paroxysmal Kinesigenic Dyskinesia - Infantile Convulsions (PKD-IC) critical interval and the Episodic Kinesigenic Dyskinesia 2 (EKD2) locus. Unusual findings in our pedigree were early infantile onset of the dyskinesias in one patient and generalized seizures as adults in two, adding to previous observations of phenotypic overlap between epileptic and non-epileptic paroxysmal disorders. Further clinical and genetic studies are needed to elucidate whether PKD and PKD-IC are allelic disorders with age-dependent phenotypic expression.

Adolescent↗
PubMed1991

[Paroxysmal choreoathetosis].

Paroxysmal Choreoathetosis (PC) is a rare disorder which is characterized by spells of involuntary movements of the limbs, the head and the trunk lasting from a minute to several hours. PC is classified in two main types. In Paroxysmal Dystonic Choreoathetosis (PDC) the attacks are prolonged and precipitated by alcohol, emotion or fatigue, in Paroxysmal Kinesigenic Choreoathetosis (PKC) the attacks last less than 5 minutes and are precipitated by sudden movements. Although the pathophysiology of the disorder ist still uncertain, it is supposed to be an extrapyramidal disorder. Clinical features, therapeutical and differential diagnostic aspects are discussed. There is a certain risk that the disorder is misinterpreted as psychogenic or hysterical in nature. The mostly good response to medication can help the patients avoid superimposed emotional problems.

Anticonvulsants↗
PubMed1998

Choreoathetoid movements associated with rapid adjustment to methadone.

Choreatiform hyperkinesias are known to be occasional movement abnormalities during intoxications with cocaine but not opiates. This is a case report of euphoria and choreoathetoid movements both transiently induced by rapid adjustment to the selective mu-opioid receptor agonist methadone in an inpatient previously abusing heroine and cocaine. In addition, minor EEG abnormalities occurred. Possible underlying neurobiological phenomena are discussed.

Adult↗
PubMedDate not supplied

[Degenerative diseases of the extrapyramidal and spinocerebellar system--differential diagnostic references].

Degenerative disorders of the extrapyramidal and spinocerebellar system often have a genetic cause; however, pathogenesis usually is still unknown. Disturbance of neurotransmitter metabolism in Parkinson disease or in some dystonic syndromes offers new therapeutic approaches; in choreatic or athetoid syndromes biochemical factors are only known in some parts. Clinical and neurophysiological differentiation of various dyskinetic disorders is essential to reach better understanding. In children early signs and symptoms are seen which may be caused by different factors and are difficult to diagnose. Similar problems are to be seen in hereditary ataxias which sometimes begin in childhood: Clinical findings are essential to plan for additional investigations.

Athetosis↗
PubMedDate not supplied

[Results of hand surgery operations in spastic-athetotic paresis].

Between 1974 and 1986 a total of 123 patients with a spastic or spastic athetotic paresis of the upper limb underwent surgery. The interval between surgery and follow-up examination was between one and 13 years. There were 73 cases of hemiparesis and 50 dipareses or tetrapareses due to perinatal cerebral paresis and 35 cases due to a variety of causes. The patients were aged between 6 and 58 years, the majority between 8 and 28 years. All contractures in the arm and hand region were treated at a single sitting. The sole exception to this was surgery for swan-neck deformity of the long fingers. In none of the cases was a wrist arthrodesis indicated. As regards the elimination of the previously existing malpositions, some of which were severe, and the cosmetic outcome, the results were good in all cases. The postoperative reduction was also preserved through the subsequent years, until completion of growth. Also, the difference in growth between flexors and extensors had no detectable negative influence on the long-term results of surgery. Only in a few isolated cases was limited revisional surgery necessary to improve the result as regards extension in the elbow joint and the posture of the wrist joint, which it had not been possible to treat satisfactorily at the first sitting. Two patients with a pronounced athetotic component manifested unsatisfactory results in several respects, or overcorrection of extension in the wrist joint: special caution is called for here. As far as necessary, corresponding corrective surgery was performed simultaneously on the lower limbs.

Adolescent↗
PubMedDate not supplied

[Joubert syndrome combined with unilateral facial paralysis: a rare variant of Joubert syndrome].

We report the case of a mature newborn infant, which aroused attention 8 hours after birth due to an unexplainable paroxysmal tachypnea together with subsequently prolonged apnea. The combination of tachypnea, athetoid movement patterns, apraxia of the tongue, nystagmus and dysplasia of the cerebellum with dilation of the 4th ventricle, led to the diagnosis of Joubert-Syndrome. The child also showed a variation of a connatal unilateral facial nerve palsy, which has not previously been described in connection with a Joubert-Syndrome.

Apnea↗
PubMed1984

Paroxysmal kinesigenic choreoathetosis.

A twelve and a half-year-old boy is reported who presented with paroxysmal kinesigenic choreoathetosis. Family history lead to the diagnosis. The differential diagnosis to paroxysmal dystonic choreoathetosis of Mount and Reback (1940) is discussed, and treatment is commented upon.

Athetosis↗
PubMed1987

The use of a computerised database for the diagnosis of a rare neurological syndrome.

A database which runs on an office microcomputer is being developed for the diagnosis of genetically determined neurological disorders. At present about 1100 conditions with their clinical features and 3000 references are stored in the database. We discuss a family with 3 sibs affected by a unique neurological disorder and show how the database is used. The 3 sibs, 4, 5 and 10 years old, show the same clinical course characterized by congenital cataracts, microcephaly, hypotonia, mental retardation, pyramidal signs and choreoathetoid movements starting in early childhood. The parents are first cousins of Bangladeshi origin. This condition does not appear in published report and is not listed in the database. It can therefore be concluded that the sibs have a unique autosomal recessive disorder.

Athetosis↗
PubMed1981

[A method of measuring centrally determined disturbances of muscle tone (author's transl)].

A technique is described (myotonography) for recording the centrally determined disturbances of muscle tone, whereby the mechanical and electro-myographic changes in the affected muscle groups are accessible to objective and quantitative study. The efficacity and reliability of the method are given and the clinically distinguishable forms of central disturbances of tonus are shown in typical tracings. These are of particular value for assessing the progress of an illness and the results of treatment. In addition it facilitates the interpretation of the patho-physiological processes. The apparatus is so constructed that in the same way as in a clinical examination, a passive extension and flexion of the elbow joint is reproduced over a range of about 100 degrees. In this way it is possible to record the stretch resistance of the extremities as well as the superficial electromyogram of the flexors and extensors.

Adolescent↗
PubMed1983

Reduced optimality in pre- and perinatal conditions in dyskinetic cerebral palsy--distribution and comparison to controls.

The optimality concept developed by Prechtl was adopted to investigate a series of 108 dyskinetic cerebral palsy (CP) cases. The results were compared to those from a control series of 229 newborns. The dyskinetic cases, as compared to the controls, had significantly more reduced optimality scores. Reduced optimality was especially pronounced in the partum and postpartum periods, but was to a highly significant degree also found in the prepartum period. Dystonics and choreoathetotics differed in their pattern of reduced optimality. Dystonics had more reduced optimality in items related to deficient intrauterine growth, intra- and extrauterine asphyxia-choreoathetotics in items related to preterm birth, postpartal hypoxia and hyperbilirubinemia. Term cases had more reduced optimality scores (median 7 vs 1) compared to term controls than preterm cases compared to preterm controls (median 9 vs 4.5). The pattern of reduced optimality in term cases corresponded primarily to that among the dystonics, and the pattern in preterm cases mainly to that of the choreoathetotics. Prepartum warning signals for further partum and postpartum reduced optimality related to the brain cell damage, were maternal disorder, blood group incompatibility and leanness at birth (weight for length less than or equal to -1.0 SD). The latter condition was found to discriminate for reduced optimality in the partum period.

Athetosis↗